Surgical management of hereditary spherocytosis

Abstract

BACKGROUND: Hereditary spherocytosis is a benign hematologic disease, which needs surgical treatment when medical therapy fails. Currently, the surgical strategies consist mainly in total or partial splenectomy, which can be performed either in open or in laparoscopic fashion. In this study, we analyzed our series of splenectomies for hereditary spherocytosis and we discuss about the surgical management, reviewing the Literature.

MATERIAL AND METHODS: Twenty-seven patients (mean age 16.5 years, range 8 – 30 years) affected by hereditary spherocytosis were retrospectively evaluated. Indication to surgery was based on hemolysis severity. Thirteen patients were submitted to laparoscopic splenectomy and 14 to open splenectomy, after preventive specific vaccinations. Cholecystectomy for associated cholelithiasis was performed during the same operation in 4 laparoscopic patients and in 6 open patients.

RESULTS: Main reasons for performing splenectomy were anemia unresponsive to iron supplementation in 7 patients (42%), splenomegaly in 6 patients (37%), and jaundice in 4 cases (21%). All the patients had a severe disease with hemoglobin level below 80 g/L, median reticulocytes count 6,5%, median value of indirect bilirubin concentration 2,0 mg/dL. Indications to splenectomy were increased need for red cell transfusions in 11 patients (66%) and symptoms related to cholelithiasis in 6 patients (34%). A post-operative early complication was observed after open splenectomy, consisting in a pancreatic fistula, which was treated conservatively. No post-operative complications were observed after laparoscopic splenectomy. Neither intra-operative complications nor conversions to open surgery were recorded during the laparoscopic approach. In a long-term follow-up, no infective complications were recorded.

CONCLUSIONS. According to our results, total splenectomy is associated with good results and few complications. In our opinion, it remains the best therapeutic option in selected adult patients non-responder to the medical treatment.