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Choledochocystic Disease in a Western Center: a 30-Year Experience
Maitham a. Moslim*, Hideo Takahashi, R Matthew Walsh, Gareth Morris-Stiff
General Surgery, Cleveland Clinic Foundation, Cleveland Heights, OH
BACKGROUND: Choledochocystic (CC) disease is a rare condition that has a varied presentation. The aim of this study was to report as single experience in the diagnosis and management of the condition at a Western center.
METHODS: With institutional review board approval, the departmental database was interrogated to identify all patients who underwent surgical treatment of CC disease. The presentation, investigation, management, surgical pathology, post-operative course and follow-up were documented. All CC were classified as per the Todani system.
RESULTS: 67 patients were identified including 15 children and 52 adults, with 76.1% being females. The mean age at diagnosis of CC was 3.7 years for children, and 44.6 years for adults. 48 patients (62.7%) were symptomatic, with the most common presentations including: (n=34); jaundice/deranged hepatic function (n=10); and acute pancreatitis (n=9). Two of 19 patients with incidental CC were diagnosed on prenatal ultrasound. Types of CC included: I (n=49, 73.1%); II (n=1, 1.5%), IV (n=9, 13.4%) and V (n=8, 12%). Malignancy was concomitant in 5 pathology specimens (gallbladder adenocarcinoma [n=2], cholangiocarcinoma [n= 3]), and atypia without cancer was reported in 3 patients. The median interval from diagnosis to operation was 95 days. 48 patients underwent excision of CC with Roux-en-Y hepatojejunostomy, and 8 resection with heptatoduodenostomy. 6 patients underwent liver resection with Roux-en-Y hepaticojejunostomy including right lobectomy (n=3), left lobectomy for (n=2) and gallbladder resection for CC associated with a gallbladder cancer (n=1). 5 patients underwent orthotopic liver transplantation (Type V [n=4], incidental type I cyst in native liver with primary biliary cirrhosis [n=1]). The surgical approach was open (n=57), laparoscopic (n=3), robotic (n=6) and laparoscopic converted to open due to gallbladder cancer (n=1). Post-operative complications included intra-abdominal fluid collections (n=8), bile leak (n=6), anastomotic stenosis (n=6), malignancy (CCA [n=2], recurrence of gallbladder cancer [n=2], post-transplant lymphoproliferative disease [n=1]), cholangitis (n=4), pancreatitis (n=4), CBD stump lithiasis (n=4), ileus (n=3), liver failure (n=3), incisional hernia (n=3) and others (n=22). 5 patients (7.5%) expired due to recurrent CCA (n=3), pneumonia in the setting of recurrent gallbladder cancer (n=1) and hepatic failure (n=1). The median follow up interval from the diagnosis to the last surgical clinic visit was 25 months.
CONCLUSIONS: The study has demonstrated the varied presentation and management of CC arising in a western population. Malignant change is not uncommon and often detected incidentally at resection of CC.
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