Splenectomy in cystic fibrosis

Splenectomy in cystic fibrosis remains controversial, mainly because the spleen is considered indispensable in this disease for its important immunological function in defence against frequent infections. Furthermore, centres that perform liver transplantation prefer this approach even after early cystic fibrosis‐related liver disease becomes obvious, as it seems more aetiological.

The article by Linnane et al¹ is an interesting contribution to this debate. Our recently published findings² largely confirm that article although we have doubts on the correctness of the reported indications. Although several of the >500 patients we have followed up over the past 50 years had a gigantic spleen, splenic rupture was never observed, and at splenectomy the surgeons each time recorded a firm thickening of the splenic capsule. In the literature, spontaneous splenic rupture is mostly described in association with infections, inflammatory disease or malignancy—that is, conditions in which the spleen enlarges over a short period of time—whereas no such association is reported in cystic fibrosis. We therefore question “risk of rupture” in itself as a true indication for splenectomy. It might be difficult to decide on the appropriate timing for operation when this is the main indication, as no information is available on the fragility of variably enlarged spleens.

Although we agree that low platelets are indeed no single indication for splenectomy, it can be justified in individual cases because of the risk of complications. A 19‐year‐old patient recently underwent splenectomy with splenorenal shunt out of fear of cerebral haemorrhage. He had a thrombopenia of 35 000/mm³, and frequent cutaneous bleeding after severe cough attacks. His spleen span was 25 cm, and placement of transjugular intrahepatic portosystemic shunt had not resulted in any amelioration. Four months after removal of a 1.7 kg heavy spleen, platelets were at 550 000/mm³ and all skin signs had disappeared. Even if it can be asserted that the thrombopenia is no real reason for concern, knowing that it has normalised is reassuring. We confirm improvement of pulmonary function and a positive effect on nutritional status, which was maintained even 5 years later. From the present article and our own results, it is clear that splenectomy can have many beneficial effects while of delaying the need for liver transplantation for several years. However, it should be performed only in highly controlled conditions, with provision for a rigorous follow‐up and constant awareness of the danger of overwhelming sepsis.