Combined laparoscopic splenectomy and cholecystectomy for the treatment ofhereditary spherocytosis: Is it safe and effective?

Hereditary spherocytosis is the most common red blood cell membrane disorde r and often is associated with hemolytic crisis and premature cholelithiasi s. Splenectomy is the only effective therapy for this disorder and often it is performed in combination with cholecystectomy. Conventional surgery req uires a wide upper abdominal incision for correct exposure of the gallbladd er and spleen. Laparoscopic cholecystectomy and splenectomy have been perfo rmed safely worldwide. We report our experience with seven patients (one ma le and six female, average age 12 years) who underwent combined laparoscopi c splenectomy and cholecystectomy for hereditary spherocytosis. The patient was placed in supine position and the procedure performed with a five-troc ar technique. Cholecystectomy was performed first, then splenectomy was ach ieved and the spleen removed by morcellation into a retrieval bag (five cas es) or via a 4- to 5-cm left subcostal incision (two cases). No patient req uired conversion to open technique or blood transfusion. The mean blood los s was 162 mL, mean operative time 207 minutes, mean spleen size 14.5 cm, an d 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 e ffective for the treatment of hereditary spherocytosis.