Sickle cell disease and the general surgeon.

Sickle cell disease (SCD) is a common genetic disorder which represents a major medical problem in certain parts of the world. It is characterized by chronic haemolytic anaemia and vaso-occlusive crises, which can lead to widespread vascular occlusion by sickled red blood cells leading to multiple organ infarctions. In this respect, SCD can be considered as a multisystem disease presenting, to various surgical disciplines, a wide variety of surgical ailments. As surgical management of these patients is associated with high morbidity and mortality, close collaboration between medical and surgical services is essential. Greater population mobility makes recognition of the surgical implications of this disease of paramount importance in surgical practice. This article reviews surgical problems with which SCD patients may present to general surgeons and discusses their management.

The safety of laparoscopic cholecystectomy in sickle cell disease: An update.

As surgery in sickle cell patients is associated with high morbidity, this study aims to establish the safety of minimally invasive surgery in this high-risk group. Over a four-year period, 71 sickle cell patients underwent laparoscopic cholecystectomy (LC) for cholelithiasis. Five patients had asymptomatic gallstones. Preoperative gastroscopy and endoscopic retrograde cholangiography were performed in 7 and 14 patients, respectively. Forty-two patients were given simple blood transfusions, while 13 received partial exchange transfusions. The mean operative time was 80 minutes and the conversion rate was 5.6%. There were 10 (14%) postoperative complications, the majority of which were respiratory and wound-related. One patient (1.4%) died as a result of postoperative vaso-occlusive crisis. The median hospital stay was 2.5 days. We believe that laparoscopic cholecystectomy is safe in patients with sickle cell hemoglobinopathy who are particularly at risk of developing pigmented gallstones. Therefore, the use of minimally invasive surgery is encouraged in any sickle cell patient undergoing operative intervention.

Laparoscopic cholecystectomy in patients with sickle cell disease.

Over a year (October 1992-September 1993), 30 patients (23 males and 7 females) with sickle cell anaemia and symptomatic gallstones underwent laparoscopic cholecystectomy (LC). The mean age was 26 years (range 15-44 years). The indications were: acute cholecystitis in four patients and long-standing biliary colic in the other 26. Twenty-four patients (80%) had a previous history of abdominal sickle cell crises. The preoperative HbF and HbS levels ranged from 18 to 33% and from 66 to 77.2%, respectively. The haemoglobin level ranged from 8.6 to 12 g% (mean 9.7 g%). Blood transfusion was given preoperatively to 19 patients (63%) and three patients needed perioperative transfusion. The mean operative time was 75 min (range 60-100 min). One of the four emergency cases was converted to open cholecystectomy due to difficult anatomy and inability to grasp a thick-walled, distended gallbladder. Two patients developed minor chest infections and one had an acute vaso-occlusive crisis on the fifth postoperative day and died despite the appropriate treatment (morbidity 6.6%, mortality 3.3%). The median hospital stay was 2 days (range 1-5 days). We believe that LC can be conducted safely in sickle cell patients with gallstones with minimal morbidity and mortality.