Send letter to journal:
Re: Indications for surgery in hereditary spherocytosis

Dear Editor,

In the excellent review of hereditary spherocytosis (HS) by Bolton- Maggs [1], I was particularly interested to read the advice regarding surgery. There is general agreement on the following points:

i) cholecystectomy is indicated for patients with symptomatic gallstones and for those with asymptomatic stones who are undergoing splenectomy for haematological reasons (since the gallstones will otherwise persist and are likely to cause symptoms).

ii) prophylactic cholecystectomy at the time of splenectomy is not indicated in children with HS who do not have gallstones since the degree of haemolysis is sufficiently decreased by splenectomy to prevent subsequent gallstone formation [2].

Paediatric surgeons should also be aware of two additional points relating to surgery in HS patients:

iii) cholecystolithotomy (in which the gallbladder is opened and the stones are removed but a cholecystectomy is not performed) at the time of splenectomy is associated with a risk of recurrent gallstones [3]. Cholecystectomy is a better option.

iv) At the time of splenectomy it is important to identify and remove any accessory spleens. There are several reports of recurrent haemolytic anaemia in HS patients with a retained accessory spleen, sometimes occurring many years after splenectomy [4,5].

I was surprised to read that “if a child requires surgery for gallbladder disease complicating HS, the spleen should be removed at the same time”. Is there good evidence to support this recommendation? As stated in the review, splenectomy in childhood is associated with long term risks of potentially life-threatening infection. Moreover, with laparoscopic techniques which require small incisions there is no particular advantage in removing the spleen just because the patient is undergoing abdominal surgery. The need for concomitant splenectomy in such cases should be judged on its own merits as in children with HS who do not have gallstones.

During the past 10 years, I have performed a cholecystectomy in 9 children with HS and symptomatic gallbladder stones. One had had a previous splenectomy and two underwent concomitant splenectomy on the advice of a paediatric haematologist. Six children, aged 7-13 years (none of whom were severely anaemic), underwent cholecystectomy alone and they continue to remain well and transfusion independent 1-5 years later. They have not come to harm by deferring splenectomy until it is indicated for haematologic reasons and have avoided the hazard of post-splenectomy sepsis during a period when they are more at risk of this complication.

References

(1). Bolton-Maggs PHB. Hereditary spherocytosis; new guidelines. Arch Dis Child 2004;89:809-812

(2). Sandler A, Winkel G, Kimura K, Soper R. The role of prophylactic cholecystectomy during splenectomy in children with hereditary spherocytosis. J Pediatr Surg 1999;34:1077-8

(3). Robertson JFR, Carachi R, Sweet EM, Raine PAM. Cholelithiasis in childhood: a follow-up study. J Pediatr Surg 1988;23:246-249

(4). Bart JB, Appel MF. Recurrent hemolytic anemia secondary to acessory spleens. South Med J 1978;71:608-9