Choledochal cysts: a ten year experience.
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.
Division of Pediatric Surgery, Children's Hospital of Buffalo, New York, USA.
S Y
SY
Rha
M C
MC
Stovroff
P L
PL
Glick
J E
JE
Allen
R R
RR
Ricketts
D000293:Adolescent; D000328:Adult; D000713:Anastomosis, Roux-en-Y; D002648:Child; D002675:Child, Preschool; D002760:Cholangiopancreatography, Endoscopic Retrograde; D002761:Cholangitis / Q000209:etiology; D015529:Choledochal Cyst / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007098:Imino Acids; D007223:Infant; D007231:Infant, Newborn; D007565:Jaundice / Q000209:etiology; D008099:Liver / Q000473:pathology; D008297:Male; D015609:Organotechnetium Compounds; D011247:Pregnancy; D012189:Retrospective Studies; D019780:Technetium Tc 99m Lidofenin; D016216:Ultrasonography, Prenatal
D007098:Imino Acids; D015609:Organotechnetium Compounds; D019780:Technetium Tc 99m Lidofenin