Gallstones in Sickle Cell Disease: A Single Institution Experience

Background: There is an increased incidence of gallstones in patients with sickle cell disease (SCD), due to haemolysis. Complications of gallstones include cholecystitis, pancreatitis and cholangiopathy and gallstones can trigger an acute sickle cell crisis. It is not known whether patients with asymptomatic gallstones would benefit from elective cholecystectomy to avoid such complications.

Method: Electronic patient records of all 767 adult SCD patients attending clinic at King’s College Hospital, London between 1^st Jan 2003 and 31^st Dec 2013 were retrospectively reviewed to identify cases of gallstones. Medical records and steady state blood values were analysed in all those patients with an ultrasound of the biliary tree during this time period.

Results: Amongst the cohort of 767 patients with SCD, 481 (62.7%) were HbSS, 244 (31.8%) HbSC, 35 (4.6%) HbSB+, 6 (0.8%) HbSB0 and 1 (0.1%) HbSHFPH genotype. 43% were male. Mean age at the end of the study period was 36.6 +/- 12.5 years. 344 patients had an ultrasound scan of the biliary tree during the time period of the study. 38 of the 344 patients scanned had had a cholecystectomy prior to 2003. Of the remaining 306 patients with an ultrasound scan, 134 had gallstones identified within the gallbladder. The 134 patients with gallstones comprised 119 (88.8%) HbSS, 12 (9.0%) HbSC, 2 (1.5%) HbSB+ and 1 (0.7%) HbSB0. 39.6% were male. Mean age at the end of the study period was 35.4 +/- 12.2 years.

Of the 134 patients with gallstones identified during the study, 35 developed serious complications directly relating to cholelithiasis (5 pancreatitis, 4 acute cholangitis, 8 choledocholithiasis and 18 isolated cholecystitis) and 8 of these patients required sphincterotomies +/- stone removal with endoscopic retrograde cholangio-pancreatogram.

34 of the 134 patients with gallstones went on to have a cholecystectomy during the 11 year study period. Of these 34, 3 had recorded surgical complications following cholecystectomy (2 bile leaks, 1 hepatic injury). All 3 cases had gallstone-related complications prior to the cholecystectomy.

Discussion: Our findings of cholelithiasis in 134 of the 306 of sickle cell disease patients scanned, is similar to incidence reported in the literature. Notably, we documented a high incidence of complications associated with cholelithiasis. Furthermore, there were higher than expected rates of surgical complications in cholecystectomy undertaken following the development of a complication relating to gallstones. These findings make routine screening for cholelithiasis followed by elective cholecystectomy for positive cases an attractive approach.