Biliary Disease in Infants and Children

Abstract

In judicandus ye are a physician, in curando, a surgeon. The patient asks for cure -surgery -and not for theory -medicine -it is the doctor who needs the latter. That is: there can be no surgeon who is not also a physician; the latter begets the surgeon and the surgeon tests the physician by the result of his work. Paracelsus O B J EC T I V E S• Become familiar with the diagnostic approach to the child with jaundice • Understand the pathogenesis and treatment strategies used in children with biliary atresia • Classify and discuss the treatment strategies in children with choledochal cysts • List the varying types of progressive familial intrahepatic cholestasis (PFIC)

Massive hydrops of the gallbladder mimicking a choledochal cyst

“…Acute hydrops of the gallbladder has been described in association with Kawasaki syndrome (KS) in 3% to 13% of patients [10]. The gastrointestinal symptoms in KS also include abdominal pain and cholestatic jaundice, which may also represent an atypical and confusing onset of the syndrome [4]. Our patient, apart from the abdominal and hepatobiliary symptoms, and remitting fever, did not have or develop other clinical features of either classical or atypical/ incomplete Kawasaki syndrome.…”

“…In AHGB, the acute distension occurs most often secondary to a preceding illness or to a focus of infection elsewhere in the body [4]. The pathogenesis is unknown, but various mechanisms are thought to be involved including transient and self-limiting obstruction of the cystic duct.…”

“…The most common symptoms and signs of AHGB unrelated to KS are abdominal pain, nausea, vomiting, right upper abdominal tenderness, and/or right upper abdominal mass which closely overlap those findings noted in choledochal cyst in older children. Acute hydrops of the gallbladder may accompany or follow upper respiratory tract staphylococcal or streptococcal infection, with associated toxin production [4]. Less frequently, a number of other infections (leptospirosis, pseudomonas sepsis, Epstein-Barr virus infection, viral hepatitis, salmonella enteric fever) may be associated with AHGB.…”

“…Radiologic diagnosis may be difficult as the condition may be mistaken for a choledochal cyst or AHGB [4]. Finally, the association of choledochal cyst and GB hydrops has been described and it was suggested that the choledochal cyst caused biliary stasis, which in turn induced acalculous GB dilation and inflammation [12].…”

“…An unusual pathologic condition termed acute hydrops of the gallbladder (AHGB) is mostly confined to the pediatric age group and is characterized by acute massive distension of the gallbladder (GB) in the absence of stones, bacteria, or congenital malformations. This acute distension often occurs in conjunction with a systemic illness and/or an infectious focus elsewhere in the body and is becoming more commonly recognized and diagnosed in pediatric practice [3,4]. Here we describe a febrile, cholestatic child affected by AHGB that mimicked a choledochal cyst both clinically and on ultrasound (US) examination and discuss the differential diagnosis of the 2 entities.…”