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 progressive hepatic fibrosis

Clinical manifestations include late-onset jaundice (after two weeks of life), choluria, hepatomegaly, and acholic stools

The surgical approach for Kasai portoenterostomy can be performed laparoscopically with antibiotic prophylaxis

In Mexico and Argentina, low rates of organ donation and limited transplant availability make it critical to perform the Kasai procedure as early as possible, ideally before 120 days of age

The prognosis for a child depends on whether they are transplanted within the first year of life versus their survival with the Kasai procedure

Laparoscopic Kasai requires an excellent pediatric laparoscopic surgeon; the technique is relatively simple but the complete dissection of the porta hepatis is complicated due to the need for magnification

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

Four surgical objectives for biliary atresia are: (1) confirm diagnosis with good operative cholangiography, (2) establish permanent bile flow free of cholangitis, (3) delay or avoid transplantation, and (4) preserve the portal vein

Bile flow success is clinically interpreted by clearance of jaundice

Key technical points for laparoscopic Kasai include meticulous dissection, perfect identification of hepatic artery bifurcation and portal vein, 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 intravenously if there are coagulation abnormalities, otherwise oral antibiotics

Steroid protocol: intravenous methylprednisolone boluses for the first 5 days (8-4 mg/kg/day), then oral prednisone starting day 6, tapered over weeks from 5 mg/kg/day to 2 mg/kg/day to 1 mg/kg every other day

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

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

Kasai failure is defined as inadequate bile drainage requiring transplantation in the first year post-Kasai; if the patient deteriorates rapidly after Kasai, transplant in the first year represents a failed Kasai

Approximately one-third of patients will have early Kasai failure, one-third will have functioning Kasai allowing growth and delayed transplant, and one-third can live well long-term with the Kasai without transplant

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

Intraoperative studies, preferably laparoscopic cholangiography, are recommended; if the patient is under 60 days old, Kasai surgery is recommended

The younger the patient at time of Kasai, the greater the possibility of success

The most frequent long-term complication is cholangitis; the more portoenterostomy anastomoses, the greater the risk of stenosis and occlusion

Liver transplantation is indicated for patients with progressive cholestasis despite Kasai