Daniel von Allmen

1113 statements · 24 topics · summaries given as host listed separately

Adrenal Tumors · guest expert Aerodigestive / ENT · guest expert Biliary Atresia · guest expert Colorectal / ARM & Hirschsprung · guest expert Etiologies (Gastroschisis/NEC/Atresia/Volvulus) · guest expert Fetal Surgery · guest expert Intestinal Failure · guest expert Intestinal Rehab · guest expert Intestinal Transplant · guest expert Neuroblastoma · guest expert Soft Tissue Sarcoma (lymph nodes) · guest expert Wilms Tumor · guest expert

Featured statements

If I have a patient who has a stricture that's been dilated 2 or 3 times, my next move would be to address reflux. Most of the patients we leave on anti-reflux medications when they are discharged from the hospital. I would argue that virtually all patients have some degree of gastroesophageal reflux. If they have a stricture that is not responsive to dilations, it dilates easily and then restrictures. I personally am very aggressive about doing a fundoplication on those patients
quote · Esophageal Atresia
I completely agree, and I think that you have to be very careful. I personally like to just use a blade, electric cautery and with a little buzz and then mostly blunt dissection with the flat end of the blade, and I think that. That can help you and staying right on the esophagus, which is usually very thickened because it's an obstructed piece of bowel. I would rather be in the esophagus than be in the trachea up high in the chest.
quote · Esophageal Atresia
You can get extraordinary length on this. And Mike and I have done a few cases for kids who had disasters, multiple operations elsewhere, who had caustic injuries that were involved all the way up to the pharynx, where we had to do a lot of work just on the pharynx to get that open, and then literally sew the colon interposition to the pharynx and then down to the stomach, which is obviously tough to do with a gastric pull-up.
quote · Fetal Surgery
I've not found personally that an oscillator makes a whole lot of difference. I think if the child has a, has a large fistula, and typically it's actually, I believe this tends to be a bigger problem in younger, that is more premature infants who have more significant lung disease, and their compliance of their lungs is really what drives the The air into the GI tract as opposed to into the lungs.
quote · Tracheoesophageal Fistula
Our method here is that if we have a child with no gas in the abdomen and we take them to the OR and put a G-tube in, many times we'll do, we'll put something up the distal esophagus at that point and just get a fluoro shot. But then we'd wait a couple of weeks and we have our patients go down to interventional radiology where we have a protocol for measuring the gap.
quote · Fetal Surgery
I pass the interposition posterior to the stomach, which leaves the vascular pedicle along the spine. And you can mobilize the colon. You can divide the gastric dual aporotomy, divide the colon attachment to the stomach, and then mobilize that sigmoid redundancy transhiatally, and then reinestimose the colon to the stomach.
quote · Long-gap Esophageal Atresia

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Daniel's statements about Adrenal Tumors 48 statements

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Topics in 10: Neuroblastoma

Statement In younger patients, neuroblastoma is often picked up either prenatally on ultrasound, or in younger kids (two-year-old or three-year-old) as a solid abdominal mass. Open
Statement When neuroblastoma patients have metastatic disease, they may present with either bony pain or potentially neurologic symptoms from cord compression. Open
Quotation So in the younger patients, they're oftentimes picked up either prenatally on ultrasound, or in younger kids, say two-year-old or three-year-old, it might be picked up as a solid abdominal mass. Open
Statement When considering neuroblastoma as part of the differential diagnosis, it is important to get catecholamines (either urine or serum) as one of the most diagnostic laboratory tests for this tumor. Open
Statement Most children with suspected neuroblastoma would get a cross-sectional imaging study, either a CT scan or an MRI. Open
Statement If imaging suggests neuroblastoma (central abdominal mass or adrenal mass rather than kidney mass), the next test would be a nuclear medicine study, typically an MIBG study. Open
Statement The MIBG study is helpful for confirming the diagnosis of neuroblastoma and can also demonstrate metastatic disease. Open
Quotation About 10% of neuroblastomas are MIBG negative. Open
Statement About 10% of neuroblastomas are MIBG negative. Open
Statement Some centers, including Cincinnati Children's Hospital, would get a PET scan looking for tumor uptake as well as potential metastatic disease. Open
Statement In the INRGSS system, tumors that are localized are categorized as L1; if localized but have image-defined risk factors (encasing nerves or vessels), they are L2; if they have metastatic disease, they are M. Open
Statement There is a special category MS for children less than 18 months of age who have metastases to either the bone marrow or the skin. Open
Statement Based on the most recent iteration of the neuroblastoma staging system (INRGSS), it is possible to assign a stage before any invasive procedure is performed. Open
Statement The prior neuroblastoma staging system required tissue diagnosis before assigning a stage. Open
Statement The INRGSS pre-biopsy staging system was specifically created to allow studies from different centers in different countries to be compared based on the pre-surgical staging of the patient. Open
Statement Some surgeons would approach resection of a localized neuroblastoma with laparoscopy depending on the size of the tumor. Open
Statement A child with an adrenal mass on the right side and a positive MIBG scan but no evidence of metastases could potentially be treated with a primary resection of the mass via laparotomy. Open
Statement For very large masses that encase the aorta, cava, or other major vasculature, all you really want is tissue for diagnosis, which can be obtained through open biopsy, laparoscopic biopsy, or core needle biopsies done by an interventional radiologist. Open
Statement In addition to NMIC status, you want to look for 1P and 11Q deletions and Shimada histology in neuroblastoma biopsy tissue. Open
Statement The most important biologic risk determinant to obtain from neuroblastoma biopsy tissue is the NMIC status. Open
Statement Neuroblastoma risk is divided about 50-50 between the low risk categories and the high risk category, with a smaller percentage being intermediate risk. Open
Statement Biologic risk determinants from biopsy will tell you what risk category the patient falls into: very low risk, low risk, intermediate risk, or high risk. Open
Quotation NMIC and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement Patients with high risk neuroblastoma receive aggressive chemotherapy including peripheral stem cell transplant times 2, aggressive surgery with the goal of greater than 90% resection of the tumor, followed by radiation, immunotherapy after chemotherapy, and potentially retinoic acid therapy. Open
Statement NMIC amplification and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement For intermediate risk neuroblastoma, the goal at the time of debulking or resecting the primary tumor is to achieve at least a 50% response from the initial volume of the primary tumor through the combination of neoadjuvant chemotherapy and surgical resection. Open
Statement Intermediate risk neuroblastoma tumors get varying cycles of chemotherapy based on the biologic risk factors they have. Open
Statement Jed Nocturne led a study through the Children's Oncology Group looking at patients less than six months of age with either a prenatally diagnosed or shortly postnatally diagnosed localized mass, showing these patients can be observed with the expectation that the vast majority will avoid any type of surgical procedure. Open
Statement The low risk neuroblastoma group, depending on the actual age of the patient and how it is diagnosed, could potentially be followed simply with observation. Open
Statement Patients with metastatic neuroblastoma typically receive four or five cycles of neoadjuvant chemotherapy and then are reassessed. Open
Statement In patients with MS disease, simple observation can be the treatment path. Open
Statement MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow (specifically not bone, not cortical bone) and is less than 18 months of age. Open
Statement If the metastatic disease is progressing on neoadjuvant chemotherapy, then surgery is not indicated. Open
Statement If the tumor and metastatic disease are responding to neoadjuvant chemotherapy, one would attack the primary tumor site with a resection, with many advocating for attempting a greater than 90% resection. Open
Quotation MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow, specifically not bone, not cortical bone, and is less than 18 months of age. Open
Statement If MS disease patients progress or develop respiratory issues because of an enlarging liver mass, treatment might be elected because of the complication of the size of the tumor, but the tumor itself usually does not have to be treated. Open
Statement You can biopsy the skin lesions in MS disease and that will give you the diagnosis. Open

Neuroblastoma

Statement Adrenal hemorrhage is the most common cause of prenatal suprarenal mass, especially with history of fetal stress Open
Statement The GetNucturne study showed that prenatal/neonatal neuroblastomas can be safely observed with careful ultrasound surveillance, with many patients spared surgery Open
Statement Lymph node status in neuroblastoma does not change therapy, unlike Wilms tumor Open
Statement Approximately 10% of neuroblastomas are not MIBG-avid, which is when PET scan may be useful Open
Statement Tumor shrinkage in neuroblastoma plateaus after cycle 2-3 of chemotherapy per LaQuaglia and Von Allman studies Open
Statement COG high-risk study showed >90% resection improved event-free survival but not overall survival (approximately 245 patients) Open
Statement European neuroblastoma group study (approximately 1000 cases) showed >90% resection improved both event-free survival and overall survival Open
Statement Approximately 70% of high-risk neuroblastoma patients can achieve >90% resection Open
Statement Neuroblastoma theoretically does not invade vessel adventitia, though invasion has been observed Open
Statement There is zero correlation between surgeon operative note description of resection extent and post-operative imaging findings Open
Quotation There is zero correlation, zero. There is absolutely no correlation between the findings on post-op imaging and the findings that are reported in the op note. Open
Daniel's statements about Aerodigestive / ENT 6 statements

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Aerodigestive & Esophageal Surgery - Difficult Tracheal Esophageal Fistula

Statement If a patient is aspirating from above (oropharyngeal or esophageal source), an anti-reflux procedure does not help and may make them worse. Open
Statement Routine bronchoscopy is now standard practice for all type C TEF repairs at Cincinnati Children's, often done in collaboration with ENT colleagues. Open
Statement Dual scoping (simultaneous bronchoscopy and esophagoscopy) allows scopes to 'shake hands' across a fistula, light transillumination through tissue, and injection of saline or air to reveal subtle openings. Open
Quotation Button batteries are bad. Open
Quotation The tissue damage extends beyond what you can appreciate with the naked eye, and the progression of it probably extends beyond what you would think is the normal time frame. Open
Statement In a patient with multiple bronchoesophageal fistulas and chronic bronchiectasis, lobectomy with resection of the esophageal pseudo-diverticulum can be performed safely; the esophagus can be primarily closed if not strictured. Open
Daniel's statements about Appendicitis 12 statements

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open
Daniel's statements about Biliary Atresia 12 statements

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open
Daniel's statements about CHARGE Syndrome 12 statements

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Aerodigestive & Esophageal Surgery - Difficult Tracheal Esophageal Fistula

Statement If a patient is aspirating from above (oropharyngeal or esophageal source), an anti-reflux procedure does not help and may make them worse. Open
Statement If a patient is aspirating from above (oropharyngeal or esophageal source), an anti-reflux procedure does not help and may make them worse. Open
Statement Routine bronchoscopy is now standard practice for all type C TEF repairs at Cincinnati Children's, often done in collaboration with ENT colleagues. Open
Statement Routine bronchoscopy is now standard practice for all type C TEF repairs at Cincinnati Children's, often done in collaboration with ENT colleagues. Open
Statement Dual scoping (simultaneous bronchoscopy and esophagoscopy) allows scopes to 'shake hands' across a fistula, light transillumination through tissue, and injection of saline or air to reveal subtle openings. Open
Statement Dual scoping (simultaneous bronchoscopy and esophagoscopy) allows scopes to 'shake hands' across a fistula, light transillumination through tissue, and injection of saline or air to reveal subtle openings. Open
Quotation Button batteries are bad. Open
Quotation Button batteries are bad. Open
Quotation The tissue damage extends beyond what you can appreciate with the naked eye, and the progression of it probably extends beyond what you would think is the normal time frame. Open
Quotation The tissue damage extends beyond what you can appreciate with the naked eye, and the progression of it probably extends beyond what you would think is the normal time frame. Open
Statement In a patient with multiple bronchoesophageal fistulas and chronic bronchiectasis, lobectomy with resection of the esophageal pseudo-diverticulum can be performed safely; the esophagus can be primarily closed if not strictured. Open
Statement In a patient with multiple bronchoesophageal fistulas and chronic bronchiectasis, lobectomy with resection of the esophageal pseudo-diverticulum can be performed safely; the esophagus can be primarily closed if not strictured. Open
Daniel's statements about Colorectal / ARM & Hirschsprung 17 statements

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Neuroblastoma

Statement Adrenal hemorrhage is the most common cause of prenatal suprarenal mass, especially with history of fetal stress Open
Statement The GetNucturne study showed that prenatal/neonatal neuroblastomas can be safely observed with careful ultrasound surveillance, with many patients spared surgery Open
Statement Lymph node status in neuroblastoma does not change therapy, unlike Wilms tumor Open
Statement Approximately 10% of neuroblastomas are not MIBG-avid, which is when PET scan may be useful Open
Statement Tumor shrinkage in neuroblastoma plateaus after cycle 2-3 of chemotherapy per LaQuaglia and Von Allman studies Open
Statement COG high-risk study showed >90% resection improved event-free survival but not overall survival (approximately 245 patients) Open
Statement European neuroblastoma group study (approximately 1000 cases) showed >90% resection improved both event-free survival and overall survival Open
Statement Approximately 70% of high-risk neuroblastoma patients can achieve >90% resection Open
Statement Neuroblastoma theoretically does not invade vessel adventitia, though invasion has been observed Open
Quotation There is zero correlation, zero. There is absolutely no correlation between the findings on post-op imaging and the findings that are reported in the op note. Open
Statement There is zero correlation between surgeon operative note description of resection extent and post-operative imaging findings Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open
Daniel's statements about Congenital Lung Lesions (CPAM) 30 statements

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Complications and Beyond

Statement Baseline chest X-ray may be useful if patient later presents with respiratory symptoms and consolidation, providing comparison Open
Statement Baseline chest X-ray may be useful if patient later presents with respiratory symptoms and consolidation, providing comparison Open
Statement If crura come together easily during paraesophageal hernia repair, primary closure without mesh is preferred Open
Statement If crura come together easily during paraesophageal hernia repair, primary closure without mesh is preferred Open
Statement Posterior defect is the usual problem site in paraesophageal hernia; mesh should cover the posterior repair Open
Statement Posterior defect is the usual problem site in paraesophageal hernia; mesh should cover the posterior repair Open
Statement Waiting only 3-4 weeks for revision risks operating during maximal inflammation period Open
Statement Waiting only 3-4 weeks for revision risks operating during maximal inflammation period Open
Quotation I hate this operation, whether it's open or laparoscopic, because you're trying to do a mechanical fix to a physiologic problem, and it's uh miserable. Open
Statement Fundoplication attempts mechanical fix for physiologic problem, making it inherently difficult operation Open
Quotation I hate this operation, whether it's open or laparoscopic, because you're trying to do a mechanical fix to a physiologic problem, and it's uh miserable. Open
Statement Fundoplication attempts mechanical fix for physiologic problem, making it inherently difficult operation Open
Statement Gastric disconnect is excellent operation in cognitively impaired patients who don't eat much and have refractory reflux or multiple failed fundoplications Open
Statement Gastric disconnect is excellent operation in cognitively impaired patients who don't eat much and have refractory reflux or multiple failed fundoplications Open
Statement Dextrocardia makes thoracoscopic visualization challenging for EA/TEF repair Open
Statement Dextrocardia makes thoracoscopic visualization challenging for EA/TEF repair Open
Statement For unstable EA/TEF patient, laparotomy with G-tube and vessel loop around GE junction allows fistula control and ventilation, deferring thoracic repair until patient stabilizes Open
Statement For unstable EA/TEF patient, laparotomy with G-tube and vessel loop around GE junction allows fistula control and ventilation, deferring thoracic repair until patient stabilizes Open
Statement Bronchoscopy should be performed in all EA/TEF cases to identify additional fistulas Open
Statement Bronchoscopy should be performed in all EA/TEF cases to identify additional fistulas Open
Statement Tracheal reconstruction for esophageal lung can be performed via median sternotomy: divide trachea, repair esophagus posteriorly, reconstruct trachea and fish-mouth abnormal bronchus to create lung connection Open
Statement Tracheal reconstruction for esophageal lung can be performed via median sternotomy: divide trachea, repair esophagus posteriorly, reconstruct trachea and fish-mouth abnormal bronchus to create lung connection Open
Statement If initial TEF ligation is performed too far from trachea (2cm), significant esophageal length is lost, potentially preventing primary anastomosis Open
Statement If initial TEF ligation is performed too far from trachea (2cm), significant esophageal length is lost, potentially preventing primary anastomosis Open
Statement Magnet anastomosis can be attempted when esophageal ends are tacked together but gap prevents primary anastomosis Open
Statement Magnet anastomosis can be attempted when esophageal ends are tacked together but gap prevents primary anastomosis Open
Quotation It'll be great if it works. Open
Quotation It'll be great if it works. Open
Statement Tracheal reconstruction for esophageal lung is performed on cardiopulmonary bypass Open
Statement Tracheal reconstruction for esophageal lung is performed on cardiopulmonary bypass Open
Daniel's statements about Congenital Pulmonary Airway Malformation 15 statements

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Complications and Beyond

Statement Baseline chest X-ray may be useful if patient later presents with respiratory symptoms and consolidation, providing comparison Open
Statement If crura come together easily during paraesophageal hernia repair, primary closure without mesh is preferred Open
Statement Posterior defect is the usual problem site in paraesophageal hernia; mesh should cover the posterior repair Open
Statement Waiting only 3-4 weeks for revision risks operating during maximal inflammation period Open
Statement Fundoplication attempts mechanical fix for physiologic problem, making it inherently difficult operation Open
Quotation I hate this operation, whether it's open or laparoscopic, because you're trying to do a mechanical fix to a physiologic problem, and it's uh miserable. Open
Statement Gastric disconnect is excellent operation in cognitively impaired patients who don't eat much and have refractory reflux or multiple failed fundoplications Open
Statement Dextrocardia makes thoracoscopic visualization challenging for EA/TEF repair Open
Statement For unstable EA/TEF patient, laparotomy with G-tube and vessel loop around GE junction allows fistula control and ventilation, deferring thoracic repair until patient stabilizes Open
Statement Bronchoscopy should be performed in all EA/TEF cases to identify additional fistulas Open
Statement Tracheal reconstruction for esophageal lung can be performed via median sternotomy: divide trachea, repair esophagus posteriorly, reconstruct trachea and fish-mouth abnormal bronchus to create lung connection Open
Statement If initial TEF ligation is performed too far from trachea (2cm), significant esophageal length is lost, potentially preventing primary anastomosis Open
Statement Magnet anastomosis can be attempted when esophageal ends are tacked together but gap prevents primary anastomosis Open
Quotation It'll be great if it works. Open
Statement Tracheal reconstruction for esophageal lung is performed on cardiopulmonary bypass Open
Daniel's statements about Enterocolitis 45 statements

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Hirschprung's Disease - Daniel von Allmen

Statement Contrast enema plus suction rectal biopsy is the standard initial workup for newborn distal bowel obstruction suspected to be Hirschsprung disease, not upper GI series. Open
Statement Laparoscopic-assisted pull-through is preferred over purely transanal approach because contrast enema underestimates transition zone level in approximately 8% of cases (Manu Proctor's data). Open
Quotation You don't want to be confused by the facts Open
Statement Female patients may have higher incidence of long-segment Hirschsprung (50-50 ratio for total colonic aganglionosis in girls) compared to males. Open
Quotation I've been burned so many times by that, what looks like an obvious low transition zone and you end up just chasing it and chasing it Open
Quotation We actually published a paper of false positive contrast enemas in newborns. So there was a transition zone that looked like Hirschsprung's, and it was not Hirschprung. Open
Statement Tissue diagnosis is mandatory before operating for Hirschsprung disease; contrast enema alone is insufficient due to false-positive transition zones in newborns. Open
Statement In sick patients with enterocolitis, diversion may be necessary before obtaining tissue diagnosis if pathology results will be delayed several days. Open
Quotation there is an occasional patient who is really sick from enterocolitis and you may not wanna wait for a tissue diagnosis. It might take a couple of days to get. Open
Quotation There are two definitions for ultra short segment Hirschmann's disease that people use, and these kinds of discussions get get foiled because one person's using one definition, another person's using another one. Open
Statement Ultra-short segment Hirschsprung has two definitions: (1) aganglionosis less than 5cm from dentate line, or (2) absent recto-anal inhibitory reflex with normal ganglion cells (internal sphincter achalasia). The latter is not true Hirschsprung disease. Open
Quotation it's hard for me to imagine how Doctor Pena could not believe in that. You know, why would there be a disease where there's a minimum of 5 centimeters of a ganglionosis? Open
Statement Manometry showing normal recto-anal inhibitory reflex rules out Hirschsprung disease in older cooperative children (age 5-6+); if reflex is absent, biopsy is still required due to false-negative manometry. Open
Quotation if there's a normal reflex. Uh-huh. Now, if they don't have a normal reflex, then, then you have to biopsy them because the, the, there can be false, false, whatever you want, I don't know, negative, positive, false absence of the of the reflex Open
Statement Hypertrophic nerves on biopsy indicate Hirschsprung disease; they should not be present in normal anal canal biopsies even though ganglion cells are normally absent in the anal canal. Open
Statement The anal canal in adults is 3-4 centimeters long (versus 1cm in infants), so biopsies at 3cm in a 16-year-old may actually be sampling anal canal rather than rectum. Open
Statement Cecal perforation in Hirschsprung disease usually occurs with shorter-segment disease (not total colonic aganglionosis) because the cecum becomes most distended, similar to rectal cancer causing cecal perforation. Open
Quotation the cecum gets more distended and pops. So, uh, I would close the cecal perforation, bring out a loop ileostomy, and if the baby was stable enough, I would do some serial biopsies in the colon Open
Statement For cecal perforation from Hirschsprung: close the perforation, create loop ileostomy, and perform serial colonic biopsies if patient is stable to identify transition zone level. Open
Quotation I tend to wait longer in long segment disease because they have terrible perianal excoriation if you, if you pull them through too early. Open
Statement Long-segment Hirschsprung patients should wait 6-12 months after diversion before definitive pull-through to allow stoma output to thicken and prevent severe perianal excoriation. Open
Statement When transition zone is in the cecum or ascending colon, pulling down the cecum as a pouch leads to stasis and enterocolitis; ileal Duhamel (sacrificing the cecum) may have better outcomes. Open
Quotation my experience with, um, with a very, very short pull, pull through like using the cecum essentially have not been very good. So in general, when I'm faced with that anatomy, I just do a, treat it like total colon disease and I do an ileal duhamel. Open
Quotation when it's just the cecum, it's, it's this big bag of stuff, and, and they end up with a lot of stasis and enterocolitis. Open
Statement If transition zone is at hepatic flexure or beyond, preserve the colon; if only cecum is ganglionic, consider ileal Duhamel instead. Open
Statement Suction rectal biopsy should not be performed in children older than approximately 15-18 months due to patient cooperation issues; open rectal biopsy under general anesthesia is preferred. Open
Quotation you gotta, even though you might, the, the specimen may come out, the logistics of taking a 2 year old and, uh, holding them they can fight back. They're too old. Open
Statement In older children (age 3+) with severely dilated colon from Hirschsprung, primary pull-through without diversion risks anastomotic complications; leveling colostomy with resection of dilated segment is preferred. Open
Quotation We've been really leaning towards doing diversion after just to protect our anastomosis. Open
Quotation if we do this, take the, try to resect these colons transanally. The stretch that we put on the sphincter is enormous, and their, uh, continence postoperatively is definitely, definitely suffers. Open
Quotation that thing that you showed, um, is so mammothly dilated, I don't think that would close. That's why I was initially going to say, Divert, but I think that that's not going to come down no matter how long you wait. Open
Statement Massively dilated colon in older Hirschsprung patients may shrink after 6-8 months of diversion in younger children (age 3) but is less likely to shrink in adolescents (age 17). Open
Quotation I have had success in, uh, getting that distal bowel to, uh, shrink in size. Um-hum. With the stoma for 6 or 8 months. Open
Quotation the older the kid, the less likely that it's going to shrink down in size. But, uh, at this age OK. Uh, I think it will. Open
Quotation patient was at a well-known institution in Ohio. And had, was seen by GI and had multiple biopsies that were normal. Those were done by colonoscopy, um, and then, so the chart read no Hirschprung's disease Open
Statement Colonoscopic biopsies for Hirschsprung diagnosis in older children can be unreliable and miss the diagnosis; full-thickness rectal biopsy is more reliable. Open
Statement Initial management of Hirschsprung-associated enterocolitis: IV broad-spectrum antibiotics and saline rectal irrigations. Consider diversion only if patient has peritonitis, sepsis, or SIRS. Open
Quotation just caution to watch out what type of biopsy was performed and how Hirschprung's was ruled out. It's, it's an argument for the manometry uh diagnosis because biopsies in an older child can be unreliable. Open
Statement Botox injection decreases the number of hospitalizations for enterocolitis in post-pull-through Hirschsprung patients, though it does not work in all cases. Open
Statement Chronic oral metronidazole is used liberally for recurrent Hirschsprung-associated enterocolitis, sometimes for months; symptoms may recur when medication is stopped. Open
Quotation we have a lot of kids on chronic, uh, metronidazole. And when they, you know, they can be on it for three months and then you try taking them off and they start getting symptoms again. Open
Quotation we actually published a, uh, that Botox decreased the number of hospitalizations for enterocolitis in, in those children. It doesn't always work, though. Open
Quotation The incidence of enterocolitis is significantly higher in children with trisomy 21, as much as double the incidence in genetically normal kids. Open
Statement Incidence of enterocolitis is significantly higher (approximately double) in children with trisomy 21 compared to genetically normal children with Hirschsprung disease. Open
Statement For recurrent enterocolitis post-pull-through: perform exam under anesthesia with biopsies to rule out residual aganglionosis or distal obstruction before treating the enterocolitis itself. Open
Daniel's statements about Esophageal Atresia 276 statements

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Aerodigestive & Esophageal Surgery - Difficult Tracheal Esophageal Fistula

Statement If a patient is aspirating from above (oropharyngeal or esophageal source), an anti-reflux procedure does not help and may make them worse. Open
Statement Routine bronchoscopy is now standard practice for all type C TEF repairs at Cincinnati Children's, often done in collaboration with ENT colleagues. Open
Statement Dual scoping (simultaneous bronchoscopy and esophagoscopy) allows scopes to 'shake hands' across a fistula, light transillumination through tissue, and injection of saline or air to reveal subtle openings. Open
Quotation Button batteries are bad. Open
Quotation The tissue damage extends beyond what you can appreciate with the naked eye, and the progression of it probably extends beyond what you would think is the normal time frame. Open
Statement In a patient with multiple bronchoesophageal fistulas and chronic bronchiectasis, lobectomy with resection of the esophageal pseudo-diverticulum can be performed safely; the esophagus can be primarily closed if not strictured. Open

Tracheoesophageal Fistula with Dr. Daniel von Allmen

Quotation It certainly is an interest that we have here with a whole group here in Cincinnati, but it can be an incredibly difficult, challenging problem. It's one of the great cases in pediatric surgery and one of the most difficult cases, so it really runs the whole spectrum for sure. Open
Statement Cincinnati Children's has a multidisciplinary aerodigestive center that includes ENT airway surgeons, GI, pulmonary, general surgeons, nurse practitioners, geneticists, and dietitians. Open
Statement Injecting air into the NG tube and obtaining a plain film helps visualize distention of the proximal esophageal pouch. Open
Quotation If I can't see a distended proximal pouch, then I worry. I worry that either one of two things either that the diagnosis is incorrect and in fact it is not an esophageal atresia, or that there could be a proximal fistula which is allowing that proximal pouch to decompress. Open
Statement Lack of visible proximal pouch distention raises concern for either incorrect diagnosis or presence of a proximal fistula allowing pouch decompression. Open
Statement Preoperative echocardiogram is essential to assess cardiac anatomy and identify right-sided aortic arch. Open
Statement Renal ultrasound and spine evaluation for tethered cord are needed but are not necessary before addressing esophageal atresia. Open
Statement Conventional wisdom suggests left thoracotomy for right-sided aortic arch, though the operation is somewhat more difficult with the heart more in the way. Open
Statement If right-sided arch is discovered intraoperatively during right thoracotomy, it is possible to complete the operation from that side, though some reports suggest higher incidence of swallowing problems. Open
Statement In a stable, larger baby not requiring intubation, it is reasonable to wait until full resources are available (Sunday or Monday) rather than operating emergently on Saturday. Open
Statement Prolonged delay risks colonization of the GI tract and soiling of the lungs. Open
Statement Oscillator ventilation has not been found to make a significant difference in managing infants with large fistulas and abdominal distention. Open
Statement Air preferentially entering the GI tract rather than lungs is a bigger problem in more premature infants with poor lung compliance. Open
Statement Treating with surfactant and improving lung compliance helps as much as changing ventilator type in managing fistula-related ventilation problems. Open
Statement One risk in managing the deteriorating infant with TEF is waiting too long to make a decision, which can lead to an emergency situation with inability to ventilate. Open
Quotation One of the risks is to wait too long to make a decision, and, and I've made that mistake myself, and then it becomes an emergency if you can't ventilate the child. Open
Statement Bronchoscopic placement of Fogarty balloon in the fistula is difficult unless expertise and equipment are immediately available. Open
Statement G-tube decompression may paradoxically worsen ventilation by creating even lower resistance pathway, allowing more air to flow from trachea to GI tract rather than to lungs. Open
Statement Laparotomy with vessel loop around gastroesophageal junction with gentle traction (Rommel tourniquet technique) can be used to stabilize critically ill infant, with G-tube placement and delayed definitive repair after several days. Open
Statement In esophageal atresia with associated duodenal atresia, if the child is stable, consider fixing duodenal atresia first to avoid repairing esophagus upstream from an obstruction. Open
Statement Both esophageal and duodenal atresia can potentially be repaired in the same operative setting if the child is stable enough. Open
Statement Routine intraoperative bronchoscopy documents fistula location, assesses for proximal fistula, and most importantly identifies laryngeal clefts that are easily missed. Open
Quotation We have seen that many times in patients who are referred who've had multiple thoracotomies. Nobody ever did a bronchoscopy and the child actually has a laryngeal cleft. Open
Statement Laryngeal clefts have been seen many times in referred patients who had multiple thoracotomies but never had bronchoscopy. Open
Statement The rate of missed second fistula is approximately 1%. Open
Statement High fistulas exist on a spectrum from essentially a trifurcation of the carina to fistulas high enough to be approachable through the neck. Open
Statement Endotracheal tube positioning depends on fistula location: for fistula at carina (trifurcation), tube should be placed higher; for high fistula, tube should be placed distal to fistula but not into the fistula itself. Open
Statement Extrapleural approach offers the advantage of potentially limiting soilage of pleural space if there is a postoperative leak. Open
Statement Dividing the azygos vein has no morbidity, gives better access, and frequently guides to the fistula location. Open
Statement For open repair, the azygos is typically ligated and divided with suture. Open
Statement The 3mm Just Right surgical sealer is an ideal device for dividing the azygos in this size patient. Open
Statement To establish extrapleural plane, place heel of right angle on ribs and spread to nicely show the plane, then mobilize pleura posteriorly to expose azygos. Open
Statement Mobilizing pleura up around apex of lung is important to allow access for proximal pouch mobilization. Open
Statement Distal esophageal segment is controlled with vessel loop, allowing proximal dissection to identify the fistula site. Open
Statement Contrary to standard teaching that distal esophagus should not be mobilized, it can be mobilized extensively especially laterally, all the way to the diaphragm. Open
Statement Medial mobilization of distal esophagus requires more caution due to blood supply concerns. Open
Statement Extensive proximal pouch mobilization provides the most important length gain for anastomosis. Open
Statement For proximal dissection, blade cautery with light buzz followed by mostly blunt dissection with flat end of blade, staying right on the thickened esophagus, is preferred; better to enter esophagus than trachea high in chest. Open
Quotation I would rather be in the esophagus than be in the trachea up high in the chest. Open
Statement For large tracheal injury, sleeve resection is possible given tracheal mobility; pericardium or autologous tissue should be interposed between trachea and esophageal repair. Open
Statement 5-0 PDS (absorbable monofilament) is the preferred suture for esophageal anastomosis; silk is not favored. Open
Statement Myotomies are not favored because they potentially create even more dysfunctional esophageal segment; motility is already abnormal in esophageal atresia. Open
Quotation I personally am not a big fan of myotomy. I think it potentially creates an even more dysfunctional segment of the esophagus than already exists. The motility is clearly abnormal in patients with oesophageal atresia, and doing a myotomy just makes that problem worse. Open
Statement For long-gap atresia when ends cannot be brought together, approach is to ligate distal end after dividing fistula, tack both ends on tension to prevertebral fascia, close, allow patient to grow 2-3 weeks, then return for anastomosis. Open
Statement Traction or pressure is a strong stimulus to growth throughout the body including cardiovascular system and lungs. Open
Statement For thoracoscopic Foker technique, traction sutures are placed in proximal and distal ends, brought out through trocar sites above and below (crossed), tensioned, with return to OR within a week for anastomosis. Open
Statement Internal Foker (tacking ends to prevertebral fascia) is preferred over classic Foker with prolonged paralysis and sequential tensioning. Open
Statement For thoracoscopic TEF repair, patient is rotated past 90 degrees to allow gravity to move lungs away, with standard port placement: camera center, posterior port inferiorly, anterior port superiorly. Open
Statement 3mm instruments are used for thoracoscopic TEF repair. Open
Quotation I'm less worried about them falling off the esophagus than I am having them fall off a blood vessel. Open
Statement Thoracoscopic visualization is very helpful for mobilizing the proximal pouch. Open
Statement The anastomosis is the most difficult part of thoracoscopic TEF repair and requires significant minimally invasive experience. Open
Quotation I think that's one of the challenges with the thoracoscopic repair of a TEF is to actually sew the anastomosis. Open
Statement Simulation courses for thoracoscopic TEF repair will be a great advantage for trainees given the infrequency of these cases. Open
Quotation To me, the important part, and I beat on our residents about this when they're doing this anastomosis, is you have to be able to see. You want to make sure that you incorporate mucosa on every stitch. Open
Statement Every stitch must incorporate mucosa, which requires good visualization; the hanging stitch helps visualize lumens of both proximal and distal ends. Open
Statement Ventilator management during thoracoscopy depends more on anesthesiologist comfort and attention than ventilator type (oscillator vs. conventional). Open
Statement Endotracheal tube can be positioned to selectively ventilate left lung during thoracoscopy, aided by preoperative bronchoscopy. Open
Statement A little CO2 insufflation with time will collapse the lung as long as anesthesiologist is not fighting with positive pressure; anesthesiologists can get scared by initial shunting but things settle down. Open
Statement Early extubation is preferred when patient had good lung function preoperatively and operation went smoothly, even potentially in the operating room, to avoid positive pressure on tracheal repair and instead have negative intratracheal pressure with spontaneous breathing. Open
Quotation I'm equally concerned that positive pressure is putting pressure on my tracheal repair, and I would much rather have the patient be breathing spontaneously to have, in fact, the opposite effect of a negative pressure in the trachea. Open
Quotation I do honestly, I'm a little bit old school in that. I still get an esophagram. Open
Statement Contrast esophagram is obtained at 5-7 days postoperatively before pulling chest drain. Open
Statement Small TLS drains are used rather than formal chest tubes, especially for open extrapleural approach. Open
Quotation I do not actually. I again, a practice of mine that has changed based on our results from the Midwest Pediatric Surgical Consortium study in which the patients who Had a trans anastomotic tube had a much higher stricture and leak rate than patients that did not have that. Open
Statement For stricture on esophagram, dilation is delayed several weeks unless stricture is critically tight, because proximal pouch is always dilated making anastomosis always appear narrowed. Open
Quotation Unless it's an incredibly tight stricture, I would wait several weeks before doing a dilation unless I was worried there was going to be a complete obstruction. Open
Statement If contrast flows freely down distal esophagus, dilation can be held off several weeks. Open
Statement Dilations are performed aggressively, typically waiting one week between dilation and restudy, then potentially dilating again. Open
Statement Balloon (radial) dilation is less traumatic to tissue than bougie dilators and is preferred. Open
Statement If case goes well and postoperative study looks great, routine follow-up esophagrams are not obtained; patients are studied only if they present with symptoms suggesting stricture. Open
Quotation I would absolutely wait and do nothing. I would leave the drain in and usually that presents with some saliva in the drain, and I would just wait, and the vast majority of those close. Open
Statement For small anastomotic leak in stable child, conservative management with drain in place is appropriate; vast majority close spontaneously. Open
Statement Leak increases risk of postoperative stricture. Open
Statement Indications for operative intervention for leak are worsening clinical status, wide open leak, or large uncontrolled pneumothorax. Open
Statement Repeat esophagram is typically performed one week after initial leak identification; if small outpouching persists but nothing comes out of tube and contrast doesn't extend further, leak is probably healed and tube can be removed. Open
Statement Pediatric surgeons do not do a fantastic job of long-term follow-up with esophageal atresia patients. Open
Quotation I think that first of all I would make the comment that that's a great point and it's interesting to participate in the International oesophageal atresia meeting that occurs every 2 years and to go and see the patients because that meeting is multidisciplinary, including even patients, and you see these patients who come back who are in their 20s or 30s or 40s and they talk about the long term issues that they have and we tend to follow patients until they're, you know, until they're eating well and then they're gone. Open
Statement At International Esophageal Atresia meetings, patients in their 20s-40s describe long-term issues that surgeons are unaware of because follow-up typically ends at age 18 or when eating well. Open
Statement Patients with recurrent strictures presenting with food bolus obstruction are seen and dilated on an ongoing basis. Open
Quotation The patients that worry me more honestly are the patients who have ongoing reflux, and we don't know the long term impact of that on things like Barrett's esophagus and Potential long term risk for malignant change and those sorts of things. Open
Statement Greater concern is patients with ongoing reflux; long-term impact on Barrett's esophagus and malignant change risk is unknown. Open
Statement If stricture has been dilated 2-3 times, next move is to address reflux. Open
Statement Most patients are discharged on anti-reflux medications. Open
Quotation I would argue that virtually all patients have some degree of gastroesophageal reflux. Open
Statement Virtually all esophageal atresia patients have some degree of gastroesophageal reflux. Open
Statement For stricture not responsive to dilations (dilates easily then restrictures), aggressive approach is fundoplication. Open
Statement Despite poor esophageal motility, Nissen fundoplication is still performed but made loose and short, using 2 or at most 3 stitches for the wrap. Open
Statement For pure esophageal atresia, approach is G-tube placement with calibration of gap length using distal catheter pushed up distal esophagus and NG tube in proximal esophagus, wait 4-6 weeks, and attempt primary repair once ends are within 2 vertebral bodies. Open

Complications and Beyond

Statement Baseline chest X-ray may be useful if patient later presents with respiratory symptoms and consolidation, providing comparison Open
Statement If crura come together easily during paraesophageal hernia repair, primary closure without mesh is preferred Open
Statement Posterior defect is the usual problem site in paraesophageal hernia; mesh should cover the posterior repair Open
Statement Waiting only 3-4 weeks for revision risks operating during maximal inflammation period Open
Quotation I hate this operation, whether it's open or laparoscopic, because you're trying to do a mechanical fix to a physiologic problem, and it's uh miserable. Open
Statement Fundoplication attempts mechanical fix for physiologic problem, making it inherently difficult operation Open
Statement Gastric disconnect is excellent operation in cognitively impaired patients who don't eat much and have refractory reflux or multiple failed fundoplications Open
Statement Dextrocardia makes thoracoscopic visualization challenging for EA/TEF repair Open
Statement For unstable EA/TEF patient, laparotomy with G-tube and vessel loop around GE junction allows fistula control and ventilation, deferring thoracic repair until patient stabilizes Open
Statement Bronchoscopy should be performed in all EA/TEF cases to identify additional fistulas Open
Statement Tracheal reconstruction for esophageal lung can be performed via median sternotomy: divide trachea, repair esophagus posteriorly, reconstruct trachea and fish-mouth abnormal bronchus to create lung connection Open
Statement If initial TEF ligation is performed too far from trachea (2cm), significant esophageal length is lost, potentially preventing primary anastomosis Open
Statement Magnet anastomosis can be attempted when esophageal ends are tacked together but gap prevents primary anastomosis Open
Quotation It'll be great if it works. Open
Statement Tracheal reconstruction for esophageal lung is performed on cardiopulmonary bypass Open

Tracheoesophageal Fistula with Dr. Daniel von Allmen

Quotation It certainly is an interest that we have here with a whole group here in Cincinnati, but it can be an incredibly difficult, challenging problem. It's one of the great cases in pediatric surgery and one of the most difficult cases, so it really runs the whole spectrum for sure. Open
Statement Cincinnati Children's has a multidisciplinary aerodigestive center including ENT airway surgeons, GI, pulmonary, general surgeons, nurse practitioners, geneticists, and dietitians Open
Statement The aerodigestive center typically receives more complicated patients referred from outside through either esophageal or airway arms Open
Statement Physical examination should assess for scaphoid versus full or distended abdomen and respiratory distress Open
Quotation I personally like to try to pass the NG tube myself, having been burned with NG tubes that wouldn't go down, and the patient was started on treatment as an oesophageal atresia and subsequently found that the NG tube passes just fine. Open
Statement The surgeon should personally attempt to pass the NG tube rather than relying on nursing reports, as tubes reported as not passing sometimes pass easily Open
Statement Injecting air into the NG tube and obtaining a plain film helps visualize proximal pouch distention and assess distal GI gas pattern Open
Quotation If I can't see a distended proximal pouch, then I worry. I worry that either one of two things either that the diagnosis is incorrect and in fact it is not an esophageal atresia, or that there could be a proximal fistula which is allowing that proximal pouch to decompress. Open
Statement Lack of visible distended proximal pouch raises concern for either incorrect diagnosis or presence of proximal fistula allowing pouch decompression Open
Statement Contrast studies can be performed if there is high suspicion of unusual anatomy, as long as the radiologist knows the esophagus may be obstructed Open
Statement Echocardiogram is the most important preoperative study to assess cardiac anatomy and rule out right-sided aortic arch Open
Quotation The most important one prior to taking the patient to the operating room is to get an echocardiogram, and the utility of that is, is obviously to assess the cardiac anatomy, but more as importantly, I would say is to assess to be sure that the patient does not have a right-sided aortic arch. Open
Statement Renal ultrasound and spine evaluation for tethered cord are needed but are elective and not necessary before addressing esophageal atresia Open
Statement Conventional wisdom is to perform left thoracotomy for right-sided aortic arch Open
Statement Left thoracotomy for right-sided arch is somewhat more difficult with the heart more in the way and harder proximal pouch mobilization Open
Statement It is possible to complete the repair from the right side if right-sided arch is discovered intraoperatively, though some reports suggest higher incidence of swallowing problems Open
Statement In a stable larger baby not intubated, it is reasonable to wait until all resources are available in the middle of the day, even waiting 1-2 days Open
Statement Should not wait a long time before repair due to risk of colonizing GI tract and soiling lungs Open
Statement Large fistula with distention tends to be a bigger problem in more premature infants with significant lung disease, where poor lung compliance drives air into GI tract Open
Statement Oscillator ventilation does not make a significant difference in managing large fistulas with abdominal distention Open
Quotation I've not found personally that an oscillator makes a whole lot of difference. I think if the child has a, has a large fistula, and typically it's actually, I believe this tends to be a bigger problem in younger, that is more premature infants who have more significant lung disease, and their compliance of their lungs is really what drives the The air into the GI tract as opposed to into the lungs. Open
Statement Treating with surfactant and improving lung compliance helps as much as changing ventilator type Open
Quotation I think this is a difficult clinical scenario, and, and I think one of the risks is to wait too long to make a decision, and I've made that mistake myself, and then it becomes an emergency if you can't ventilate the child. Open
Statement Risk of waiting too long to make a decision in worsening distention can lead to emergency situation where child cannot be ventilated Open
Statement Bronchoscopic Fogarty balloon placement sounds good but is difficult unless expertise and equipment are immediately available Open
Statement G-tube decompression may paradoxically worsen ventilation by making stomach lower resistance, allowing more air to go there instead of lungs Open
Statement For reasonably stable but worsening distention, would take child urgently to OR for right thoracotomy and fistula ligation Open
Quotation Decompressing the stomach may just allow that to become, in effect, the even lower resistance and allow more air to go there, making it even more difficult to ventilate. You relieve the abdominal distention, but you don't fix the flow of gas from the Trachea to the GI tract as opposed to from the trachea to the lungs. Open
Statement Laparotomy with vessel loop around esophagogastric junction at hiatus with gentle traction (Rommel tourniquet technique) can temporize severe cases, leaving in place for several days before definitive repair Open
Statement For associated duodenal atresia in stable child, would potentially fix duodenal atresia first to avoid fixing esophagus upstream from obstruction Open
Statement Both duodenal and esophageal atresia could potentially be fixed at same time if child is old enough and stable enough Open
Quotation We have seen that many times in patients who are referred who've had multiple thoracotomies. Nobody ever did a bronchoscopy and the child actually has a laryngeal cleft Open
Quotation That is one of the things that I have changed in my management after coming here and having exposure to the patient population that we see, the complex patients that we see with the esophageal center and the neurodigestive center, and The airway surgeons here have convinced me that it is really important to do a bronchoscopy Open
Statement Von Allmen changed practice to always perform intraoperative bronchoscopy after exposure to Cincinnati's complex patient population Open
Statement Bronchoscopy documents fistula location, assesses for proximal fistula, and most importantly rules out laryngeal cleft which is easily missed Open
Statement Many referred patients with multiple thoracotomies never had bronchoscopy and actually have laryngeal cleft Open
Statement Second fistula occurs in approximately 1% of cases and can be very difficult to diagnose Open
Statement High fistulas can range from trifurcation of carina (suggesting difficulty getting ends together) to very high fistulas potentially approachable through neck Open
Statement Bronchoscopy allows guidance of ET tube placement by anesthesiologist based on fistula location Open
Statement For fistula at carina (trifurcation), ET tube should be placed higher as it cannot pass the fistula; for high fistula, tube should be placed distal to fistula but not into the fistula itself Open
Statement Extrapleural approach offers advantage of potentially limiting soilage of pleural space if there is postoperative leak Open
Quotation I do an extra plural approach and that's the way I was trained. I think it offers the advantage of potentially limiting any soilage of the plural space if there is a leak postoperatively. Open
Quotation I think there's no morbidity associated with that, so dividing the azygus gives you better access. In fact, frequently will guide you to the fistula. Open
Statement Dividing azygos has no morbidity, gives better access, and frequently guides to the fistula Open
Statement Open azygos division is done by ligation and division; thoracoscopically can use energy devices or clips Open
Quotation It is amazing what you can divide with the electric cautery, with the hook electric cautery if you just get used to it, and I learned that doing actually robotic Nissans that you can divide the short gastrics in a teenager with the with the hook electric cautery. Open
Statement 3mm surgical sealer is ideal device for this size patient and vessel Open
Statement Hook cautery can safely divide vessels if done slowly going up and down to ensure coagulation, learned from robotic Nissen experience dividing short gastrics Open
Statement Important to mobilize pleura up around apex of lung to have access for proximal pouch mobilization Open
Statement After azygos division, identify distal esophagus and control with vessel loop, then dissect proximally to identify fistula site Open
Statement Spreading heel of right angle on ribs nicely shows extrapleural plane when going through intercostal muscles Open
Statement Muscle-sparing thoracotomy can make exposure more difficult; has not seen significant morbidity from standard posterolateral thoracotomy Open
Statement Standard teaching has been not to mobilize distal esophagus, but can actually mobilize it significantly especially laterally all the way to diaphragm Open
Statement Extensive proximal pouch mobilization gives the most length to get ends together Open
Statement Thoracoscopic approach advantage is clearer visualization for proximal pouch mobilization Open
Quotation I think we have learned that, you know, the standard teaching has been that you shouldn't mobilize the distal esophagus at all, and I think we've learned that in fact you can mobilize that segment a fair amount, especially laterally. Open
Statement Must be careful with medial mobilization of distal esophagus due to blood supply concerns Open
Statement Risk of entering trachea during proximal dissection; must be very careful using blade cautery with light buzz and mostly blunt dissection with flat end of blade Open
Statement Better to be in esophagus than trachea during high chest dissection; esophagus is thickened from obstruction Open
Quotation I completely agree, and I think that you have to be very careful. I personally like to just use a blade, electric cautery and with a little buzz and then mostly blunt dissection with the flat end of the blade, and I think that. That can help you and staying right on the esophagus, which is usually very thickened because it's an obstructed piece of bowel. I would rather be in the esophagus than be in the trachea up high in the chest. Open
Statement For significant tracheal defect, could perform sleeve resection as trachea is incredibly mobile, then place pericardium or autologous tissue between trachea and esophageal repair Open
Statement Personal preference is 5-0 PDS - absorbable monofilament suture; not a fan of silk Open
Statement Not a fan of myotomies as they potentially create even more dysfunctional esophageal segment; motility is already clearly abnormal in esophageal atresia Open
Statement For cases too tight for primary repair, would ligate ends, tack both on tension to prevertebral fascia, close, let patient grow for couple weeks, then return to put together Open
Quotation I personally am not a big fan of myotomy. I think it. Potentially creates an even more dysfunctional segment of the esophagus than already exists. The motility is clearly abnormal in patients with oesophageal atresia, and doing a myotomy just makes that problem worse. Open
Quotation I believe very firmly in the physiology of traction or pressure. Actually it's sort of the reverse, is a very strong stimulus to growth all over the place in the cardiovascular system in the lungs Open
Statement Traction or pressure is very strong stimulus to growth throughout cardiovascular system and lungs Open
Statement Would not do classic Foker technique with prolonged paralysis and sequential suture tensioning Open
Statement For thoracoscopic approach, can place traction sutures in proximal and distal ends brought out through crossed trocar sites with tension, allowing return within a week for anastomosis Open
Statement Foker technique requires patients intubated and paralyzed spending weeks in ICU, which is probably worth it if you get good result Open
Statement Rusty Jennings and Foker published series in JPS showing 98% success getting ends together in primary atresia versus only 67% in secondary approaches after previous surgeries Open
Quotation They published a series a year ago or so in JPS looking at their results, and, and it was a very carefully well documented report of their results, and the data are striking if you Have a patient who has a primary atresia that you address, then in 98% of patients they're able to get the two ends of the esophagus together. In contrast, if it's a patient who's had previous surgeries and it's a secondary approach, then only 2/3 of those patients ever get the esophagus together. Open
Statement Standard port placement: camera in center, posterior port inferiorly, anterior port superiorly Open
Statement For thoracoscopic procedure, rotate patient past 90 degrees to allow gravity to move lungs out of way Open
Statement Uses 3mm instruments for thoracoscopic TEF repair Open
Statement Sewing the anastomosis is one of the challenges with thoracoscopic TEF repair and requires most experience with minimally invasive techniques Open
Statement Simulation courses for TEF repair will be great advantage for trainees as these cases are not done often enough to get practice Open
Quotation I'm less worried about them falling off the esophagus than I am having them fall off a blood vessel. Open
Quotation I think as with most minimally invasive operations, I think it's important to do the same operation or at least the same quality operation that you would do open and Frankly, I think that's one of the challenges with the thoracoscopic repair of a TEF is to actually sew the anastomosis. Open
Statement Thoracoscopic visualization is very helpful for mobilizing proximal pouch Open
Statement Would use clips for dividing fistula thoracoscopically, less worried about them falling off esophagus than blood vessel Open
Statement Important to do same quality operation thoracoscopically as would do open Open
Statement Thoracoscopic ventilation management depends more on anesthesiologist than ventilator type; need anesthesiologist comfortable with procedure who pays attention during operation Open
Quotation I think it's more dependent on the anesthesiologist than it is on the type of ventilator, and you have to have an anesthesiologist who's comfortable with doing this and understands what you're trying to accomplish and is paying attention while you're actually doing the operation because they can either help you a lot or hurt you a lot with how they ventilate the child. Open
Statement Anesthesiologists can get scared seeing initial shunting but things settle down if they wait Open
Statement Little CO2 insufflation with some time will collapse lung as long as anesthesiologist isn't fighting with positive pressure Open
Statement Concern about reintubation exists, but equally concerned that positive pressure puts pressure on tracheal repair Open
Statement If patient had good lung function preoperatively and operation went smoothly, advocate extubating as soon as possible, even conceivably in operating room Open
Statement Prefer spontaneous breathing with negative pressure in trachea rather than positive pressure Open
Quotation I agree it is a concern that the patient may require reintubation. However, I'm equally concerned that positive pressure is putting pressure on my tracheal repair, and I would much rather have the patient be breathing spontaneously to have, in fact, the opposite effect of a negative pressure in the trachea. Open
Statement Gets contrast study at 5-7 days before pulling chest drain, timing depends on avoiding weekends Open
Quotation I do honestly, I'm a little bit old school in that. I still get an esophagram. I usually wait for about 5 days. Open
Statement Uses small TLS drain rather than formal chest tube, especially for open extrapleural approach Open
Statement Does not use transanastomotic tube based on Midwest Pediatric Surgical Consortium study showing much higher stricture and leak rate with transanastomotic tubes Open
Quotation I do not actually. I again, a practice of mine that has changed based on our results from the Midwest Pediatric Surgical Consortium study in which the patients who Had a trans anastomotic tube had a much higher stricture and leak rate than patients that did not have that. Open
Statement Midwest consortium study was presented at APSA and should be published in JPS soon Open
Quotation Unless it's an incredibly tight stricture, I would wait several weeks before doing a dilation unless I was worried there was going to be a complete obstruction. Open
Statement Unless incredibly tight stricture risking complete obstruction, would wait several weeks before first dilation Open
Statement Always appears to be narrowing at anastomosis because proximal pouch is dilated; as long as patent with free contrast flow distally, would hold off on dilation Open
Quotation There's always reported a narrowing because the proximal pouch is always dilated, so it always looks like there's a narrowing at the anastomosis. As long as it's patent and contrast flows freely down the distal esophagus, I would hold off on. Uh, dialing it, dilating it probably several weeks if possible. Open
Statement Huge spectrum from very tight strictures requiring many dilations to kids fine after one dilation Open
Statement Fairly aggressive with dilations: dilate, wait 1-2 weeks, restudy, potentially dilate again Open
Statement If case goes well and post-op study looks great, does not get routine follow-up esophagrams; studies based on clinical symptoms suggesting stricture Open
Quotation I think that again, having trained using bougie dilators, a balloon or radial dilation is, is less traumatic for the tissue Open
Statement Dilations done with GI colleagues in aerodigestive center for bigger kids or with interventional radiologists Open
Statement Balloon or radial dilation is less traumatic for tissue than bougie dilators Open
Quotation I would absolutely wait and do nothing. I would leave the drain in and usually that presents with some saliva in the drain, and I would just wait, and the vast majority of those close. Open
Statement For small leak with child not sick, would absolutely wait and do nothing, leaving drain in; vast majority close Open
Statement Would only go to OR for leak if child getting sicker, wide open leak, or large uncontrollable pneumothorax Open
Statement Even reasonably significant leaks will heal, then can deal with stricture postoperatively Open
Statement Sometimes little outpouching where leak happened makes it unclear if still leaking; if nothing from tube and nothing goes further, leak probably healed and would remove tube Open
Statement Usually waits one week between esophagrams for leak; if child continues to do well, restudies Open
Quotation I think that first of all I would make the comment that that's a great point and it's interesting to participate in the International oesophageal atresia. A meeting that occurs every 2 years and to go and see the patients because that meeting is multidisciplinary, including even patients, and you see these patients who come back who are in their 20s or 30s or 40s and they talk about the long term issues that they have and we tend to follow patients until they're, you know, until they're eating well and then they're gone Open
Statement International esophageal atresia meeting occurs every 2 years and is multidisciplinary including patients Open
Statement Patients in their 20s-40s at international meeting discuss long-term issues; surgeons tend to follow until eating well or age 18 then never see them again Open
Statement Have a lot to learn about long-term complications; personally follows patients for at least couple years which is probably not long enough Open
Statement More concerning are patients with ongoing reflux due to unknown long-term impact on Barrett's esophagus and potential malignant change Open
Quotation The patients that worry me more honestly are the patients who have ongoing reflux, and we don't know the long term impact of that on things like Barrett's esophagus and Potential long term risk for malignant change and those sorts of things. Open
Statement Biggest challenges in long-term follow-up are reflux and recurrent strictures Open
Quotation If I have a patient who has a stricture that's been dilated 2 or 3 times, my next move would be to address reflux. Most of the patients we leave on anti-reflux medications when they are discharged from the hospital. I would argue that virtually all patients have some degree of gastroesophageal reflux. If they have a stricture that is not responsive to dilations, it dilates easily and then restrictures. I personally am very aggressive about doing a fundoplication on those patients Open
Statement If patient has stricture dilated 2-3 times, next move would be to address reflux Open
Statement Most patients left on anti-reflux medications when discharged from hospital Open
Statement Very aggressive about fundoplication for strictures not responsive to dilations (dilates easily then restrictures) Open
Statement Virtually all patients have some degree of gastroesophageal reflux Open
Statement Would wait 4-6 weeks to see how close ends come together, then attempt primary repair once within 2 vertebral bodies Open
Statement For pure esophageal atresia, approach is G-tube placement with calibration of gap length using distal catheter pushed up and NG tube in proximal pouch Open
Quotation I do, but as with most Nissans, I make them loose and I make them short, so I usually use two or at most 3 stitches for the wrap and make sure that it is loose around the distal esophagus. Open
Statement Does Nissen fundoplication in patients with poor esophageal motility but makes them loose and short, using 2 or at most 3 stitches Open

Esophageal Replacement with Dr. Dan von Allmen

Quotation We have a tendency to say TEF when we mean esophageal atresia, so just be very careful when you're talking about these things that you're clear about what you're talking about, because when you say, oh, the patient had a TEF when really. They had is a pure esophageal atresia. That's just not correct. Open
Statement Esophageal atresia incidence is 1 in 4500 in the United States with slight male predominance Open
Statement Recurrence risk of esophageal atresia among parents who have one affected child is 0.5 to 2% Open
Statement Prenatal ultrasound detects only about half of esophageal atresia cases, primarily detecting pure atresia due to significant proximal pouch dilation Open
Statement The most common type of esophageal atresia is proximal atresia with distal fistula Open
Statement VACTERL is an association, not a syndrome, requiring 3 of the defects for diagnosis Open
Statement Esophageal atresia associated with significant cardiac anomalies and low birth weight has only 27 to 30% survival rate Open
Statement G-tube decompression or needle decompression helps temporarily but air still follows the low resistance pathway out of the G-tube instead of going into the lungs Open
Statement In type C fistula with respiratory distress, air continuously goes into the intestine via the low resistance pathway instead of into the lungs, causing increased stomach size and affecting respiratory compliance Open
Statement Putting vessel loop around distal esophagus is easier than dividing the fistula when in trouble during surgery Open
Statement Every child with tracheoesophageal fistula or esophageal atresia needs bronchoscopy due to 10-15% association of airway issues and to help plan surgery Open
Quotation We believe quite strongly that every child with a tracheoesophageal fistula or esophageal atresia needs a bronchoscopy. Open
Statement Virtually all kids with esophageal atresia have some element of tracheomalacia, though clinical significance varies Open
Quotation I personally believe that there is some Growth in the esophagus and typically our approach is to wait several weeks. I think once you get past probably 4 to 6 weeks, there's not going to be any more growth. Open
Statement There is growth in the esophagus over time, but once past 4 to 6 weeks there is not going to be any more growth Open
Statement For significant gap, approach is to support child with enteral bolus feedings to put stretch on the distal esophagus and get sequential gap studies, then take to OR at 6 weeks Open
Statement For thoracoscopy, patient positioning should be almost 45 degrees from prone, pushed to one side of bed, with camera port looking straight down at esophagus providing better visualization than open surgery Open
Statement The azygos vein guides to the level of the fistula because the fistula typically occurs right about the level of the azygos vein Open
Statement When placing clip on fistula, it must be flush with the trachea to avoid leaving a long pouch that can pool secretions and cause problems Open
Statement Anesthesiologist needs to push down repeatedly to help find the proximal pouch during surgery Open
Statement If CO2 skyrockets on ET tube during thoracoscopy, this indicates a hole in the trachea as CO2 from thoracoscopy goes up the trachea and out the endotracheal tube Open
Quotation You would much rather put a hole in the esophageal pouch than in the trachea. Open
Statement It is safer to put a hole in the esophageal pouch than in the trachea during dissection Open
Statement Foker procedure uses traction sutures brought out through chest wall with sequential tightening, as traction is a stimulus for esophageal growth Open
Statement Most common esophageal replacement is gastric pull-up, but other options include reverse gastric tube, colon interposition, and duodenal interposition, with none being clearly better than others Open
Statement Secondary Foker cases spent mean of 110 days in ICU and were paralyzed for more than 1 month Open
Statement In primary Foker cases, esophagus was successfully brought together 96% of the time, versus only 2/3 of the time in secondary cases after previous operation Open
Quotation I would say that none is any better than the other. Open
Statement Primary Foker cases spent more than 1 month in ICU on average and were paralyzed for more than 2 weeks Open
Statement Reverse gastric tube is created from greater curve of stomach and requires preserving gastroepiploic artery when placing initial G-tube, but has many problems Open
Statement Colon interposition advantage is tremendous length, allowing anastomosis to pharynx in caustic ingestion cases with no remaining esophagus Open
Quotation We were taught initially that these are hard to revise because you damage the blood supply to the colon, but that's actually not true. Open
Statement Colon interposition conduits can be revised despite teaching that revision damages blood supply Open
Statement Biggest challenge with colon interposition is that conduits dilate and elongate over time, with food collecting and not emptying Open
Statement In institutional series of first 13 colon interpositions, 12 of 13 patients were NPO at presentation, with no graft loss after initial case Open
Statement When performing initial repair with field already dissected, placing 3 posterior tracheopexy stitches takes 15 minutes and avoids need to operate through previously operated field later Open
Statement Posterior tracheopexy involves putting sutures into membranous trachea to hold it back for tracheomalacia management Open
Quotation As surgeons, we're frequently trained that we need to be the captain of the ship, and it's all about us, and we need to do everything. And one of the best things that happened to me when I came here and started doing these esophageal cases was partnering with our ENT group. Open
Daniel's statements about Etiologies (Gastroschisis/NEC/Atresia/Volvulus) 6 statements

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open
Daniel's statements about Fetal Surgery 52 statements

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Treatment for Long Gap Esophageal Atresia: Esophageal Elongation and Replacement

Statement At Cincinnati Children's, if a child has no gas in the abdomen, they place a G-tube and may put something up the distal esophagus for a fluoro shot, then wait a couple of weeks for a protocol gap measurement in interventional radiology. Open
Quotation Our method here is that if we have a child with no gas in the abdomen and we take them to the OR and put a G-tube in, many times we'll do, we'll put something up the distal esophagus at that point and just get a fluoro shot. But then we'd wait a couple of weeks and we have our patients go down to interventional radiology where we have a protocol for measuring the gap. Open
Statement At Cincinnati Children's, if a child has no gas in the abdomen, they place a G-tube and may put something up the distal esophagus for a fluoro shot, then wait a couple of weeks for a protocol gap measurement in interventional radiology. Open
Quotation Our method here is that if we have a child with no gas in the abdomen and we take them to the OR and put a G-tube in, many times we'll do, we'll put something up the distal esophagus at that point and just get a fluoro shot. But then we'd wait a couple of weeks and we have our patients go down to interventional radiology where we have a protocol for measuring the gap. Open
Quotation The physiology, which I believe in intensely, is that stretch is a very strong promoter of growth. And then if you put things on tension, they will actually grow over time. That's how the cardiovascular system develops in utero. Open
Quotation But the philosophy here is that with traction, you can get the two ends of the esophagus to grow. And if you can get them to grow far enough, you can put them together. Open
Quotation But the philosophy here is that with traction, you can get the two ends of the esophagus to grow. And if you can get them to grow far enough, you can put them together. Open
Quotation The physiology, which I believe in intensely, is that stretch is a very strong promoter of growth. And then if you put things on tension, they will actually grow over time. That's how the cardiovascular system develops in utero. Open
Statement Stretch is a very strong promoter of growth, and if you put things on tension they will actually grow over time, which is how the cardiovascular system develops in utero. Open
Statement The philosophy of traction-based elongation is that with traction, you can get the two ends of the esophagus to grow, and if you can get them to grow far enough, you can put them together. Open
Statement The philosophy of traction-based elongation is that with traction, you can get the two ends of the esophagus to grow, and if you can get them to grow far enough, you can put them together. Open
Statement Stretch is a very strong promoter of growth, and if you put things on tension they will actually grow over time, which is how the cardiovascular system develops in utero. Open
Quotation Obviously, you look at the result of getting the esophagus together and intact esophagus in 96% of patients in the primary group, about two thirds of patients in the secondary group. Open
Quotation About two thirds of patients with the primary repair were able to get the full oral nutrition and about 10% of the patients who had the secondary repair. Open
Statement In the Boston Group 2015 study, the primary group (de novo cases) achieved an intact esophagus in 96% of patients, while the secondary group (patients with previous operations) achieved this in about two thirds of patients. Open
Statement In the Boston Group 2015 study, the primary group had a median ICU stay of 70 days with a couple of weeks being paralyzed, and the secondary group had a median ICU stay of 110 days with a month of being paralyzed. Open
Statement In the Boston Group 2015 study, about two thirds of patients with primary repair were able to get full oral nutrition and about 10% of patients who had secondary repair achieved full oral nutrition. Open
Quotation There was a great article published by the Boston Group 2015. Divides the cases into two groups, a primary group and a secondary group. Secondary being patients who had had operations previously in the primary group, being cases that they saw de novo. Open
Quotation But these procedures are not without some morbidity. And you look at the ICU stay is a median of 70 days with a couple of weeks being paralyzed for the primary group and 110 days for the secondary group with a month of being paralyzed. Open
Quotation You divide the esophageal stump at the esophageal hiatus and mobilize the fundus and pull the fundus up and you can pull it up either in the anterior or posterior mediastinum. Open
Statement The surgical group from INOEA recommends gastric pull up as the first option for esophageal replacement, dividing the esophageal stump at the esophageal hiatus and mobilizing the fundus to pull it up in either the anterior or posterior mediastinum. Open
Statement The colon can be used as an interposition for esophageal replacement. Open
Quotation You can actually use the colon as an interposition as well. I personally was trained to do colon interpositions. Open
Quotation You could use the stomach. That would be called a gastric transposition. The surgical group from the INOEA, their recommendation, the recommendation for the first option is a gastric pull up. Open
Quotation You can actually use the colon as an interposition as well. I personally was trained to do colon interpositions. Open
Statement The colon can be used as an interposition for esophageal replacement. Open
Statement Common problems with colonic interposition are that the colon can dilate and become tortuous, and it's not uncommon to get a sigmoid sink drain deformity just above the diaphragm. Open
Quotation Common problems with this are that they dilate and they can become tortuous. It's not uncommon to get kind of a sigmoid sink drain deformity just above the diaphragm. Open
Statement Common problems with colonic interposition are that the colon can dilate and become tortuous, and it's not uncommon to get a sigmoid sink drain deformity just above the diaphragm. Open
Quotation Common problems with this are that they dilate and they can become tortuous. It's not uncommon to get kind of a sigmoid sink drain deformity just above the diaphragm. Open
Quotation So I wouldn't pretend to say that these don't need to be revised sometimes, but it is not impossible to revise them and the kids tend to do pretty well. Open
Quotation I was taught initially that you can't fix that and that it's too dangerous and that you'll risk the blood supply to the colon interposition. But I found that actually that's not really true. Open
Quotation I pass the interposition posterior to the stomach, which leaves the vascular pedicle along the spine. And you can mobilize the colon. You can divide the gastric dual aporotomy, divide the colon attachment to the stomach, and then mobilize that sigmoid redundancy transhiatally, and then reinestimose the colon to the stomach. Open
Statement Von Allman was initially taught that sigmoid redundancy in colonic interposition can't be fixed and that it's too dangerous because it will risk the blood supply, but he found that this is not really true. Open
Statement Von Allman passes the colonic interposition posterior to the stomach, which leaves the vascular pedicle along the spine, allowing mobilization of the colon by dividing the gastric duodenotomy and colon attachment to the stomach, then mobilizing the sigmoid redundancy transhiatally and reanastomosing the colon to the stomach. Open
Statement Von Allman was initially taught that sigmoid redundancy in colonic interposition can't be fixed and that it's too dangerous because it will risk the blood supply, but he found that this is not really true. Open
Statement Colonic interpositions sometimes need to be revised, but it is not impossible to revise them and the kids tend to do pretty well. Open
Statement Von Allman passes the colonic interposition posterior to the stomach, which leaves the vascular pedicle along the spine, allowing mobilization of the colon by dividing the gastric duodenotomy and colon attachment to the stomach, then mobilizing the sigmoid redundancy transhiatally and reanastomosing the colon to the stomach. Open
Statement Colonic interpositions sometimes need to be revised, but it is not impossible to revise them and the kids tend to do pretty well. Open
Quotation I was taught initially that you can't fix that and that it's too dangerous and that you'll risk the blood supply to the colon interposition. But I found that actually that's not really true. Open
Quotation I pass the interposition posterior to the stomach, which leaves the vascular pedicle along the spine. And you can mobilize the colon. You can divide the gastric dual aporotomy, divide the colon attachment to the stomach, and then mobilize that sigmoid redundancy transhiatally, and then reinestimose the colon to the stomach. Open
Quotation So I wouldn't pretend to say that these don't need to be revised sometimes, but it is not impossible to revise them and the kids tend to do pretty well. Open
Quotation You can get extraordinary length on this. And Mike and I have done a few cases for kids who had disasters, multiple operations elsewhere, who had caustic injuries that were involved all the way up to the pharynx, where we had to do a lot of work just on the pharynx to get that open, and then literally sew the colon interposition to the pharynx and then down to the stomach, which is obviously tough to do with a gastric pull-up. Open
Statement You can get extraordinary length with colonic interposition, allowing treatment of cases with caustic injuries extending to the pharynx by sewing the colon to the pharynx and down to the stomach, which is tough to do with a gastric pull-up. Open
Quotation You can get extraordinary length on this. And Mike and I have done a few cases for kids who had disasters, multiple operations elsewhere, who had caustic injuries that were involved all the way up to the pharynx, where we had to do a lot of work just on the pharynx to get that open, and then literally sew the colon interposition to the pharynx and then down to the stomach, which is obviously tough to do with a gastric pull-up. Open
Statement You can get extraordinary length with colonic interposition, allowing treatment of cases with caustic injuries extending to the pharynx by sewing the colon to the pharynx and down to the stomach, which is tough to do with a gastric pull-up. Open
Quotation I don't think we know we should do the studies to understand that. But I would say that this gets back to David van der Zee's comment about, go have a cup of coffee and come back and it'll be fine. Clearly, that's not growth, that's stretch. Open
Statement Von Allman states we don't know whether esophageal elongation is growth or stretch and should do studies to understand that, but notes that tension is a very good physiologic growth promoter in other organs, making this an area ripe for more basic science. Open
Quotation And yet there's very good physiologic data that tension is a growth promoter, not necessarily in the esophagus, but in other organs. So I think that it's an area ripe for a little more basic science. Open
Quotation I don't think we know we should do the studies to understand that. But I would say that this gets back to David van der Zee's comment about, go have a cup of coffee and come back and it'll be fine. Clearly, that's not growth, that's stretch. Open
Statement Von Allman states we don't know whether esophageal elongation is growth or stretch and should do studies to understand that, but notes that tension is a very good physiologic growth promoter in other organs, making this an area ripe for more basic science. Open
Quotation And yet there's very good physiologic data that tension is a growth promoter, not necessarily in the esophagus, but in other organs. So I think that it's an area ripe for a little more basic science. Open
Daniel's statements about Gastroesophageal Reflux 7 statements

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Gastoesophageal Reflux: Update Course 2015

Quotation Do they have data to support that statement, the significant morbidity? Open
Statement Immediate morbidity and mortality of laparoscopic fundoplication, even in infants, is very low and it is a safe operation Open
Quotation There's lots of articles that many in this room have authored that show that the immediate morbidity and mortality of a fundoplication, of a laparoscopic fundoplication, even in infants is very low. It's a safe operation. Open
Quotation Do PPIs stop vomiting? No. I, I never understood that acid reflux less acidic, right? Open
Statement PPIs do not stop vomiting; they make refluxed material less acidic Open
Quotation I started doing is I put an NG tube down, and if they do well with an NG tube, I just do a G tube. If they don't, I do a Nissan. That's as simple as I make it. Open
Statement Trial of bolus NG tube feeding predicts whether infant needs fundoplication: if they do well with NG tube, perform G-tube alone; if they don't, perform Nissen Open
Daniel's statements about Gastroesophageal Reflux Disease 7 statements

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Gastoesophageal Reflux: Update Course 2015

Quotation Do they have data to support that statement, the significant morbidity? Open
Statement Immediate morbidity and mortality of laparoscopic fundoplication, even in infants, is very low and it is a safe operation Open
Quotation There's lots of articles that many in this room have authored that show that the immediate morbidity and mortality of a fundoplication, of a laparoscopic fundoplication, even in infants is very low. It's a safe operation. Open
Statement PPIs do not stop vomiting; they make refluxed material less acidic Open
Quotation Do PPIs stop vomiting? No. I, I never understood that acid reflux less acidic, right? Open
Quotation I started doing is I put an NG tube down, and if they do well with an NG tube, I just do a G tube. If they don't, I do a Nissan. That's as simple as I make it. Open
Statement Trial of bolus NG tube feeding predicts whether infant needs fundoplication: if they do well with NG tube, perform G-tube alone; if they don't, perform Nissen Open
Daniel's statements about High-risk Neuroblastoma 14 statements

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Neuroblastoma: Update Course 2014

Quotation I think that the, the main controversy, at least from my perspective, that still persists is what to do with the high-risk patients. Open
Statement Survival rate for high-risk neuroblastoma patients is in the 38 to 40% range Open
Quotation The difference between what a surgeon says they did in the operating room and and what the postoperative imaging says they did in the operating room, and those are not necessarily the same. Open
Statement Surgeon-reported degree of resection showed only 66% concordance with radiologist assessment of postoperative imaging in tandem transplant pilot study Open
Statement Repeat study in recent COG high-risk trial showed same 66% concordance rate between surgeon op notes and postoperative imaging, with surgeons underestimating and radiologists overcalling resection completeness Open
Quotation We don't have a very good definition of what is a greater than 90% resection. Open
Quotation The more chemotherapy you give or other agents, and now things like MIBG, which is local radiation, um. Uh, that you actually make the tumor more fibrotic, and it makes that technique of getting down on the vessels and splitting it off much more difficult. Open
Statement More chemotherapy or other agents like MIBG may make tumor more fibrotic and make subadventitial dissection more difficult Open
Statement Complication rate for aggressive neuroblastoma resection is approximately 30% morbidity with mortality less than 1% Open
Statement High-risk neuroblastoma treatment includes tandem peripheral blood stem cell transplants, with second transplant given as soon as patient recovers from first, followed by immunotherapy and Retin-A Open
Quotation This is very important to support the aggressive approach that everybody in this room has suggested that they would, uh, that they would pursue. There actually now is some data to support that. Open
Statement COG 3973 study's inability to demonstrate overall survival benefit may be type 2 error due to smaller sample size (230 vs 1,300 patients in European study) Open
Quotation If you're not comfortable taking care of them, you should send them to somebody that is. Open
Statement Immunotherapy is effective in neuroblastoma in the setting of minimal residual disease Open
Daniel's statements about Intestinal Failure 12 statements

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open
Daniel's statements about Intestinal Rehab 12 statements

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open
Daniel's statements about Intestinal Transplant 6 statements

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Quotation I personally used to use them in my practice, but based on this study have stopped using them. Open
Statement Dr. von Almen personally used steroids after Kasai in the past but has stopped based on the START trial results. Open
Quotation The whole point of this is to preserve the native liver. Open
Statement Two proposed mechanisms for steroid benefit in biliary atresia: reducing ongoing inflammation to preserve ductules, and acting as a choleretic to maintain bile flow (analogous to diuresis after kidney transplant). Open
Quotation I think that you have to always be careful about generalizing studies, and in this study it is specifically testing a fairly long course, 1413 week course of high dose steroids. Open
Statement The START trial specifically tested a 13-week course of high-dose steroids; the lack of benefit applies to that regimen. Open
Daniel's statements about Long-gap Esophageal Atresia 21 statements

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Treatment for Long Gap Esophageal Atresia: Esophageal Elongation and Replacement

Statement At Cincinnati Children's, if a child has no gas in the abdomen, they place a G-tube and may put something up the distal esophagus for a fluoro shot, then wait a couple of weeks for a protocol gap measurement in interventional radiology. Open
Quotation Our method here is that if we have a child with no gas in the abdomen and we take them to the OR and put a G-tube in, many times we'll do, we'll put something up the distal esophagus at that point and just get a fluoro shot. But then we'd wait a couple of weeks and we have our patients go down to interventional radiology where we have a protocol for measuring the gap. Open
Statement The philosophy of traction-based elongation is that with traction, you can get the two ends of the esophagus to grow, and if you can get them to grow far enough, you can put them together. Open
Statement Stretch is a very strong promoter of growth, and if you put things on tension they will actually grow over time, which is how the cardiovascular system develops in utero. Open
Quotation The physiology, which I believe in intensely, is that stretch is a very strong promoter of growth. And then if you put things on tension, they will actually grow over time. That's how the cardiovascular system develops in utero. Open
Quotation But the philosophy here is that with traction, you can get the two ends of the esophagus to grow. And if you can get them to grow far enough, you can put them together. Open
Quotation You can actually use the colon as an interposition as well. I personally was trained to do colon interpositions. Open
Statement The colon can be used as an interposition for esophageal replacement. Open
Quotation Common problems with this are that they dilate and they can become tortuous. It's not uncommon to get kind of a sigmoid sink drain deformity just above the diaphragm. Open
Statement Common problems with colonic interposition are that the colon can dilate and become tortuous, and it's not uncommon to get a sigmoid sink drain deformity just above the diaphragm. Open
Quotation So I wouldn't pretend to say that these don't need to be revised sometimes, but it is not impossible to revise them and the kids tend to do pretty well. Open
Statement Colonic interpositions sometimes need to be revised, but it is not impossible to revise them and the kids tend to do pretty well. Open
Statement Von Allman was initially taught that sigmoid redundancy in colonic interposition can't be fixed and that it's too dangerous because it will risk the blood supply, but he found that this is not really true. Open
Statement Von Allman passes the colonic interposition posterior to the stomach, which leaves the vascular pedicle along the spine, allowing mobilization of the colon by dividing the gastric duodenotomy and colon attachment to the stomach, then mobilizing the sigmoid redundancy transhiatally and reanastomosing the colon to the stomach. Open
Quotation I was taught initially that you can't fix that and that it's too dangerous and that you'll risk the blood supply to the colon interposition. But I found that actually that's not really true. Open
Quotation I pass the interposition posterior to the stomach, which leaves the vascular pedicle along the spine. And you can mobilize the colon. You can divide the gastric dual aporotomy, divide the colon attachment to the stomach, and then mobilize that sigmoid redundancy transhiatally, and then reinestimose the colon to the stomach. Open
Statement You can get extraordinary length with colonic interposition, allowing treatment of cases with caustic injuries extending to the pharynx by sewing the colon to the pharynx and down to the stomach, which is tough to do with a gastric pull-up. Open
Quotation You can get extraordinary length on this. And Mike and I have done a few cases for kids who had disasters, multiple operations elsewhere, who had caustic injuries that were involved all the way up to the pharynx, where we had to do a lot of work just on the pharynx to get that open, and then literally sew the colon interposition to the pharynx and then down to the stomach, which is obviously tough to do with a gastric pull-up. Open
Quotation And yet there's very good physiologic data that tension is a growth promoter, not necessarily in the esophagus, but in other organs. So I think that it's an area ripe for a little more basic science. Open
Quotation I don't think we know we should do the studies to understand that. But I would say that this gets back to David van der Zee's comment about, go have a cup of coffee and come back and it'll be fine. Clearly, that's not growth, that's stretch. Open
Statement Von Allman states we don't know whether esophageal elongation is growth or stretch and should do studies to understand that, but notes that tension is a very good physiologic growth promoter in other organs, making this an area ripe for more basic science. Open
Daniel's statements about Metastatic Disease 37 statements

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Topics in 10: Neuroblastoma

Statement In younger patients, neuroblastoma is often picked up either prenatally on ultrasound, or in younger kids (two-year-old or three-year-old) as a solid abdominal mass. Open
Statement When neuroblastoma patients have metastatic disease, they may present with either bony pain or potentially neurologic symptoms from cord compression. Open
Quotation So in the younger patients, they're oftentimes picked up either prenatally on ultrasound, or in younger kids, say two-year-old or three-year-old, it might be picked up as a solid abdominal mass. Open
Statement When considering neuroblastoma as part of the differential diagnosis, it is important to get catecholamines (either urine or serum) as one of the most diagnostic laboratory tests for this tumor. Open
Statement If imaging suggests neuroblastoma (central abdominal mass or adrenal mass rather than kidney mass), the next test would be a nuclear medicine study, typically an MIBG study. Open
Statement The MIBG study is helpful for confirming the diagnosis of neuroblastoma and can also demonstrate metastatic disease. Open
Statement Most children with suspected neuroblastoma would get a cross-sectional imaging study, either a CT scan or an MRI. Open
Statement About 10% of neuroblastomas are MIBG negative. Open
Quotation About 10% of neuroblastomas are MIBG negative. Open
Statement Some centers, including Cincinnati Children's Hospital, would get a PET scan looking for tumor uptake as well as potential metastatic disease. Open
Statement Based on the most recent iteration of the neuroblastoma staging system (INRGSS), it is possible to assign a stage before any invasive procedure is performed. Open
Statement There is a special category MS for children less than 18 months of age who have metastases to either the bone marrow or the skin. Open
Statement In the INRGSS system, tumors that are localized are categorized as L1; if localized but have image-defined risk factors (encasing nerves or vessels), they are L2; if they have metastatic disease, they are M. Open
Statement The INRGSS pre-biopsy staging system was specifically created to allow studies from different centers in different countries to be compared based on the pre-surgical staging of the patient. Open
Statement The prior neuroblastoma staging system required tissue diagnosis before assigning a stage. Open
Statement Some surgeons would approach resection of a localized neuroblastoma with laparoscopy depending on the size of the tumor. Open
Statement A child with an adrenal mass on the right side and a positive MIBG scan but no evidence of metastases could potentially be treated with a primary resection of the mass via laparotomy. Open
Statement For very large masses that encase the aorta, cava, or other major vasculature, all you really want is tissue for diagnosis, which can be obtained through open biopsy, laparoscopic biopsy, or core needle biopsies done by an interventional radiologist. Open
Statement In addition to NMIC status, you want to look for 1P and 11Q deletions and Shimada histology in neuroblastoma biopsy tissue. Open
Statement The most important biologic risk determinant to obtain from neuroblastoma biopsy tissue is the NMIC status. Open
Statement Neuroblastoma risk is divided about 50-50 between the low risk categories and the high risk category, with a smaller percentage being intermediate risk. Open
Statement Biologic risk determinants from biopsy will tell you what risk category the patient falls into: very low risk, low risk, intermediate risk, or high risk. Open
Statement Patients with high risk neuroblastoma receive aggressive chemotherapy including peripheral stem cell transplant times 2, aggressive surgery with the goal of greater than 90% resection of the tumor, followed by radiation, immunotherapy after chemotherapy, and potentially retinoic acid therapy. Open
Quotation NMIC and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement NMIC amplification and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement For intermediate risk neuroblastoma, the goal at the time of debulking or resecting the primary tumor is to achieve at least a 50% response from the initial volume of the primary tumor through the combination of neoadjuvant chemotherapy and surgical resection. Open
Statement Intermediate risk neuroblastoma tumors get varying cycles of chemotherapy based on the biologic risk factors they have. Open
Statement The low risk neuroblastoma group, depending on the actual age of the patient and how it is diagnosed, could potentially be followed simply with observation. Open
Statement Jed Nocturne led a study through the Children's Oncology Group looking at patients less than six months of age with either a prenatally diagnosed or shortly postnatally diagnosed localized mass, showing these patients can be observed with the expectation that the vast majority will avoid any type of surgical procedure. Open
Quotation MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow, specifically not bone, not cortical bone, and is less than 18 months of age. Open
Statement If MS disease patients progress or develop respiratory issues because of an enlarging liver mass, treatment might be elected because of the complication of the size of the tumor, but the tumor itself usually does not have to be treated. Open
Statement In patients with MS disease, simple observation can be the treatment path. Open
Statement MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow (specifically not bone, not cortical bone) and is less than 18 months of age. Open
Statement If the metastatic disease is progressing on neoadjuvant chemotherapy, then surgery is not indicated. Open
Statement If the tumor and metastatic disease are responding to neoadjuvant chemotherapy, one would attack the primary tumor site with a resection, with many advocating for attempting a greater than 90% resection. Open
Statement Patients with metastatic neuroblastoma typically receive four or five cycles of neoadjuvant chemotherapy and then are reassessed. Open
Statement You can biopsy the skin lesions in MS disease and that will give you the diagnosis. Open
Daniel's statements about Neuroblastoma 114 statements

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Neuroblastoma: Update Course 2014

Quotation I think that the, the main controversy, at least from my perspective, that still persists is what to do with the high-risk patients. Open
Quotation I think that the, the main controversy, at least from my perspective, that still persists is what to do with the high-risk patients. Open
Statement Survival rate for high-risk neuroblastoma patients is in the 38 to 40% range Open
Statement Survival rate for high-risk neuroblastoma patients is in the 38 to 40% range Open
Quotation The difference between what a surgeon says they did in the operating room and and what the postoperative imaging says they did in the operating room, and those are not necessarily the same. Open
Quotation The difference between what a surgeon says they did in the operating room and and what the postoperative imaging says they did in the operating room, and those are not necessarily the same. Open
Statement Surgeon-reported degree of resection showed only 66% concordance with radiologist assessment of postoperative imaging in tandem transplant pilot study Open
Statement Surgeon-reported degree of resection showed only 66% concordance with radiologist assessment of postoperative imaging in tandem transplant pilot study Open
Statement Repeat study in recent COG high-risk trial showed same 66% concordance rate between surgeon op notes and postoperative imaging, with surgeons underestimating and radiologists overcalling resection completeness Open
Statement Repeat study in recent COG high-risk trial showed same 66% concordance rate between surgeon op notes and postoperative imaging, with surgeons underestimating and radiologists overcalling resection completeness Open
Quotation We don't have a very good definition of what is a greater than 90% resection. Open
Quotation We don't have a very good definition of what is a greater than 90% resection. Open
Statement Memorial Sloan Kettering data suggests biggest volume response of neuroblastoma tumor occurs with first two cycles of chemotherapy, with very little response after that Open
Quotation The more chemotherapy you give or other agents, and now things like MIBG, which is local radiation, um. Uh, that you actually make the tumor more fibrotic, and it makes that technique of getting down on the vessels and splitting it off much more difficult. Open
Quotation The more chemotherapy you give or other agents, and now things like MIBG, which is local radiation, um. Uh, that you actually make the tumor more fibrotic, and it makes that technique of getting down on the vessels and splitting it off much more difficult. Open
Statement More chemotherapy or other agents like MIBG may make tumor more fibrotic and make subadventitial dissection more difficult Open
Statement More chemotherapy or other agents like MIBG may make tumor more fibrotic and make subadventitial dissection more difficult Open
Statement Complication rate for aggressive neuroblastoma resection is approximately 30% morbidity with mortality less than 1% Open
Statement Complication rate for aggressive neuroblastoma resection is approximately 30% morbidity with mortality less than 1% Open
Statement High-risk neuroblastoma treatment includes tandem peripheral blood stem cell transplants, with second transplant given as soon as patient recovers from first, followed by immunotherapy and Retin-A Open
Statement High-risk neuroblastoma treatment includes tandem peripheral blood stem cell transplants, with second transplant given as soon as patient recovers from first, followed by immunotherapy and Retin-A Open
Statement German study of 278 stage 4 high-risk neuroblastoma patients achieved complete resection in almost half and >90% resection in another quarter (75% total with >90% resection) Open
Statement German study showed overall survival 45%, event-free survival 33%, and local progression-free survival 58% in stage 4 neuroblastoma Open
Statement German study showed no difference in overall survival, event-free survival, or local progression-free survival based on completeness of resection in stage 4 neuroblastoma Open
Statement German study concluded aggressive surgery is not justified in stage 4 neuroblastoma, that limited operations decrease complications, and there is limited impact on patient outcome Open
Statement European study included 1,324 high-risk neuroblastoma patients (stages 2, 3, and 4) and achieved 76% with >95% resection Open
Statement European neuroblastoma study showed 0.5% mortality and 10% morbidity (30% including lesser complications) Open
Statement European study showed significant improvement in event-free survival and overall survival with aggressive resection in high-risk neuroblastoma - first study to show overall survival benefit Open
Quotation This is very important to support the aggressive approach that everybody in this room has suggested that they would, uh, that they would pursue. There actually now is some data to support that. Open
Quotation This is very important to support the aggressive approach that everybody in this room has suggested that they would, uh, that they would pursue. There actually now is some data to support that. Open
Statement European study concluded >95% resection results in improvement in event-free survival in high-risk neuroblastoma Open
Statement COG 3973 study of approximately 230 high-risk neuroblastoma patients showed significant improvement in local relapse-free survival and event-free survival but not overall survival Open
Statement COG 3973 study's inability to demonstrate overall survival benefit may be type 2 error due to smaller sample size (230 vs 1,300 patients in European study) Open
Statement COG 3973 study's inability to demonstrate overall survival benefit may be type 2 error due to smaller sample size (230 vs 1,300 patients in European study) Open
Quotation If you're not comfortable taking care of them, you should send them to somebody that is. Open
Quotation If you're not comfortable taking care of them, you should send them to somebody that is. Open
Statement European neuroblastoma procedures are performed in more than 200 hospitals yet still demonstrated survival improvements Open
Statement Older data shows worse survival in neuroblastoma when kidney is removed, likely because single kidney limits chemotherapy dosing Open
Quotation If you can't be informed, be opinionated Open
Statement Immunotherapy is effective in neuroblastoma in the setting of minimal residual disease Open
Statement Immunotherapy is effective in neuroblastoma in the setting of minimal residual disease Open

Neuroblastoma

Statement Other differential diagnoses for suprarenal mass include neuroblastoma, pulmonary sequestration below the diaphragm, and misdiagnosed renal anomaly Open
Statement Adrenal hemorrhage is the most common differential diagnosis for prenatal suprarenal mass, more common with history of fetal stress Open
Statement MIBG scan is the next step if catecholamines are elevated Open
Statement Radiologists are quite good at identifying adrenal hemorrhage on ultrasound Open
Statement In perinatal phase, most common metastatic sites are liver, bone, skin, and lymph nodes Open
Statement Nocktern study data supports observation of prenatal neuroblastoma with careful ultrasound surveillance Open
Statement Case report: child with observed prenatal adrenal mass that resolved presented at age 3 with widely metastatic high-risk neuroblastoma Open
Quotation The only caution I would raise is that we had a case of a child who presented with exactly this scenario, was observed, the adrenal mass went away and age at age three she presented with widely metastatic high risk neuroblastoma. Open
Statement Lymph node status in neuroblastoma is not as important for therapy changes as in Wilms tumor Open
Statement Primary concern in stage MS with liver involvement is mass effect causing respiratory compromise Open
Quotation The most common thing that we worry about is is the mass, is the mass effect of the tumor in the liver, which can be really dramatic and causes respiratory compromise. Open
Statement Classic findings of stage MS (high catecholamines, blue blebs on skin, liver metastasis, adrenal mass) may not require biopsy Open
Statement 10% of neuroblastomas are not MIBG avid Open
Statement PET scan may detect metastases in MIBG-negative neuroblastomas Open

Topics in 10: Neuroblastoma

Statement In younger patients, neuroblastoma is often picked up either prenatally on ultrasound, or in younger kids (two-year-old or three-year-old) as a solid abdominal mass. Open
Quotation So in the younger patients, they're oftentimes picked up either prenatally on ultrasound, or in younger kids, say two-year-old or three-year-old, it might be picked up as a solid abdominal mass. Open
Statement When neuroblastoma patients have metastatic disease, they may present with either bony pain or potentially neurologic symptoms from cord compression. Open
Statement When considering neuroblastoma as part of the differential diagnosis, it is important to get catecholamines (either urine or serum) as one of the most diagnostic laboratory tests for this tumor. Open
Statement The MIBG study is helpful for confirming the diagnosis of neuroblastoma and can also demonstrate metastatic disease. Open
Statement Most children with suspected neuroblastoma would get a cross-sectional imaging study, either a CT scan or an MRI. Open
Statement If imaging suggests neuroblastoma (central abdominal mass or adrenal mass rather than kidney mass), the next test would be a nuclear medicine study, typically an MIBG study. Open
Quotation About 10% of neuroblastomas are MIBG negative. Open
Statement Some centers, including Cincinnati Children's Hospital, would get a PET scan looking for tumor uptake as well as potential metastatic disease. Open
Statement About 10% of neuroblastomas are MIBG negative. Open
Statement In the INRGSS system, tumors that are localized are categorized as L1; if localized but have image-defined risk factors (encasing nerves or vessels), they are L2; if they have metastatic disease, they are M. Open
Statement Based on the most recent iteration of the neuroblastoma staging system (INRGSS), it is possible to assign a stage before any invasive procedure is performed. Open
Statement There is a special category MS for children less than 18 months of age who have metastases to either the bone marrow or the skin. Open
Statement The prior neuroblastoma staging system required tissue diagnosis before assigning a stage. Open
Statement The INRGSS pre-biopsy staging system was specifically created to allow studies from different centers in different countries to be compared based on the pre-surgical staging of the patient. Open
Statement A child with an adrenal mass on the right side and a positive MIBG scan but no evidence of metastases could potentially be treated with a primary resection of the mass via laparotomy. Open
Statement Some surgeons would approach resection of a localized neuroblastoma with laparoscopy depending on the size of the tumor. Open
Statement For very large masses that encase the aorta, cava, or other major vasculature, all you really want is tissue for diagnosis, which can be obtained through open biopsy, laparoscopic biopsy, or core needle biopsies done by an interventional radiologist. Open
Statement The most important biologic risk determinant to obtain from neuroblastoma biopsy tissue is the NMIC status. Open
Statement In addition to NMIC status, you want to look for 1P and 11Q deletions and Shimada histology in neuroblastoma biopsy tissue. Open
Statement Biologic risk determinants from biopsy will tell you what risk category the patient falls into: very low risk, low risk, intermediate risk, or high risk. Open
Statement Neuroblastoma risk is divided about 50-50 between the low risk categories and the high risk category, with a smaller percentage being intermediate risk. Open
Statement Patients with high risk neuroblastoma receive aggressive chemotherapy including peripheral stem cell transplant times 2, aggressive surgery with the goal of greater than 90% resection of the tumor, followed by radiation, immunotherapy after chemotherapy, and potentially retinoic acid therapy. Open
Quotation NMIC and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement NMIC amplification and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement For intermediate risk neuroblastoma, the goal at the time of debulking or resecting the primary tumor is to achieve at least a 50% response from the initial volume of the primary tumor through the combination of neoadjuvant chemotherapy and surgical resection. Open
Statement Intermediate risk neuroblastoma tumors get varying cycles of chemotherapy based on the biologic risk factors they have. Open
Statement Jed Nocturne led a study through the Children's Oncology Group looking at patients less than six months of age with either a prenatally diagnosed or shortly postnatally diagnosed localized mass, showing these patients can be observed with the expectation that the vast majority will avoid any type of surgical procedure. Open
Statement The low risk neuroblastoma group, depending on the actual age of the patient and how it is diagnosed, could potentially be followed simply with observation. Open
Statement If the metastatic disease is progressing on neoadjuvant chemotherapy, then surgery is not indicated. Open
Statement If the tumor and metastatic disease are responding to neoadjuvant chemotherapy, one would attack the primary tumor site with a resection, with many advocating for attempting a greater than 90% resection. Open
Statement MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow (specifically not bone, not cortical bone) and is less than 18 months of age. Open
Statement In patients with MS disease, simple observation can be the treatment path. Open
Statement If MS disease patients progress or develop respiratory issues because of an enlarging liver mass, treatment might be elected because of the complication of the size of the tumor, but the tumor itself usually does not have to be treated. Open
Quotation MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow, specifically not bone, not cortical bone, and is less than 18 months of age. Open
Statement Patients with metastatic neuroblastoma typically receive four or five cycles of neoadjuvant chemotherapy and then are reassessed. Open
Statement You can biopsy the skin lesions in MS disease and that will give you the diagnosis. Open

Neuroblastoma

Statement Adrenal hemorrhage is the most common cause of prenatal suprarenal mass, especially with history of fetal stress Open
Statement The GetNucturne study showed that prenatal/neonatal neuroblastomas can be safely observed with careful ultrasound surveillance, with many patients spared surgery Open
Statement Lymph node status in neuroblastoma does not change therapy, unlike Wilms tumor Open
Statement Approximately 10% of neuroblastomas are not MIBG-avid, which is when PET scan may be useful Open
Statement Tumor shrinkage in neuroblastoma plateaus after cycle 2-3 of chemotherapy per LaQuaglia and Von Allman studies Open
Statement COG high-risk study showed >90% resection improved event-free survival but not overall survival (approximately 245 patients) Open
Statement European neuroblastoma group study (approximately 1000 cases) showed >90% resection improved both event-free survival and overall survival Open
Statement Approximately 70% of high-risk neuroblastoma patients can achieve >90% resection Open
Statement Neuroblastoma theoretically does not invade vessel adventitia, though invasion has been observed Open
Statement There is zero correlation between surgeon operative note description of resection extent and post-operative imaging findings Open
Quotation There is zero correlation, zero. There is absolutely no correlation between the findings on post-op imaging and the findings that are reported in the op note. Open

Update Course Rewind: Highlighting Collaboration from Surgery & Interventional Radiology in the OR 2024

Statement A hybrid OR is not required for image-guided surgery; collaboration can occur in regular ORs with ultrasound machines or in dual settings where patients go to interventional radiology for localization then to the OR. Open
Quotation So it's important to understand that you don't have to have a hybrid OR to be able to do this type of collaboration. Open
Quotation And I think this is technology that many people have and don't realize that they have available at their institutions even if they're not. Do it within a hybrid OR space. Open
Statement Cone beam CT technology is available at many institutions even if they don't have a hybrid OR space, though people may not realize they have it. Open
Statement During neuroblastoma excisions when dissecting near the aorta, ultrasound can be used every 5 minutes to identify the celiac takeoff and reorient the surgeon. Open
Statement Ultrasound can be used at the end of cases to confirm vessel patency, such as looking at flow in kidney transplants, portal flow after Mesorex bypass, or renal flow after challenging neuroblastoma excisions where the renal hilum has been skeletonized. Open
Statement Pulmonary nodule localization can be done with wire, coil, or dye depending on institutional preference. Open
Statement At one institution (Laurie), pulmonary nodule localization is performed in two locations: first in pre-op CT where interventional radiologists use CT guidance to place a coil next to the nodule, then in the OR using fluoroscopy under two orthogonal planes to ensure accurate nodule identification and removal. Open
Statement Using coil localization combined with dye eliminates the risk of wire displacement. Open
Statement Vascular malformations are often managed in multidisciplinary VLC clinics where interventional radiologists offer sclerotherapy and pediatric surgeons offer resection, but sometimes a combination approach is best. Open
Statement For vascular malformations not amenable to sclerotherapy alone, interventional radiologists can perform angiography, directly inject contrast, and place glue to ensure complete resection of small outpouchings or legs. Open
Daniel's statements about Soft Tissue Sarcoma (lymph nodes) 59 statements

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Topics in 10: Neuroblastoma

Quotation So in the younger patients, they're oftentimes picked up either prenatally on ultrasound, or in younger kids, say two-year-old or three-year-old, it might be picked up as a solid abdominal mass. Open
Statement In younger patients, neuroblastoma is often picked up either prenatally on ultrasound, or in younger kids (two-year-old or three-year-old) as a solid abdominal mass. Open
Statement When neuroblastoma patients have metastatic disease, they may present with either bony pain or potentially neurologic symptoms from cord compression. Open
Statement When considering neuroblastoma as part of the differential diagnosis, it is important to get catecholamines (either urine or serum) as one of the most diagnostic laboratory tests for this tumor. Open
Statement Most children with suspected neuroblastoma would get a cross-sectional imaging study, either a CT scan or an MRI. Open
Statement The MIBG study is helpful for confirming the diagnosis of neuroblastoma and can also demonstrate metastatic disease. Open
Statement If imaging suggests neuroblastoma (central abdominal mass or adrenal mass rather than kidney mass), the next test would be a nuclear medicine study, typically an MIBG study. Open
Statement About 10% of neuroblastomas are MIBG negative. Open
Statement Some centers, including Cincinnati Children's Hospital, would get a PET scan looking for tumor uptake as well as potential metastatic disease. Open
Quotation About 10% of neuroblastomas are MIBG negative. Open
Statement There is a special category MS for children less than 18 months of age who have metastases to either the bone marrow or the skin. Open
Statement Based on the most recent iteration of the neuroblastoma staging system (INRGSS), it is possible to assign a stage before any invasive procedure is performed. Open
Statement In the INRGSS system, tumors that are localized are categorized as L1; if localized but have image-defined risk factors (encasing nerves or vessels), they are L2; if they have metastatic disease, they are M. Open
Statement The INRGSS pre-biopsy staging system was specifically created to allow studies from different centers in different countries to be compared based on the pre-surgical staging of the patient. Open
Statement The prior neuroblastoma staging system required tissue diagnosis before assigning a stage. Open
Statement Some surgeons would approach resection of a localized neuroblastoma with laparoscopy depending on the size of the tumor. Open
Statement A child with an adrenal mass on the right side and a positive MIBG scan but no evidence of metastases could potentially be treated with a primary resection of the mass via laparotomy. Open
Statement For very large masses that encase the aorta, cava, or other major vasculature, all you really want is tissue for diagnosis, which can be obtained through open biopsy, laparoscopic biopsy, or core needle biopsies done by an interventional radiologist. Open
Statement In addition to NMIC status, you want to look for 1P and 11Q deletions and Shimada histology in neuroblastoma biopsy tissue. Open
Statement The most important biologic risk determinant to obtain from neuroblastoma biopsy tissue is the NMIC status. Open
Statement Neuroblastoma risk is divided about 50-50 between the low risk categories and the high risk category, with a smaller percentage being intermediate risk. Open
Statement Biologic risk determinants from biopsy will tell you what risk category the patient falls into: very low risk, low risk, intermediate risk, or high risk. Open
Statement NMIC amplification and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement Patients with high risk neuroblastoma receive aggressive chemotherapy including peripheral stem cell transplant times 2, aggressive surgery with the goal of greater than 90% resection of the tumor, followed by radiation, immunotherapy after chemotherapy, and potentially retinoic acid therapy. Open
Quotation NMIC and age greater than 18 months are the most important prognostic determinants for neuroblastoma. Open
Statement For intermediate risk neuroblastoma, the goal at the time of debulking or resecting the primary tumor is to achieve at least a 50% response from the initial volume of the primary tumor through the combination of neoadjuvant chemotherapy and surgical resection. Open
Statement Intermediate risk neuroblastoma tumors get varying cycles of chemotherapy based on the biologic risk factors they have. Open
Statement Jed Nocturne led a study through the Children's Oncology Group looking at patients less than six months of age with either a prenatally diagnosed or shortly postnatally diagnosed localized mass, showing these patients can be observed with the expectation that the vast majority will avoid any type of surgical procedure. Open
Statement The low risk neuroblastoma group, depending on the actual age of the patient and how it is diagnosed, could potentially be followed simply with observation. Open
Statement If the tumor and metastatic disease are responding to neoadjuvant chemotherapy, one would attack the primary tumor site with a resection, with many advocating for attempting a greater than 90% resection. Open
Statement If MS disease patients progress or develop respiratory issues because of an enlarging liver mass, treatment might be elected because of the complication of the size of the tumor, but the tumor itself usually does not have to be treated. Open
Quotation MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow, specifically not bone, not cortical bone, and is less than 18 months of age. Open
Statement MS disease is specifically for patients who have a primary site with metastatic disease to the liver, the skin, or the bone marrow (specifically not bone, not cortical bone) and is less than 18 months of age. Open
Statement In patients with MS disease, simple observation can be the treatment path. Open
Statement Patients with metastatic neuroblastoma typically receive four or five cycles of neoadjuvant chemotherapy and then are reassessed. Open
Statement If the metastatic disease is progressing on neoadjuvant chemotherapy, then surgery is not indicated. Open
Statement You can biopsy the skin lesions in MS disease and that will give you the diagnosis. Open

Neuroblastoma

Statement Adrenal hemorrhage is the most common cause of prenatal suprarenal mass, especially with history of fetal stress Open
Statement The GetNucturne study showed that prenatal/neonatal neuroblastomas can be safely observed with careful ultrasound surveillance, with many patients spared surgery Open
Statement Lymph node status in neuroblastoma does not change therapy, unlike Wilms tumor Open
Statement Approximately 10% of neuroblastomas are not MIBG-avid, which is when PET scan may be useful Open
Statement Tumor shrinkage in neuroblastoma plateaus after cycle 2-3 of chemotherapy per LaQuaglia and Von Allman studies Open
Statement COG high-risk study showed >90% resection improved event-free survival but not overall survival (approximately 245 patients) Open
Statement European neuroblastoma group study (approximately 1000 cases) showed >90% resection improved both event-free survival and overall survival Open
Statement Approximately 70% of high-risk neuroblastoma patients can achieve >90% resection Open
Statement Neuroblastoma theoretically does not invade vessel adventitia, though invasion has been observed Open
Quotation There is zero correlation, zero. There is absolutely no correlation between the findings on post-op imaging and the findings that are reported in the op note. Open
Statement There is zero correlation between surgeon operative note description of resection extent and post-operative imaging findings Open

Update Course Rewind: Highlighting Collaboration from Surgery & Interventional Radiology in the OR 2024

Statement A hybrid OR is not required for image-guided surgery; collaboration can occur in regular ORs with ultrasound machines or in dual settings where patients go to interventional radiology for localization then to the OR. Open
Quotation So it's important to understand that you don't have to have a hybrid OR to be able to do this type of collaboration. Open
Quotation And I think this is technology that many people have and don't realize that they have available at their institutions even if they're not. Do it within a hybrid OR space. Open
Statement Cone beam CT technology is available at many institutions even if they don't have a hybrid OR space, though people may not realize they have it. Open
Statement During neuroblastoma excisions when dissecting near the aorta, ultrasound can be used every 5 minutes to identify the celiac takeoff and reorient the surgeon. Open
Statement Ultrasound can be used at the end of cases to confirm vessel patency, such as looking at flow in kidney transplants, portal flow after Mesorex bypass, or renal flow after challenging neuroblastoma excisions where the renal hilum has been skeletonized. Open
Statement Pulmonary nodule localization can be done with wire, coil, or dye depending on institutional preference. Open
Statement At one institution (Laurie), pulmonary nodule localization is performed in two locations: first in pre-op CT where interventional radiologists use CT guidance to place a coil next to the nodule, then in the OR using fluoroscopy under two orthogonal planes to ensure accurate nodule identification and removal. Open
Statement Using coil localization combined with dye eliminates the risk of wire displacement. Open
Statement Vascular malformations are often managed in multidisciplinary VLC clinics where interventional radiologists offer sclerotherapy and pediatric surgeons offer resection, but sometimes a combination approach is best. Open
Statement For vascular malformations not amenable to sclerotherapy alone, interventional radiologists can perform angiography, directly inject contrast, and place glue to ensure complete resection of small outpouchings or legs. Open
Daniel's statements about Tracheoesophageal Fistula 282 statements

Open the Tracheoesophageal Fistula collection →

Aerodigestive & Esophageal Surgery - Difficult Tracheal Esophageal Fistula

Statement If a patient is aspirating from above (oropharyngeal or esophageal source), an anti-reflux procedure does not help and may make them worse. Open
Statement Routine bronchoscopy is now standard practice for all type C TEF repairs at Cincinnati Children's, often done in collaboration with ENT colleagues. Open
Statement Dual scoping (simultaneous bronchoscopy and esophagoscopy) allows scopes to 'shake hands' across a fistula, light transillumination through tissue, and injection of saline or air to reveal subtle openings. Open
Quotation Button batteries are bad. Open
Quotation The tissue damage extends beyond what you can appreciate with the naked eye, and the progression of it probably extends beyond what you would think is the normal time frame. Open
Statement In a patient with multiple bronchoesophageal fistulas and chronic bronchiectasis, lobectomy with resection of the esophageal pseudo-diverticulum can be performed safely; the esophagus can be primarily closed if not strictured. Open

Tracheoesophageal Fistula with Dr. Daniel von Allmen

Quotation It certainly is an interest that we have here with a whole group here in Cincinnati, but it can be an incredibly difficult, challenging problem. It's one of the great cases in pediatric surgery and one of the most difficult cases, so it really runs the whole spectrum for sure. Open
Statement Cincinnati Children's has a multidisciplinary aerodigestive center that includes ENT airway surgeons, GI, pulmonary, general surgeons, nurse practitioners, geneticists, and dietitians. Open
Statement Injecting air into the NG tube and obtaining a plain film helps visualize distention of the proximal esophageal pouch. Open
Quotation If I can't see a distended proximal pouch, then I worry. I worry that either one of two things either that the diagnosis is incorrect and in fact it is not an esophageal atresia, or that there could be a proximal fistula which is allowing that proximal pouch to decompress. Open
Statement Lack of visible proximal pouch distention raises concern for either incorrect diagnosis or presence of a proximal fistula allowing pouch decompression. Open
Statement Preoperative echocardiogram is essential to assess cardiac anatomy and identify right-sided aortic arch. Open
Statement Renal ultrasound and spine evaluation for tethered cord are needed but are not necessary before addressing esophageal atresia. Open
Statement Conventional wisdom suggests left thoracotomy for right-sided aortic arch, though the operation is somewhat more difficult with the heart more in the way. Open
Statement If right-sided arch is discovered intraoperatively during right thoracotomy, it is possible to complete the operation from that side, though some reports suggest higher incidence of swallowing problems. Open
Statement In a stable, larger baby not requiring intubation, it is reasonable to wait until full resources are available (Sunday or Monday) rather than operating emergently on Saturday. Open
Statement Prolonged delay risks colonization of the GI tract and soiling of the lungs. Open
Statement Oscillator ventilation has not been found to make a significant difference in managing infants with large fistulas and abdominal distention. Open
Statement Air preferentially entering the GI tract rather than lungs is a bigger problem in more premature infants with poor lung compliance. Open
Statement Treating with surfactant and improving lung compliance helps as much as changing ventilator type in managing fistula-related ventilation problems. Open
Statement One risk in managing the deteriorating infant with TEF is waiting too long to make a decision, which can lead to an emergency situation with inability to ventilate. Open
Quotation One of the risks is to wait too long to make a decision, and, and I've made that mistake myself, and then it becomes an emergency if you can't ventilate the child. Open
Statement Bronchoscopic placement of Fogarty balloon in the fistula is difficult unless expertise and equipment are immediately available. Open
Statement G-tube decompression may paradoxically worsen ventilation by creating even lower resistance pathway, allowing more air to flow from trachea to GI tract rather than to lungs. Open
Statement Laparotomy with vessel loop around gastroesophageal junction with gentle traction (Rommel tourniquet technique) can be used to stabilize critically ill infant, with G-tube placement and delayed definitive repair after several days. Open
Statement In esophageal atresia with associated duodenal atresia, if the child is stable, consider fixing duodenal atresia first to avoid repairing esophagus upstream from an obstruction. Open
Statement Both esophageal and duodenal atresia can potentially be repaired in the same operative setting if the child is stable enough. Open
Statement Routine intraoperative bronchoscopy documents fistula location, assesses for proximal fistula, and most importantly identifies laryngeal clefts that are easily missed. Open
Statement Laryngeal clefts have been seen many times in referred patients who had multiple thoracotomies but never had bronchoscopy. Open
Quotation We have seen that many times in patients who are referred who've had multiple thoracotomies. Nobody ever did a bronchoscopy and the child actually has a laryngeal cleft. Open
Statement The rate of missed second fistula is approximately 1%. Open
Statement High fistulas exist on a spectrum from essentially a trifurcation of the carina to fistulas high enough to be approachable through the neck. Open
Statement Endotracheal tube positioning depends on fistula location: for fistula at carina (trifurcation), tube should be placed higher; for high fistula, tube should be placed distal to fistula but not into the fistula itself. Open
Statement Extrapleural approach offers the advantage of potentially limiting soilage of pleural space if there is a postoperative leak. Open
Statement Dividing the azygos vein has no morbidity, gives better access, and frequently guides to the fistula location. Open
Statement For open repair, the azygos is typically ligated and divided with suture. Open
Statement The 3mm Just Right surgical sealer is an ideal device for dividing the azygos in this size patient. Open
Statement To establish extrapleural plane, place heel of right angle on ribs and spread to nicely show the plane, then mobilize pleura posteriorly to expose azygos. Open
Statement Mobilizing pleura up around apex of lung is important to allow access for proximal pouch mobilization. Open
Statement Distal esophageal segment is controlled with vessel loop, allowing proximal dissection to identify the fistula site. Open
Statement Contrary to standard teaching that distal esophagus should not be mobilized, it can be mobilized extensively especially laterally, all the way to the diaphragm. Open
Statement Medial mobilization of distal esophagus requires more caution due to blood supply concerns. Open
Statement Extensive proximal pouch mobilization provides the most important length gain for anastomosis. Open
Statement For proximal dissection, blade cautery with light buzz followed by mostly blunt dissection with flat end of blade, staying right on the thickened esophagus, is preferred; better to enter esophagus than trachea high in chest. Open
Quotation I would rather be in the esophagus than be in the trachea up high in the chest. Open
Statement For large tracheal injury, sleeve resection is possible given tracheal mobility; pericardium or autologous tissue should be interposed between trachea and esophageal repair. Open
Statement 5-0 PDS (absorbable monofilament) is the preferred suture for esophageal anastomosis; silk is not favored. Open
Statement Myotomies are not favored because they potentially create even more dysfunctional esophageal segment; motility is already abnormal in esophageal atresia. Open
Quotation I personally am not a big fan of myotomy. I think it potentially creates an even more dysfunctional segment of the esophagus than already exists. The motility is clearly abnormal in patients with oesophageal atresia, and doing a myotomy just makes that problem worse. Open
Statement For long-gap atresia when ends cannot be brought together, approach is to ligate distal end after dividing fistula, tack both ends on tension to prevertebral fascia, close, allow patient to grow 2-3 weeks, then return for anastomosis. Open
Statement Traction or pressure is a strong stimulus to growth throughout the body including cardiovascular system and lungs. Open
Statement For thoracoscopic Foker technique, traction sutures are placed in proximal and distal ends, brought out through trocar sites above and below (crossed), tensioned, with return to OR within a week for anastomosis. Open
Statement Internal Foker (tacking ends to prevertebral fascia) is preferred over classic Foker with prolonged paralysis and sequential tensioning. Open
Statement For thoracoscopic TEF repair, patient is rotated past 90 degrees to allow gravity to move lungs away, with standard port placement: camera center, posterior port inferiorly, anterior port superiorly. Open
Statement 3mm instruments are used for thoracoscopic TEF repair. Open
Statement Thoracoscopic visualization is very helpful for mobilizing the proximal pouch. Open
Quotation I'm less worried about them falling off the esophagus than I am having them fall off a blood vessel. Open
Statement The anastomosis is the most difficult part of thoracoscopic TEF repair and requires significant minimally invasive experience. Open
Quotation I think that's one of the challenges with the thoracoscopic repair of a TEF is to actually sew the anastomosis. Open
Statement Simulation courses for thoracoscopic TEF repair will be a great advantage for trainees given the infrequency of these cases. Open
Quotation To me, the important part, and I beat on our residents about this when they're doing this anastomosis, is you have to be able to see. You want to make sure that you incorporate mucosa on every stitch. Open
Statement Every stitch must incorporate mucosa, which requires good visualization; the hanging stitch helps visualize lumens of both proximal and distal ends. Open
Statement Ventilator management during thoracoscopy depends more on anesthesiologist comfort and attention than ventilator type (oscillator vs. conventional). Open
Statement Endotracheal tube can be positioned to selectively ventilate left lung during thoracoscopy, aided by preoperative bronchoscopy. Open
Statement A little CO2 insufflation with time will collapse the lung as long as anesthesiologist is not fighting with positive pressure; anesthesiologists can get scared by initial shunting but things settle down. Open
Statement Early extubation is preferred when patient had good lung function preoperatively and operation went smoothly, even potentially in the operating room, to avoid positive pressure on tracheal repair and instead have negative intratracheal pressure with spontaneous breathing. Open
Quotation I'm equally concerned that positive pressure is putting pressure on my tracheal repair, and I would much rather have the patient be breathing spontaneously to have, in fact, the opposite effect of a negative pressure in the trachea. Open
Statement Contrast esophagram is obtained at 5-7 days postoperatively before pulling chest drain. Open
Quotation I do honestly, I'm a little bit old school in that. I still get an esophagram. Open
Statement Small TLS drains are used rather than formal chest tubes, especially for open extrapleural approach. Open
Quotation I do not actually. I again, a practice of mine that has changed based on our results from the Midwest Pediatric Surgical Consortium study in which the patients who Had a trans anastomotic tube had a much higher stricture and leak rate than patients that did not have that. Open
Quotation Unless it's an incredibly tight stricture, I would wait several weeks before doing a dilation unless I was worried there was going to be a complete obstruction. Open
Statement For stricture on esophagram, dilation is delayed several weeks unless stricture is critically tight, because proximal pouch is always dilated making anastomosis always appear narrowed. Open
Statement If contrast flows freely down distal esophagus, dilation can be held off several weeks. Open
Statement Dilations are performed aggressively, typically waiting one week between dilation and restudy, then potentially dilating again. Open
Statement Balloon (radial) dilation is less traumatic to tissue than bougie dilators and is preferred. Open
Statement If case goes well and postoperative study looks great, routine follow-up esophagrams are not obtained; patients are studied only if they present with symptoms suggesting stricture. Open
Quotation I would absolutely wait and do nothing. I would leave the drain in and usually that presents with some saliva in the drain, and I would just wait, and the vast majority of those close. Open
Statement For small anastomotic leak in stable child, conservative management with drain in place is appropriate; vast majority close spontaneously. Open
Statement Leak increases risk of postoperative stricture. Open
Statement Indications for operative intervention for leak are worsening clinical status, wide open leak, or large uncontrolled pneumothorax. Open
Statement Repeat esophagram is typically performed one week after initial leak identification; if small outpouching persists but nothing comes out of tube and contrast doesn't extend further, leak is probably healed and tube can be removed. Open
Statement Pediatric surgeons do not do a fantastic job of long-term follow-up with esophageal atresia patients. Open
Quotation I think that first of all I would make the comment that that's a great point and it's interesting to participate in the International oesophageal atresia meeting that occurs every 2 years and to go and see the patients because that meeting is multidisciplinary, including even patients, and you see these patients who come back who are in their 20s or 30s or 40s and they talk about the long term issues that they have and we tend to follow patients until they're, you know, until they're eating well and then they're gone. Open
Statement At International Esophageal Atresia meetings, patients in their 20s-40s describe long-term issues that surgeons are unaware of because follow-up typically ends at age 18 or when eating well. Open
Statement Patients with recurrent strictures presenting with food bolus obstruction are seen and dilated on an ongoing basis. Open
Statement Greater concern is patients with ongoing reflux; long-term impact on Barrett's esophagus and malignant change risk is unknown. Open
Quotation The patients that worry me more honestly are the patients who have ongoing reflux, and we don't know the long term impact of that on things like Barrett's esophagus and Potential long term risk for malignant change and those sorts of things. Open
Statement If stricture has been dilated 2-3 times, next move is to address reflux. Open
Statement Most patients are discharged on anti-reflux medications. Open
Quotation I would argue that virtually all patients have some degree of gastroesophageal reflux. Open
Statement Virtually all esophageal atresia patients have some degree of gastroesophageal reflux. Open
Statement For stricture not responsive to dilations (dilates easily then restrictures), aggressive approach is fundoplication. Open
Statement Despite poor esophageal motility, Nissen fundoplication is still performed but made loose and short, using 2 or at most 3 stitches for the wrap. Open
Statement For pure esophageal atresia, approach is G-tube placement with calibration of gap length using distal catheter pushed up distal esophagus and NG tube in proximal esophagus, wait 4-6 weeks, and attempt primary repair once ends are within 2 vertebral bodies. Open

Tracheoesophageal Fistula with Dr. Daniel von Allmen

Quotation It certainly is an interest that we have here with a whole group here in Cincinnati, but it can be an incredibly difficult, challenging problem. It's one of the great cases in pediatric surgery and one of the most difficult cases, so it really runs the whole spectrum for sure. Open
Statement Cincinnati Children's has a multidisciplinary aerodigestive center including ENT airway surgeons, GI, pulmonary, general surgeons, nurse practitioners, geneticists, and dietitians Open
Statement The aerodigestive center typically receives more complicated patients referred from outside through either esophageal or airway arms Open
Statement Physical examination should assess for scaphoid versus full or distended abdomen and respiratory distress Open
Quotation I personally like to try to pass the NG tube myself, having been burned with NG tubes that wouldn't go down, and the patient was started on treatment as an oesophageal atresia and subsequently found that the NG tube passes just fine. Open
Statement The surgeon should personally attempt to pass the NG tube rather than relying on nursing reports, as tubes reported as not passing sometimes pass easily Open
Statement Injecting air into the NG tube and obtaining a plain film helps visualize proximal pouch distention and assess distal GI gas pattern Open
Statement Lack of visible distended proximal pouch raises concern for either incorrect diagnosis or presence of proximal fistula allowing pouch decompression Open
Quotation If I can't see a distended proximal pouch, then I worry. I worry that either one of two things either that the diagnosis is incorrect and in fact it is not an esophageal atresia, or that there could be a proximal fistula which is allowing that proximal pouch to decompress. Open
Statement Contrast studies can be performed if there is high suspicion of unusual anatomy, as long as the radiologist knows the esophagus may be obstructed Open
Quotation The most important one prior to taking the patient to the operating room is to get an echocardiogram, and the utility of that is, is obviously to assess the cardiac anatomy, but more as importantly, I would say is to assess to be sure that the patient does not have a right-sided aortic arch. Open
Statement Echocardiogram is the most important preoperative study to assess cardiac anatomy and rule out right-sided aortic arch Open
Statement Renal ultrasound and spine evaluation for tethered cord are needed but are elective and not necessary before addressing esophageal atresia Open
Statement Conventional wisdom is to perform left thoracotomy for right-sided aortic arch Open
Statement Left thoracotomy for right-sided arch is somewhat more difficult with the heart more in the way and harder proximal pouch mobilization Open
Statement It is possible to complete the repair from the right side if right-sided arch is discovered intraoperatively, though some reports suggest higher incidence of swallowing problems Open
Statement In a stable larger baby not intubated, it is reasonable to wait until all resources are available in the middle of the day, even waiting 1-2 days Open
Statement Should not wait a long time before repair due to risk of colonizing GI tract and soiling lungs Open
Quotation I've not found personally that an oscillator makes a whole lot of difference. I think if the child has a, has a large fistula, and typically it's actually, I believe this tends to be a bigger problem in younger, that is more premature infants who have more significant lung disease, and their compliance of their lungs is really what drives the The air into the GI tract as opposed to into the lungs. Open
Statement Large fistula with distention tends to be a bigger problem in more premature infants with significant lung disease, where poor lung compliance drives air into GI tract Open
Statement Oscillator ventilation does not make a significant difference in managing large fistulas with abdominal distention Open
Statement Treating with surfactant and improving lung compliance helps as much as changing ventilator type Open
Quotation I think this is a difficult clinical scenario, and, and I think one of the risks is to wait too long to make a decision, and I've made that mistake myself, and then it becomes an emergency if you can't ventilate the child. Open
Statement Risk of waiting too long to make a decision in worsening distention can lead to emergency situation where child cannot be ventilated Open
Quotation Decompressing the stomach may just allow that to become, in effect, the even lower resistance and allow more air to go there, making it even more difficult to ventilate. You relieve the abdominal distention, but you don't fix the flow of gas from the Trachea to the GI tract as opposed to from the trachea to the lungs. Open
Statement For reasonably stable but worsening distention, would take child urgently to OR for right thoracotomy and fistula ligation Open
Statement Bronchoscopic Fogarty balloon placement sounds good but is difficult unless expertise and equipment are immediately available Open
Statement G-tube decompression may paradoxically worsen ventilation by making stomach lower resistance, allowing more air to go there instead of lungs Open
Statement Laparotomy with vessel loop around esophagogastric junction at hiatus with gentle traction (Rommel tourniquet technique) can temporize severe cases, leaving in place for several days before definitive repair Open
Statement For associated duodenal atresia in stable child, would potentially fix duodenal atresia first to avoid fixing esophagus upstream from obstruction Open
Statement Both duodenal and esophageal atresia could potentially be fixed at same time if child is old enough and stable enough Open
Quotation That is one of the things that I have changed in my management after coming here and having exposure to the patient population that we see, the complex patients that we see with the esophageal center and the neurodigestive center, and The airway surgeons here have convinced me that it is really important to do a bronchoscopy Open
Quotation We have seen that many times in patients who are referred who've had multiple thoracotomies. Nobody ever did a bronchoscopy and the child actually has a laryngeal cleft Open
Statement Von Allmen changed practice to always perform intraoperative bronchoscopy after exposure to Cincinnati's complex patient population Open
Statement Bronchoscopy documents fistula location, assesses for proximal fistula, and most importantly rules out laryngeal cleft which is easily missed Open
Statement Many referred patients with multiple thoracotomies never had bronchoscopy and actually have laryngeal cleft Open
Statement Second fistula occurs in approximately 1% of cases and can be very difficult to diagnose Open
Statement High fistulas can range from trifurcation of carina (suggesting difficulty getting ends together) to very high fistulas potentially approachable through neck Open
Statement Bronchoscopy allows guidance of ET tube placement by anesthesiologist based on fistula location Open
Statement For fistula at carina (trifurcation), ET tube should be placed higher as it cannot pass the fistula; for high fistula, tube should be placed distal to fistula but not into the fistula itself Open
Quotation I do an extra plural approach and that's the way I was trained. I think it offers the advantage of potentially limiting any soilage of the plural space if there is a leak postoperatively. Open
Statement Extrapleural approach offers advantage of potentially limiting soilage of pleural space if there is postoperative leak Open
Quotation I think there's no morbidity associated with that, so dividing the azygus gives you better access. In fact, frequently will guide you to the fistula. Open
Statement Dividing azygos has no morbidity, gives better access, and frequently guides to the fistula Open
Statement Open azygos division is done by ligation and division; thoracoscopically can use energy devices or clips Open
Quotation It is amazing what you can divide with the electric cautery, with the hook electric cautery if you just get used to it, and I learned that doing actually robotic Nissans that you can divide the short gastrics in a teenager with the with the hook electric cautery. Open
Statement Hook cautery can safely divide vessels if done slowly going up and down to ensure coagulation, learned from robotic Nissen experience dividing short gastrics Open
Statement 3mm surgical sealer is ideal device for this size patient and vessel Open
Statement Spreading heel of right angle on ribs nicely shows extrapleural plane when going through intercostal muscles Open
Statement Important to mobilize pleura up around apex of lung to have access for proximal pouch mobilization Open
Statement After azygos division, identify distal esophagus and control with vessel loop, then dissect proximally to identify fistula site Open
Statement Muscle-sparing thoracotomy can make exposure more difficult; has not seen significant morbidity from standard posterolateral thoracotomy Open
Statement Standard teaching has been not to mobilize distal esophagus, but can actually mobilize it significantly especially laterally all the way to diaphragm Open
Statement Extensive proximal pouch mobilization gives the most length to get ends together Open
Quotation I think we have learned that, you know, the standard teaching has been that you shouldn't mobilize the distal esophagus at all, and I think we've learned that in fact you can mobilize that segment a fair amount, especially laterally. Open
Statement Must be careful with medial mobilization of distal esophagus due to blood supply concerns Open
Statement Thoracoscopic approach advantage is clearer visualization for proximal pouch mobilization Open
Quotation I completely agree, and I think that you have to be very careful. I personally like to just use a blade, electric cautery and with a little buzz and then mostly blunt dissection with the flat end of the blade, and I think that. That can help you and staying right on the esophagus, which is usually very thickened because it's an obstructed piece of bowel. I would rather be in the esophagus than be in the trachea up high in the chest. Open
Statement Better to be in esophagus than trachea during high chest dissection; esophagus is thickened from obstruction Open
Statement Risk of entering trachea during proximal dissection; must be very careful using blade cautery with light buzz and mostly blunt dissection with flat end of blade Open
Statement For significant tracheal defect, could perform sleeve resection as trachea is incredibly mobile, then place pericardium or autologous tissue between trachea and esophageal repair Open
Statement Personal preference is 5-0 PDS - absorbable monofilament suture; not a fan of silk Open
Quotation I personally am not a big fan of myotomy. I think it. Potentially creates an even more dysfunctional segment of the esophagus than already exists. The motility is clearly abnormal in patients with oesophageal atresia, and doing a myotomy just makes that problem worse. Open
Statement Not a fan of myotomies as they potentially create even more dysfunctional esophageal segment; motility is already clearly abnormal in esophageal atresia Open
Statement For cases too tight for primary repair, would ligate ends, tack both on tension to prevertebral fascia, close, let patient grow for couple weeks, then return to put together Open
Statement Traction or pressure is very strong stimulus to growth throughout cardiovascular system and lungs Open
Quotation I believe very firmly in the physiology of traction or pressure. Actually it's sort of the reverse, is a very strong stimulus to growth all over the place in the cardiovascular system in the lungs Open
Statement Would not do classic Foker technique with prolonged paralysis and sequential suture tensioning Open
Statement For thoracoscopic approach, can place traction sutures in proximal and distal ends brought out through crossed trocar sites with tension, allowing return within a week for anastomosis Open
Statement Foker technique requires patients intubated and paralyzed spending weeks in ICU, which is probably worth it if you get good result Open
Statement Rusty Jennings and Foker published series in JPS showing 98% success getting ends together in primary atresia versus only 67% in secondary approaches after previous surgeries Open
Quotation They published a series a year ago or so in JPS looking at their results, and, and it was a very carefully well documented report of their results, and the data are striking if you Have a patient who has a primary atresia that you address, then in 98% of patients they're able to get the two ends of the esophagus together. In contrast, if it's a patient who's had previous surgeries and it's a secondary approach, then only 2/3 of those patients ever get the esophagus together. Open
Statement For thoracoscopic procedure, rotate patient past 90 degrees to allow gravity to move lungs out of way Open
Statement Standard port placement: camera in center, posterior port inferiorly, anterior port superiorly Open
Statement Uses 3mm instruments for thoracoscopic TEF repair Open
Quotation I'm less worried about them falling off the esophagus than I am having them fall off a blood vessel. Open
Statement Thoracoscopic visualization is very helpful for mobilizing proximal pouch Open
Statement Simulation courses for TEF repair will be great advantage for trainees as these cases are not done often enough to get practice Open
Statement Important to do same quality operation thoracoscopically as would do open Open
Statement Sewing the anastomosis is one of the challenges with thoracoscopic TEF repair and requires most experience with minimally invasive techniques Open
Statement Would use clips for dividing fistula thoracoscopically, less worried about them falling off esophagus than blood vessel Open
Quotation I think as with most minimally invasive operations, I think it's important to do the same operation or at least the same quality operation that you would do open and Frankly, I think that's one of the challenges with the thoracoscopic repair of a TEF is to actually sew the anastomosis. Open
Quotation I think it's more dependent on the anesthesiologist than it is on the type of ventilator, and you have to have an anesthesiologist who's comfortable with doing this and understands what you're trying to accomplish and is paying attention while you're actually doing the operation because they can either help you a lot or hurt you a lot with how they ventilate the child. Open
Statement Thoracoscopic ventilation management depends more on anesthesiologist than ventilator type; need anesthesiologist comfortable with procedure who pays attention during operation Open
Statement Anesthesiologists can get scared seeing initial shunting but things settle down if they wait Open
Statement Little CO2 insufflation with some time will collapse lung as long as anesthesiologist isn't fighting with positive pressure Open
Statement Prefer spontaneous breathing with negative pressure in trachea rather than positive pressure Open
Quotation I agree it is a concern that the patient may require reintubation. However, I'm equally concerned that positive pressure is putting pressure on my tracheal repair, and I would much rather have the patient be breathing spontaneously to have, in fact, the opposite effect of a negative pressure in the trachea. Open
Statement If patient had good lung function preoperatively and operation went smoothly, advocate extubating as soon as possible, even conceivably in operating room Open
Statement Concern about reintubation exists, but equally concerned that positive pressure puts pressure on tracheal repair Open
Statement Uses small TLS drain rather than formal chest tube, especially for open extrapleural approach Open
Quotation I do honestly, I'm a little bit old school in that. I still get an esophagram. I usually wait for about 5 days. Open
Statement Gets contrast study at 5-7 days before pulling chest drain, timing depends on avoiding weekends Open
Statement Does not use transanastomotic tube based on Midwest Pediatric Surgical Consortium study showing much higher stricture and leak rate with transanastomotic tubes Open
Quotation I do not actually. I again, a practice of mine that has changed based on our results from the Midwest Pediatric Surgical Consortium study in which the patients who Had a trans anastomotic tube had a much higher stricture and leak rate than patients that did not have that. Open
Statement Midwest consortium study was presented at APSA and should be published in JPS soon Open
Quotation Unless it's an incredibly tight stricture, I would wait several weeks before doing a dilation unless I was worried there was going to be a complete obstruction. Open
Quotation There's always reported a narrowing because the proximal pouch is always dilated, so it always looks like there's a narrowing at the anastomosis. As long as it's patent and contrast flows freely down the distal esophagus, I would hold off on. Uh, dialing it, dilating it probably several weeks if possible. Open
Statement Always appears to be narrowing at anastomosis because proximal pouch is dilated; as long as patent with free contrast flow distally, would hold off on dilation Open
Statement Unless incredibly tight stricture risking complete obstruction, would wait several weeks before first dilation Open
Statement Fairly aggressive with dilations: dilate, wait 1-2 weeks, restudy, potentially dilate again Open
Statement If case goes well and post-op study looks great, does not get routine follow-up esophagrams; studies based on clinical symptoms suggesting stricture Open
Statement Huge spectrum from very tight strictures requiring many dilations to kids fine after one dilation Open
Quotation I think that again, having trained using bougie dilators, a balloon or radial dilation is, is less traumatic for the tissue Open
Statement Dilations done with GI colleagues in aerodigestive center for bigger kids or with interventional radiologists Open
Statement Balloon or radial dilation is less traumatic for tissue than bougie dilators Open
Statement For small leak with child not sick, would absolutely wait and do nothing, leaving drain in; vast majority close Open
Quotation I would absolutely wait and do nothing. I would leave the drain in and usually that presents with some saliva in the drain, and I would just wait, and the vast majority of those close. Open
Statement Even reasonably significant leaks will heal, then can deal with stricture postoperatively Open
Statement Would only go to OR for leak if child getting sicker, wide open leak, or large uncontrollable pneumothorax Open
Statement Usually waits one week between esophagrams for leak; if child continues to do well, restudies Open
Statement Sometimes little outpouching where leak happened makes it unclear if still leaking; if nothing from tube and nothing goes further, leak probably healed and would remove tube Open
Quotation I think that first of all I would make the comment that that's a great point and it's interesting to participate in the International oesophageal atresia. A meeting that occurs every 2 years and to go and see the patients because that meeting is multidisciplinary, including even patients, and you see these patients who come back who are in their 20s or 30s or 40s and they talk about the long term issues that they have and we tend to follow patients until they're, you know, until they're eating well and then they're gone Open
Statement International esophageal atresia meeting occurs every 2 years and is multidisciplinary including patients Open
Statement Patients in their 20s-40s at international meeting discuss long-term issues; surgeons tend to follow until eating well or age 18 then never see them again Open
Statement Have a lot to learn about long-term complications; personally follows patients for at least couple years which is probably not long enough Open
Quotation The patients that worry me more honestly are the patients who have ongoing reflux, and we don't know the long term impact of that on things like Barrett's esophagus and Potential long term risk for malignant change and those sorts of things. Open
Statement More concerning are patients with ongoing reflux due to unknown long-term impact on Barrett's esophagus and potential malignant change Open
Statement Biggest challenges in long-term follow-up are reflux and recurrent strictures Open
Statement If patient has stricture dilated 2-3 times, next move would be to address reflux Open
Statement Virtually all patients have some degree of gastroesophageal reflux Open
Statement Most patients left on anti-reflux medications when discharged from hospital Open
Statement Very aggressive about fundoplication for strictures not responsive to dilations (dilates easily then restrictures) Open
Quotation If I have a patient who has a stricture that's been dilated 2 or 3 times, my next move would be to address reflux. Most of the patients we leave on anti-reflux medications when they are discharged from the hospital. I would argue that virtually all patients have some degree of gastroesophageal reflux. If they have a stricture that is not responsive to dilations, it dilates easily and then restrictures. I personally am very aggressive about doing a fundoplication on those patients Open
Statement Would wait 4-6 weeks to see how close ends come together, then attempt primary repair once within 2 vertebral bodies Open
Statement For pure esophageal atresia, approach is G-tube placement with calibration of gap length using distal catheter pushed up and NG tube in proximal pouch Open
Quotation I do, but as with most Nissans, I make them loose and I make them short, so I usually use two or at most 3 stitches for the wrap and make sure that it is loose around the distal esophagus. Open
Statement Does Nissen fundoplication in patients with poor esophageal motility but makes them loose and short, using 2 or at most 3 stitches Open

Treatment for Long Gap Esophageal Atresia: Esophageal Elongation and Replacement

Statement At Cincinnati Children's, if a child has no gas in the abdomen, they place a G-tube and may put something up the distal esophagus for a fluoro shot, then wait a couple of weeks for a protocol gap measurement in interventional radiology. Open
Quotation Our method here is that if we have a child with no gas in the abdomen and we take them to the OR and put a G-tube in, many times we'll do, we'll put something up the distal esophagus at that point and just get a fluoro shot. But then we'd wait a couple of weeks and we have our patients go down to interventional radiology where we have a protocol for measuring the gap. Open
Quotation But the philosophy here is that with traction, you can get the two ends of the esophagus to grow. And if you can get them to grow far enough, you can put them together. Open
Quotation The physiology, which I believe in intensely, is that stretch is a very strong promoter of growth. And then if you put things on tension, they will actually grow over time. That's how the cardiovascular system develops in utero. Open
Statement The philosophy of traction-based elongation is that with traction, you can get the two ends of the esophagus to grow, and if you can get them to grow far enough, you can put them together. Open
Statement Stretch is a very strong promoter of growth, and if you put things on tension they will actually grow over time, which is how the cardiovascular system develops in utero. Open
Quotation You can actually use the colon as an interposition as well. I personally was trained to do colon interpositions. Open
Statement The colon can be used as an interposition for esophageal replacement. Open
Quotation Common problems with this are that they dilate and they can become tortuous. It's not uncommon to get kind of a sigmoid sink drain deformity just above the diaphragm. Open
Statement Common problems with colonic interposition are that the colon can dilate and become tortuous, and it's not uncommon to get a sigmoid sink drain deformity just above the diaphragm. Open
Statement Von Allman was initially taught that sigmoid redundancy in colonic interposition can't be fixed and that it's too dangerous because it will risk the blood supply, but he found that this is not really true. Open
Quotation I was taught initially that you can't fix that and that it's too dangerous and that you'll risk the blood supply to the colon interposition. But I found that actually that's not really true. Open
Quotation So I wouldn't pretend to say that these don't need to be revised sometimes, but it is not impossible to revise them and the kids tend to do pretty well. Open
Quotation I pass the interposition posterior to the stomach, which leaves the vascular pedicle along the spine. And you can mobilize the colon. You can divide the gastric dual aporotomy, divide the colon attachment to the stomach, and then mobilize that sigmoid redundancy transhiatally, and then reinestimose the colon to the stomach. Open
Statement Von Allman passes the colonic interposition posterior to the stomach, which leaves the vascular pedicle along the spine, allowing mobilization of the colon by dividing the gastric duodenotomy and colon attachment to the stomach, then mobilizing the sigmoid redundancy transhiatally and reanastomosing the colon to the stomach. Open
Statement Colonic interpositions sometimes need to be revised, but it is not impossible to revise them and the kids tend to do pretty well. Open
Statement You can get extraordinary length with colonic interposition, allowing treatment of cases with caustic injuries extending to the pharynx by sewing the colon to the pharynx and down to the stomach, which is tough to do with a gastric pull-up. Open
Quotation You can get extraordinary length on this. And Mike and I have done a few cases for kids who had disasters, multiple operations elsewhere, who had caustic injuries that were involved all the way up to the pharynx, where we had to do a lot of work just on the pharynx to get that open, and then literally sew the colon interposition to the pharynx and then down to the stomach, which is obviously tough to do with a gastric pull-up. Open
Quotation I don't think we know we should do the studies to understand that. But I would say that this gets back to David van der Zee's comment about, go have a cup of coffee and come back and it'll be fine. Clearly, that's not growth, that's stretch. Open
Statement Von Allman states we don't know whether esophageal elongation is growth or stretch and should do studies to understand that, but notes that tension is a very good physiologic growth promoter in other organs, making this an area ripe for more basic science. Open
Quotation And yet there's very good physiologic data that tension is a growth promoter, not necessarily in the esophagus, but in other organs. So I think that it's an area ripe for a little more basic science. Open

Esophageal Replacement with Dr. Dan von Allmen

Quotation We have a tendency to say TEF when we mean esophageal atresia, so just be very careful when you're talking about these things that you're clear about what you're talking about, because when you say, oh, the patient had a TEF when really. They had is a pure esophageal atresia. That's just not correct. Open
Statement Esophageal atresia incidence is 1 in 4500 in the United States with slight male predominance Open
Statement Recurrence risk of esophageal atresia among parents who have one affected child is 0.5 to 2% Open
Statement Prenatal ultrasound detects only about half of esophageal atresia cases, primarily detecting pure atresia due to significant proximal pouch dilation Open
Statement The most common type of esophageal atresia is proximal atresia with distal fistula Open
Statement Esophageal atresia associated with significant cardiac anomalies and low birth weight has only 27 to 30% survival rate Open
Statement VACTERL is an association, not a syndrome, requiring 3 of the defects for diagnosis Open
Statement In type C fistula with respiratory distress, air continuously goes into the intestine via the low resistance pathway instead of into the lungs, causing increased stomach size and affecting respiratory compliance Open
Statement G-tube decompression or needle decompression helps temporarily but air still follows the low resistance pathway out of the G-tube instead of going into the lungs Open
Statement Putting vessel loop around distal esophagus is easier than dividing the fistula when in trouble during surgery Open
Quotation We believe quite strongly that every child with a tracheoesophageal fistula or esophageal atresia needs a bronchoscopy. Open
Statement Every child with tracheoesophageal fistula or esophageal atresia needs bronchoscopy due to 10-15% association of airway issues and to help plan surgery Open
Statement Virtually all kids with esophageal atresia have some element of tracheomalacia, though clinical significance varies Open
Quotation I personally believe that there is some Growth in the esophagus and typically our approach is to wait several weeks. I think once you get past probably 4 to 6 weeks, there's not going to be any more growth. Open
Statement There is growth in the esophagus over time, but once past 4 to 6 weeks there is not going to be any more growth Open
Statement For significant gap, approach is to support child with enteral bolus feedings to put stretch on the distal esophagus and get sequential gap studies, then take to OR at 6 weeks Open
Statement For thoracoscopy, patient positioning should be almost 45 degrees from prone, pushed to one side of bed, with camera port looking straight down at esophagus providing better visualization than open surgery Open
Statement The azygos vein guides to the level of the fistula because the fistula typically occurs right about the level of the azygos vein Open
Statement When placing clip on fistula, it must be flush with the trachea to avoid leaving a long pouch that can pool secretions and cause problems Open
Statement Anesthesiologist needs to push down repeatedly to help find the proximal pouch during surgery Open
Statement It is safer to put a hole in the esophageal pouch than in the trachea during dissection Open
Statement If CO2 skyrockets on ET tube during thoracoscopy, this indicates a hole in the trachea as CO2 from thoracoscopy goes up the trachea and out the endotracheal tube Open
Quotation You would much rather put a hole in the esophageal pouch than in the trachea. Open
Statement Foker procedure uses traction sutures brought out through chest wall with sequential tightening, as traction is a stimulus for esophageal growth Open
Statement Primary Foker cases spent more than 1 month in ICU on average and were paralyzed for more than 2 weeks Open
Statement In primary Foker cases, esophagus was successfully brought together 96% of the time, versus only 2/3 of the time in secondary cases after previous operation Open
Statement Secondary Foker cases spent mean of 110 days in ICU and were paralyzed for more than 1 month Open
Statement Most common esophageal replacement is gastric pull-up, but other options include reverse gastric tube, colon interposition, and duodenal interposition, with none being clearly better than others Open
Quotation I would say that none is any better than the other. Open
Statement Reverse gastric tube is created from greater curve of stomach and requires preserving gastroepiploic artery when placing initial G-tube, but has many problems Open
Statement Colon interposition advantage is tremendous length, allowing anastomosis to pharynx in caustic ingestion cases with no remaining esophagus Open
Statement Colon interposition conduits can be revised despite teaching that revision damages blood supply Open
Quotation We were taught initially that these are hard to revise because you damage the blood supply to the colon, but that's actually not true. Open
Statement Biggest challenge with colon interposition is that conduits dilate and elongate over time, with food collecting and not emptying Open
Statement In institutional series of first 13 colon interpositions, 12 of 13 patients were NPO at presentation, with no graft loss after initial case Open
Statement Posterior tracheopexy involves putting sutures into membranous trachea to hold it back for tracheomalacia management Open
Statement When performing initial repair with field already dissected, placing 3 posterior tracheopexy stitches takes 15 minutes and avoids need to operate through previously operated field later Open
Quotation As surgeons, we're frequently trained that we need to be the captain of the ship, and it's all about us, and we need to do everything. And one of the best things that happened to me when I came here and started doing these esophageal cases was partnering with our ENT group. Open
Daniel's statements about Wilms Tumor 11 statements

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Neuroblastoma

Statement Adrenal hemorrhage is the most common cause of prenatal suprarenal mass, especially with history of fetal stress Open
Statement The GetNucturne study showed that prenatal/neonatal neuroblastomas can be safely observed with careful ultrasound surveillance, with many patients spared surgery Open
Statement Lymph node status in neuroblastoma does not change therapy, unlike Wilms tumor Open
Statement Approximately 10% of neuroblastomas are not MIBG-avid, which is when PET scan may be useful Open
Statement Tumor shrinkage in neuroblastoma plateaus after cycle 2-3 of chemotherapy per LaQuaglia and Von Allman studies Open
Statement COG high-risk study showed >90% resection improved event-free survival but not overall survival (approximately 245 patients) Open
Statement European neuroblastoma group study (approximately 1000 cases) showed >90% resection improved both event-free survival and overall survival Open
Statement Approximately 70% of high-risk neuroblastoma patients can achieve >90% resection Open
Statement Neuroblastoma theoretically does not invade vessel adventitia, though invasion has been observed Open
Statement There is zero correlation between surgeon operative note description of resection extent and post-operative imaging findings Open
Quotation There is zero correlation, zero. There is absolutely no correlation between the findings on post-op imaging and the findings that are reported in the op note. Open

Summaries Daniel gave as host · 152 summaries

Recaps of other experts' statements, not Daniel's own clinical position.

Summaries Daniel gave as host · Appendicitis 14 summaries

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Summaries Daniel gave as host · Biliary Atresia 14 summaries

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Summaries Daniel gave as host · Colorectal / ARM & Hirschsprung 7 summaries

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Summaries Daniel gave as host · Enterocolitis 1 summary

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Hirschprung's Disease - Daniel von Allmen

Host summary Daniel von Allmen summarizing a resource: the baby's first enema should be a barium enema Open
Summaries Daniel gave as host · Esophageal Atresia 6 summaries

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Tracheoesophageal Fistula with Dr. Daniel von Allmen

Host summary Daniel von Allmen summarizing a resource: I think credit to Rusty Jennings in Boston and Dr. Folker, who is the consultant there with them. They published a series a year ago or so in JPS looking at their results, and, and it was a very carefully well documented report of their results, and the data are striking if you Have a patient who has a primary atresia that you address, then in 98% of patients they're able to get the two ends of the esophagus together. In contrast, if it's a patient who's had previous surgeries and it's a secondary approach, then only 2/3 of those patients ever get the esophagus together. Open
Host summary Daniel von Allmen summarizing a resource: Rusty Jennings and Dr. Foker published data showing 98% success rate getting esophageal ends together in primary atresia, but only 67% success in secondary approaches after previous surgeries. Open
Host summary Daniel von Allmen summarizing a resource: Foker technique requires patients to be intubated and paralyzed for weeks in ICU, which is significant cost but may be worth it for good result. Open
Host summary Daniel von Allmen summarizing a resource: The Atlanta group uses a hanging stitch technique: first stitch between the two ends is brought out through the chest to hold anastomosis up in the air for subsequent stitches, similar to duodenal atresia repair. Open
Host summary Daniel von Allmen summarizing a resource: Transanastomotic tubes are not used based on Midwest Pediatric Surgical Consortium data showing much higher stricture and leak rates in patients with transanastomotic tubes. Open

Tracheoesophageal Fistula with Dr. Daniel von Allmen

Host summary Daniel von Allmen summarizing a resource: Atlanta group uses stay stitch between two ends brought out of chest to hold anastomosis up for subsequent stitches, similar to duodenal atresia technique Open
Summaries Daniel gave as host · Etiologies (Gastroschisis/NEC/Atresia/Volvulus) 7 summaries

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Summaries Daniel gave as host · Fetal Surgery 10 summaries

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Treatment for Long Gap Esophageal Atresia: Esophageal Elongation and Replacement

Host summary Daniel von Allmen summarizing a resource: There was a great article published by the Boston Group 2015. Divides the cases into two groups, a primary group and a secondary group. Secondary being patients who had had operations previously in the primary group, being cases that they saw de novo. Open
Host summary Daniel von Allmen summarizing a resource: But these procedures are not without some morbidity. And you look at the ICU stay is a median of 70 days with a couple of weeks being paralyzed for the primary group and 110 days for the secondary group with a month of being paralyzed. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, about two thirds of patients with primary repair were able to get full oral nutrition and about 10% of patients who had secondary repair achieved full oral nutrition. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, the primary group had a median ICU stay of 70 days with a couple of weeks being paralyzed, and the secondary group had a median ICU stay of 110 days with a month of being paralyzed. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, the primary group (de novo cases) achieved an intact esophagus in 96% of patients, while the secondary group (patients with previous operations) achieved this in about two thirds of patients. Open
Host summary Daniel von Allmen summarizing a resource: About two thirds of patients with the primary repair were able to get the full oral nutrition and about 10% of the patients who had the secondary repair. Open
Host summary Daniel von Allmen summarizing a resource: Obviously, you look at the result of getting the esophagus together and intact esophagus in 96% of patients in the primary group, about two thirds of patients in the secondary group. Open
Host summary Daniel von Allmen summarizing a resource: The surgical group from INOEA recommends gastric pull up as the first option for esophageal replacement, dividing the esophageal stump at the esophageal hiatus and mobilizing the fundus to pull it up in either the anterior or posterior mediastinum. Open
Host summary Daniel von Allmen summarizing a resource: You could use the stomach. That would be called a gastric transposition. The surgical group from the INOEA, their recommendation, the recommendation for the first option is a gastric pull up. Open
Host summary Daniel von Allmen summarizing a resource: You divide the esophageal stump at the esophageal hiatus and mobilize the fundus and pull the fundus up and you can pull it up either in the anterior or posterior mediastinum. Open
Summaries Daniel gave as host · Gastroesophageal Reflux 3 summaries

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Gastoesophageal Reflux: Update Course 2015

Host summary Daniel von Allmen summarizing the discussion: In adult literature, after about 15 years almost all fundoplications are undone Open
Host summary Daniel von Allmen summarizing the discussion: If you look at the adult literature for fund application, after about 15 years, almost all of them are, are, are undone. And so I don't know why we think that ours is going to be any different. We just don't follow our patients past 18. Open
Host summary Daniel von Allmen summarizing the discussion: Neurologically impaired patients do worse than neurologically normal patients after fundoplication according to historical data Open
Summaries Daniel gave as host · Gastroesophageal Reflux Disease 3 summaries

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Gastoesophageal Reflux: Update Course 2015

Host summary Daniel von Allmen summarizing the discussion: In adult literature, after about 15 years almost all fundoplications are undone Open
Host summary Daniel von Allmen summarizing the discussion: If you look at the adult literature for fund application, after about 15 years, almost all of them are, are, are undone. And so I don't know why we think that ours is going to be any different. We just don't follow our patients past 18. Open
Host summary Daniel von Allmen summarizing the discussion: Neurologically impaired patients do worse than neurologically normal patients after fundoplication according to historical data Open
Summaries Daniel gave as host · High-risk Neuroblastoma 13 summaries

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Neuroblastoma: Update Course 2014

Host summary Daniel von Allmen summarizing the discussion: Memorial Sloan Kettering data suggests biggest volume response of neuroblastoma tumor occurs with first two cycles of chemotherapy, with very little response after that Open
Host summary Daniel von Allmen summarizing the discussion: German study of 278 stage 4 high-risk neuroblastoma patients achieved complete resection in almost half and >90% resection in another quarter (75% total with >90% resection) Open
Host summary Daniel von Allmen summarizing the discussion: German study showed overall survival 45%, event-free survival 33%, and local progression-free survival 58% in stage 4 neuroblastoma Open
Host summary Daniel von Allmen summarizing the discussion: German study showed no difference in overall survival, event-free survival, or local progression-free survival based on completeness of resection in stage 4 neuroblastoma Open
Host summary Daniel von Allmen summarizing the discussion: German study concluded aggressive surgery is not justified in stage 4 neuroblastoma, that limited operations decrease complications, and there is limited impact on patient outcome Open
Host summary Daniel von Allmen summarizing the discussion: European study included 1,324 high-risk neuroblastoma patients (stages 2, 3, and 4) and achieved 76% with >95% resection Open
Host summary Daniel von Allmen summarizing the discussion: European neuroblastoma study showed 0.5% mortality and 10% morbidity (30% including lesser complications) Open
Host summary Daniel von Allmen summarizing the discussion: European study showed significant improvement in event-free survival and overall survival with aggressive resection in high-risk neuroblastoma - first study to show overall survival benefit Open
Host summary Daniel von Allmen summarizing the discussion: European study concluded >95% resection results in improvement in event-free survival in high-risk neuroblastoma Open
Host summary Daniel von Allmen summarizing the discussion: COG 3973 study of approximately 230 high-risk neuroblastoma patients showed significant improvement in local relapse-free survival and event-free survival but not overall survival Open
Host summary Daniel von Allmen summarizing the discussion: European neuroblastoma procedures are performed in more than 200 hospitals yet still demonstrated survival improvements Open
Host summary Daniel von Allmen summarizing the discussion: Older data shows worse survival in neuroblastoma when kidney is removed, likely because single kidney limits chemotherapy dosing Open
Host summary Daniel von Allmen summarizing the discussion: If you can't be informed, be opinionated Open
Summaries Daniel gave as host · Intestinal Failure 14 summaries

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Summaries Daniel gave as host · Intestinal Rehab 14 summaries

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open

Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Summaries Daniel gave as host · Intestinal Transplant 7 summaries

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Biliary Atresia, Appendicitis, Intestinal Failure, and Anesthetic...

Host summary Daniel von Allmen summarizing the discussion: The START trial randomized 140 infants with biliary atresia to high-dose steroids (IV for 2 weeks, oral for 2 weeks, taper over 9 weeks) versus placebo within 72 hours of Kasai portoenterostomy. Open
Host summary Daniel von Allmen summarizing the discussion: The primary outcome was serum total bilirubin <1.5 mg/dL with native liver at 6 months post-Kasai; the study was powered to detect a 25% absolute treatment difference. Open
Host summary Daniel von Allmen summarizing the discussion: High-dose steroid therapy after Kasai did not result in a statistically significant treatment difference in bile drainage at 6 months: 58.6% in the steroid group versus 48.6% in placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Survival without liver transplant at 24 months was not statistically different: 58.7% for steroids versus 49.4% for placebo. Open
Host summary Daniel von Allmen summarizing the discussion: Adverse events were common in both groups (near 80%), largely reflecting severe underlying liver dysfunction; steroid treatment was associated with earlier onset of serious adverse events. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the paper, and I believe them, feel that anything less than a 25% difference is probably not worth the potential risk of the early complications associated with the steroids. Open
Host summary Daniel von Allmen summarizing the discussion: The authors of the START trial believe anything less than a 25% difference in bile drainage is not worth the potential early complications of steroids. Open
Summaries Daniel gave as host · Long-gap Esophageal Atresia 10 summaries

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Treatment for Long Gap Esophageal Atresia: Esophageal Elongation and Replacement

Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, about two thirds of patients with primary repair were able to get full oral nutrition and about 10% of patients who had secondary repair achieved full oral nutrition. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, the primary group had a median ICU stay of 70 days with a couple of weeks being paralyzed, and the secondary group had a median ICU stay of 110 days with a month of being paralyzed. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, the primary group (de novo cases) achieved an intact esophagus in 96% of patients, while the secondary group (patients with previous operations) achieved this in about two thirds of patients. Open
Host summary Daniel von Allmen summarizing a resource: About two thirds of patients with the primary repair were able to get the full oral nutrition and about 10% of the patients who had the secondary repair. Open
Host summary Daniel von Allmen summarizing a resource: But these procedures are not without some morbidity. And you look at the ICU stay is a median of 70 days with a couple of weeks being paralyzed for the primary group and 110 days for the secondary group with a month of being paralyzed. Open
Host summary Daniel von Allmen summarizing a resource: Obviously, you look at the result of getting the esophagus together and intact esophagus in 96% of patients in the primary group, about two thirds of patients in the secondary group. Open
Host summary Daniel von Allmen summarizing a resource: There was a great article published by the Boston Group 2015. Divides the cases into two groups, a primary group and a secondary group. Secondary being patients who had had operations previously in the primary group, being cases that they saw de novo. Open
Host summary Daniel von Allmen summarizing a resource: You could use the stomach. That would be called a gastric transposition. The surgical group from the INOEA, their recommendation, the recommendation for the first option is a gastric pull up. Open
Host summary Daniel von Allmen summarizing a resource: You divide the esophageal stump at the esophageal hiatus and mobilize the fundus and pull the fundus up and you can pull it up either in the anterior or posterior mediastinum. Open
Host summary Daniel von Allmen summarizing a resource: The surgical group from INOEA recommends gastric pull up as the first option for esophageal replacement, dividing the esophageal stump at the esophageal hiatus and mobilizing the fundus to pull it up in either the anterior or posterior mediastinum. Open
Summaries Daniel gave as host · Neuroblastoma 13 summaries

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Neuroblastoma: Update Course 2014

Host summary Daniel von Allmen summarizing the discussion: Memorial Sloan Kettering data suggests biggest volume response of neuroblastoma tumor occurs with first two cycles of chemotherapy, with very little response after that Open
Host summary Daniel von Allmen summarizing the discussion: German study of 278 stage 4 high-risk neuroblastoma patients achieved complete resection in almost half and >90% resection in another quarter (75% total with >90% resection) Open
Host summary Daniel von Allmen summarizing the discussion: German study showed overall survival 45%, event-free survival 33%, and local progression-free survival 58% in stage 4 neuroblastoma Open
Host summary Daniel von Allmen summarizing the discussion: German study showed no difference in overall survival, event-free survival, or local progression-free survival based on completeness of resection in stage 4 neuroblastoma Open
Host summary Daniel von Allmen summarizing the discussion: German study concluded aggressive surgery is not justified in stage 4 neuroblastoma, that limited operations decrease complications, and there is limited impact on patient outcome Open
Host summary Daniel von Allmen summarizing the discussion: European study included 1,324 high-risk neuroblastoma patients (stages 2, 3, and 4) and achieved 76% with >95% resection Open
Host summary Daniel von Allmen summarizing the discussion: European neuroblastoma study showed 0.5% mortality and 10% morbidity (30% including lesser complications) Open
Host summary Daniel von Allmen summarizing the discussion: European study showed significant improvement in event-free survival and overall survival with aggressive resection in high-risk neuroblastoma - first study to show overall survival benefit Open
Host summary Daniel von Allmen summarizing the discussion: European study concluded >95% resection results in improvement in event-free survival in high-risk neuroblastoma Open
Host summary Daniel von Allmen summarizing the discussion: COG 3973 study of approximately 230 high-risk neuroblastoma patients showed significant improvement in local relapse-free survival and event-free survival but not overall survival Open
Host summary Daniel von Allmen summarizing the discussion: European neuroblastoma procedures are performed in more than 200 hospitals yet still demonstrated survival improvements Open
Host summary Daniel von Allmen summarizing the discussion: Older data shows worse survival in neuroblastoma when kidney is removed, likely because single kidney limits chemotherapy dosing Open
Host summary Daniel von Allmen summarizing the discussion: If you can't be informed, be opinionated Open
Summaries Daniel gave as host · Tracheoesophageal Fistula 16 summaries

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Tracheoesophageal Fistula with Dr. Daniel von Allmen

Host summary Daniel von Allmen summarizing a resource: I think credit to Rusty Jennings in Boston and Dr. Folker, who is the consultant there with them. They published a series a year ago or so in JPS looking at their results, and, and it was a very carefully well documented report of their results, and the data are striking if you Have a patient who has a primary atresia that you address, then in 98% of patients they're able to get the two ends of the esophagus together. In contrast, if it's a patient who's had previous surgeries and it's a secondary approach, then only 2/3 of those patients ever get the esophagus together. Open
Host summary Daniel von Allmen summarizing a resource: Rusty Jennings and Dr. Foker published data showing 98% success rate getting esophageal ends together in primary atresia, but only 67% success in secondary approaches after previous surgeries. Open
Host summary Daniel von Allmen summarizing a resource: Foker technique requires patients to be intubated and paralyzed for weeks in ICU, which is significant cost but may be worth it for good result. Open
Host summary Daniel von Allmen summarizing a resource: The Atlanta group uses a hanging stitch technique: first stitch between the two ends is brought out through the chest to hold anastomosis up in the air for subsequent stitches, similar to duodenal atresia repair. Open
Host summary Daniel von Allmen summarizing a resource: Transanastomotic tubes are not used based on Midwest Pediatric Surgical Consortium data showing much higher stricture and leak rates in patients with transanastomotic tubes. Open

Tracheoesophageal Fistula with Dr. Daniel von Allmen

Host summary Daniel von Allmen summarizing a resource: Atlanta group uses stay stitch between two ends brought out of chest to hold anastomosis up for subsequent stitches, similar to duodenal atresia technique Open

Treatment for Long Gap Esophageal Atresia: Esophageal Elongation and Replacement

Host summary Daniel von Allmen summarizing a resource: There was a great article published by the Boston Group 2015. Divides the cases into two groups, a primary group and a secondary group. Secondary being patients who had had operations previously in the primary group, being cases that they saw de novo. Open
Host summary Daniel von Allmen summarizing a resource: About two thirds of patients with the primary repair were able to get the full oral nutrition and about 10% of the patients who had the secondary repair. Open
Host summary Daniel von Allmen summarizing a resource: But these procedures are not without some morbidity. And you look at the ICU stay is a median of 70 days with a couple of weeks being paralyzed for the primary group and 110 days for the secondary group with a month of being paralyzed. Open
Host summary Daniel von Allmen summarizing a resource: Obviously, you look at the result of getting the esophagus together and intact esophagus in 96% of patients in the primary group, about two thirds of patients in the secondary group. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, the primary group (de novo cases) achieved an intact esophagus in 96% of patients, while the secondary group (patients with previous operations) achieved this in about two thirds of patients. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, the primary group had a median ICU stay of 70 days with a couple of weeks being paralyzed, and the secondary group had a median ICU stay of 110 days with a month of being paralyzed. Open
Host summary Daniel von Allmen summarizing a resource: In the Boston Group 2015 study, about two thirds of patients with primary repair were able to get full oral nutrition and about 10% of patients who had secondary repair achieved full oral nutrition. Open
Host summary Daniel von Allmen summarizing a resource: The surgical group from INOEA recommends gastric pull up as the first option for esophageal replacement, dividing the esophageal stump at the esophageal hiatus and mobilizing the fundus to pull it up in either the anterior or posterior mediastinum. Open
Host summary Daniel von Allmen summarizing a resource: You could use the stomach. That would be called a gastric transposition. The surgical group from the INOEA, their recommendation, the recommendation for the first option is a gastric pull up. Open
Host summary Daniel von Allmen summarizing a resource: You divide the esophageal stump at the esophageal hiatus and mobilize the fundus and pull the fundus up and you can pull it up either in the anterior or posterior mediastinum. Open