Spontaneous biliary perforation in a child with features of Ivemark syndrome

Abstract

A case of spontaneous biliary perforation is reported for the following noteworthy aspects: its rare mode of presentation as an acute intestinal obstruction; its presentation in late infancy; and its association with Ivemark syndrome.

Congenital pancreatic cyst with Ivemark II syndrome: a rare case

“…Ivemark II syndrome, also referred to as the renal-pancreatic dysplasia sequence, includes polycystic kidneys, enlarged pancreas, pancreatic cysts, liver anomalies, absent or undeveloped spleen, heart anomalies, dilated bile ducts, and dilated pancreatic ducts. In contrast, the Ivemark I syndrome or asplenia-cardiac anomaly is defined by the association of heart malformation, absent spleen or multiple spleens, and visceral heterotaxia [8,9].…”

“…To date, 29 cases of solitary CPC [1][2][3][4][6][7][8][9][10] have been reported in the literature. It is believed that these cysts are caused by an anomalous development of the pancreatic ductal system, wherein sequestered segments of the primitive secretory ductal system give rise to microscopic or macroscopic cystic lesions [4,5].…”

Rare cause of ascites and jaundice in infancy: Spontaneous bile duct perforation

“…Associated conditions such as Ivemark syndrome, cystic fibrosis, typhoid fever and resolving scarlet fever have been reported so far; however, the association of EBS, CAH and SBDP has not been reported before. 5 Although it is known that generalised EBS rarely affects mucosal surfaces, we hypothesised that the mural weakness secondary to EBS and one of the gallbladder stones passing into the bile duct might have a key role in the association of these two disorders. Necrosis due to increased intraductal pressure secondary to obstruction or direct erosion by stone might have led to perforation.…”

Spontaneous biliary peritonitis: Is bed side diagnosis possible?

“…Nontraumatic perforation of the bile duct is difficult to diagnose because of its non-specific presentation, usually diagnosed intra-operatively 7 , [7,12,13] In most cases of SBDP surgical intervention is performed for diffuse peritonitis or unstable vital signs but none of patients in our series had presented with features of overt peritonitis (wide table 1) because biliary peritonitis is usually a sterile chemical inflammation and hence may not create signs similar to bacterial peritonitis secondary to bowel perforation. [14] Main presenting features in our series were progressive abdominal distension, clinically evident jaundice with failure to thrive and recent change in color of stool which is contrary to the recently published series where most common presenting features were pain [10] and vomiting.…”