Combined laparoscopic splenectomy and cholecystectomy for the treatment of hereditary spherocytosis: is it safe and effective?

Hereditary spherocytosis is the most common red blood cell membrane disorder and often is associated with hemolytic crisis and premature cholelithiasis. Splenectomy is the only effective therapy for this disorder and often it is performed in combination with cholecystectomy. Conventional surgery requires a wide upper abdominal incision for correct exposure of the gallbladder and spleen. Laparoscopic cholecystectomy and splenectomy have been performed safely worldwide. We report our experience with seven patients (one male and six female, average age 12 years) who underwent combined laparoscopic splenectomy and cholecystectomy for hereditary spherocytosis. The patient was placed in supine position and the procedure performed with a five-trocar technique. Cholecystectomy was performed first, then splenectomy was achieved and the spleen removed by morcellation into a retrieval bag (five cases) or via a 4- to 5-cm left subcostal incision (two cases). No patient required conversion to open technique or blood transfusion. The mean blood loss was 162 mL, mean operative time 207 minutes, mean spleen size 14.5 cm, and median postoperative hospital stay 4 days. No perioperative mortality or major complications occurred in our series. After a median follow-up of 18 months all patients showed sharp hematologic improvement. Despite the small number of cases, we consider the combined laparoscopic approach safe and effective for the treatment of hereditary spherocytosis.