SURGICAL MANAGEMENT OF BILIARY ATRESIA
Saing H
Department of Surgery, University of Hong Kong
Medical Centre, Queen Mary Hospital, Hong Kong
Objective: To evaluate the surgical treatment and results of
portoenterostomy (PE) for biliary atresia (BA).
Methods: A review of the literature, together with analysis of the
results of portoenterostomy on 93 patients with BA was performed.
Results: While short-term disappearance of jaundice of approximately 60%
is achieved when PE is performed before 70 days of age, PE performed beyond
90 days can still achieve 30% to 40% clearance of jaundice. The most common complication of
cholangitis may be present in 40% to 60% of patients. Various modifications, including
total or partial diversion of the biliary conduit, have been introduced but
none of these procedures can modify the incidence of cholangitis. Portal hypertension (35% to 75%)
with variceal bleeding (20% to 60%) is the most serious complication even
in jaundice-free survivors.
Injection sclerotherapy can successfully control variceal bleeding
in the majority of cases.
Hypersplenism is also troublesome in long-term survivors and may
lead to alimentary tract bleeding.
Partial splenic embolization is recommended as an effective means of
controlling hypersplenism; this treatment modality avoids overwhelming
infection after splenectomy and encephalopathy after shunt surgery. Following PE, 30% to 50% of
children will have a good long-term survival rate. The remaining 50% to 70% will
require liver transplantation (LTX), most likely within the first two years
of age, and 70% to 80% of BA patients with liver transplants will become
long-term survivors with good physical and mental development.
Conclusions: PE remains the treatment of choice for BA. PE should be complemented by LTX in
indicated patients. LTX should
be the first line of treatment only in BA patients who present very late.