Marc Levitt

1438 statements · 5 topics · summaries given as host listed separately

Colorectal / ARM & Hirschsprung · guest expert Etiologies (Gastroschisis/NEC/Atresia/Volvulus) · guest expert Intestinal Rehab · guest expert Motility / Pseudo-obstruction · guest expert Pediatric Robotic Surgery · episode host Single Ventricle / HLHS · guest expert

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I think the key to deciding whether to dive into a perineon posterior sagittally is where is the rectum? You want to know what structure you will find if you make a posterior sagittal or even a mini posterior sagittal incision. And there is no question in my mind that if I open posterior sagittally here, the first structure I would find would be rectum. Because the danger is that you go in posterior sagittally, you don't know where the rectum is, and you find something midline and white, like the urethra or the bladder neck or the bladder itself. And that's why we do colostomies. And that's why we do distal colostograms. So we know exactly where the rectum is, and we know whether we should approach it perineally or whether we should do it laparoscopically.

quote · Anorectal Malformation

It's fascinating actually that, you know, of those four procedures, the only one that actually leaves behind virtually no Hirschprungs is the Swenson. The suave leaves behind the outer rectal wall. The Duhamel leaves behind the original rectum, and the ravine leaves behind the original rectum as well. Amazingly, many, many of those patients did perfectly well, and I think that has to do with the fact that the ganglionic bowel, if it's good, can overcome a lot.

quote · Appendicitis

By definition, you're leaving behind 1 centimeter of columnar epithelium, that of course is Hirschberg's disease, but the ganglionic bowel is able to overcome that. And of course you're leaving behind the smooth muscle, the internal sphincter, which has a problem by definition with relaxation. However, that can be overcome by good ganglionated bowel pushing through and eventually the baby recognizing how to relax that sphincter over time.

quote · Appendicitis

I get asked all the time about the stimulator. It's the same electrical stimulator that anesthesia uses for their train of four. And then there's a connection that you can make that has little pins. Really, really inexpensive. And you just have to tell your anesthesiologist not to give skeletal muscle relaxant because it's a little bit weaker than the traditional stimulator, which was super expensive.

quote · Anorectal Malformation

If you go for a TUM, And it doesn't reach, what do you do? Well, then you need to go into the abdomen and continue your dissection of the urogenital immobilization. Now, on occasion, that's enough, and it does reach, but if it doesn't reach at that point, now you are in some serious trouble, because now you have to do the separation. And guess what? You've already dissected the anterior urethra.

quote · Cloaca

Sena on the other hand pushes out the stool and to compensate for that push which would normally make the stool very liquid. We give as was mentioned water soluble fiber to provide a little bit of bulk and therefore the stool that gets pushed out by the sena is a single well formed section of stool and the the patient can feel that much more.

quote · Anorectal Malformations & Cloacal Reconstruction

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Marc's statements about Anorectal Malformation 870 statements

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How I Do It Levitt PSARP

Statement A no-fistula anorectal malformation defect is managed very similarly to a bulbar urethral fistula. Open

Statement The key to starting any anorectal malformation repair is a good imaging study. Open

Quotation Obviously the key to starting any such case is a good imaging study. Open

Statement Fistula levels can be classified anatomically: bladder neck fistula is at the deltoid level (C), rectoprostatic fistula is at the triceps level (B), and rectobulbar fistula is at the elbow of the urethral curve or distal (A). Open

Statement It is important to mark the sphincter location before making the posterior sagittal incision because once the incision is made, it is hard to know exactly where the sphincter center is. Open

Statement The sphincters must be cut perfectly in the midline so that they can be easily reconstructed. Open

Statement Without a good distal colostogram, the midline whitish structure at the center of the dissection could be the urinary tract rather than the rectum. Open

Quotation If you don't have a good distal colostogram, this midline whitish structure could very easily be the urinary tract. Open

Quotation You don't want to hurt the urethra. Open

Statement Lateral dissection should be performed before turning attention anteriorly during rectal mobilization. Open

Quotation That is the critical moment. You want to separate the rectum from the urinary tract below without injuring the urinary tract. Open

Statement The initial anterior dissection to separate rectum from urinary tract is a submucosal dissection for the first few millimeters. Open

Quotation Imagine you are literally dropping down the urinary tract as the rectum is lifted up. Open

Quotation If you're not sure, go lateral. Open

Quotation When you're lateral, any fat you see means you can get closer to the rectum. Open

Statement During lateral dissection, any fat seen means you can get closer to the rectum safely. Open

Statement The lower the rectum is positioned, the longer is the common wall between rectum and urinary tract. Open

Statement A lower rectum is easier to repair in one sense but harder because there is a longer dissection adjacent to the urethra. Open

Statement A rectum at the bulbar level is too low to approach laparoscopically and is much safer to approach posterior sagittally. Open

Quotation That is way too low in my opinion to approach laparoscopically, much safer to approach such an operation posterior sagittally. Open

Statement Approaching a low rectum laparoscopically risks leaving behind distal rectum, a remnant of the urethral fistula, or a roof. Open

Quotation If you see fat, you can get closer. Open

Statement The rectum must be in the correct dissection plane or it will not mobilize properly. Open

Quotation You must be in the correct plane, otherwise the rectum does not mobilize. Open

Statement When closing the muscle complex, taking a bite of the rectum helps to avoid prolapse. Open

Statement The rectum should lie adjacent to, not constricted by, the muscle complex. Open

Statement As much rectum as possible should be preserved during the repair. Open

Statement The anoplasty is performed with 16 sutures under slight tension so that when stitches are cut, the rectum will gently retract and appear like a normal anus. Open

Statement Dilations begin at 2 weeks postoperatively. Open

Statement Colostomy closure can take place 2 to 3 months after PSARP once the anus has reached its desired size. Open

Complications of Anorectal Malformations with Dr. Marc Levitt

Statement Anorectal malformations are commonly not properly diagnosed or completely missed in the newborn period. Open

Statement Male babies with perineal fistula may pass meconium and have the malformation go unnoticed, typically presenting in the first year of life with severe constipation. Open

Statement In perineal fistula, the hole is too small and anterior to the center of the sphincters, causing the rectum and sigmoid to dilate as stool passes through a tiny fistulous orifice. Open

Statement The standard practice of checking temperature on the forehead or ear rather than rectally means the anus may not be examined, potentially missing malformations. Open

Statement Relocating a perineal fistula into the sphincters does not completely fix constipation, though it improves anatomy by making the hole adequately sized and lined by mucosa. Open

Statement Patients with uncorrected perineal fistula may have some continence with formed stool but will soil with loose stool or athletic activity because sphincter contraction cannot completely close the anteriorly located hole. Open

Statement A newborn anus should accept a size 12 Hagar dilator and a one-year-old should accept size 15. Open

Statement A bucket handle skin tag is consistent with perineal fistula; a probe can be passed underneath it even when the fistula itself is not visible. Open

Statement Diagnosing perineal fistula in females is probably the most confounding thing in pediatric colorectal surgery, with many patients either missed or overdiagnosed. Open

Statement Criteria for perineal fistula in females: hole too close to vagina (inadequate perineal body), inadequate hole size by Hagar dilators, and hole not centered in sphincter. Open

Statement If the hole is adequate size and centered in the sphincter, even if appearing slightly anterior with short perineal body, the patient does not need surgery; the perineal body will lengthen with growth. Open

Statement Examination under anesthesia with stimulation can confirm whether a questionable hole is properly centered within the sphincter. Open

Statement An anesthesia nerve stimulator costing $150 with appropriate needle probes works as well as commercial $15,000 sphincter stimulators. Open

Quotation You don't need to order those because you can take the anesthesia stimulator, which is $150 and then put these two nice little probes into the stimulator and connected to little needles and you get a beautiful sphincteric response. Open

Statement In rectourethral fistula, there is no anal opening and no hope for a hole; some babies pee meconium, making the diagnosis obvious. Open

Statement Rectourethral fistulas should not be approached primarily because the rectum location (bladder neck, prostatic, or bulbar level) is unknown; attempting posterior sagittal incision may find urinary tract structures instead of rectum. Open

Statement Rectourethral fistula patients should be managed with colostomy and distal colostogram, except in exceedingly rare cases where cross-table lateral at 20 hours shows very low rectum allowing safe primary posterior sagittal approach. Open

Statement Cloaca can be missed in the newborn period; a recent case presented at 6 months with constipation when someone finally noticed no anus. Open

Statement Ambiguous genitalia (clitoromegaly from endocrine stimulation) with urogenital sinus has a completely normal anus, distinguishing it from cloaca which has no anus. Open

Statement Cloaca patients have no endocrine problem and two completely normal ovaries, unlike urogenital sinus with virilization. Open

Statement The most common colostomy error is opening too distal in the sigmoid, restricting the ultimate pull-through by the location of the colostomy or mucous fistula. Open

Statement Incompletely diverting loop colostomies allow stool to spill across and contaminate the distal segment, leading to urinary tract infections. Open

Statement Transverse colostomies can prolapse and, with large rectourethral fistula, the left colon absorbs urine causing acidosis; they also make distal colostogram difficult. Open

Statement Preferred technique is very proximal sigmoid colostomy leaving entire sigmoid for pull-through, with tiny flat mucous fistula separated from proximal stoma. Open

Statement Prolapse is related to colon mobility: mid-transverse colostomy both sides can prolapse, hepatic flexure only distal prolapses, proximal sigmoid only distal can prolapse because left colon is fixed to retroperitoneum. Open

Statement Ileostomies prolapse frequently because they are free-floating unless tacked to anterior abdominal wall. Open

Quotation I actually make mark the anoplasty before I make the incision. I think you can get lost when you're looking at a bunch of jumping muscles from a stimulator. Open

Statement The anoplasty site should be marked before making the incision by drawing a circle around the pinkish ellipse where it stimulates on the skin surface, preventing confusion when anatomy is disrupted. Open

Statement Without pre-marking, surgeons can choose the wrong place when seeing muscles jumping with stimulator after opening posterior sagittal incision, requiring re-operation despite good muscle potential. Open

Quotation Really, really good surgeons have put anuses in crazy places, and I think it's because they don't have a sense of what's the center because everything's disrupted once it's open. Open

Statement The distal colostogram is an absolutely vital study; many mistakes result from poorly done studies and misinterpretation. Open

Statement The colostogram must answer: where is the rectum, how low is it, is it reachable posterior sagittally or better laparoscopically, and what is its relationship to the urinary tract. Open

Statement Common colostogram error is insufficient contrast and pressure, giving false impression of high rectum and no fistula. Open

Quotation If you see that flattening of the rectum, you know the radiologist did not give enough of contrast, enough pressure, because you need to overcome the PC line because that's the sphincters. Open

Statement If the distal rectum shows a straight line corresponding to the pubococcygeal line, the radiologist did not give enough contrast or pressure; more pressure will show bulging rectum and fistula. Open

Quotation I like to look at the urethra and think of it as a reverse C. Or let's say an elbow, and I think if the fistula is at the elbow or below, it's a bulbar fistula, and if the fistula is above the elbow, it's a prostatic fistula. Open

Statement Fistula classification using urethra as reverse C or elbow: fistula at or below elbow is bulbar, above elbow is prostatic, at bladder neck is bladder neck fistula. Open

Statement Bulbous rectum may be reachable posterior sagittally and hard laparoscopically due to girth; tapered rectum is better approached laparoscopically. Open

Statement Opening posterior sagittally without knowing rectum location will find a whitish shiny structure that may be bladder neck, not rectum. Open

Statement Adjunct techniques to locate rectum include balloon catheter in mucous fistula inflated with fluid or gastroscope to look for light, though the speaker has not used these. Open

Quotation You should never go to the operating room without knowing exactly what anatomy you're going to expect to find. Open

Statement Never go to the operating room without knowing exactly what anatomy to expect from a proper distal colostogram; the key question is where is the rectum and is it the most posterior structure. Open

Statement Bulbar or low prostatic rectum with bulge is more easily approached posterior sagittally; high prostatic tapered rectum is best served by laparoscopy; bladder neck fistulas are certainly best by laparoscopy. Open

Statement Laparoscopy for bulging rectum below peritoneal reflection at low prostatic or bulbar level requires unnecessary extra work and risks leaving remnant of original fistula (roof) if surgeon is timid. Open

Statement Posterior sagittal approach for high rectum is very difficult to mobilize and risks urinary tract injury. Open

Quotation I never look at it that way. I think that laparoscopy replaces laparotomy. It's an elegant dissection from above, but don't give away the advantages of the PSARP. Open

Statement Laparoscopy replaces laparotomy as elegant dissection from above but should not give away advantages of PSARP; a mini-PSARP during laparoscopy allows safe pelvic entry and rectal tacking to prevent prolapse. Open

Quotation I like to call mine a laparoscopic assisted PSARP. I think that's a better terminology. Open

Statement Preferred terminology is laparoscopic-assisted PSARP rather than laparoscopy versus PSARP. Open

Statement Prolapse prevention: proper levator closure, tacking rectum to posterior edge of muscle complex for 3-4 stitches, not dissecting rectum more than necessary, avoiding excessive trimming. Open

Statement Prolapse occurs in about 3% of cases, particularly in those without great muscles. Open

Statement Rectal prolapse causes bleeding, mucus, and can inhibit bowel control in patients with good muscle potential because they cannot close the opening with prolapsed tissue through it. Open

Statement Prolapse more than 3 millimeters should be treated; ideal time is when colostomy is still present. Open

Statement For circumferential prolapse, performing half the circumference in two different ambulatory settings is preferred by families over hospitalization and avoids need for dilation since half is untouched. Open

Statement Perineal body dehiscence prevention requires complete anterior rectal wall separation from posterior vaginal wall to reach areolar plane, avoiding tension on anoplasty. Open

Statement Perineal body dehiscence is the most common cause of reoperation in female ARM repairs. Open

Statement Traditional postoperative management is NPO for 7 days on 10% dextrose (hyperalimentation only if longer than 7 days); recently trialing clear liquids only for a week to avoid hard stool while allowing some oral intake. Open

Statement Daily perineal examination during the first week is critical; if dehiscence is detected on day 5-8, taking the patient back to OR to re-suture can salvage the repair, but by 3-4 weeks later nothing can be done. Open

Quotation If you see it's opening, I actually will take them back to the OR and re-suture the perineal body. You can actually save it by doing that. Open

Statement Laparoscopy causes trouble if dissecting a rectum that is too low, getting too close to urinary tract or being too timid and leaving remnant of original fistula (distal rectum). Open

Statement For high rectums, particularly bladder neck fistulas, dissection to make the rectum reach with good blood supply is challenging; the IMA must be preserved because prior colostomy may have disrupted left colic collaterals. Open

Statement The rectum has excellent intramural blood supply from the IMA; taking tiny distal vessels along the rectal wall preserves this, but taking IMA or branches too close to aorta will cause rectal necrosis. Open

Statement The biggest PSARP problem is exploring without knowing rectum location and finding bladder neck, urethra, seminal vesicles, vas deferens, or ectopic ureter instead of distal rectum. Open

Quotation There are some famous cases of pull through bladder neck made into beautiful anoplasties. And then post op, the patient was draining liquid out their anoplasty, and in fact it was the bladder neck. Open

Statement Famous cases exist of bladder neck being pulled through and made into beautiful anoplasties, with the patient postoperatively draining liquid (urine) from the anoplasty. Open

Statement To determine continence potential, assess three factors: original malformation type, sacral quality and calculated sacral ratio, and spine quality (ARM continence index). Open

Statement Three A's in continence index (malformation type, sacrum, spine) predicts continence; three C's predicts incontinence; intermediate grades are being quantified through data collection. Open

Statement Bulbar fistula with sacral ratio of 1 and normal spine should absolutely have bowel control; bladder neck fistula with sacral ratio 0.4 and tethered cord or myelomeningocele has no chance of good bowel control. Open

Statement For 4-year-old with soiling and continence potential, first step is mechanical cleaning with bowel management enemas to gain confidence, then when older try switching to laxatives for voluntary bowel movements. Open

Statement If patient cannot be weaned from enemas, consider antegrade option like Malone procedure. Open

Statement Indications for redo pull-through: any continence potential with imperfect anatomy including improperly located anus, anal stricture, rectal prolapse, or remnant of original fistula (posterior urethral diverticulum). Open

Statement Redoing anoplasty to center rectum in sphincter can change a patient to have continence potential; success is very good when the right patient is selected. Open

Statement One of the biggest problems with anorectal malformations is that surgical errors do not become apparent for years, unlike most surgical complications which are evident immediately. Open

Quotation I think one of the biggest problems with anorectal malformations is that if you don't get it right, you don't really know for a few years. Most things in surgery, if you don't do it right, you know right away. Open

Quotation How are you supposed to as a surgeon, know what to fix about your technique if your problems are only becoming obvious years later? And I think that's why there is so much morbidity in colorectal. Open

Posterior Sagittal Anorectaplasty in a Female: Pediatric Colorectal...

Quotation This is a very good representation. I just have a very good question. I have a very good use. Open

Statement In babies this small, MRI has difficulty delineating uterine and ovarian structures Open

Statement Train-of-four box from anesthesia machine is a less expensive alternative to dedicated nerve stimulator for muscle mapping Open

Quotation I don't see a vagina. No, I don't see a vaginal opening at all. Open

Statement Lateral dissection defines the anterior plane in rectal mobilization - first step is to establish clean lateral plane Open

Quotation Lateral defines the anterior. First step, lateral. I want a nice, clean lateral plane. Open

Statement Lateral dissection defines the anterior plane in rectal mobilization - first step is to establish clean lateral plane Open

Quotation Lateral defines the anterior. First step, lateral. I want a nice, clean lateral plane. Open

Statement In absent vagina situations with recto-vestibular fistula, the urethra is characteristically enlarged Open

Statement In this patient population, there is thick wall between rectum and urethra in absent vagina situations Open

Statement Surgeon would not perform independent examination under anesthesia in straightforward newborn primary anorectal malformation case, but would examine at time of repair Open

Statement When fat is visible during rectal dissection, surgeon can safely dissect closer to the bowel wall Open

Statement Best time to create neovagina is when rectum has been mobilized; waiting creates scarred perineum making later reconstruction more difficult Open

Quotation I think technically it's the ideal time to do the neovagina when the rectum has been mobilized. I think that would be a mistake. I think it would be a very scarred perineum to then put a neovagina through. Open

Statement Best time to create neovagina is when rectum has been mobilized; waiting creates scarred perineum making later reconstruction more difficult Open

Statement Staple lines should be removed from neovaginal segment rather than left in place Open

Statement Surgeon prefers colon over small bowel for neovagina because small bowel mesentery is more tenuous and colon has more robust blood supply Open

Statement Standard neovaginal length in infant is 7-8 centimeters based on normal vaginal length in babies Open

Quotation I don't know what's going to happen with our technology and our ability to think with uteruses. I mean, who would have thought a year ago that there would be such a thing as a uterine transplantation? Open

Statement Neovagina should be tacked to pelvic fascia or posterior bladder to prevent prolapse and ensure it grows with the patient Open

Statement Six months after breast budding is appropriate timing for pelvic ultrasound and vaginoscopy to assess Müllerian structures and menstrual patency Open

Quotation I think it's a lot of torture to make them go through vasomal dilations. Open

Statement Surgeon does not dilate neovaginas post-operatively, anticipating some patients will need minor revision but avoiding torture of vaginal dilations Open

Statement Six months after breast budding is appropriate timing for pelvic ultrasound and vaginoscopy to assess Müllerian structures and menstrual patency Open

Statement MRI has limited ability to determine presence of vaginal lumen in very young patients unless there is clear hematocolpos or hydrocolpos Open

Quotation I don't think I would have changed a thing. I don't think we would have changed the preoperative workup. I wouldn't do an independent EUA. I would examine the patient at the time of the PSAR. Open

Posterior Sagittal Anorectaplasty-Female Part III: Pediatric Colorectal...

Quotation I don't think that's the case. I find the small bowel mesentery a bit tenuous. So I prefer, I think the colon is a little bit more robust. From a blood supply point of view. Open

Statement Colon is preferred over small bowel for neovagina construction because small bowel mesentery is more tenuous and colon has more robust blood supply. Open

Quotation I don't think that's the case. I find the small bowel mesentery a bit tenuous. So I prefer, I think the colon is a little bit more robust. From a blood supply point of view. Open

Quotation I just normally in a baby do about 78 centimeters because that's what a normal vaginal length looks like in a. I think there's no science behind that at all. Open

Statement 7-8 centimeters is used for neovaginal conduit length based on normal vaginal length in an infant, though there is no science behind this measurement. Open

Quotation I just normally in a baby do about 78 centimeters because that's what a normal vaginal length looks like in a. I think there's no science behind that at all. Open

Statement The family agreed to preserve structures that did not need removal, acknowledging uncertainty about future technology and outcomes. Open

Statement Uterine transplantation has become successful within the past year, representing advancement in reproductive technology. Open

Quotation I mean, who would have thought a year ago that there would be such a thing as a uterine transplantation. The successful. Open

Statement Uterine transplantation has become successful within the past year, representing advancement in reproductive technology. Open

Quotation I mean, who would have thought a year ago that there would be such a thing as a uterine transplantation. The successful. Open

Statement Neovagina should be tacked to pelvic fascia or posterior bladder to prevent prolapse and allow it to grow and lengthen into the pelvis. Open

Statement Vaginal dilation is not performed post-operatively; a certain percentage will need introitoplasty later, but dilation is considered torture for patients. Open

Quotation Well, I, I don't dilate the, I don't dilate these vaginas. And I anticipate that a certain percentage of them will need a little bit of an internalplasty later, but I think it's a lot of torture to make them go through vaginal dilations. Open

Statement Vaginal dilation is not performed post-operatively; a certain percentage will need introitoplasty later, but dilation is considered torture for patients. Open

Statement Six months after breast budding, Müllerian structures must be monitored for dilation and menstrual function assessed. Open

Statement Vaginoscopy should be performed after breast budding to determine if a cervix is present and if structures are larger. Open

Statement If no cervix is found on vaginoscopy, there is a difficult decision between empirical removal of Müllerian structures or waiting for complications. Open

Statement In patients without a cervix, some develop pelvic inflammatory disease episodes requiring removal, while others remain asymptomatic. Open

Statement Anal transitional epithelium (dentate line) should be preserved during anoplasty rather than excised. Open

Statement Pelvic MRI in infants struggles to identify vaginal lumen unless there is clearly hematocolpos or hydrocolpos; it is less helpful than desired. Open

Quotation It's always promises to be much better than it is, and. The very young patient Open

Statement MRI can frequently identify fallopian tubes, ovaries, and uterine anatomy, but determining the presence and characteristics of the vaginal lumen is very challenging with low confidence. Open

Statement Keeping fluid in the vagina during MRI imaging is difficult once the patient is in the magnet. Open

Statement No preoperative workup including independent examination under anesthesia, MRI, or better scoping would have changed the surgical approach in this case. Open

Quotation So I don't think I would have changed a thing. I don't think we would have changed the preoperative workup. I wouldn't do an independent EUA. I would examine the patient at the time of the PSA. Open

Statement No preoperative workup including independent examination under anesthesia, MRI, or better scoping would have changed the surgical approach in this case. Open

Quotation None of the imaging hinted at this, and even the office exam looked amazingly normal from a, a gynecologic point of view. Open

Statement None of the imaging or office examination hinted at the Müllerian anatomy found intraoperatively; the office exam looked amazingly normal from a gynecologic point of view. Open

Statement When the neovagina is connected to the patient's native vagina, it is already tethered into the abdomen and does not require additional tacking to the bladder. Open

ARMs in Female Patients: Pediatric Colorectal Controversies 2014

Quotation I think a lot of retracted anoplasties are Related to not getting it loose enough and you sometimes do a redo, you find that this plane, this plane which separates it hasn't been touched before. Open

Statement In every single female redo, the surgeon finds areolar tissue that had never been dissected by the original surgeon, suggesting inadequate anterior rectal wall mobilization led to perineal body disruption. Open

Quotation And in every single redo of a female, I find that perineal, I'm sorry, that areolar tissue that had never been dissected by the original surgeon. We know it. We say, see, no one, no one was ever here. Open

Statement The common perineal groove is often associated with an anorectal malformation and the vast majority will become normal skin over time if observed. Open

Statement Marc Levitt performs vestibular repairs primarily without a colostomy, either in the newborn period or within the first 3-4 months depending on the child's condition. Open

Quotation My personal practice is I wait, um, until the perineal body is healed, which usually is around day 6 or 7. Open

Statement Marc Levitt waits until the perineal body is healed (around day 6 or 7) before feeding after primary vestibular repair without colostomy, based on experience with redos in patients who were fed early. Open

Statement By watching the perineal body carefully and keeping the patient NPO, the surgeon can intervene and re-suture the perineal body on day 6 or 7 if needed, preventing complete dehiscence. Open

Quotation I believe if you are watching the perineal body carefully, you have a way to intervene without a dehiscence. You can actually take the patient back to the operating room on day 6 or 7 and re-suture the perineal body. Open

Statement In parts of the world without hyperalimentation, 10% dextrose can be used for NPO periods up to 7 days in healthy, robust children, though not in undernourished infants. Open

Quotation As a surgeon, you have to have a feeling and you have to do it the way you're used to and and feel like you're giving your patient a good result. Open

Statement About 2 to 5% of vestibular fistulas have a vaginal septum that should be identified at the time of primary repair. Open

Statement The ideal time to remove a vaginal septum is when the rectum is being mobilized and the perineal body is open, though many patients have had rectal repairs with the septum ignored. Open

Statement The alternative approach is to use the rectum as vagina and mobilize more proximal rectum down as neo-rectum, but this should only be done if the patient is unlikely to be continent due to spinal anomaly or absent sacrum, since rectum has value for continence. Open

Quotation I would only do that if I knew that the patient was unlikely to be continent, like they had a spinal anomaly or an absent sacrum. Otherwise I would do exactly as Don described. Open

Statement In vestibular fistula with absent vagina, the rectum separates very nicely from the urethra with thick fibrous tissue that is not nearly as adherent as rectum to posterior vagina, creating space for the neo-vagina. Open

Statement The ideal time to create a neo-vagina in ARM patients is when fixing the rectum because the perineal body is open, and it's technically easier when children are younger because the sigmoid pedicle reaches more easily in a shorter pelvis. Open

Quotation I think from a technical point of view, it's easier when they're younger because that pedicle, this pedicle reaches much easier when the child is young and doesn't have as long a pelvis. Open

Statement There are two different types of cloacas that should be distinguished: lower ones (13 centimeters or less common channel) and complicated ones (13 centimeters or greater), and making this distinction helps avoid trouble. Open

Statement About 50% of cloacas have a duplicated gynecologic system. Open

Statement Marc Levitt recommends just diverting the patient initially and dealing with vaginostomy without scoping, then doing endoscopy at 2-3 months of age when visualization is better and there's no rush. Open

Quotation I would pretty much base on how big the thing is. And if it's a monster hydrocopos and it comfortably reaches the abdominal wall, you can do a sutured tubeless vaginostomy. And if it's a little bit lower than that, your ideal is to do a tube vaginostomy, but I would suggest using a curled tube. Rather than a straight tube like a pesser or Malanot. Open

Statement For tube vaginostomy, a curled tube should be used rather than a straight tube, because as hydrocolpos inflammation resolves and it recedes from the abdominal wall, straight tubes fall out at about 2 months. Open

Quotation What happens with the hydroculpos is it recedes from the abdominal wall, the inflammation goes away, and then at about 2 months, the tube falls out. Whereas if you have a curled tube, that doesn't, uh, that doesn't happen. Open

Statement If using intermittent catheterization for hydrocolpos, the teaching should be done under ultrasound guidance because the tube can go into right vagina, left vagina, bladder, or rectum, and you may go three days without draining the correct structure. Open

Quotation I would add to that that that first, the teaching should be under ultrasound. Because, um, you can have that tube go into the right vagina, the left vagina, the bladder, or the rectum, and you may go three days without draining the right vagina, for example. Open

Statement In most patients with cloaca and hydrocolpos, draining the hydrocolpos alone is sufficient and bladder drainage is rarely needed. Open

Statement When there is a duplicated vaginal system in cloaca, both sides must be drained or only one side's hydronephrosis will improve. Open

Complications of Anorectal Malformations with Dr. Marc Levitt

Statement Anorectal malformations are commonly not properly diagnosed or completely missed in the newborn period. Open

Statement Male babies with perineal fistula may pass meconium and no one notices anything wrong with their anorectal anatomy, typically presenting in the first year of life with severe constipation. Open

Statement The standard practice of checking temperature on forehead or ear rather than rectally makes it easier to miss anorectal malformations in newborns. Open

Statement Relocating a perineal fistula into the sphincters improves anatomy but does not completely fix constipation; patients will inherently have some constipation requiring aggressive treatment. Open

Statement If a perineal fistula is not centered in the sphincter, patients with loose stool will soil, and athletic activity will cause soiling because sphincter squeeze cannot completely close the hole. Open

Statement A newborn anus should accept a size 12 Hagar dilator and a 1-year-old should accept a size 15. Open

Statement In females, diagnostic criteria for perineal fistula are: hole too close to vagina (inadequate perineal body), inadequate hole size by Hagar dilators, and hole not centered in sphincter. Open

Statement If a female's anal opening is adequate size and centered in the sphincter, even if appearing slightly anterior with short perineal body, no surgery is needed; the perineal body will lengthen with growth. Open

Statement An anesthesia nerve stimulator costing $150 with appropriate needle probes works as well as commercial $15,000 stimulators for identifying sphincter muscles. Open

Quotation I think that operation of a colostomy needs to be taken very seriously, and every pediatric surgeon who deals with newborns, which is the art of our specialty, needs to be really, really good at this because there's a lot of morbidity that's conveyed to a patient with an improperly done colostomy. Open

Statement The most common colostomy error is opening too distal in the sigmoid, restricting the ultimate pull-through by the location of the colostomy or mucous fistula. Open

Statement Incompletely diverting loop colostomies allow stool to spill across and contaminate the distal segment, leading to urinary tract infections. Open

Statement With transverse colostomy and large rectourethral fistula, the left colon absorbs urine which doesn't exit the mucous fistula, potentially causing acidosis from urine absorption. Open

Statement Prolapse risk depends on colostomy location: mid-transverse allows bilateral prolapse, hepatic flexure allows only distal prolapse, proximal sigmoid allows only distal prolapse because left colon is fixed to retroperitoneum. Open

Quotation I actually make mark the anoplasty before I make the incision. I think you can get lost when you're looking at a bunch of jumping muscles from a stimulator. Open

Statement Marking the sphincter ellipse on skin surface before making any incision prevents confusion from muscle stimulation after dissection is open, avoiding misplaced anoplasty. Open

Statement A distal colostogram showing flattening of the rectum corresponding to the pubococcygeal line indicates insufficient contrast or pressure; more pressure is needed to overcome the sphincters and reveal the true rectal position and fistula. Open

Statement Fistula level is determined by viewing the urethra as a reverse C or elbow: fistula at or below the elbow is bulbar, above the elbow is prostatic, at bladder neck is bladder neck fistula. Open

Statement Bulbous rectum on colostogram may be reachable posterior sagittally and difficult laparoscopically; tapered rectum is better approached laparoscopically. Open

Statement Opening posterior sagittally without knowing exact rectal location risks finding and potentially mobilizing bladder neck instead of rectum. Open

Quotation You should never go to the operating room without knowing exactly what anatomy you're going to expect to find. Open

Statement Bulbar and low prostatic fistulas with bulbous rectum are best approached posterior sagittally; high prostatic with tapered rectum and bladder neck fistulas are best approached laparoscopically. Open

Statement Attempting laparoscopy for bulging rectum below peritoneal reflection at low prostatic or bulbar level risks leaving behind a remnant of the original fistula (roof) if the surgeon is timid. Open

Statement Laparoscopy replaces laparotomy, not PSARP; a mini-PSARP during laparoscopy allows safe entry through peritoneal reflection and tacking rectum to posterior muscle complex to prevent prolapse. Open

Quotation I think that laparoscopy replaces laparotomy. It's an elegant dissection from above, but don't give away the advantages of the PSARP. Open

Statement Rectal prolapse occurs in about 3% of cases, particularly in patients without great muscles. Open

Statement Rectal prolapse more than 3 millimeters should be treated because it causes bleeding, mucus, and can inhibit bowel control in patients with good muscle potential. Open

Statement Circumferential prolapse can be trimmed in two separate ambulatory sessions (half circumference each), avoiding hospitalization and eliminating need for dilation since half the circumference remains untouched. Open

Statement Complete anterior rectal wall mobilization to the areolar plane between rectum and vagina is essential to avoid tension on the anoplasty that can lead to perineal body dehiscence. Open

Statement Perineal body dehiscence is the most common cause of reoperation in female ARM repairs. Open

Statement Clear liquids only for one week postoperatively prevents hard stool formation while allowing more stool volume, showing good perineal body healing results without traditional 7-day NPO period. Open

Statement If perineal body dehiscence is recognized on days 5-8, taking the patient back to OR to re-suture can salvage the repair; by 3-4 weeks the entire perineal body is dehisced and unsalvageable. Open

Statement During laparoscopic approach for high rectums, the IMA must be preserved because prior colostomy may have disrupted collaterals down the left colic, making the rectum completely dependent on IMA blood supply. Open

Statement Continence potential in ARM patients is predicted by three factors: original malformation type, sacral ratio, and spine quality (ARM continence index). Three A's predicts continence, three C's predicts incontinence. Open

Statement A bulbar fistula with sacral ratio of 1 and normal spine should have bowel control; a bladder neck fistula with sacral ratio of 0.4 and tethered cord or myelomeningocele has no chance of good bowel control. Open

Statement Initial management of soiling 4-year-old with continence potential is bowel management with enemas to achieve cleanliness, then trial of laxatives when older and more mature to attempt voluntary bowel movements. Open

Statement Indications for redo pull-through include any patient with continence potential who has improperly located anus, anal stricture, rectal prolapse, or remnant of original fistula (posterior urethral diverticulum). Open

Quotation If you don't do it right, you don't really know for a few years. Most things in surgery, if you don't do it right, you know right away. Open

Statement The major problem with anorectal malformations is that surgical errors may not become apparent for years; an improperly placed anus appears successful initially but presents with soiling at age 4. Open

The Colorectal Quiz Episode 1: ARM - Low Bulbar Fistula

Statement Anorectal malformation patients require screening for VACTERL association: vertebral abnormalities (plain X-ray), cardiac defects (exam and echo), esophageal atresia (NG tube pass), renal abnormalities (kidney ultrasound), and limb abnormalities (physical exam). Open

Quotation We want to make sure we've checked for any cardiac defect, both on exam and on echo. Open

Statement Sacral ratio measurement should wait until the child is 3 months of age for true measurements, though early imaging gives a feel for pelvic development. Open

Statement Spinal ultrasound in anorectal malformation patients should include evaluation of the presacral space to screen for presacral masses. Open

Statement Presacral masses are rare in typical imperforate anus but occur in almost half of anal stenosis or rectal atresia cases, which require MRI evaluation. Open

Quotation The sacrum looks quite normal. I bet this kid is gonna have a pretty normal sacral ratio, certainly greater than 0.7. Which connotes a very good prognosis for bowel control and really peace of mind for the family. Open

Statement A sacral ratio greater than 0.7 connotes very good prognosis for bowel control and provides peace of mind for families regarding potty training and school readiness at 4 years of age. Open

Quotation They want to know what's going to happen to this baby in 4 years when they need to potty train and go to school. Open

Quotation I think the key to deciding whether to dive into a perineum and posterior sagittal is, where is the rectum? Open

Statement The key to deciding whether to perform primary posterior sagittal anorectoplasty is knowing where the rectum is located; the danger is finding midline white structures like urethra, bladder neck, or bladder instead of rectum. Open

Quotation The danger is that you go in posterior sagittal, you don't know where the rectum is, and you find something midline and white, like the urethra, the bladder neck or the bladder itself. Open

Statement Colostomies and distal colostograms are performed to know exactly where the rectum is and whether to approach it perineally or laparoscopically. Open

Quotation I will tell you that the surgeon who sent me this case, they did not do that. They felt more comfortable doing a colostomy, and I wanna tell you, that was the safe thing to do. Bravo to them. That was the right choice. Open

Statement Colostomy is the safe choice for anorectal malformation repair, though it carries risks of complications from both the colostomy creation and the subsequent closure. Open

Quotation We are giving the child a colostomy. And whatever complications can happen from that, and we are giving the child a colostomy closure, and whatever complications can happen from that. Open

Statement Surgeons have performed primary anorectoplasty on low anorectal malformations without knowing about a fistula, resulting in children later urinating out of the anus. Open

Quotation This is sort of scary, because if you went in and grabbed this rectum and did a primary anoplasty. And did not know there was a fistula, and unfortunately, I have seen this done by some very good surgeons where they went in, did a beautiful anoplasty, but ignored the fistula, and the child down the road started peeing out their anus. Open

Quotation When I do that, I open up the posterior wall of the rectum and I inspect the anterior wall of the rectum. Open

Statement During primary posterior sagittal anorectoplasty, the surgeon should open the posterior wall of the rectum and inspect the anterior wall to rule out a fistula. Open

Statement 95% of Down syndrome patients with imperforate anus have no fistula, but 5% do have a fistula, so distal colostogram is still indicated. Open

Quotation 95% of Down's patients have no fistula, but 5% do. So I would still do a distal colostogram, and some of them might have a fistula. Open

Colorectal Quiz Episode 2: When to redo a PSARP

Statement The original malformation in Case 1 was a prostatic fistula. Open

Statement The patient in Case 1 has a tethered cord and a sacral ratio of 0.66. Open

Statement Visual cues for identifying correct sphincter location include the anal dimple, a midline raised area where the sphincters are, the ellipse, color change, indentation or raised area, and appropriate perineal body length. Open

Statement Case 2 patient was born with a vestibular fistula, has a normal spine and an excellent sacrum, indicating a much better prognosis for bowel control. Open

Statement The electrical stimulator used is the same one that anesthesia uses for their train of four, with an inexpensive connection with little pins. Open

Quotation I get asked all the time about the stimulator. It's the same electrical stimulator that anesthesia uses for their train of four. And then there's a connection that you can make that has little pins. Really, really inexpensive. And you just have to tell your anesthesiologist not to give skeletal muscle relaxant because it's a little bit weaker than the traditional stimulator, which was super expensive. Open

Statement You have to tell your anesthesiologist not to give skeletal muscle relaxant because the stimulator is a little bit weaker than the traditional stimulator. Open

Statement If you know the anatomy is off, you should do the redo, and there's an advantage to getting the anatomy right the younger the child is. Open

Statement For a two-year-old with a mislocated anus or bad prolapse, offer a redo and let them live in diapers for a year or two with better anatomy, then see if they can successfully potty train. Open

Statement Many patients present after the age of potty training because they're incontinent, and evaluation reveals the reason is they don't have the best operation—their anus isn't in the right place. Open

Statement For patients presenting with incontinence after potty training age, do the redo and usually add a Malone at the same time so they can learn how to get control with their new anatomy before attempting voluntary bowel movements. Open

Statement The process of learning control with new anatomy after redo and Malone may take 6 to 12 months. Open

Statement For a patient with a mislocated anus that's 50% within the sphincter complex, three and a half years old and fecally incontinent, one approach is to redo them, do a Malone, get them perfectly clean mechanically, then see if they can develop bowel control. Open

Statement If patients haven't declared their continence yet because they're not old enough to do so from a behavioral point of view, give them a chance—they may succeed. Open

Colorectal Quiz Episode 13: Newborn ARM Part 2

Statement Indications for surgery in female ARM include: hole too small, hole not in center of sphincter, and inadequate perineal body Open

Quotation You must achieve a hole in the center of the sphincter with an adequate peroneal body. And those are the indications for surgery. Open

Statement If anal opening is adequately sized, surrounded by sphincter, and has a perineal body (albeit short), no surgery is indicated Open

Quotation One of our professors like to say, and we trained at the same place, Jason and I, the great Mount Sinai in New York, it's very hard to improve on an asymptomatic patient. Open

Statement Short perineal body will grow over time and there is nothing to do about it surgically Open

Statement If half the fistula is within sphincter complex and half outside, patient will leak stool because they cannot close the hole, making surgery worthy Open

Quotation I think that if you don't have a sphincter on the anterior aspect of the analplasty, they will leak stool because they won't be able to close the hole. Open

Statement Cloaca patients do not need endocrine workup or steroids and there is no question of gender assignment - they are female Open

Statement Cloaca patients are still being misdiagnosed as ambiguous genitalia, with some babies not having proper gender assignment for one to two weeks Open

Statement Cloaca presents with single perineal orifice and hypertrophied area around clitoral hood is typical, not ambiguous genitalia Open

Quotation That little hypertrophied area around the clitoral hood on the right photo is fairly typical, and it is not, and I repeat, not ambiguous genitalia. Open

Quotation This patient has no endocrine problem, does not need steroids, does not need an endocrinologic workup. This is a cloaca. There's no question of their gender assignment. It's a female. Open

Quotation You really want to push down and flatten the perineal body. That is the key. You want to see if the perineal body is normal or not. Open

Statement Key to perineal exam is to push down and flatten the perineal body to assess if it is normal Open

Statement Normal anus is centered within sphincter, of adequate size, and perineal body is of normal length properly distanced from vestibule Open

The Colorectal Quiz Episode 23: Hirschsprung Disease - The Soiling Patient...

Quotation Let's talk dentate line, shall we? Open

Quotation Remember, the three components of continence are quality of sphincters, quality of dentate line, and motility. Open

Statement The three components of continence are quality of sphincters, quality of dentate line, and motility. Open

Statement In Hirschsprung disease, two sphincters are of concern: the external sphincter (under voluntary control) and the internal sphincter (which tends not to relax due to absent rectoanal inhibitory reflex). Open

Statement If both internal and external sphincters have been overstretched, both become problematic. Open

Statement A patient who has voluntary bowel movements during the day but soils at night indicates working external sphincters but non-functioning internal sphincters; when sleeping, they relax the external sphincter and lose control. Open

Quotation One very typical scenario that we see, let me ask you, what do you think it means if you have a patient that has voluntary bowel movements during the day and has accidents when they go to sleep. Open

Quotation So at night, they poop, but during the day, they have control. What does that mean to you here? What, what is working and what is not working? Open

Statement Loss of dentate line can occur with overstretching and some preservation of external sphincter, resulting in daytime control but nighttime soiling. Open

Quotation I'm, I'm, I'm gonna sign off and you guys just, Open

Statement The rectum (not the anal canal) has proprioception capacity to detect stretch, which is the signal that stool is accumulating and it's time to hold stool and find a bathroom. Open

Quotation I actually, if I may add one more thing, I also think that there is a proprioception concept, the stretch. Open

Statement In anorectal malformation patients, the rectum should be preserved because rectal stretch provides proprioception. Open

Quotation That's called proprioception, and that's your signal. Uh oh, stool is accumulating. It's time to exert some authority over my external sphincter, hold the stool in and find a bathroom. Open

Statement When the internal sphincter is functioning properly, it relaxes at the time of rectal stretch. Open

Statement Giving anorectal malformation patients stool softeners is problematic because they never feel the stretch; loose stool just flows and they will never have control of that. Open

Quotation This is why in ARM patients, in erectal malformation patients, giving them a stool softener is so problematic, because basically, they never feel the stretch. They just have loose stool flowing. Open

Statement ARM patients are much better off with bulk (kicked out by a laxative) than a stool softener that slowly oozes out. Open

Quotation So they are much better off with bulk, which is then kicked out by a laxative, than a stool softener that just slowly oozes out, they'll never have control of that. Open

Quotation And they said, well, I'm not yet farting with confidence. Open

Statement Loose stool is the enemy if you have borderline continence or even completely normal continence, because you don't know for sure that it's there. Open

Quotation And what that says is so important because loose stool. Is your enemy if you have borderline continence. It's your enemy if you have completely normal continents. Right? Loose stool is hard to deal with, because you don't know for sure that it's there. Open

Statement We are very dependent on the stretch and bulk of stool in the rectum; that's when the external sphincter goes into motion and the internal sphincter relaxes. Open

Quotation We are very dependent on the stretch, on the bulk of the stool in the rectum, and then that's when the external sphincter goes into motion, and the internal sphincter relaxes. Open

Statement Hirschsprung patients with absolutely intact sphincters are dependent on that stretch; they don't have a rectum (it's been removed) and their sigmoid has taken over that job. Open

Quotation Patients with Hirschprung's disease, with absolutely intact sphincters. Are dependent on that stretch, remember, they don't have a rectum, it's been removed. Their sigmoid has now taken over that job. Open

Statement Hirschsprung patients with injured sphincters are particularly in trouble regarding dependence on stool bulk and stretch. Open

Quotation If they have injured sphincters, they're particularly in trouble. Open

Quotation This is a trick question because this patient's never had any surgery. This patient is about to get a biopsy to rule out Hirschrung's disease, and I took this picture of a absolutely totally normal, never touched dentate line. Open

Quotation I do think it's a myth to say that this patient cannot have bowel control. I think this patient can develop bowel control, provided their sphincters are working. Open

Statement A patient with missing dentate line can develop bowel control provided their sphincters are working, but they will be very sensitive to loose stool. Open

Quotation They will be very sensitive to loose stool. They won't be good at detecting that something is there unless they have bulk, but if they have the right diet and the right stool consistency, And sphincters that are intact, we can get this patient through, but it's really challenging if they have a missing dentate. Open

Statement Patients with missing dentate line won't be good at detecting that something is there unless they have bulk, but with the right diet, right stool consistency, and intact sphincters, they can achieve control. Open

Quotation So here you're about to go train in a wonderful children's hospital. If I asked you which side of this anoplasty did the fellow do, and which side did the faculty member do, what, what would your, what would your answer be? Open

Quotation Very good. You are ready for your fellowship. Open

Statement Patients with horrific diaper rash and perineal excoriation related to no dentate line and no intact sphincters need temporary stomas; some may need permanent stomas. Open

Quotation I mean, this is just absolutely the worst. These patients, there are two of them here, show a horrific diaper rash, perineal excoriation related to no dentate line, no intact sphincters. Open

Quotation These patients needed temporary stomas, and I think the one on the right needed a permanent stoma. Open

Statement In a soiling Hirschsprung patient with no obstruction, no distention, no enterocolitis, and 3 stools a day, that's a patient who needs management of a slow-moving colon. Open

Quotation I mean, the bottom line is we have solved the obstruction. The Hirschprung's is an obstruction problem. Now the tough part is getting them clean. Two separate and independent challenges. Open

Statement Hirschsprung disease is an obstruction problem; once that's solved, the tough part is getting patients clean—two separate and independent challenges. Open

Statement The vast majority of Hirschsprung patients get clean on their own and have great success stories. Open

Quotation Most of them get clean on their own. That's why Hirshprung's vast majority of patients have a great, great success stories. Open

Statement Clinicians need to determine if Hirschsprung patients are too slow or too fast, how to manipulate their motility, and understand if they have the mechanisms needed for continence (sphincters and dentate line). Open

Quotation But then we need to know, are they too slow or are they too fast? How do we manipulate their motility, and we need to understand inherently, do they have the mechanisms needed for continence, i.e., their sphincters and their dentate line. Open

Statement For hypermotile patients, skin care is vitally important; a cyanoacrylate-based barrier is very helpful. Open

Statement Wound care improvements for perineums in Hirschsprung or any hypermotile patient have dramatically improved over the last 4-5 years. Open

Statement Proton pump inhibitors are helpful to reduce the acidity of stool in hypermotile patients. Open

Statement Some antacid products can be taken orally as liquid and put on the skin to reduce acidity and help excoriation. Open

Statement Small volume enemas are a very helpful maneuver for hypermotile patients. Open

Statement Water-soluble fiber (not water-insoluble) produces bulky stool and is helpful for hypermotile patients. Open

Statement Loperamide is very helpful medicine for hypermotile patients; the maximum dose is 0.5 to 0.8 mg per kilogram divided daily based on patient weight. Open

Statement Cholestyramine is the next level of treatment after loperamide for hypermotile patients. Open

Statement Hyoscyamine (Levsin) 0.125 mg tablet every six hours has been used for hypermotile patients. Open

Statement Diphenoxylate-atropine (Lomotil) is almost never used because it has cardiac side effects. Open

Statement Tincture of opium is useful for slowing stool but is a controlled substance and difficult to get prescribed. Open

Statement If everything checks out with the pull-through and patients are still not emptying, Botox may be needed to help patients train and control their non-relaxing sphincters rather than being withholders. Open

Quotation But I will say, the hardest group of patients of all the soilers that we take care of. Which include anorectal malformation, Hirschsprung's, functional constipation, and spinal. The worst group, the hardest group is definitely Hirschprung's, without question, because the sphincters are so troublesome. Open

Statement Of all soiling patients (anorectal malformation, Hirschsprung, functional constipation, spinal), the hardest group is definitely Hirschsprung without question, because the sphincters are so troublesome. Open

Statement Of Hirschsprung patients, the hypermotile are much harder to manage than the hypomotile. Open

Quotation Um, but of that group, the hypermodal are much harder than the hypomodal. Open

Statement With systematic strategies—knowing if patients have potential for bowel control and manipulating motility accordingly—many Hirschsprung patients who were told they could never be clean can achieve cleanliness. Open

Quotation But with strategies, you can really get a lot of these patients clean that were told they could never be clean. Um, if you sort of have your methodology, you know if they have potential for bowel control. And then you manipulate the motility as we've, as we've discussed. Open

Colorectal Quiz Episode 28: Female ARM Management - Perineal Fistula

Statement Conversations about female perineal fistula management are more time-consuming than those about cloaca, and patients seek multiple opinions for this relatively benign malformation. Open

Quotation the conversations that go around a female, the perineal fistula are more time-consuming than the conversations that need to happen about a cloaca. Open

Statement If the hole is in the center of the sphincter with adequate lumen and a perineal body is present, the patient does not need surgery. Open

Quotation if the hole is in the center of the sphincter or it's in somewhat of the sphincter, and if the hole is enough of a lumen, and if there's a perineal body, that patient does not need to be touched. Open

Statement If the hole is too small or outside of the sphincter, surgery is required. Open

Statement There are five valid management options for perineal fistula: colostomy then repair, primary repair, dilation then repair, simultaneous colostomy and repair, and dilation alone. Open

Statement Diverting with a colostomy does not necessarily prevent perineal body dehiscence, and colostomy carries significant morbidity including closure complications. Open

Quotation diverting with a colostomy doesn't necessarily stop that from happening and You have the morbidity of a colostomy and of the colostomy closure, which is nothing to sneeze at. Open

Quotation this is not an emergency. They are passing stool, so the diversion of stool is not the reason for the colostomy, unless it's a very, very tiny fistula, and they can't pass stool, but that can be managed by, by dilation. Open

Statement Dilation alone is potentially acceptable but could be problematic if the fistulous distal end will not grow, leading to proximal distension. Open

Statement Anal stenosis (position 5 on the classification) requires screening for Currarino syndrome. Open

Statement A vestibular fistula is not a vaginal fistula because the posterior vaginal wall is intact with no fistula to it. Open

Quotation this vestibular fistula is not a vaginal fistula because the vaginal wall, the posterior vaginal wall is intact. There's no fistula to it. Open

Statement True vaginal fistulas are exceedingly rare in anorectal malformations. Open

Statement Some perineal fistulas (position 4 on the classification) can be managed with posterior wall mobilization without touching the anterior wall. Open

Statement Surgeons performing vaginoscopy should look for single versus duplicated cervix, distal vaginal atresia, and vaginal septum. Open

Quotation Incidence of vaginal atresia is quite rare, and vaginal septums are a little bit more common, somewhere around 3 to 5% of vestibulars. Open

Statement The incidence of distal vaginal atresia is quite rare in anorectal malformations. Open

Statement Vaginal septums occur in approximately 3 to 5% of vestibular fistulas. Open

Statement Perineal fistulas can be associated with distal vaginal atresia, though less commonly than vestibular fistulas. Open

Statement For patients with anorectal malformations and ureteral abnormalities, differential renal function assessment (such as DMSA scan) is important to determine whether to reimplant the ureter or remove a non-functional kidney. Open

Quotation absent kidney, quote unquote. And it's usually not absent, it's more non-functional and it's often multicystic and dysplastic, not absent Open

Statement Absent kidneys in anorectal malformation patients are usually not truly absent but rather non-functional, often multicystic and dysplastic. Open

Quotation every surgeon takes care of an interectal malformation, ought to know the type of malformation, the status of the spine, i.e., tethered cord or myelomeningocele, or in most cases, luckily normal, and the status of the sacrum Open

Statement Every surgeon caring for anorectal malformations should know the malformation type, spinal status (tethered cord, myelomeningocele, or normal), and sacral anatomy including sacral ratio. Open

Statement A patient with a low-type anorectal malformation (such as perineal fistula) but with associated spinal pathology has a different prognosis for bowel control than the same malformation with a normal spine. Open

Quotation this is an interesting patient with a very low type of malformation, however, with an associated spinal problem, and therefore their prognosis is not the same as a perineal fistula patient with a normal spine Open

Statement Sacral ratio calculation is valuable for informing family conversations about potential for bowel control in anorectal malformation patients. Open

Colorectal Quiz Episode 29: Female ARM-Post Op Management

Quotation So are you trying to say that I am a conservative surgeon because I may have a different protocol? Open

Statement Alberto Pena's original protocol mandated 7 days NPO with central line and hyperalimentation after ARM repair, feeding only on day 7 if healed. Open

Quotation And I had a fellow that said, why exactly are you keeping these patients NPO? Open

Quotation I had a fellow that said, why exactly are you keeping these patients NPO? And I said, well, because I don't want them to stool, because if they stool, they're more likely to hiss their perineal body. And the fellow said, you know, they still stool. Even if they're NPO. And I said yes, but not as much. Open

Statement A study by Carlos Reck (now in Vienna, Austria) compared NPO for 7 days versus clear liquids for 7 days and found the same amount of stool output in both groups. Open

Quotation Same amount of poop. Open

Quotation So then I came to the conclusion that it's not the pooping that's the problem, it's the hard pooping that's the problem. Open

Statement The problem is not stool passage itself but hard stool passage that can disrupt the perineal body anastomosis. Open

Statement Dr. Levitt's current protocol is regular IV (no PICC line), clear liquids or breast milk for 5 days, with very low dehiscence rate. Day 5 provides better healing than day 1-2. Open

Statement There is no published article showing post-op day 1 regular diet (not breast milk, but actual food or formula) with a very low dehiscence rate. Open

Quotation And invariably, a perineal body dehiscence usually leads to no perineal body over several months, and needing for a redo because the anterior anoplasty has no sphincter around it, it's split. Open

Statement Perineal body dehiscence usually leads to no perineal body over several months, requiring redo surgery because the anterior anoplasty has no sphincter around it (it's split). Open

Statement Nearly every redo case Dr. Levitt sees for perineal body dehiscence involved patients who were fed right away and discharged home. Open

Quotation And every single one of those that I see nearly, I ask them what was the feeding protocol, and in invariably they were fed right away and discharged to home. Open

Quotation All right, my friend, give them clear liquids. You're gonna make a lot happier families and kids. We tested it. We have a nice paper about clear liquids. Open

Quotation Richard Wood and I ran a randomized controlled trial of dilation and non-dilation for primary PSAP. Cloacass were excluded, and families knew that they were going to be randomized into one of two groups, and the backup plan, if a patient developed um a stricture was dilation, plus or minus a Heineke McCulitz anoplasty. Open

Statement A randomized controlled trial by Richard Wood and Dr. Levitt compared dilation versus non-dilation for primary PSARP (cloacas excluded). Families were randomized and knew the backup plan for stricture was dilation ± Heineke-Mikulicz anoplasty. Open

Statement In the dilation trial, both groups (dilation and non-dilation) had stricture rates somewhere between 10 and 20%. Open

Statement Many patients' anoplasties look absolutely fine 8 weeks later at colostomy closure if they were never touched with a dilator, provided the anoplasty was healthy with no tension and good blood supply. Open

Statement The dilation study was prompted by asking families their biggest concern about ARM care, and by far number one was dilations. This was family-driven research, not doctor-driven problem-solving. Open

Quotation And what prompted the paper was, and this was Richard's idea, was to ask the families, what is their biggest concern relative to care of patients with an anorectal malformation, and by far, number one was dilations. Open

Quotation This was a real family-driven research. Open

Statement The non-dilation protocol offers families a choice: dilate twice daily for 4 months, or accept a 10-15% risk of stricture requiring Heineke-Mikulicz anoplasty, with the child already going under anesthesia in 8 weeks for colostomy closure. Open

Statement Jack Langer's protocol is to see patients weekly in clinic and pass a dilator himself rather than having families do it at home. Open

Quotation We don't know the answer to that question. That's a more longer term follow up. Open

Quotation Have you negatively affected their continence 3 years hence? And I don't know the answer to that question. Open

Statement Full continence can be restored with a redo operation for stricture, and data is available showing this. One indication for redo is stricture. Open

Quotation I personally have come to the conclusion that the amount of morbidity that we're putting families through by dilating them, and the minimal risk to them of even needing an intervention. And then if they do need an intervention, it's relatively minor, and the vast majority of those, they're already undergoing surgery for their colostomy closure. Open

Statement The vast majority of patients needing intervention for stricture in the non-dilation protocol are already undergoing surgery for colostomy closure, making the intervention relatively minor. Open

Statement Dr. Levitt has yet to meet a family that has chosen dilation when presented with the non-dilation option and its risks/benefits. Open

Quotation I can tell you, I have yet to, I have yet to meet a family that has chosen dilation. Open

Quotation I think the good news is that there's no right answer, we just have to all work together and suffer together on these difficult problems and try to, uh, improve lives as best we can. Open

Statement In Ghana, a colleague makes anoplasties slightly bigger knowing patients won't return for follow-up, anticipating some contraction will occur. Open

Statement For redo ARM cases, Dr. Levitt makes the anoplasty a little bigger knowing there will be contraction. Redos are not dilated at all, but are examined under anesthesia at one month to check for early stricture. Open

Quotation We don't dilate the redos, period. We EUA them at a month just to make sure no early stricture is developing. Open

Statement For primary repairs, Dr. Levitt makes the lumen match what the maximal rectal lumen can be. With good mobilization (not overdoing it, throwing away as little rectum as possible), the anoplasty is usually a good size, about Hegar 13 or 14 at the end. Open

Quotation In the primaries, I basically make the lumen what the rectum needs, what the maximal rectal lumen can be. And whatever that is, that fills the sphincter, that's how I make the anoplasty. Open

Is Multi-disciplinary care the future of medicine?

Statement Most families who have a baby born with an anorectal malformation never heard that was something that could happen and are in shock. Open

Statement The Babylonian Talmud from 2000 years ago describes that if an infant whose anus is not visible is born, they should be rubbed with oil and stood in the sun, and where it shows transparent should be torn crosswise with a barley grain, representing an early description of anoplasty. Open

Statement The first PSARP (posterior sagittal anorectoplasty) was performed in 1980, making modern functional reconstruction techniques much more recent than other congenital surgeries like esophageal atresia and diaphragmatic hernia from the 1950s. Open

Statement Before modern techniques, anorectal malformation patients survived but suffered from constipation and fecal incontinence due to poor surgical technique, unlike esophageal atresia patients who did not survive without good repair. Open

Statement Alberto Pena, who performed the first PSARP in 1980, is considered the father of pediatric colorectal surgery. Open

Statement In 1992, Alberto Pena was functioning as a lone provider handling colorectal surgery, urology, gynecology, psychology, and social work for his patients. Open

Quotation can I speak to the colorectal surgeon, he would say, you got him. That's me. All right, can you patch me through now to the urologist? He would say I do the urology as well. Do you have a gynecologist on your staff? I got that covered. Psychology, social work, I handle it all. Open

Statement Babies with anorectal malformations are almost always full term and healthy with no prenatal expectation of the problem. Open

Statement When families learn their baby has an anorectal malformation, their primary question is whether their child will be normal at age 4 during potty training, not about surgical technique elegance. Open

Quotation will my child be normal when they're age 4 and are supposed to be potty training. That is their question. Open

Statement A good anatomic reconstruction without a good functional result accomplishes very little, analogous to correcting scoliosis but leaving the child unable to walk. Open

Quotation Correct the scoliosis and the child can't walk, you haven't accomplished very much. Open

Statement Children's National is the World Center for Cloacal Care, having treated 32 cloacas in the last 4 years, while most centers see one cloaca every 5 years. Open

Statement Cloacal defects may be prenatally diagnosed, particularly when there is a large pelvic mass, hydronephrosis, or a missing kidney identified on ultrasound. Open

Statement Hydrocolpos (vagina filled with urine) in newborns with cloaca can depress respiration, cause kidney failure, and cause hydronephrosis, requiring management in specialized NICUs. Open

Statement Patients with anorectal malformations often have associated neurologic problems, may have a single kidney or some degree of chronic kidney disease, and may have abnormal gynecologic systems including duplicated Mullerian systems. Open

Statement The quality of the sacrum and spine are used to predict continence and allow confident counseling of families even on the first day of life. Open

Statement A patient with a favorable malformation, good quality sacrum, and normal spine will have continence in 4 years after reconstruction. Open

Statement A patient born with myelomeningocele will not be continent in 4 years, but bowel management programs can achieve functional outcomes. Open

Statement The word 'cloaca' comes from French meaning 'sewer,' and birds have cloacas that combine poop and pee together in a single exit. Open

Statement The Malone procedure involves taking the appendix, connecting it to the belly button, and using it to flush the colon once daily, resulting in no bowel movements for the next 24 hours. Open

Statement The center performs approximately 100 Malone procedures per year. Open

Statement The Malone procedure does not require continence or sphincters; as long as poop comes out at a specific time with no poop for 24 hours afterward, the child can wear normal underwear. Open

Statement If a patient does not have an appendix, one can be surgically created for the Malone procedure. Open

Statement Pediatric surgery fellows perform about 14 anorectal malformation cases in 2 years of training. Open

Statement A practicing pediatric surgeon performs about one anorectal malformation case per year. Open

Statement Insufficient case volume is one reason for the high number of redo operations seen in colorectal surgery. Open

Statement In the UK, biliary atresia and bladder exstrophy are specifically focused at certain centers and cannot be operated on in other centers because the country recognized the expertise available through the collaborative model. Open

Statement In collaborative surgery for patients with myelomeningocele and both urinary and fecal incontinence, a piece of colon can be used for bladder augmentation while simultaneously making the remaining colon shorter and easier to empty, solving both problems in one operation. Open

Statement The appendix can be split to create both a Malone and a Mitrofanoff procedure, which is only possible with a collaborative model. Open

Statement There are 600 newborns with anorectal malformations born per year in the United States. Open

Colorectal Quiz Episode 38: Transitional Care in Colorectal Surgery

Quotation I encourage you to do examinations under anesthesia. This is a very valuable maneuver for ARM patients who have had a previous surgery. Open

Statement Examination under anesthesia is a valuable maneuver for ARM patients with previous surgery, allowing assessment of anoplasty prolapse, stricture, and proper location via electrical stimulation. Open

Statement In males, cystoscopy is added to evaluation to rule out remnant fistula and assess bladder mucosa and emptying; in females, cystoscopy can identify inappropriate fistulas. Open

Quotation I was just going to say from a very functional point of view, what what's missing here is sphincters anterior to the anoplasty. Open

Statement The functional problem is absence of sphincter anterior to the anoplasty: when the patient squeezes, the sphincter cannot close the anterior aspect and stool slips out, whereas proper concentric sphincter placement would successfully close the hole. Open

Quotation So when the patient with very good sphincters tries to squeeze those sphincters, they simply cannot close the anterior aspect of the anus, and stool can slip out of there, whereas if the anoplasty was properly located and the sphincter was concentric. They would successfully close the hole, and that's the incontinence problem Open

Quotation it's the most common problem in females that Jason and I have done redos for, uh, because there simply is no circle Open

Statement The most common problem in females requiring redo surgery is absence of a circular sphincter (an O), either from improper initial placement or dehiscence of the perineal body leaving a C-shaped sphincter; the surgical goal is to get muscle in front of the anus. Open

Statement The strategy of non-diversion with postoperative clear liquids (not NPO/TPN) produces soft watery stool that does not harm the perineal repair, whereas real food produces harder stool that can disrupt the repair; patients and families are much happier avoiding PICC lines and TPN. Open

Quotation my impression is that if you give someone real food. They make harder stool which can then blow through your perineal repair, whereas if you give the patient clear liquids, the stools that they produce are very soft and very watery, which don't do anything really to the wound care Open

Statement At 5 days, if perineal healing looks good, diet is advanced with laxatives to keep stool liquidy; the problem is hard stool going through the repair, not stool itself, as long as the wound is kept clean. Open

Quotation I think the problem is hard stool going through your repair, not stool. As long as you're keeping the wound clean, we have not had a problem, and I have not, uh, needed to divert, uh, such a patient. Open

Statement The ideal transition model begins psychologic discussion in early teenage years, engages friendly adult colleagues, and involves joint clinic visits and collaborative operating; most pediatric surgeons understand this because they have received calls from adult surgeons encountering unfamiliar pediatric conditions like malrotation. Open

Statement Not all pediatric colorectal surgeons need to physically operate in the adult setting if there are interested adult colleagues; in Jason Frischer's model, he has privileges at the adult hospital but functions primarily as an assistant while the adult surgeon (Ian Piquette) provides adult care expertise. Open

Statement In the United States, the age line for pediatric vs. adult care is very blurry, which is problematic; patients over age 21 (or possibly 18) at children's hospitals face credentialing issues where pediatric nurse practitioners cannot write orders due to licensing restrictions. Open

Statement If a 40-year-old at a children's hospital needs ICU care, the intensivists are pediatric-trained, not adult-trained, creating a safety concern. Open

Statement Bowel management expertise needs to be passed not just doctor-to-doctor but also nurse-to-nurse and advanced practice provider (NP/PA) to advanced practice provider. Open

Statement Adult colleagues should be introduced to the Malone appendicostomy (which many have never heard of) and the use of Peristeen for self-controlled enemas in adults; these are tricks learned in the pediatric population. Open

Statement In the United States, financial pressures favor operations, but many ARM patients do not require operations—they just need medical management that can change their life positively; one week of bowel management can achieve continence in a patient who has been soiling for decades. Open

Statement The presented case converted a patient with 27 years of fecal incontinence to normal bowel control by changing anatomy, but this is not always an option; medical management is important, and transition will require devoted colleagues not solely motivated by procedures. Open

Statement A patient-held 'passport' template documenting all previous surgical procedures, dates, and medical history should be created and distributed to parent organizations so each family is responsible for maintaining their own record to hand to any new care provider. Open

Statement Transitional care is being done well in several places worldwide, including Paris where pediatric and adult surgeons join each other's clinics for the first couple of visits. Open

Colorectal Quiz: Episode 49 – Collaborating for Kids: Colorectal & Pelvic Solutions (with a Little Help from AI)

Quotation I was expecting lots of errors and a very computer sounding experience, and it wasn't that at all. Open

Quotation The computer read, and I put that in quotes, the article fully and then knew what was coming later in the article to get us as the listener excited about it. Open

Statement A urologist produced a review article outline in minutes using Notebook LM with 30 articles, a task that previously took two weeks. Open

Statement AI can identify gaps in knowledge across a set of uploaded research articles. Open

Colorectal Quiz: Episode 49 – Collaborating for Kids: Colorectal & Pelvic Care (with Help from AI)

Quotation We did something crazy. I uploaded an article that Jason Fisher and I wrote together about the collaborative model in colorectal care, published in seminars in pediatric surgery and loaded that article using AI into a podcast format. Open

Quotation I was expecting lots of errors and a very computer sounding experience, and it wasn't that at all. It was a lovely two human sounding voices that were chatting about the article that Jason and I wrote. Open

Statement The AI-generated podcast from the uploaded article was exceedingly accurate and featured human-sounding voices that foreshadowed content coming later in the article Open

Quotation The computer read, and I put that in quotes, the article fully and then knew what was coming later in the article to get us as the listener excited about it. Open

Statement A neurologist colleague produced a review article outline in minutes using Notebook LM with 30 articles that previously took 2 weeks to complete manually Open

Statement Notebook LM identified gaps in knowledge from the uploaded articles and could recognize when new articles filled those gaps Open

Colorectal Quiz Episode 1 - Low Bulbar Fistua

Statement VACTERL workup for anorectal malformation includes vertebral abnormalities (plain X-ray), cardiac defects (exam and echo), esophageal atresia (NG tube passage), renal abnormalities (kidney ultrasound), and limb abnormalities (physical exam). Open

Statement True sacral ratio measurements should wait until the child is 3 months of age, though early imaging gives a sense of pelvic development. Open

Statement Spinal ultrasound should include evaluation of the presacral space to look for presacral masses. Open

Statement In anal stenosis or rectal atresia defects, presacral masses are found almost half the time, and those patients require MRI. Open

Statement Cross-table lateral can be obtained at bedside in the neonatal unit by placing the baby on a bump under the buttocks. Open

Statement A sacral ratio greater than 0.7 connotes very good prognosis for bowel control. Open

Statement The key to deciding whether to perform posterior sagittal anorectoplasty is knowing where the rectum is located. Open

Quotation the danger is that you go in posterior sagitary, you don't know where the rectum is, and you find something midline and white, like the urethra, the bladder neck or the bladder itself. Open

Statement The danger of blind posterior sagittal approach is finding a midline white structure like the urethra, bladder neck, or bladder itself instead of the rectum. Open

Statement Colostomies and distal colostograms are performed to know exactly where the rectum is and whether to approach it perineally or laparoscopically. Open

Statement Colostomy is the safe choice for anorectal malformation repair, though it carries risks of complications from both the colostomy creation and the subsequent closure. Open

Statement If primary anorectoplasty is performed without recognizing a fistula, the child may later urinate through the anus. Open

Quotation I have seen this done by some very good surgeons where they went in, did a beautiful anoplasty, but ignored the fistula, and the child down the road started peeing out their anus. Open

Statement During primary posterior sagittal repair, the posterior wall of the rectum should be opened and the anterior wall inspected to rule out fistula. Open

Statement Distal colostogram should still be performed in Down syndrome patients with imperforate anus because some may have a fistula despite the lower prevalence. Open

Statement 95% of patients with Down syndrome and imperforate anus have no fistula, but 5% do have a fistula. Open

Episode 19 - Interview with Dr Marc Levitt, Chief of Colorectal & Pelvic Reconstruction, Children‘s

Quotation I was the kid who, when I came home, there was a broken vase. All the pieces were sitting on the table, a bit of glue, and my mom communicated to me my responsibility. So I was the one putting those things back together in the house. Open

Quotation there's a specialty that's called pediatric surgery. You can actually do both. Open

Quotation the idea was born that we must have a collaborative approach with lots of disciplines represented to help a complicated patient. Open

Statement The multidisciplinary approach with multiple disciplines represented to help complicated ARM patients was conceived during Levitt's medical school elective with Alberto Pena. Open

Statement The first ever colorectal center was launched in Cincinnati in 2005 by Levitt and Pena. Open

Quotation if you inspire a smart mind to focus on this field, then you get that many more exponentially exciting ideas. Open

Statement Training surgeons in ARM specialty provides greater impact than individual patient care because trained surgeons help hundreds or thousands more patients. Open

Quotation if they are inspired to then do that in their own city and develop their own team, they help so many hundreds of more patients, thousands, than I could ever possibly help just by taking care of those patients myself. Open

Statement The bowel management program with focused nursing attention for 4-5 days plus long-term follow-up is a major advance in ARM care. Open

Statement Integration of urology and bladder management has revolutionized ARM care, with kidney transplant now exceedingly rare compared to previously being not uncommon. Open

Statement Better urologic care has dramatically reduced renal disease in ARM patients. Open

Statement The standard PSARP incision has gotten smaller and understanding of anatomy has improved since 2005. Open

Statement Laparoscopy has been added to many kinds of ARM cases as a technical innovation. Open

Statement Transition programs for ARM patients moving to adult care are the most behind aspect of the field compared to other areas like congenital heart disease and cystic fibrosis. Open

Statement There are only 3 surgeons in the entire world trained in both pediatric and adult colorectal surgery: Ali Gisher in Columbus Ohio, Erin Teeple in Wilmington Delaware, and a surgeon named Mark in Munich Germany. Open

Statement Paris at Necker Children's Hospital has done an amazing job with formal transition to adult programs, with teenagers meeting pediatric and adult providers together before transitioning to adult facility visits. Open

Statement No parent has ever known anything about ARM when their baby is born, creating shock when a colorectal problem is announced. Open

Statement ARM surgery typically requires 3 surgeries within the first year of life: colostomy, reconstructive operation, and colostomy closure. Open

Statement ARM surgery requires months to years of tinkering and may require lifelong adjustments, unlike typical surgery where the problem is immediately solved. Open

Statement Parents care more about functional results than anatomic reconstruction, but surgeons are often very proud of their anatomic creations. Open

Statement A complex colorectal operation takes about 4 hours, but delivering a good result requires 96 more hours of tinkering, almost all of which is nursing care. Open

Quotation I like to say that the complex colorectal operation takes about 4 hours, but to deliver a good result, you need 96 more hours of tinkering. Almost all of that is nursing care. Open

Statement If a proper colostomy is made, most families would agree they'd rather change a colostomy bag than a diaper. Open

Statement If the anus is misaligned with sphincters due to surgical error, the problem won't be discovered until potty training fails 4 years later, making it difficult for surgeons to improve their technique. Open

Statement Studies have shown cardiac surgery in adults gets better results with higher surgical volume. Open

Statement In typical US pediatric surgery training, fellows do approximately 12 ARM cases over 2 years of training. Open

Statement The average finished pediatric surgeon performs 1 ARM case per year. Open

Statement Levitt advocates strongly for pull-through surgery and colostomy closure rather than permanent colostomy, stating he wants every kid to be typical and normal without a colostomy. Open

Quotation I don't want any kid to have a colostomy. I want everyone, I want those kids typical, normal, just like all the other kids. Open

Quotation the pull through should be done, the colostomy should be closed, and with proper bowel management and good nursing care, I'm very confident that we can get patients clean. Open

Statement With proper bowel management and good nursing care, Levitt is very confident patients can be gotten clean mechanically. Open

Statement The Malone appendicostomy is an advance that allows patients without innate anatomy or sphincters to be cleaned mechanically by emptying the colon once daily. Open

Statement Most patients would prefer being cleaned through the Malone route than having a permanent stoma. Open

Statement Permanent colostomy may be more practical for rare patients who don't ambulate. Open

Statement The vast majority of ARM patients can be perfectly clean with stool flowing through the normal anal route via bowel management. Open

Statement Many patients actually develop bowel control even if predicted they would never achieve it, with proper nursing care and medical regimen tinkering. Open

Quotation you would be amazed how many patients actually develop bowel control. Even if they were predicted that they would never achieve bowel control again with proper nursing care and tinkering with their medical regimen. Open

Statement Levitt and Richard Wood conducted a randomized controlled trial comparing standard dilation protocol versus no dilation after primary PSARP. Open

Quotation it's not the unanswered questions that matter the most, it's the unquestioned answers. Open

Statement The dilation study found that whether dilations were done or not, there was a similar 15% risk of developing a stricture. Open

Statement Strictures can be treated straightforwardly with a minor Heineke-Mikulicz anoplasty to enlarge the stricture at the time of colostomy closure. Open

Statement Levitt's center no longer performs routine dilations, allowing families to avoid daily dilation burden and accept the 10-15% stricture risk manageable at colostomy closure. Open

Statement Doing a good anoplasty with no tension and good blood supply affects the likelihood of getting a stricture. Open

Statement Skin irritation after colostomy closure is a short-term problem lasting about 2 weeks because the skin has never seen stool. Open

Statement The distal unused bowel segment needs to learn to adapt and absorb water to thicken stool after colostomy closure. Open

Statement Post-closure skin irritation is managed with good perineal creams, frequent diaper changes, and preventing stool from sitting on skin, typically resolving by 3 weeks. Open

Statement At 3 weeks post-closure, the problem switches to constipation, which many surgeons fail to manage aggressively. Open

Statement Unrecognized constipation causes colonic dilation over time, making the colon harder to empty by age 4 when potty training begins. Open

Statement Almost all of Levitt's patients are on laxatives about 3-4 weeks after colostomy closure to keep them flowing. Open

Statement Starting at about age 2.5 years, the goal is achieving a good bowel movement pattern of 1-2 well-formed stools per day. Open

Statement Achieving good bowel pattern requires diet with bulk fiber, water-soluble fiber, and a laxative with senna or bisacodyl that provokes stool. Open

Statement Stool softeners do not help ARM children and actually make them worse because they prevent sensation development needed for potty training. Open

Statement ARM children have a surgically created anal canal without typical sensation and somewhat good or not-so-good sphincters, missing two key continence components. Open

Statement ARM patients are dependent on feeling rectal stretch from stool filling to develop bowel control. Open

Statement Loose stool from stool softeners prevents feeling stretch, preventing development of bowel control. Open

Statement Bulky stool (not constipated but with enough bulk to feel stretch) allows most patients to translate into successful potty training with one stool per day. Open

Statement If potty training doesn't succeed, bowel management should begin at age 3-4 to get children clean and in normal underwear. Open

Statement Enemas should be introduced when the child should be in normal underwear according to family choice, which varies but should be by age 4-5 for all children. Open

Statement Bowel management is nursing-run by nurse practitioners or advanced practice providers. Open

Statement The first responsibility in bowel management is assessing patient anatomy, which should be fixed first if there are anatomic issues. Open

Statement Bowel management with enemas can still be done even without perfect anatomy, with anatomic correction performed later. Open

Statement Bowel management involves mechanical emptying of the colon with an enema containing saline plus an additive that provokes stool emptying. Open

Statement The Malone procedure connects the appendix to the umbilicus for more comfortable and independent enema administration. Open

Statement The goal of bowel management is to empty the colon with a flush, then have nothing pass for the next 24 hours, achieving a clean child in normal underwear for 23.5 hours. Open

Statement Bowel management program typically lasts about a week, with 4-5 days of X-rays. Open

Statement An early Cincinnati study claimed 95% bowel management success at one week, but this short-term result is misleading. Open

Statement Levitt and Wood's follow-up study documented bowel management results at 1 year, showing over 80% success for ARM patients. Open

Statement Among the 4 patient categories (ARM, Hirschsprung's, spinal disorders, functional constipation), Hirschsprung's patients are the hardest for bowel management because they don't have good functioning sphincters or their sphincters are sometimes too good. Open

Statement A lot of tinkering is required during the year after bowel management initiation, with X-rays and flush adjustments when accidents occur. Open

Statement About 75% of ARM patients should be able to develop their own bowel control and have voluntary bowel movements based on good operation, good quality sacrum, and good quality spine. Open

Statement About 25% of ARM patients will not achieve their own bowel control even with perfect operation because they don't have the best sphincters or have spinal issues affecting bowel function. Open

Statement Many patients choose the Malone appendicostomy route to the umbilicus rather than rectal enemas for bowel management. Open

Statement In Europe, many patients use Peristeen for enema administration. Open

Statement Levitt performs over 100 Malone procedures per year laparoscopically as a one-hour procedure with most patients staying one night in hospital. Open

Statement The Malone procedure is very low investment of patient time and surgical morbidity, and is straightforward if done frequently. Open

Statement The Malone makes it much easier for older children to administer their own enema without parental help. Open

Statement A full-fledged colorectal center requires integrated psychology, child life services, and social work rather than surgeons calling them only when help is needed. Open

Statement An integrated psychologist should meet every patient and decide whether they can be of use rather than waiting for surgeon referral. Open

Statement The burden of therapy has been ignored but is an enormous component of ARM care that every collaborative center should recognize. Open

Statement The dilation study was prompted by investigating burden of therapy and recognizing the cost to families of achieving correct anus size. Open

Statement Many ARM problems are physiologic rather than psychologic, requiring anatomic correction and bowel management first before psychological intervention. Open

Statement Many patients who received significant psychological help actually needed re-operation and good bowel management, after which psychological and emotional problems resolved. Open

Statement One week of proper bowel management can transform an entire family's emotional state. Open

Statement Fecal incontinence is a physiologic problem, not a psychologic problem, though psychology still needs to be managed. Open

Statement Patient engagement through family networks and social media has dramatically improved ARM care worldwide. Open

Statement Greg Ryan did not meet another ARM patient until his adult life, 52 years after his surgery. Open

Statement The origin of modern ARM care began in Melbourne Australia with Dr. Kelly, who learned from Dr. Stevens and taught Dr. Pena, who revised and improved the operation and launched it in 1980. Open

APSP Online Webinar | Marc Levitt & team | Bowel Management Program for Fecal incontinence

Statement For children under age 2-3 (before potty training age), laxatives are used primarily to prevent constipation; timing of bowel movements is not critical. As children approach potty training, temporary enemas help retrain the colon by giving a period without stool so they can learn what being clean feels like. Open

Statement Before starting laxative therapy, obtain abdominal X-ray to ensure no large stool burden or fecal impaction, because laxatives prevent constipation but are not strong enough to treat existing severe constipation and may cause vomiting if given with impaction present. Open

Quotation The laxative that we use are really great at preventing constipation, but they're not so great at treating constipation. Open

Statement Senna is the preferred stimulant laxative because it is a natural herbal medication derived from Senna plant leaves that irritates bowel lining to increase peristalsis. Starting dose is 2mg/kg with no fixed maximum; titrate based on patient tolerance and effect. Open

Quotation We don't use lax in our practice as a maintenance medication because it's an osmotic laxative and it often softens the stool, but it doesn't stimulate a bowel movement. Open

Statement Miralax (polyethylene glycol) is not used as maintenance medication because it is an osmotic laxative that softens stool without stimulating bowel movement, and loose stools make it difficult for ARM patients to sense rectal/colonic tension needed to recognize need to defecate. Open

Quotation It also makes it very difficult for our patients with anorectal malformations to sense the need to use the bathroom because their stools become so loose, um, that they can't feel any tension in the rectum or colon to to make it obvious that they need that they that there is stool there and they need to go to the bathroom. Open

Statement Literature supports no long-term complications or contraindications to prolonged Senna use. Senna burns to buttocks skin occur only in smaller/younger patients on high doses with prolonged stool-skin contact; prevented by appropriate dosing and frequent diaper changes. Open

Statement Senna takes approximately 12 hours to take effect, so evening dosing typically results in morning bowel movement. During initial titration, have patients sit on toilet first thing in morning and after every meal/large snack to establish their pattern. Open

Statement Bisacodyl is second-line stimulant laxative (also irritates bowel lining) but tends to provoke frequent smaller bowel movements rather than fewer consolidated movements that patients prefer. Available in pill form that can be crushed; side effects include cramping and nausea. Open

Statement If patient on Senna does not stool for 24 hours, give Fleet enema from below before increasing Senna dose to avoid making patient sick by increasing medication while stool accumulates. Open

Statement Water-soluble fiber is essential with Senna to bulk stools that become loose from accelerated colonic transit. Nutrisource is preferred because it is flavorless, unlike orange-flavored Metamucil/Citrucel. Start 2 grams twice daily, spread throughout day as dose increases for best efficacy. Open

Statement Pectin and Nutrisource work best in hypermotile patients. Titrate fiber based on stool frequency and consistency; no maximum dose except Nutrisource capped at 6 scoops. Beyond 4-6 grams/day fiber, add other interventions rather than continuing to increase fiber. Open

Statement Water-soluble fibers (Citrucel, Metamucil, pectin, Nutrisource) have different effects than water-insoluble fibers (Benefiber, Fiber One bars, dietary fiber), which have more laxative effect. Families are often unaware of this distinction. Open

Statement A subset of ARM and Hirschsprung patients have hypermotility (colon moves too fast) causing incontinence from frequent stooling rather than constipation. These patients need to be slowed down with constipating diet, water-soluble fiber, loperamide, hyoscyamine, and possibly small-volume nightly enemas. Open

Statement For hypermotile patients, small-volume nightly enemas (approximately half the volume used in large-volume enemas) can keep them clean overnight and help consolidate to 1-2 bowel movements daily, avoiding need for additional medications with side effects. Open

Statement Laxative foods that accelerate colonic transit include high-sugar foods, berries, and high-fat/greasy/fried foods. Constipating foods include bananas, rice, pasta, and bread. Families often don't realize dietary changes cause bowel management program failures. Open

Statement A paper from Mexico City comparing Miralax and Senna for ARM patients found Senna much more effective. Miralax makes stool soft without helping it come out, losing the rectal stretch that is probably the most important factor for ARM patients to feel stool and have control. Open

Quotation What mirrorlex does is it makes the stool soft. It doesn't help it come out and when a stool is soft, you lose the stretch of the rectum and I think the stretch of the rectum is probably the most important factor of whether an arm patient can feel stool and have control. Open

Quotation Sena on the other hand pushes out the stool and to compensate for that push which would normally make the stool very liquid. We give as was mentioned water soluble fiber to provide a little bit of bulk and therefore the stool that gets pushed out by the sena is a single well formed section of stool and the the patient can feel that much more. Open

Statement Senna pushes out stool (which would normally make it very liquid), so water-soluble fiber is given to provide bulk, resulting in a single well-formed section of stool that the patient can feel much better than soft stool from Miralax. Open

Statement In Duhamel pull-through patients, the colon is usually hypomotile. Contrast studies should be evaluated for Duhamel pouch size, presence of spur, and any areas of persistent narrowing that might indicate stricture. Open

Statement For refractory functional constipation cases, workup should include anorectal manometry to assess for recto-anal inhibitory reflex (RAIR). If RAIR is absent (no internal sphincter relaxation), biopsy for Hirschsprung and treat with Botox for possible internal sphincter achalasia. Open

Statement Functional constipation patients who cannot successfully empty on laxatives or find them difficult to take are good candidates for either rectal enemas (if tolerated) or antegrade enemas with Malone flush, with most responding very well to antegrade flush. Open

2019 Chandler Lecture: Dr. Marc Levitt

Statement In medicine, the colorectal surgeon may take care of a colorectal problem and not really worry about the bladder issue, and 6 months later the urologist meets the patient and realizes they have to do some intervention after the patient already had a laparotomy for their colon problem—completely uncoordinated care. Open

Statement A pediatric surgery fellow does about 14 anorectal malformation cases in 2 years, and a graduate does about 1 case per year. Open

Statement There are about 600 newborns with an anorectal malformation born per year in the United States. Open

Statement Complications of anorectal malformations include fecal incontinence, urinary incontinence, renal dysfunction and the need for renal transplant, sexual dysfunction, and infertility. Open

Statement In colorectal surgery, if you do an operation as a 6 month old and put the anus outside of the sphincters, the 6 month old won't know that until they are 4 years old and trying to potty train—the surgeon will never figure out they didn't do a great job and will keep doing the same mistake over and over again. Open

Statement The speaker's current practice is about 60% reoperative surgery. Open

Statement Patients with anorectal malformations many times have associated spinal problems, and if you don't collaborate upfront with neurosurgery, you may discover a neurologic problem 5 years later when the patient already has renal damage. Open

Statement Patients with cloacal malformations many times have Mullerian anomalies and hemisystems—if you don't think about gynecology when they're 6 months old during cloacal reconstruction, you're setting yourself up for a very difficult problem 13 years down the road when they have pelvic pain because their Mullerian structure is not draining menstrual blood. Open

Statement Knowing the associated sacral and spinal problems allows us to predict continence and have a conversation with a family in the neonatal unit about whether their child will very likely be continent with proper management, or whether there's not really a lot of chance for continence because of the associated spinal problem. Open

Statement Rectourethral fistulas are classified as bladder neck fistula (at the deltoid level), prostatic fistula (at the tricep level), or bulbar fistula (at the elbow level)—each diagnosis has a different surgical approach and a different potential outcome. Open

Statement Common technical errors in anorectal malformation repair include anus placed too far posterior (outside the sphincter), stricture, remnant of the original fistula left behind, and rectal prolapse—these are solvable with better technique. Open

Statement Redoing patients with misplaced anus can dramatically improve their functional outcome and quality of life—we were able to convert a good number of these patients to continence by making them have better anatomy. Open

Statement The surgeon's responsibility is to get good anatomy, but after achieving that goal there's a functional outcome that's relevant—you can do a perfectly good Nissen but it requires some tinkering over time to try to get the best possible result for that patient. Open

Statement The colostomy and ileostomy closure surgical site infection rate was 22.4%, which is pretty standard in the literature. Open

Statement Implementing a GI bundle—most importantly changing instruments and gloves before closing the fascia—reduced the wound infection rate to 7.9%. Open

Statement The organism that ultimately grew in the wounds was resistant to the preoperative antibiotic (cefoxitin) given within an hour of incision in every circumstance. Open

Statement Changing the preoperative antibiotic from cefoxitin to Unasyn (chosen with infectious disease collaboration based on wound culture data) reduced the wound infection rate to 2.2%. Open

Statement In cloacal malformation repair, you have to measure both the common channel length and the urethral length and make a plan accordingly—if the urethra is going to ultimately be shorter than 1.5 centimeters, bringing the bladder neck down to the perineum makes a wet (incontinent) patient. Open

Statement If you leave enough of the urethra behind at the end of cloacal reconstruction (>1.5 cm), you could have a dry patient 4 years hence. Open

Statement In a 6 year old with myelomeningocele who has urinary and fecal incontinence, you can take out the sigmoid to improve bowel care but keep it on its mesentery and roll it over to use for bladder augmentation, and share the appendix between Malone (antegrade enema) and Mitrofanoff (catheterizable channel)—the only way to do that is with coordinated care between urology and colorectal surgery. Open

Statement The proximal appendix becomes the antegrade access for enemas for patients that are fecally incontinent but can be made clean by a flush every single day, and the distal piece stays on its blood supply and can be implanted into the bladder for intermittent catheterization for urine—they can be rendered dry and clean for urine and stool in a single operation. Open

Statement There are certain patients with severe functional constipation that need surgical solutions—in the adult world that's a subtotal colectomy ileorectal anastomosis, but in the pediatric world before the entire colon has dilated up we may be able to get away with just a segmental resection of the sigmoid. Open

Time for some patient driven change | Marc Levitt | TEDxColumbus

Statement In medicine, complex problems are often approached without coordinated planning, analogous to building a bridge by having cement layers arrive Monday, steel workers Tuesday, architect Wednesday, and engineers Thursday. Open

Statement Some children are born without an opening for stool (anorectal malformation). Open

Statement A cloacal malformation is a condition where the bladder, gynecologic system, and colon all converge into one opening. Open

Statement Cloacal malformation is not compatible with life without surgical intervention. Open

Statement Once surgically corrected, cloacal malformation has unique implications on the child's quality of life. Open

Statement In cloacal malformation, the bladder, gynecologic, and colorectal structures are anatomically adjacent to each other, and each is handled by a different type of doctor. Open

Statement Effective management of cloacal malformation requires doctors from different specialties to work together. Open

Statement Twenty-five years ago, the speaker's mentor attempted to provide colorectal surgery, urology, and gynecology care for these patients as a single provider. Open

Quotation if the phone rang and someone said, can I speak to the colorectal surgeon, he would say, Speaking. Well, actually, I'd like now to speak to the urologist, and he would say, you got him. And the gynecologist, that's me Open

Statement As medicine became more complicated, a single provider became incapable of solving all problems in complex malformation cases without gathering a multidisciplinary team. Open

Statement The speaker's vision was to unite GI motility specialists, colorectal surgeons, gynecologic surgeons, and urologic surgeons to work together in a unified approach for complex children. Open

Statement Surgeons, particularly, like to be in charge and do not like to share patients or be told they cannot go first in an operation when another team needs to go first for the patient's benefit. Open

Statement Hospitals face logistical challenges with collaborative care including billing, clinic scheduling with all doctors present on the same day, and managing surgeon productivity when a 10-hour operation requires a specialist for only 2 hours. Open

Statement When a surgeon is needed for a portion of a long operation, they cannot leave to do other work because they must be available when needed while the patient is under anesthesia. Open

Statement Patients with complex malformations face significant burdens when specialists are in different cities, including scheduling multiple appointments at different times and dates, travel costs, and sibling care logistics. Open

Statement There are clinical circumstances where a child benefits from one operation where tissues can be shared between surgical teams. Open

Statement In a case where a segment of colon was not working and the bladder was too small, a section of colon could be removed from the fecal stream and added to the bladder to augment it, performed simultaneously in one operation. Open

Statement Typically, bladder augmentation would be performed first, and 6 months later someone would perform the colonic work as a separate operation. Open

Statement Implementing collaborative care requires deep infrastructure including coordinators, administrative assistants, schedulers, and multiple types of specialized nurses (floor, operating room, clinic). Open

Statement Nationwide Children's Hospital provided institutional support for the collaborative care model, whereas other institutions where the speaker had attempted this approach did not successfully adopt it. Open

Statement The siloed approach to subspecialty care was the traditional way of practicing medicine. Open

Statement In the collaborative model, 1 + 1 equals 3, meaning the sum is greater than the individual parts. Open

Statement The collaborative care model could be applied to other hospital programs, such as a limb program requiring orthopedic surgery and rehabilitation medicine, where teams meet before the patient arrives and see the patient together on the same day. Open

Quotation In one week, you will be clean and in normal underwear. Open

Quotation because of the collaborative process, we are able to not only make those promises, but we are able to keep them. Open

cincinnati children's hospital medical team in Korle Bu

Quotation We sought out Ghana because we thought Ghana would be an ideal location to create a West African center for colorectal problems. Open

Statement Ghana was selected as an ideal location to create a West African center for colorectal problems because they already had very good expertise and infrastructure to handle complex cases. Open

Quotation We felt that they had already a very good expertise and an infrastructure that they could handle complex cases. Open

Quotation And I had the dream of creating in Ghana a center where children from all of Africa could come for colorectal problems. Open

Statement The vision is to create in Ghana a center where children from all of Africa could come for colorectal problems. Open

Colorectal Quiz: Episode 49 - Collaborating for Kids: Colorectal & Pelvic Solutions (with a Little Help from AI)

Quotation we did something crazy i uploaded an article that jason frischer and i wrote together about the collaborative model in colorectal care published in seminars in pediatric surgery and loaded that article using ai into a podcast format Open

Quotation i was expecting lots of errors and a very computer sounding experience and it wasn't that at all it was a lovely two human sounding voices that were chatting about the article that jason and i wrote Open

Quotation it foreshadowed things the computer read and i put that in quotes the article fully and then knew what was coming later in the article to get us as the listener excited about it Open

Statement NotebookLM creates an expert from only the provided document, not pulling information from external sources like other chatbots. Open

Colorectal Quiz: Episode 43

Quotation It's very important to know the type of malformation, the quality of the sacrum, and the quality of the spine and give the patient's family some estimate of the likelihood that they will or not be continent. Open

Quotation I mean, I have to say, I'm sort of speechless that we're talking about physics with Jason Frischer. Open

Statement Small bowel volvulus around the appendix can occur but is rare, seen in only two or three cases. Open

Statement When doing a neo-Malone, try to orient the channel so the catheter enters into the right colon rather than refluxing into the ileum. Open

Statement When doing plication, pass the tube after each stitch to ensure it passes in the desired direction. Open

Statement Lone Star ring and pins can be placed in the umbilicus to get excellent exposure to visualize a tiny Malone hole. Open

Statement Ultrasound can be used to find the appendix around the umbilicus and needle localize the lumen for access rescue. Open

Statement Appendicitis in a Malone is impossible unless the hole closes, because there is no obstruction in pathophysiology. Open

Quotation It's impossible. Well, unless the hole closes. Unless the hole closes. Open

Statement Do not take out the appendix in a first Crohn's or ARM patient or a kid with spine issues or absent sacrum or spina bifida, as they may need it for future Malone or Mitrofanoff. Open

Statement In South Africa, appendix is never removed as part of laparoscopic appendectomy for appendicitis; it is a United States practice. Open

Quotation No one is obligating you to take out the appendix. And I will tell you, in South Africa, they never take out the appendix as part of a lads. It's just not done. It's a United States thing. Open

Statement Appendix tips should be sent to pathology because neuroendocrine tumors (carcinoids) can be found; one was discovered three months after pathology insisted on receiving specimens. Open

Colorectal Quiz: Episode 40

Statement Anorectal malformation occurs more commonly in females and has a prevalence of about one in 5,000 births. Open

Quotation When you look in and you see an end of the colon and that's all you see, you do not have to do a divided colostomy. That can be the end. That can be your colostomy. Open

Quotation We've talked about this before on some of these rare circumstances. Open

Statement When you look in laparoscopically and see an end of the colon with nothing else visible, you do not have to do a divided colostomy—you can bring out that distal end as your stoma rather than interfering with the blood supply for the distal segment. Open

Statement The only potential benefit of doing a divided colostomy when encountering blind-ending colon is protecting the ultimate rectal repair, but this risks the blood supply to the distal rectum. Open

Quotation I think the smartest thing to do in this very rare case is make a true end colostomy. Open

Statement Meyer-Rokitansky-Küster-Hauser syndrome can present with anorectal malformation, creating Meyer-Rokitansky-like anatomy where ovaries and remnant tubes are present but no midline Müllerian structures. Open

Statement The more common scenario of anorectal malformation with absent vagina is a recto-vestibular fistula with completely normal urethra but no vagina in between—this is called recto-vestibular fistula with distal vaginal atresia, where the rectum ends as a fistula in the vestibule. Open

Statement In this case, the rectum ended blind and quite high in the pelvis, making it unreachable through a posterior sagittal incision. Open

Statement The foreshortened sacrum in this case suggests caudal regression, where everything below that level forgot to develop. Open

Statement Not diverting after limited posterior sagittal anorectoplasty is safe when you have a colocolonic anastomosis at the colostomy closure site and only an anoplasty with a couple of posterior sutures. Open

Statement In the past, vaginal replacement would have been done at the same time as rectal repair in these patients, but time and research have shown that colonic neovaginas are not great for patients 20 years down the road, and surgeons should try very hard to avoid them. Open

Quotation In the past, a vaginal replacement would have been done at the same time as the rectal repair in these patients. However, time and research have shown that colonic neovaginas are not great for patients 20 years down the road. And we should try very hard to avoid them. Open

Statement In most cloacas, you should be able to get the native vagina to reach without needing vaginal replacement. Open

Quotation I can tell you in most cloacas, you should be able to get the native vagina to reach. Open

Statement Vascular anomalies associated with anorectal malformation have not been much written about in the literature. Open

Statement An aberrant external iliac artery can loop up within the abdominal wall, looking very much like the obliterated umbilical artery, while actually being a blood supply to one of the extremities. Open

Quotation I vividly remember a case where we encountered an aberrant external iliac artery that looped up and actually was within the abdominal wall and looked very much like the obliterated umbilical artery and in fact was a blood supply to one of the extremities. Open

Colorectal Quiz Episode 32: Anorectal Malformations And Cardiac Anomalies

Quotation there is an anal dimple. That means... A raised area and a good change in color. Which means there's probably a good sphincter Open

Statement An anal dimple with raised area and good color change indicates there is probably a good sphincter. Open

Quotation I don't stand in the way when the NICU gets an echo. I routinely get them all Open

Statement Dilation and sending baby home is a suboptimal choice in a baby with no cardiac defect, but might be a good choice in a baby you don't want to take to the OR. Open

Statement If you could dilate a perineal fistula patient, you don't need to go to the OR at all and can let them deal with the heart. Open

Statement In a male perineal fistula, the hole isn't always easy to see and dilation is more dangerous because it's near the urethra, but with care and Hagar dilators you can get egressive stool and never go to the OR. Open

Quotation There's no rush on a vestibular. There's no rush on a perineal Open

Statement There is no rush on a vestibular fistula in a female patient or a perineal fistula, allowing time to address cardiac issues first. Open

Quotation I actually don't think there's a need to do a colostomy in a baby like this. You can dilate and then do the repair primarily later Open

Statement Colostomy is not any more or less risky than a one-hour mini-PSARP for perineal fistula. Open

Statement There is no need to do a colostomy in a baby with perineal fistula and cardiac disease; you can dilate and then do the repair primarily later. Open

Statement A turnable loop ostomy with 95-5 percentage behaves like an end colostomy, and no one knows except the surgeon that there's another side where you can do a contrast study. Open

Quotation Sort of a 95-5 percentage loop. It behaves like an end. And no one knows except for you that there's another side there you can do a contrast Open

Statement If the baby has an umbilical line, consider going into Palmer's Point instead of accessing through the umbilicus, using a Hasson technique. Open

Statement Important factors for continence include sensation in the anal canal, absence of the dentate line, quality of the spine, type of anorectal malformation, and sacral anatomy. Open

Quotation This baby has a low lesion. So, basically, just had a closely approximated perineal fistula to the anal muscular complex. So, should do really well Open

Statement A baby with a low ARM lesion (closely approximated perineal fistula to anal muscular complex) should do really well with continence. Open

Statement Sacral ratio measurement should wait until 3 months of age. Open

Quotation It's one millimeter deep. Do not dive in and try to find that fistula's tract. It will disappear, provided you have a good anoplasty with a good anterior rectal wall mobilization Open

Statement The perineal fistula tract is only one millimeter deep; do not dive in to find it as it will disappear with good anoplasty and anterior rectal wall mobilization. Open

Statement If the fistula is completely outside of the sphincteric ellipse, then full mobilization is required. Open

Colorectal Quiz Episode 29: Female ARM

Statement Dr. Levitt's current protocol is regular IV (no PICC line) and clear liquids or breast milk for five days, based on better healing by day five compared to day one or two. Open

Statement A study by Carlos Reck comparing NPO for seven days versus clear liquids for seven days found the same amount of stool output in both groups. Open

Statement The problem is not pooping itself but hard pooping that can disrupt the perineal body repair. Open

Statement Alberto Pena's historical protocol kept patients mandatory NPO for seven days with central line and hyperalimentation, feeding on day seven if healed. Open

Quotation I think the key to this will be as minimal amount of perineal body injury by surgery as possible. Open

Statement In redo cases seen by Dr. Levitt, patients were invariably fed right away and discharged home. Open

Statement Perineal body dehiscence usually leads to no perineal body over several months and requires redo surgery because the anterior anoplasty has no sphincter around it. Open

Statement A paper by Dr. Levitt found that clear liquids were no different than NPO in terms of stool output, but both groups still produced very thin, liquidy stool that would not disrupt the anastomosis. Open

Statement Many anoplasties that were never touched with a dilator look absolutely fine eight weeks later at colostomy closure if the repair was healthy with no tension and good blood supply. Open

Statement The dilation RCT was prompted by families identifying dilations as their biggest concern in caring for patients with anorectal malformation. Open

Statement A randomized controlled trial by Richard Wood and Dr. Levitt compared dilation versus non-dilation for primary PSARP (cloacas excluded), with backup plan of dilation plus or minus Heineke-Mikulicz anoplasty for strictures. Open

Statement The dilation RCT found that both dilated and non-dilated groups developed strictures somewhere between 10 and 20% of the time. Open

Statement Jack Langer's routine is to see patients every week in clinic and pass a dilator without having families do it at home. Open

Statement In the dilation RCT, four patients required redo operations for stricture: two in the dilation arm (both chose not to do dilations) and two in the non-dilation arm. Open

Statement Dr. Levitt has yet to meet a family that has chosen dilation when presented with the option of non-dilation with selective intervention. Open

Statement There is existing data showing that full continence can be restored with a redo operation, including for stricture as an indication. Open

Quotation I think the good news is that there's no right answer. We just have to all work together and suffer together on these difficult problems and try to improve lives as best we can. Open

Statement In primary repairs, Dr. Levitt makes the lumen match what the maximal rectal lumen can be, filling the sphincter, which is usually about a 13 or 14 Hegar size at the end. Open

Statement In redo cases, Dr. Levitt makes the anoplasty a little bigger knowing there will be contraction, and does not dilate redos but performs EUA at one month to check for early stricture. Open

Statement A surgeon in Ghana taught Dr. Levitt to make anoplasties a little bigger in cases where patients will not return for follow-up, knowing there will be some contraction. Open

Colorectal Quiz: Episode 2

Quotation How many patients, Jason, have you seen on laxatives that comes with an anus in the wrong place? How many patients have you seen having had a cecostomy with perfect anatomy? Open

Statement The original malformation in case 1 was a prostatic fistula and the patient has a tethered cord with a sacral ratio of 0.66 Open

Statement Sacral ratio 0.7 or greater usually means normal sphincters and good muscle tone Open

Statement The higher the malformation, the worse the prognosis for bowel control Open

Quotation the family doesn't really care how technically elegant is your analplasty. What they care about is whether that analplasty that you make is going to work, and is the child going to be clean and in normal underwear, and just like all the other kids. Open

Statement Patients with myelomeningocele have much more trouble with continence than those with tethered cord Open

Quotation a key pitfall is not do that. Mark the sphincters first, then open the PSARP, because then you don't get confused at the end when you're trying to place the analplasty in the correct location. Open

Statement A key pitfall is opening the PSARP incision first; instead, mark the sphincters first with electrical stimulation, then open the PSARP Open

Quotation I'm looking for the anal dimple. And there's like a midline where I say, and there seems to be a little raised area, which is where the sphincters are and where the analplasty ought to be. Open

Statement Anesthesiologists should not give skeletal muscle relaxant when using the stimulator because it is weaker than traditional stimulators Open

Statement The electrical stimulator used for sphincter mapping is the same one anesthesia uses for train of four Open

Statement For a two-year-old with mislocated anus or bad prolapse, offer redo and let them live in diapers for a year or two with better anatomy before potty training Open

Statement After redo with Malone, patients learn to get control with new anatomy before stopping Malone flushes and trying voluntary bowel movements; this process may take six to twelve months Open

Statement If anatomy is off, redo should be done, and there is an advantage to getting anatomy right when the child is younger Open

Statement For patients presenting after potty training age with incontinence due to mislocated anus, do the redo and usually add a Malone at the same time Open

Statement For a patient with anus 50% within sphincter complex at age three and a half with incontinence, one approach is to redo and add Malone, get them clean mechanically, then see if they develop bowel control Open

Statement If patients haven't declared continence yet because they're not old enough behaviorally, give them a chance as they may succeed with current anatomy Open

The Colorectal Quiz: Episode 1

Statement VACTERL workup for anorectal malformations includes: V (vertebral abnormalities via plain x-ray), A (anorectal malformations), C (cardiac abnormalities via exam and echo), E (esophageal atresia via NG tube passage), R (renal abnormalities via kidney ultrasound), and L (limb abnormalities via physical exam) Open

Statement Sacral ratio should be measured at three months of age for true measurement, though early measurement gives a feel for how normally the pelvis has developed Open

Quotation I always worry about a missed pre-sacral mass. Open

Quotation V, vertebral abnormalities. Plain x-ray of the abdomen tells you about the spine, make sure there's no hemivertebra. A is anorectal malformations, which is why we're having this podcast. And then C, cardiac abnormalities. We want to make sure we check for any cardiac defect, both on exam and on echo. E is esophageal atresia. So they ought to get an NG2 pass. R for renal abnormalities. So they need a kidney ultrasound. And then L, limb abnormalities. Open

Quotation it's important that if this baby was born today, we're not rushing to do anything. Because we need time to have this baby declare themselves to either be someone who's going to need a colostomy or someone who might be able to benefit from a primary repair, a meconium, if a perineal fistula bubbles up. Open

Quotation I bet this kid is going to have a pretty normal sacral ratio, certainly greater than 0.7, which connotes a very good prognosis for bowel control and really peace of mind for the family. They want to know what's going to happen to this baby in four years when they need to potty train and go to school. Open

Statement Sacral ratio greater than 0.7 connotes very good prognosis for bowel control and provides peace of mind for families regarding potty training at age four Open

Quotation I think the key to deciding whether to dive into a perineon posterior sagittally is where is the rectum? You want to know what structure you will find if you make a posterior sagittal or even a mini posterior sagittal incision. And there is no question in my mind that if I open posterior sagittally here, the first structure I would find would be rectum. Because the danger is that you go in posterior sagittally, you don't know where the rectum is, and you find something midline and white, like the urethra or the bladder neck or the bladder itself. And that's why we do colostomies. And that's why we do distal colostograms. So we know exactly where the rectum is, and we know whether we should approach it perineally or whether we should do it laparoscopically. Open

Statement Colostomies are done to know exactly where the rectum is via distal colostogram and to determine whether to approach perineally or laparoscopically Open

Statement The key to deciding whether to approach perineally via posterior sagittal is knowing where the rectum is—must be confident the first structure encountered will be rectum, not urethra, bladder neck, or bladder Open

Statement Colostomy is the safe choice and was the right decision in this case, though it carries its own complications including those from colostomy closure Open

Quotation I will tell you that the surgeon who sent me this case, they did not do that. They felt more comfortable doing a colostomy. And I want to tell you, that was the safe thing to do. Bravo to them. That was the right choice. Nothing wrong with that at all. But of course, we are giving the child a colostomy. And whatever complications can happen from that, and we are giving the child a colostomy closure and whatever complications can happen from that. So everything in medicine is a balance, but there is no question that the anal part has been made safer by having a colostomy. Open

Statement The anal repair is made safer by having a colostomy, though everything in medicine is a balance Open

Statement Very good surgeons have done beautiful anoplasties but ignored fistulas, resulting in children urinating out their anus postoperatively Open

Quotation if you went in and grabbed this rectum and did a primary analplasty and did not know there was a fistula, and unfortunately, I've seen this done by some very good surgeons where they went in, did a beautiful analplasty, but ignored the fistula. And the child down the road started peeing out their anus. Open

Statement During primary posterior sagittal approach, open the posterior wall of rectum and inspect the anterior wall to rule out fistula Open

Quotation when I do that, I open up the posterior wall of the rectum, and I inspect the anterior wall of the rectum. Open

Quotation 95% of Downs patients have no fistula, but 5% do. So I would still do a distal colostogram and some of them might have a fistula. Open

Statement Distal colostogram should still be performed in Down syndrome patients with imperforate anus despite 95% having no fistula Open

Statement 95% of Down syndrome patients with imperforate anus have no fistula, but 5% do have a fistula Open

Anorectal Malformations Complications

Statement Perineal fistulas in males are commonly missed in the newborn period because the baby passes meconium through the small fistulous opening, and no one notices the abnormal anal anatomy. These patients typically present in the first year of life with severe constipation. Open

Quotation You'd be surprised how common these malformations are not properly diagnosed or completely missed in the newborn period. Open

Statement By the time a missed perineal fistula is diagnosed, the rectum and sigmoid have dilated because stool has been passing through a very tiny fistulous orifice which is not normal anal or rectal mucosa. Open

Quotation Nowadays, the standard is to not even check a rectal temperature, is to check a temperature on the forehead or in the ear, and therefore, you don't have to look. If you don't look, you might not know. Open

Statement The current standard is to check temperature on the forehead or in the ear rather than rectally, so if you don't look at the anus, you might not know there is a malformation. Open

Statement Relocating a perineal fistula into the sphincters does not completely fix the constipation, though it improves the anatomy by making the hole adequately sized and lined by mucosa. Open

Statement Patients with uncorrected perineal fistulas can have some semblance of continence with formed stool, but with loose stool or athletic activity they will soil because they cannot completely close the anteriorly located hole when squeezing their sphincters. Open

Statement A newborn anus should accept a size 12 Hagar dilator, and a one-year-old should accept a size 15. Open

Statement A bucket handle (a lifted skin tag that you can pass a probe underneath) is consistent with a perineal fistula even if you cannot see the fistula itself. Open

Statement Perineal fistula in females is probably the most confounding diagnosis in pediatric colorectal surgery, with many patients either being missed or overdiagnosed. Open

Statement Diagnostic criteria for perineal fistula in females: inadequate perineal body (hole too close to vagina), inadequate hole size, and hole not centered in the sphincter. Open

Statement If the anal opening in a female is adequate size and centered in the sphincter, even if it appears slightly anterior with a short perineal body, that patient does not need surgery. The perineal body will lengthen with growth. Open

Statement An anesthesia nerve stimulator ($150) with appropriate needle probes works as well as a dedicated Pena stimulator ($15,000) for intraoperative sphincter mapping. Open

Quotation I got no benefit from that $15,000 times two credit, but we use the anesthesia nerve stimulator. Open

Statement The vast majority of male ARM patients have a rectourethral fistula. It is important not to approach these primarily because you don't know where the rectum is—it could be at bladder neck, prostatic, or bulbar level. Open

Statement If you open posterior sagittal looking for a rectourethral fistula without knowing the location, you will find something midline, white, and shiny that might be the urinary tract, not the rectum. Open

Statement Cloacas can be missed in the newborn period. Dr. Levitt saw a six-month-old who presented with constipation and was found to have an undiagnosed cloaca with no hint of an anal opening. Open

Quotation Believe it or not, that has happened too. I just, in fact, last week saw a six-month-old who presented at age six months with constipation, and someone finally looked and saw there was no anus. Open

Statement Ambiguous genitalia (clitoromegaly from endocrine stimulation) presents with a urogenital sinus but a completely normal anus. This is different from a cloaca, which has no anus and no endocrine problem. Open

Statement The most common colostomy error is opening too distal in the sigmoid, which restricts the ultimate pull-through by the location of the colostomy or mucous fistula. Open

Statement Incompletely diverting loop colostomies allow stool to spill across and contaminate the distal segment, leading to urinary tract infections. Open

Statement Transverse colostomies are problematic because they can prolapse, and if there is a large rectourethral fistula, the left colon absorbs all the urine (which doesn't come out the mucous fistula), causing acidosis. Open

Statement Dr. Levitt's preference is a very proximal sigmoid colostomy with separated stomas, leaving the entire sigmoid loop for the pull-through. He makes the mucous fistula very tiny and flat. Open

Statement Prolapse is related to where in the colon you choose to do the colostomy. Mid-transverse: both sides can prolapse. Hepatic flexure: only distal can prolapse. Proximal sigmoid: only distal (mucous fistula) can prolapse because left colon is fixed to retroperitoneum. Open

Quotation I actually mark the anoplasty before I make the incision. And I think you can get lost when you're looking at a bunch of jumping muscles from a stimulator. Open

Statement Dr. Levitt marks the anoplasty location by drawing a circle around the pinkish ellipse where it stimulates on the skin surface BEFORE making the incision, to avoid getting lost when looking at jumping muscles from the stimulator. Open

Quotation I'm sure that's what's happened because really, really good surgeons have put anuses in crazy places. And I think it's because they don't have a sense of what's the center because everything's disrupted once it's open. Open

Statement Really good surgeons have put anuses in crazy places because they don't have a sense of what's the center once everything is disrupted and open. Open

Statement The distal colostogram is an absolutely vital study. Many mistakes are made because of a poorly done study and misinterpretation. Open

Quotation The distal colostagram is really an absolutely vital study. And a lot of mistakes are made because of a poorly done study, first of all, and then a misinterpretation of that study. Open

Statement The basic questions the distal colostogram must answer: Where is the rectum? How low is it? Is it reachable posterior sagittally or better approached laparoscopically? What is its relationship to the urinary tract? Open

Statement The common colostogram mistake is not giving enough contrast and pressure into the distal segment, giving a false impression that the rectum is high or that there is no fistula. Open

Quotation If you see that flattening of the rectum, you know the radiologist did not give enough contrast, enough pressure. Because you need to overcome the PC line because that's the sphincters. Open

Statement If you see a straight line flattening of the rectum corresponding to the pubococcygeal line, the radiologist did not give enough contrast or pressure. You need to overcome the PC line (the sphincters compressing the distal rectum) to see the bulging rectum and fistula. Open

Statement Fistula classification: if the fistula is at the urethral 'elbow' or below, it's bulbar. Above the elbow is prostatic. At the bladder neck is bladder neck fistula. Open

Statement If the rectum is bulbous, it might be reachable posterior sagittally and hard to do laparoscopically because of the girth. If it's tapered, you're better off laparoscopically. Open

Statement If you don't know where the rectum is and open posterior sagittal, you will find a whitish, shiny structure and may think it's the rectum. Often it's the bladder neck. Open

Statement You avoid bladder neck injury by knowing exactly where the rectum is from a properly done distal colostogram. When you open posterior sagittal, you know the rectum is right under the coccyx (prostatic) or distal to the coccyx (bulbar), or it isn't posterior sagittal at all (bladder neck—do laparoscopy). Open

Quotation You should never go to the operating room without knowing exactly what anatomy you're going to expect to find. And the big question is, where is the rectum? Is it the most posterior structure? And where is it the most posterior structure? Open

Statement Dr. Levitt will do posterior sagittal for bulbar fistulas and low prostatic fistulas with a bulge. High prostatic with tapered rectum and bladder neck fistulas are best served by laparoscopy. Open

Statement If you try laparoscopy for a rectum bulging below the peritoneal reflection at low prostatic or bulbar level, you may leave behind a remnant of the original fistula (ROOF)—the distal rectum left behind that causes trouble later. Open

Quotation I never look at it that way. I think that laparoscopy replaces laparotomy. It's an elegant dissection from above. But don't give away the advantages of the PSARP. Open

Statement Laparoscopy replaces laparotomy, not PSARP. Dr. Levitt does a mini-PSARP when doing laparoscopy to safely enter the pelvis and tack the rectum to the posterior edge of the muscle complex to avoid prolapse. Open

Statement Dr. Levitt calls his approach 'laparoscopic-assisted PSARP' rather than pure laparoscopy. Open

Quotation I like to call mine a laparoscopic-assisted PSARP. I think that's a better terminology. Open

Statement Prolapse prevention: put the rectum in the right location, close the levators properly, close the posterior wall to the posterior edge of the muscle complex for 3-4 stitches, don't dissect the rectum more than necessary. Open

Statement Prolapse occurs in about 3% of cases, particularly in those without great muscles. Open

Statement Rectal prolapse causes bleeding, mucus, and for patients with good continence potential, it inhibits bowel control because they can't close the opening with prolapsed tissue through it. Open

Statement Dr. Levitt trims prolapse of more than about 3mm. For circumferential prolapse, he does half the circumference in two different ambulatory settings so families don't need hospitalization and the patient doesn't need dilation (half the circumference is untouched so they won't stricture). Open

Statement Perineal body dehiscence is the most common cause of reoperation Dr. Levitt performs in female ARM repairs. Open

Quotation Perineal body dehiscence is the most common cause of reoperation that I do is a female repair in which the perineal body dehiscs. Open

Statement The key to preventing perineal body dehiscence is mobilizing the rectum well—you must get the anterior rectal wall completely separated from the posterior vaginal wall to the areolar plane. If you don't, the anoplasty will be under tension and can pull back, leak into the perineal body space, and dehisce. Open

Statement Dr. Levitt uses 3-0 suture for perineal body closure in a baby and 4-0 Vicryl on the perineal skin, then watches the perineum very closely. Open

Statement Traditionally Dr. Levitt kept patients NPO for 7 days on 10% dextrose after female ARM repair. Recently he has been trialing clear liquids only for a week because the major problem is hard stool—clear liquids won't make hard stool. Open

Quotation If you see it's opening, I actually will take... And this happens maybe one or two cases out of about 200, I can tell you. That's not insignificant. I will take them back to the OR and re-suture the perineal body. Open

Statement If perineal body dehiscence is detected on days 5-8, Dr. Levitt will take the patient back to the OR and re-suture the perineal body, which can salvage the situation. This happens in maybe 1-2 cases out of about 200. Open

Statement Laparoscopy causes trouble if you try to dissect a rectum that's too low—you get too close to the urinary tract or you're too timid and leave behind the distal rectum (remnant of original fistula). Open

Statement For high rectums, particularly bladder neck fistulas, the dissection of the distal rectum is quite challenging to make it reach with good blood supply. You must preserve the IMA because the colostomy may have disrupted collaterals down the left colic, making the rectum completely dependent on the IMA. Open

Statement The rectum has an excellent intramural blood supply from the IMA. If you take the IMA or take branches too close to the aorta, the rectum will die because there's no collateralization down the left colic. Open

Statement The biggest problem with posterior sagittal incision is going after a rectum when you don't know where it is. You open and find the bladder neck, urethra, seminal vesicles, vas deferens, ectopic ureter—everything but the distal rectum. Open

Quotation There are some famous cases of pull through bladder neck made into beautiful anoplasties. And post-op, the patient was draining liquid out their anoplasty. And in fact, it was the bladder neck. Open

Statement There are famous cases of pull-through of bladder neck made into beautiful anoplasties, and post-op the patient was draining liquid out their anoplasty—it was the bladder neck. Open

Statement To determine if an ARM patient has potential for bowel control, Dr. Levitt looks at three factors: original type of malformation, quality of sacrum and calculated sacral ratio, and quality of spine. He calls this the ARM continence index. Open

Statement Three A's (excellent malformation type, sacrum, and spine) predicts a continent patient. Three C's predicts an incontinent patient. Dr. Levitt's group is working on quantifying the in-between grades. Open

Statement A bulbar fistula with a good sacrum (sacral ratio of 1) and normal spine should absolutely have bowel control. A bladder neck fistula with poor sacrum (sacral ratio 0.4) and tethered cord or myelomeningocele has no real chance of good bowel control. Open

Statement For a soiling 4-year-old ARM patient, Dr. Levitt's first step is to get them clean mechanically with bowel management using enemas. For those with continence potential, when they're older and more mature, he tries to switch them to laxatives to achieve voluntary bowel movements. Open

Statement Indications for redo pull-through: any patient with potential for bowel control whose anatomy is not perfect—improperly located anus, anal stricture, rectal prolapse, or remnant of original fistula (posterior urethral diverticulum). Open

Statement One of the biggest problems with ARM is that if you don't get it right, you don't know for a few years. Most surgical problems become obvious immediately, but with ARM, patients may not present with soiling until age 4, making it hard for surgeons to learn what to fix about their technique. Open

Quotation Most things in surgery, if you don't do it right, you know right away. Like, if you don't sew a hepatic artery together properly during a liver transplant, the next day, you have a thrombosed artery. If you don't put an anus in the right place, you think you did a perfectly fine operation, the patient goes home, everyone's happy, and only four years later do they come soiling. Open

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