Approaches to Biliary Atresia Care at Cincinnati Children's - Greg Tiao, MD - Controversies in the surgical management of children with biliary atresia
With Dr. Greg Tiao
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What the experts said
Biliary atresia was first recognized in the 1890s, with Dr. Holmes coining the term and Dr. Ladd describing a correctable form.
Dr. Kasai's procedure in the 1950s revolutionized biliary atresia treatment by giving children a chance at survival, whereas prior to his procedure all affected children developed progressive liver failure and died.
Liver transplantation evolved from experimental in the 1960s-1970s to a durable standard treatment by the 1980s.
Biliary atresia is currently a surgical disease with surgery (Kasai and liver transplant) being the only way to provide durable cure.
Both Kasai and liver transplant are crude interventions that give children longer life but have long-term complications.
Dr. Kasai's original procedure was described as a Roux-en-Y mucosa to hilar plate anastomosis.
Various modifications of the Kasai procedure including diverting stomas, refluxing valves, and use of patent gallbladder have been abandoned in favor of Kasai's original technique.
Cincinnati's Kasai technique is based on what Dr. Ryckman and Dr. Ballesteria learned training with Dr. Kasai in Sendai in the 1990s.
The Kasai procedure involves identifying the gallbladder as a handle and dissecting to the portal bifurcation to identify the fibrous remnant in standard biliary atresia.
Dr. Neo's 2016 paper reviewing Japanese Biliary Atresia Research Consortium data found that transection at the current dissection plane (at the break point between liver parenchyma and fibrous plate) achieves 80%+ drainage success rates.
Extended dissection into liver parenchyma showed slightly decreased drainage rates in the high 70s compared to transection at the fibrous plate-parenchyma junction.
Cincinnati's approach leaves a small amount of fibrous remnant rather than exposing actual parenchyma, providing structure for remnant micro-ductules to drain more durably.
Some North American teams perform hilar dissection extending into the liver parenchyma itself.
Cincinnati typically has two faculty members come into the operating room to agree on the proper plane of hilar dissection during Kasai procedures.
Patients with polysplenia and preduodenal portal vein (biliary-splenic malformation group) often have no hilar plate, requiring abandonment of Kasai procedure.
Cystic biliary atresia (Ladd's correctable variant) is increasingly detected in utero as a cyst in the liver hilum.
Dr. Davenport's work demonstrated that surgical intervention before 30 days of age in cystic biliary atresia patients results in more durable drainage.
Cystic biliary atresia patients are operated on before 30 days of age despite smaller bowel making the procedure more hazardous.
Cincinnati resects all cystic remnant and anastomoses the Roux limb to the hilar plate rather than to the cyst wall, based on Davenport's work showing cyst wall anastomosis is less effective for drainage.
Cincinnati pathology study of approximately 10 cystic biliary atresia patients found no epithelium in the cyst wall, supporting the rationale for not anastomosing to the cyst.
Japanese colleagues found some cystic biliary atresia patients have epithelium in the cyst wall, and those with 1mm visible left and right ducts on cholangiogram can achieve successful drainage with cyst wall anastomosis.
European data demonstrated that centers performing more than 5 Kasai procedures per year had better outcomes, showing that operative surgeon experience matters.
Earlier age at Kasai intervention correlates with better outcomes.
Cincinnati study of 227 biliary atresia patients included 26 who underwent Kasai revision, of which 77% had initially successful Kasai procedures.
Patients who underwent Kasai revision were able to live longer with their native liver.
Indications for Kasai revision include recurrent cholangitis in patients who initially had successful drainage, with the rationale being a blind loop in the Roux limb that can be shortened.
Kasai revision involves opening the Roux limb and scraping/debriding the hilar plate surface to remove inflammatory peel, not re-cutting the hilar plate.
Cincinnati does not offer Kasai revision for patients who had failed initial Kasai (never achieved drainage), only for those with initially successful Kasai who developed recurrent jaundice and cholangitis.
Biliary atresia remains the number one indication for liver transplantation.
Biliary atresia led to technical advances in liver transplantation.
Indications for transplant evaluation include failed Kasai that never drained, or successful Kasai with progressive fibrosis leading to complications including recurrent cholangitis, portal hypertension, variceal bleeding, hepatopulmonary syndrome, and growth failure.
Dr. Starzl first suggested primary transplant for biliary atresia in 1986.
In the 1990s, donor shortage and significant morbidity/mortality in smaller infants, combined with some patients achieving drainage with Kasai, led to acceptance of Kasai-first followed by transplant approach.
Cincinnati considers not offering Kasai for late-presenting patients with significant ascites on ultrasound and bridging fibrosis on liver biopsy.
Age is relative for Kasai candidacy—Cincinnati has performed successful Kasai procedures up to 120 days of age, and has found patients at 50 days with micronodular cirrhosis, ascites, and varices requiring transplant instead.
Five-year survival after liver transplantation for biliary atresia is now 90%+ according to data from the transplant registry.
California registry study of approximately 500 patients over 20 years found that prior to 2002, primary liver transplant had slightly better outcomes than transplant as salvage after failed Kasai.
Rationale for primary transplantation includes one less major procedure, less blood loss, and lower risk of bowel injury due to absence of vascularized adhesions from prior Kasai.
Vicky Eng's study of the SPLIT consortium found that only 30% of pediatric transplant recipients at 10 years had the ideal profile of stable allograft, monotherapy immunosuppression, normal growth, and no immunosuppression-induced sequelae.
Cincinnati continues the approach of Kasai followed by transplant rather than primary transplantation.
Approximately 30% of Kasai patients will be successful and avoid transplant into adolescence, adulthood, or longer, with another subset going on to transplant, resulting in overall survival of 80-90%.
Despite extensive research efforts, the field has not dramatically changed biliary atresia outcomes since the 1990s.
Performing Kasai in a 24-25 day old infant is a very different experience than in a 5-7 week old child because the bowel is much smaller.
Cincinnati typically performs two-layer anastomosis for the Roux-en-Y jejunojejunostomy, but uses single-layer technique in smaller infants, which adds more risk.
Jejunojejunostomy or Roux limb complications will make Kasai unlikely to drain.
Cincinnati aims to perform Kasai before 45 days of age, though this doesn't always happen—the most recent case was an 86-day-old patient.
Biliary atresia livers undergo an inflammatory process making them firm enough to work with even in very young infants.
A successful Kasai is defined as achieving direct bilirubin under 2 at 90 days post-procedure.
A 4-month-old with direct bilirubin of 6 two months after Kasai represents an unsuccessful/failed Kasai and would not be offered revision.
A 2-year-old who had normalized bilirubin after Kasai but developed acholic stools after cholangitis is the ideal candidate for Kasai revision.
Cholangitis can trigger an inflammatory response that sometimes does not resolve with antibiotics and short-course steroids, necessitating consideration of Kasai revision if acholic stools persist.
Patients with pigmented stools and progressive fibrosis/portal hypertension would not benefit from Kasai revision as they have ongoing liver disease rather than drainage obstruction.
Teenagers with recurrent cholangitis and significant portal hypertension represent a gray zone for Kasai revision—Cincinnati has offered it in some cases, but significant varices make the procedure less pleasant and durability is uncertain given existing liver disease.
Cincinnati typically uses a 30-35 cm Roux limb for Kasai procedures.
For patients with short gut, Cincinnati has used gallbladder, appendix, or very short Roux limbs for Kasai procedures.
Cincinnati does not use valves in the Roux limb as they don't make a difference and can cause stasis.
Good drainage in the Roux limb is key to successful Kasai, supporting use of 30-35 cm length.
The depth of hilar plate transection is based more on expert opinion than data, as there are no data within the Children's Network showing differences in outcomes between surgical teams that go into the liver versus those that don't.
Some teams are using ICG (indocyanine green) to identify ductular drainage at branching points where bile ducts typically arise, though Cincinnati's experience has not found it helpful yet.
ICG use requires careful dosing because too much ICG causes everything to turn green, obscuring useful information.