Neonatal Type 1 Choledochal Cyst

TE Herman and MJ Siegel Mallinckrodt Institute of Radiology, St Louis Children’s Hospital Department of Radiology, Washington University School of Medicine, St Louis, MO, USA

Journal of Perinatology (2007) 27, 453–454; doi:10.1038/sj.jp.7211759 dysmorphic features. The baby had a palpable right upper quadrant (RUQ) mass that measured approximately 4–5 cm. Case presentation An abdominal sonogram was performed on the ﬁrst A 3250 g full-term infant girl was born vaginally to a 19-year-old day of life (Figure 1). On the second day of life, the patient gravida 2, para 1 mother after a pregnancy complicated only was taken to the operating room where a RUQ cystogram by the discovery of an abdominal cyst at fetal sonography, was performed under laparoscopic control (Figure 2). The believed to be an ovarian cyst. The baby had Apgar scores of bilirubin was only mildly elevated at 1.6 mg% (normal 8 and 9 at 1 and 5 min, respectively, and was normal without 0.3–1.1 mg%).

Figure 1 (a) Longitudinal sonogram of liver through porta hepatis,(b) transverse sonogram through porta hepatis and (c) transverse liver sonogram at level portal vein bifurcation. A large cyst is present in the porta hepatis. It appears to be continuous with the slightly dilated biliary ducts on the longitudinal image. Intrahepatic ductal dilatation is appreciated anterior to the portal vein (white arrow).

Correspondence: Dr TE Herman, Mallinckrodt Institute of Radiology, St Louis Children’s Hospital Department of Radiology, Washington University School of Medicine, 510 South Kingshighway Blvd, St Louis, MO 63110, USA. E-mail: [email protected] Received 21 March 2007; accepted 22 March 2007 Neonatal type 1 choledochal cyst TE Herman and MJ Siegel 454

Type I choledochal cysts are associated with a significant risk of development of biliary adenocarcinomas.3 The frequency of these malignancies increases with age, but has been reported in patients as young as 12 years old.4 The lifetime risk of malignancy is 25–40%. Because of this risk of malignancy, the definitive surgical treatment should be resection of the entire choledochal cyst and as much of the biliary system as possible.3 Although 86% of cases present before 16 years of age, neonatal presentation is uncommon.5 The most common presentation of these lesions is jaundice, pain and a RUQ mass.5 The primary differential consideration is BA with a cyst (Japanese type I cyst).6–8 This is a critical differential because of the different surgical implications. Patients with BA type I cyst have a very high incidence of hepatic fibrosis. In addition to absence of demonstrable bile flow into the duodenum at cholangiography in patients with BA type I cyst, liver biopsy can also be helpful, because in typical choledochal cysts there are normal interlobar Figure 2 Intraoperative cystography. Contrast injected into the porta bile ducts. However, in BA type I cyst there are no interlobar bile 7 hepatis cyst flows into the slightly dilated intrahepatic biliary tree and ducts and bile ductular proliferation. Neonatal biliary choledochal into the duodenum and jejunum (black arrow). cysts even without atresia (those discovered in patients younger than 4 months) have been reported to develop hepatic fibrosis, Denouement and discussion making early surgical intervention warranted to prevent 6 The initial abdominal sonogram demonstrated a large RUQ cyst in progression. the porta hepatis. The main intrahepatic biliary ducts were dilated. A gallbladder was identified. The mass displaced the hepatic artery anteriorly. Echogenic debris was present in the dependent portion References of the cyst. Given this appearance, a choledochal cyst was 1 Savader SJ, Benenati JF, Venbrux AC, Mitchell SE, Widlus DM, Cameron JL considered most likely. The infant was taken to the operating room et al. Choledocahl cysts: classification and cholangiography appearance. where the cystogram demonstrated a choledochal cyst without A J Roentgenol 1991; 156: 327–331. biliary atresia (BA) as contrast flowed freely antegrade into the 2 Kimura K, Ohto M, Ono T, Tsuchiya Y, Saisho H, Kawamura K et al. duodenum and retrograde into the slightly dilated intrahepatic Congenital cystic dilatation of the common bile duct: relationship to biliary tree. anomalous pancreaticobiliary duct union. Am J Roentegenol 1977; 128: 571–577. Choledochal cysts were reportedly described first by Abraham 1 3 Yoshida H, Itai Y, Minami M, Kokubo T, Ohtomo K, Kuroda A. Biliary Vater in 1723. Choledochal cysts are cystic dilatations of the malignancies occurring in choledochal cysts. Radiology 1989; 173: extrahepatic and intrahepatic biliary tree or both. They are 389–392. approximately three times more common in women than in men. 4 Iwa N, Deguchi E, Yanagihara J, Iwai M, Matsuo H, Todo S et al. Cancer Although the etiology is not known, it has been shown that in at arising in a choledochal cyst in a 12-year-old girl. J Pediatr Surg 1990; 25: least 94% of choledochal cysts there are anomalous insertions of 1261–1263. the common bile duct into the pancreatic duct.2 It is believed that 5 Bass EM, Cremin BJ. Choledochal cysts: clinical and radiological evaluation of this allows reflux of pancreatic enzymes into the biliary tree 21 cases. Pediatr Radiol 1976; 5: 81–85. leading to ductal dilatation and scarring. Among the various 6 Suita S, Shono K, Kinugasa Y, Kubota M, Matsuo S. Influence of age on the classification schemes, that of Todani is widely used. The cystic presentation and outcome of choledochal cysts. J Pediatr Surg 1999; 34: dilatation in this patient is that of Todani type I. This comprises 1765–1768. 80–90% of all choledochal cysts.1 Todani type II are diverticular 7 Tsuchida Y, Kawarasaki H, Iwanaka T, Uchida H, Nakanishi H, Uno K. Antenatal diagnosis of biliary atresia (Type I cyst) at 19 weeks gestation: dilatations of the common bile duct. Todani type III is a differential diagnosis and etiologic implications. J Pediatr Surg 1995; 30: choledocele, a malformation of the ampulla of Vater that does not 697–699. have a high insertion of the pancreatic duct and common bile 8 Saito T, Horie H, Yoshida H, Matsunaga T, Kouchi K, Kuroda H et al. The duct. Type IV is a choledochal cyst associated with other perinatal transition of the hepatobiliary cyst size provides information about intrahepatic and extrahepatic cysts or Caroli’s disease. Type V is the condition of bile flow in biliary cystic malformation cases. J Pediatr Surg intrahepatic cystic biliary dilatation or Caroli’s disease. 2006; 41: 1397–1402.

Journal of Perinatology