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Choledochal cysts: a ten year experience
S Y Rha1, M C Stovroff, P L Glick
1Division of Pediatric Surgery, Children's Hospital of Buffalo, New York, USA.
Insights
Early diagnosis of choledochal cysts (CC) through prenatal ultrasonography enables prompt surgical intervention. Complete cyst excision and hepaticojejunostomy remain the preferred treatment for choledochal cysts, ensuring symptom-free outcomes.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Imaging
Background:
- Choledochal cyst (CC) is a rare congenital anomaly often diagnosed in childhood.
- Prognosis is significantly influenced by early detection, complete surgical excision, and reconstruction.
- This study reviews a decade of management experience, highlighting recent advancements.
Purpose of the Study:
- To evaluate the 10-year experience in managing choledochal cysts.
- To emphasize innovations in diagnosis and surgical treatment.
- To assess the long-term outcomes of surgical management.
Main Methods:
- Retrospective analysis of 16 patients with choledochal cysts treated over 10 years.
- Classification of cysts by type (1, 3, and 4).
- Surgical procedures included cyst excision with Roux-en-Y hepaticojejunostomy or sphincteroplasty.
Main Results:
- 16 patients (M:F ratio 1:4) presented with CC at a mean age of 3 years.
- Two presentation groups: neonatal (7 patients) and older children with cholangitis (9 patients).
- All patients achieved symptom-free status post-surgery; 4 cases diagnosed prenatally via ultrasonography.
Conclusions:
- Complete excision of choledochal cysts with primary hepatico-enteric anastomosis is the gold standard treatment.
- Prenatal ultrasonography is highly effective for early CC diagnosis, facilitating planned neonatal surgery and preventing complications.
Abstract:
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.