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Choledochal cyst disease. A changing pattern of presentation
P A Lipsett1, H A Pitt, P M Colombani
1Department of Surgery, Johns Hopkins University, Baltimore, Maryland.
Insights
Adults and children present choledochal cysts differently, with adults often showing acute biliary or pancreatic symptoms. Surgical excision and reconstruction are safe, effective, and reduce malignancy risk in both groups.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Hepatobiliary Surgery
Background:
- Choledochal cysts traditionally present in infants with jaundice, mass, and pain.
- Recent observations suggest increased recognition of choledochal cysts in adult patients.
Purpose of the Study:
- To compare the clinical presentation, treatment, and long-term outcomes of choledochal cysts in pediatric versus adult populations.
- To evaluate the safety and efficacy of surgical management for choledochal cysts across different age groups.
Main Methods:
- Retrospective review of 42 patients (11 children, 32 adults) treated for choledochal cysts between 1976 and 1993.
- Data collection included patient presentation, clinical evaluation, operative treatment, and long-term follow-up via records and direct contact.
Main Results:
- Children were more likely to exhibit classic symptoms (jaundice, mass, pain) or two of these signs compared to adults.
- Adults frequently presented with abdominal pain, often misdiagnosed as pancreatitis or acute biliary symptoms requiring cholecystectomy.
- The most common cyst types were fusiform extrahepatic (Type I) and combined intrahepatic/extrahepatic (Type IVA). Surgical treatment involved cyst excision and hepaticojejunostomy with no operative mortality.
- Three adults developed malignancy (cholangiocarcinoma or gallbladder cancer); long-term complications included biliary stricture and intrahepatic stones in Type IVA cysts.
Conclusions:
- Choledochal cyst presentation significantly differs between children and adults, with adults more commonly presenting with acute biliary or pancreatic symptoms.
- Surgical excision of choledochal cysts combined with biliary bypass (hepaticojejunostomy) is a safe and effective treatment for both children and adults.
- This surgical approach yields excellent long-term results and effectively minimizes the risk of developing malignancy associated with choledochal cysts.
Objective:
The authors compared the presentation, treatment, and long-term outcome of children and adults with choledochal cysts.
Summary Background Data:
The typical patient with choledochal cyst disease has been the female infant with the triad of jaundice, an abdominal mass, and pain. However, the recent experience of the authors suggested that the disease currently is recognized more commonly in adults.
Methods:
Forty-two patients (11 children, 32 adults) with choledochal cyst disease were treated primarily at this institution between 1976 and 1993. Patients presentation, clinical evaluation, and operative treatment were obtained from existing records. Long-term follow-up was obtained by records, physician, or direct patient contact.
Results:
One child--but no adults--had the classic triad of jaundice, abdominal mass, and pain. Children were more likely to have two of the three signs or symptoms (82% vs. 25%; p = < 0.05). Adult patients most commonly had abdominal pain and were thought to have pancreatitis (23%) or acute biliary tract symptoms, prompting cholecystectomy (50%). The type of choledochal cyst seen in children and adults was similar; the fusiform extrahepatic (Type I) was most common (50%), and the combined intrahepatic and extrahepatic (Type IVA) was the next most prominent (33%). For both children and adults, treatment consisted of excision of the cyst and biliary reconstruction with a hepaticojejunostomy. There was no surgical mortality. Gallbladder or cholangiocarcinoma was identified in three adults (9.7%), two of which were manifest on presentation. Long-term follow-up revealed one patient with a biliary stricture and three patients with Type IVA cysts who had intrahepatic stones.
Conclusions:
Children and adults differ in presentation of choledochal cysts, with adults commonly having acute biliary tract or pancreatic symptoms. Surgical treatment with cysts excision and biliary bypass is safe and effective in children and adults with excellent long-term results that minimize the development of malignancy.