Splenectomy in Gaucher Disease: A Call for Minimally Invasive Surgery.

Objective: The objective of this article was to demonstrate that Gaucher disease (GD) patients with refractory hypersplenism and massive splenomegaly may successfully undergo hand-assisted laparoscopic splenectomy (HALS).

Methods: This was a retrospective audit conducted at the Gaucher clinic at a national referral center over a 10-year period.

Patient Population: This study included 8 GD patients who underwent hand-assisted or conventional laparoscopic splenectomy for massive or complicated splenomegaly between the years 2007 and 2017.

Results: Seven patients underwent an elective HALS procedure because of refractory hypersplenism, whereas 1 patient underwent an urgent conventional laparoscopic splenectomy because of torsion of a wandering spleen. Only 1 patient required conversion to open surgery because of multiple adhesions from a previous partial splenectomy. The mean weight of the removed spleens was 2373 g (range, 480 to 4900 g), mean craniocaudal length of the removed spleens was 25 cm (range, 20 to 33.5 cm), and mean operating time was 150 minutes (range, 96 to 280 min). Postoperative complications were limited to 2 patients and included thrombosis of the splenic vein stump in 1 patient, and propagation of a preoperative splenic vein thrombus to the portal system, as well as an accumulation of an intra-abdominal hematoma in another patient. There was no mortality. Mean length of hospital stay was 5 days (range, 2 to 11 d).

Conclusion: HALS for GD patients with refractory hypersplenism and massive splenomegaly is safe and feasible in experienced hands.