Surgical Treatment of Biliary Tract Malformations in Newborns

Abstract

The most common biliary malformations in newborn requiring early surgical treatment are represented by biliary atresia (BA), congenital bile duct dilatation (CBDD), and solitary liver cysts (SLCs). Biliary atresia (BA) is the first cause of neonatal cholestatic jaundice. The origin of BA still remains unclear; if untreated, it usually leads to exitus within the first 2 years of life; therefore, early diagnosis is mandatory to plan a timely surgery, preferably earlier than 70 days. The Kasai operation can achieve good results in about a half of cases, but even children successfully treated suffered from liver failure, so the majority of these patients need liver transplantation later in life. Actually, the sequential use of Kasai operation and liver transplantation has dramatically increased the survival rate of patients with BA. Congenital bile duct dilatation is a relatively rare malformation of the pancreatobiliary system; the etiology remains essentially unclear. The cyst usually occurs with asymptomatic jaundice that may be mild and intermittent or progressive due to complete biliary obstruction. The surgical procedure of choice is total excision. Solitary liver cyst is a benign lesion with unknown etiology. They are often asymptomatic and identified incidentally or antenatally in the third trimester. Intervention is required among children who are symptomatic for a mass effect. On the basis of recent studies and fortieth experience of our Center, we summarize the current status of diagnosis and therapy of these biliary tract malformations.