Choledochal cyst in pediatric patients: a 10-year single institution experience (original) (raw)
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Choledochal cyst in pediatric patients: a 10 years single institution experience
Acta gastroenterologica Latinoamericana
Choledochal cysts (CCs) are rare congenital dilatations of the biliar tree. The incidence is 1:150,000. A correct diagnosis and surgical treatment are important because of the long term risks of infection and neoplasia. We report our experience with CCs in children. Patients with CC disease, who were seen at the Hospital Italiano de Buenos Aires between 1999 and 2009, were identified retrospectively from our prospectively acquired hepato-pancreatico-biliary database. Prenatal, clinical, surgical and pathological records were analysed. We included 12 children (10 girls and 2 boys), all of them affected by a cystic dilatation type I of Todani classification. Between the 4 patients with antenatal diagnosis only one presented symptoms before surgery, expressed in acolic feces. Between the 8 patients with postnatal diagnosis, the most common clinical presentation was abdominal pain, occurring in all patients. Ultrasound was used for the diagnosis of all patients. Mean age at surgery was ...
Choledochal cysts in infancy and childhood
Indian Journal of Pediatrics, 2007
Objective To study the clinical presentation, management, and results of treatment of 41 consecutive cases of choledochal cyst (CC) managed from 1999 to 2006. Methods The age of the patients ranged from 20 days to 11 years. Eleven cases were 1 year or less in age (infantile group), and 30 patients were more than 1 year old (classical pediatric group). Children less than 1 year old presented with jaundice (72%), hepatomegaly (54%) and clay-colored stools (63%); whereas those above one year in age presented with pain (83.3%) and jaundice (47.6%). Three cases had biliary perforation with localized or generalized biliary peritonitis. Ultrasonography (US) diagnosed/suggested CC in all 41 patients, however, contrast-enhanced computed tomographic (CECT) scan (n=12), radionuclide DISIDA scan (n=5), and magnetic resonance cholangiopancreatogram (MRCP) (n=3) also contributed to the diagnosis. 19 patients, including 4 from the ‘infantile’ group, had significant dilatation of intrahepatic biliary ducts. Results Nine patients from the ‘infantile’ group had cystic type I CC (type Ic), while 2 patients had fusiform dilatation of the common bile duct (type if disease). In contrast, 21 of the older patients had type If disease while 9 had type Ic disease. Operative management included primary complete excision of the cyst with a Roux-en-Y hepaticodochojejunostomy (HDJ) (n=32), Lilly’s modification of submucosal resection with a HDJ (n=8), and cyst excision with a hepaticodochoduodenostomy (HDD) (n=1). All 3 patients with biliary perforation had primary excision of the CC with a HDJ. Unusual operative findings included accessory hepatic ducts (n=2), anteriorly placed common or right hepatic arteries (n=3), and partially or completely blocked proximal extrahepatic bile ducts (n=3). There were 2 postoperative deaths, one had cirrhotic liver disease with portal hypertension, the other had deep icterus with poor general condition. Both patients succumbed to overwhelming sepsis. One patient required reexploration and refashioning of the HDJ for biliary leak. Short-term follow-up was satisfactory in all 39 patients. Conclusion Infants with CC constitute a distinct group with regard to clinical presentation and the pathological anatomy. CC should be kept in mind while evaluating neonates and infants with cholestatic jaundice, and older children with recurrent abdominal pain. Primary excision of the cyst with a HDJ provides satisfactory results in the management of the vast majority of cases of CC including those in infants, massive CC, and in the presence of biliary perforation and peritonitis.
Choledochal cyst--a different disease in newborns and infants
Journal of the College of Physicians and Surgeons--Pakistan : JCPSP, 2014
We report experience of managing Choledochal Cyst (CC) in different paediatric ages. Eleven neonates and infants (aged 0-8 months) and 24 paediatric cases (aged 2.5 - 18 years) were managed over 24 years (1988 to 2012). Neonates and infants presented with jaundice, acholic stools and abdominal mass whereas most of the paediatric cases presented with intermittent non-specific abdominal pain. Morphology of CC was mostly cystic in neonates whereas it was fusiform in majority (62%) of paediatric cases. Biliary amylase was high and correlated with the presence of abnormal pancreaticobiliary junction (PBJ) in 20 /24 paediatric patients. Obstruction at the lower end of bile duct, liver fibrosis and cirrhosis were common in neonates. In conclusion, CC in newborns and infants is different and mimic correctable Biliary Atresia (BA). Early excision of CC and biliary reconstruction is promising in neonates, infants and children and it can be performed with minimal morbidity.
Congenital Choledochal Cyst in a Toddler – Case Report and Literature Review
Romanian Journal of Pediatrics, 2019
Congenital choledochal cysts are more frequently diagnosed in children under the age of 10, occurring prevailingly in female patients. We are presenting a case of choledochal cyst in a 7-month-old male baby, which had a sudden onset with scleral-cutaneous jaundice and lack of appetite. Abdominal ultrasound revealed a transonic, hyperreflective formation located in the topography of the main bile duct, dilated intrahepatic bile ducts, and laboratory tests revealed a severe syndrome of hepatic cytolysis and cholestasis. The diagnosis was confirmed using cholangiopancreatic MRI performed in an emergency regime and required surgical intervention. Therefore, the differential diagnosis of cholestasis syndromes must rule out choledochal cyst irrespective of the patient’s age due to the complications and risk of malignization.
Choledochal cysts: lessons from a 20 year experience
Archives of Disease in Childhood, 1995
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)
Choledochal cysts: diagnosis and treatment
2012
The aim of this study is to show the different diagnostic procedures and treatment in patients diagnosed with congenital choledochal cysts. Choledochal cysts are congenital anomalies of the bile ducts and include cystic dilatation of the extrahepatic and intrahepatic biliary ducts or both. The study shows ten patients diagnosed as having choledochal cysts. Diagnosis was established by clinical and radiographic findings including: ultrasound (US), magnetic resonance cholangiopancreatograpy (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC) and cytological examination of the bile juice. In the study choledochal cysts were classified according to the Todani classification. Most common cysts were type I (six cases); type III (one case), type IVa (one case) and two patients were type V cysts (Caroli disease). The most frequent symptoms were abdominal pain, jaundice and cholangitis. US findings were sensitive for the preliminary ...
Gastroenterology Research and Practice, 2015
Background. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. Their presentations differ from children and surgical management has evolved. Methods. A retrospective review of the records of all the patients above 15 years, who underwent therapeutic intervention in our hospital, was carried out. Results. Ten cases of choledochal cyst were found; 8 female, with mean age 31 years. These included 8 cases of Todani type I and one case each of type II and type III. The predominant symptoms were abdominal pain and jaundice. Abdominal mass and past history of cholangitis and pancreatitis were seen in 2 patients. Investigations included ultrasound in 8 patients, CT in 7, ERCP in 3, and MRCP in 5. Surgical intervention included complete excision of the cyst with hepaticojejunostomy and cholecystectomy (type I), excision of the diverticulum (type II), and ERCP sphincterotomy (type III). Malignancy was not seen in any patients. The long-term postoperative complications included cholangitis in two patients. Conclusion. Choledochal cyst is rare in adults. The typical triad of abdominal pain, jaundice, and mass is uncommon in adults. The surgical strategy aims for single stage complete excision of the cyst with hepaticojejunostomy.
Biliary Cysts in Children — Long-term Follow-up in Taiwan
Journal of the Formosan Medical Association, 2006
Cystic malformation in the biliary tree is an uncommon anomaly in Western populations but comparatively frequent in Asian children. 1-7 The etiology is still controversial. 8 It is often located in the common bile duct (choledochus), and is thus referred to as choledochal cyst. 9 Alonso-Lej and
Unusual Presentations of Choledochal Cyst: Case Series and Review of Literature
Indian Journal of Surgery, 2014
Congenital cystic dilation of the biliary duct is defined as choledochal cyst (CDC). CDC presenting with the classical triad of abdominal pain, lump, and jaundice is s e e n i n o n l y 6 % c a s e s. C y s t e x c i s i o n w i t h hepaticojejunostomy is the standard treatment worldwide. We hereby report five cases of CDC with unusual presentations (gastric outlet obstruction, cyst perforation, giant cystolithiasis, giant cyst, and mixed type) and discuss the challenges faced during the diagnosis and perioperative management of these cases. diagnosis and management. Herein, we report five cases of CDC who had unusual features on presentation. Case Series Of the 29 cases of CDC managed between January 2009 and December 2013, 5 (17.2 %) cases had unusual presentation. Case 1 A 12-year-old girl presented with history of pain in the abdomen for 2 years and recurrent bilious vomiting for 7-months duration. Upper gastrointestinal endoscopy showed dilated stomach with external compression at the antropyloric region. Subsequently, contrast-enhanced computed tomography (CECT) was done, which showed type I CDC with gastric outlet obstruction (Fig. 1a). On exploration, dilated stomach with a large CDC (10×7 cm) compressing the antropyloric region was present (Fig. 1b). CDC excision with Roux-en-Y hepaticojejunostomy (RYHJ) was performed. Postoperative course was uneventful and patient is doing well after a follow up of two and half years. Case 2 A 16-year-old female presented with complaints of abdominal pain for 2 months. Abdominal ultrasonography (USG) revealed a dilated common bile duct (CBD) suggesting CDC. Though surgery was advised on index admission, patient refused the same due to personal reasons. One month later, she presented with jaundice, fever, epigastric lump, and features of local peritonitis. Liver function test showed raised serum bilirubin, 17.25 mg/dl (normal range 0-1.4 mg/dl) and liver enzymes (serum aspartate transaminase, AST), 174 IU/L