Choledochal cysts: lessons from a 20 year experience.

Abstract

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n=44), If (n=28), IVa (n=4), and V (n=2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (30/o) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n= 12), incomplete investigation of abdominal pain (n=6), and failure to note the significance of ultrasonographic findings (n= 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4-1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed. (Arch Dis Child 1995; 73: 528-531)

Two-Dimensional Ultrasonographic Prenatal Diagnosis of Choledochal Cyst: Our Experience and Literature Review

“…In rare cases with a much prolonged course, chronic biliary obstruction is associated with prolonged icterus; evolution towards a secondary biliary cirrhosis and portal hypertension has been reported in rare cases. On the contrary, an incidental diagnosis done early in infancy (or a prenatal diagnosis as in our case) before clinical symptoms appear, is much likely to be associated with the absence of any complication and in particular biliary obstruction or pancreatitis [16,17]. As in our case, asymptomatic anicteric infants should be scheduled for elective surgery even in absence of complications, as evolution towards complication is the rule.…”

Controversies in choledochal malformation

“…However, the incidence is much higher in Asian populations, and a female predominance of around 4:1 is consistently found in most series. [2][3][4] There is no obvious hereditary element in most CMs, though a recent review identified 10 pairs of familial occurrence (siblings and offspring) in the literature. [5] Conversely, six pairs of monozygotic twins have been described in which a distinct CM appeared in only one pair.…”

“…[3] Infants tend to present with obstructive jaundice, and in later childhood and adult life, patients present with recurrent abdominal pain, with or without features of acute pancreatitis (Fig. 3).…”

“…3). [2,3] Certainly, recurrent pancreatitis should be seriously considered as a cause for recurrent abdominal pain in childhood and actively excluded by the measurement of serum amylase or lipase. A value of >3 times the upper normal limit, in association with typical abdominal pain and/ or features of pancreatitis on cross-sectional imaging (the Atlanta criteria), confirms the diagnosis.…”

Endoscopic treatment of choledochal cyst type III

“…They were first described by Vatero and Ezler in 1723 7,11 . The classical clinical symptoms are abdominal pain, jaundice and fever in a pre-school children 8 .…”