Atresia de Vias Biliares - Resumen de la 5ta sesión Nacional de Residentes de Mexico

Biliary atresia is obstruction of the extrahepatic bile duct leading to cholestasis and hepatic fibrosis

Clinical manifestations include late jaundice (after two weeks of life), acholic stools, dark urine, and hepatomegaly

Surgical approach uses laparoscopic technique with specific positioning and prophylaxis

In Mexico and Argentina, low transplant availability necessitates performing Kasai surgery as early as possible, ideally before 120 days of life

A child's prognosis within the first year of life depends on whether they will be transplanted within that year

Laparoscopic approach requires an excellent pediatric laparoscopic surgeon; the technique is relatively simple with magnification but complete porta hepatis resection is complicated

There is no serious scientific publication with rigor demonstrating that laparoscopic approach is superior to open approach in any aspect

Four objectives of Kasai surgery: confirm diagnosis with good operative cholangiography, achieve permanent bile flow free of cholangitis, delay or avoid transplantation (especially important in countries with low transplant availability), and avoid portal vein injury

Key technical points for laparoscopic approach include meticulous dissection identifying hepatic artery and portal vein bifurcation, use of specific retractors or percutaneous sutures for traction, and careful division of vessels to avoid injury

Postoperative management includes antibiotics for at least 10 days IV if coagulation abnormalities present, otherwise oral antibiotics

Steroid protocol: IV methylprednisolone boluses for first 5 days (8-10 mg/kg/day), then oral prednisone starting day 6, tapering from 2 mg/kg/day to 1 mg/kg every other day over several weeks, then discontinuing after Kasai procedure

Cholangitis is the primary prognostic indicator for long-term Kasai success; a meta-analysis from Hong Kong showed repeated episodes of cholangitis are the principal prognostic factor for failure beyond 3 years

Important factors for preventing cholangitis include early clinical diagnosis and treatment with at least 21 days of IV antibiotics for severe infection

Kasai failure is defined as inadequate bile drainage requiring transplant in the first year post-Kasai; if patient deteriorates rapidly and needs transplant in first year, the Kasai has failed

Long-term outcomes: approximately one-third of patients need transplant in first year (Kasai failure), one-third have functioning Kasai allowing growth and delayed transplant, and one-third can live well with Kasai alone without transplant

Biliary atresia is the most common cause of neonatal cholestasis with predominance in females

Intraoperative cholangiography is most concordant when done laparoscopically

Surgery is not recommended if patient is less than 60 days old, as younger patients have better outcomes

Most common long-term complication is portal hypertension, which increases with larger portoenterostomy anastomosis, potentially leading to obstruction

Most experts agree on using high-dose steroids in postoperative management

Transplant is indicated for patients with cirrhosis