Priapism in a sickle cell prepuberal child

Between 1985 and 1995, six sickle cell children listed SS were hospitalized in our department for priapism persisting after 24 hours of medical treatm ent. Two cases regressed after 6 hours of treatment follow-up. Four childre n were treated by a corpus cavernosum puncture, through the gland, leading to priapism detumescence in 3 cases. In one of our cases, the persistence o f priapism induced us to perform a spongiocavernous anastomosis at the 72nd hour. One of the children developed a cerebrovascular accident 10 days aft er the beginning of the priapism. The other children have been re-examined. They have not presented any reccurence and have normal erection after pube rty. Starting from a literature review, we explain the principles we adopte d. The physiopathology of priapism in sickle cell patients remains unclear, for both high and low flows do exist. In addition to the risks related to blood products, the treatment by transfusional exchange involves neurologic al risks and must not delay any surgery. Analgesic treatment is often requi red. Corpus cavernosum puncture is efficient, with no fibrosis risk. In cas e of failure, a cavernospongious shunt, with a biopsy needle, can be perfor med at the same time. Surgical anastomoses are then proposed in case of pri apism persistance. General evolution is satisfactory. But the sickle cell patient's priapism n eeds a long-term follow-up in order to recognise any minor recurrences. The latter could be the principal cause of fibrosis and impotency.