Diagnosis and management of choledochal cysts.: A review of 10 new cases

Aim: the aim of this study was to analyze the diagnostic and therapeutic options for the various types of this rare disease. Patients and methods: 10 patients with choledochal cysts (CC) were diagnosed in our hospital since 1991. Type of cyst was established according to the Alonso-Lej classification. Results: we report 7 type-I, 1 type-III, 1 type-IVa, and 1 type-V CC cases. Clinical manifestations were abdominal pain in all cases with biliary or pancreatic features. The diagnosis was established using abdominal ultrasonography, computed tomography, and endoscopic retrograde cholangiopancreatography (ERCP). All 7 patients with type-I CC underwent total cyst excision with Roux-en-Y hepato-jejunostomy. For type-III CC an endoscopic sphincterotomy (ES) was performed, and in type-IVa CC a transductal sphincterotomy and cholecistectomy was made. The patient with Caroli's disease (type V) underwent liver transplantation. We have followed up all patients for several years without significant complications. Conclusions: CC is more frequent in childhood, but is not exceptional in the adult. Imaging techniques and ERCP play an important role in the diagnosis, and also in the treatment of type-III cysts. Therapeutic options depend on cyst type, but due to the potential malignancy of this disease total cyst excision is recommended for types I, II and IV. In type-III CC endoscopic sphyncterothomy is recommended, while liver transplantation is sometimes necessary for type V. Long-term follow-up is crucial to prevent malignant transformation except for type-III CC where this complication is very unusual.