Choledochal cysts: a ten year experience. 1996

S Y Rha, and M C Stovroff, and P L Glick, and J E Allen, and R R Ricketts
Division of Pediatric Surgery, Children's Hospital of Buffalo, New York, USA.

Choledochal cyst (CC) is a rare disorder that usually presents in childhood. Prognosis depends on early diagnosis, complete excision of the cyst, and reconstruction by hepaticojejunostomy. This report details our 10-year experience and emphasizes innovations in our management. Sixteen patients presented with CC at a mean age of 3 years. (Range, newborn to 21 years, with a M:F ratio 1:4). Two groups could be identified on the basis of age at presentation. Group I (N = 7), presented in the neonatal period, three with obstructive jaundice and four without symptoms. In Group II (N = 9), all patients presented with ascending cholangitis at a mean age of 6 years. Thirteen patients had a type 1 CC, one patient had a type 3 CC, and two had type 4 CC. The patients with type 1 and type 4 CC underwent primary cyst excision with Roux-en-Y hepaticojejunostomy, whereas the patient with type 3 CC underwent cyst excision with sphincteroplasty of the ampulla. There was only one complication of postop cholangitis that cleared with antibiotic therapy. All patients have remained free from symptoms in the follow-up period (6 months-10 years). Our four most recent cases were diagnosed in utero by prenatal ultrasonography. This led to appropriate antenatal counseling and prospectively planned neonatal surgery. These infants were asymptomatic, with no clinical signs at birth, and antenatal ultrasonography prevented diagnostic delay. Excision of the choledochal cyst and primary hepatico-enteric anastomosis is confirmed as the therapy of choice. Antenatal sonography is a sensitive method in the diagnosis of CC and offers the opportunity for early diagnosis and planned surgery before the onset of complications.

UI MeSH Term Description Entries
D007098 Imino Acids Carboxylic acids that contain an imino group (C Amino Acids, Secondary,Acids, Secondary Amino,Secondary Amino Acids,Acids, Imino
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007565 Jaundice A clinical manifestation of HYPERBILIRUBINEMIA, characterized by the yellowish staining of the SKIN; MUCOUS MEMBRANE; and SCLERA. Clinical jaundice usually is a sign of LIVER dysfunction. Icterus,Jaundice, Hemolytic,Hemolytic Jaundice,Hemolytic Jaundices,Jaundices, Hemolytic
D008099 Liver A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances. Livers
D008297 Male Males
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D002760 Cholangiopancreatography, Endoscopic Retrograde Fiberoptic endoscopy designed for duodenal observation and cannulation of VATER'S AMPULLA, in order to visualize the pancreatic and biliary duct system by retrograde injection of contrast media. Endoscopic (Vater) papillotomy (SPHINCTEROTOMY, ENDOSCOPIC) may be performed during this procedure. ERCP,Endoscopic Retrograde Cholangiopancreatography,Retrograde Cholangiopancreatography, Endoscopic,Cholangiopancreatographies, Endoscopic Retrograde,Endoscopic Retrograde Cholangiopancreatographies,Retrograde Cholangiopancreatographies, Endoscopic

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