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 treatment. Two cases regressed after 6 hours of treatment follow-up. Four children were treated by a corpus cavernosum puncture, through the gland, leading to priapism detumescence in 3 cases. In one of our cases, the persistence of priapism induced us to perform a spongiocavernous anastomosis at the 72nd hour. One of the children developed a cerebrovascular accident 10 days after the beginning of the priapism. The other children have been re-examined. They have not presented any reccurence and have normal erection after puberty. Starting from a literature review, we explain the principles we adopted. 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 neurological risks and must not delay any surgery. Analgesic treatment is often required. Corpus cavernosum puncture is efficient, with no fibrosis risk. In case of failure, a cavernospongious shunt, with a biopsy needle, can be performed at the same time. Surgical anastomoses are then proposed in case of priapism persistence. General evolution is satisfactory. But the sickle cell patient's priapism needs a long-term follow-up in order to recognise any minor recurrences. The latter could be the principal cause of fibrosis and impotency.