Indications and complications of splenectomy for children with sickle cell disease.

BACKGROUND: Sickle cell anemia (SCA), which is characterized by high hemoglobin (Hb) F level and persistent splenomegaly into the older age group (up to 18 years of age) or even adults, is one of the commonest hemoglobinopathies in the Eastern Province of Saudi Arabia. This makes them liable to develop splenic complications requiring splenectomy. This is a review of our experience in the management of 134 children with SCA who had splenectomy as part of their management at our hospital, with emphasis given to the indications and complications of splenectomy.

PATIENTS AND METHODS: The medical records of all children who had splenectomy at our hospital were retrospectively reviewed for the following: age at splenectomy, sex, Hb electrophoresis, indication for splenectomy, preoperative investigations, type of surgery, spleen weight, histology, perioperative management, and postoperative complications.

RESULTS: From 1990 to 2004, 170 children with various hematologic disorders had splenectomy at our hospital. Of these, 134 had SCA (118 had sickle cell disease and 16 had sickle-beta-thalassemia). Recurrent acute splenic sequestration crisis (ASSC) was the commonest indication for splenectomy in 103 (76.9%) patients, followed by hypersplenism in 18 (13.4%). Seven (5.2%) of our patients had splenectomy for splenic abscess (SA) and 2 had splenectomy for massive splenic infarction; 103 (61 boys, 42 girls) patients with a mean age of 7.6 years (range, 1.8-13 years) had splenectomy for ASSC. Their mean Hb F level was 20.5% (range, 9.2%-39.6%). Thirty-two of them had major attacks. Their Hb levels at the time of admission ranged from 1.4 to 4.1 g/dL (mean, 2.5 g/dL). The remaining 71 had minor recurrent attacks. Eighteen had splenectomy for hypersplenism and all had a significant increase in their blood parameters after splenectomy. Seven had splenectomy for SA. In 5 patients, Salmonella was the causative organism; in 1, it was Enterobacter sakazaki, whereas in 1, no organisms were identified. Two of our patients had splenectomy for massive splenic infarction because of persistent left upper quadrant abdominal pain, and 1 had splenectomy for splenomegaly with a nonfunctioning spleen. Twenty-eight (21%) of our patients had splenectomy and cholecystectomy. In 4 patients, this was because of symptomatic gallstones, whereas in the other 24, it was because of asymptomatic gallstones discovered on ultrasound. There was no mortality, but 8 (6%) developed postoperative complications.

CONCLUSIONS: With good perioperative management, splenectomy in children with SCA is not only safe, but also beneficial in treating SA, reducing the patients' transfusion requirements, eliminating the risks of ASSC, and eliminating the discomfort and mechanical pressure of the enlarged spleen. Abdominal ultrasound should be done routinely preoperatively for all children with SCA undergoing splenectomy, and if gallstones are discovered, they should undergo concomitant cholecystectomy. This is even so for asymptomatic gallstones. The addition of cholecystectomy to splenectomy does not increase the morbidity, but eliminates the subsequent complications of gallstones and simplifies their future management in case of abdominal crisis as the possibility of cholecystitis is eliminated.