Spontaneous Rupture of Extrahepatic Choledochal Cyst: Two Pediatric Cases and Literature Review

Abstract

Spontaneous rupture of extrahepatic choledochal cysts is very rare. Neonatal cases generally present with biliary ascites, and older children with acute abdomen. Although the cause is unclear, accumulation of protein plugs in an anomalous pancreaticobiliary junction, irritation of the cyst wall due to reflux of pancreatic juice, and weakness due to a developmental error such as common channel syndrome have all been suggested to play contributing roles. The treatment of choice is complete excision of the cyst and hepatico-jejunostomy. In newborns who are in a poor condition, the primary treatment should be simple peritoneal drainage.

Traumatic Rupture of Choledochal Cyst in Children

“…7 A spontaneous rupture of a CC accounts for 1.8% to 18% of all CCs in children. 8 Traumatic rupture is the rarest clinical manifestation of a CC, and only 8 cases have been reported in English literature. 6 Among these cases, only 2 were reported in children.…”

Spontaneous perforation of the bile duct in a neonate: Drainage or resection?

“…2 Although rare, spontaneous perforation of the bile duct is the third most common cause of surgical jaundice in infants, after biliary atresia and choledochal cyst. 1,6 Unlike the aforementioned diseases, spontaneous perforation of the bile duct can deteriorate and cause death due to severe sepsis developing in a few days or even hours.…”

“…1e3 The etiology is unknown, but there are many possible causes, such as congenital mural weakness of the common bile duct, relative ischemia of its anterior wall due to posterolateral arterial supply of the bile duct, and anatomic anomalies (such as cyst, diverticulum, and distal biliary obstruction). 2,4 Diagnosis is usually confirmed with surgical exploration, and good prognosis depends on early surgical intervention. 1,2 We report on a 10-day-old female neonate with spontaneous perforation and nearly total transection of the bile duct at the junction of the cystic duct and common hepatic duct, whose condition was managed by cholecystectomy and biliary reconstruction.…”

Bilome géant compliquant une angiocholite lithiasique

“…Chez le nourrisson et chez l'enfant, les ruptures « spontanées » de l'arbre biliaire sont plus fréquentes mais de présentation clinique totalement différente (la constitution d'un bilome n'est pas la règle). Elles sont en rapport avec une perforation de la portion extrahépatique de l'arbre biliaire, siège d'une malformation kystique, souvent à la jonction de la VBP et du canal cystique [7,8]. La multiplicité des localisations des bilomes est remarquable et s'explique par le siège de la perforation de l'arbre biliaire et par la disposition des ligaments périhépatiques [2,9].…”