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Priapism in a sickle cell prepuberal child
J F Colombani1, P Peluchon, G Elana
1Service de Chirurgie Pédiatrique, CHRU P. Zobda Quitman, Fort-de-France, Martinique.
Insights
Priapism in sickle cell disease (SCD) patients requires prompt intervention. Surgical options like corpus cavernosum puncture and spongiocavernous anastomosis offer effective detumescence, preventing long-term complications such as fibrosis and impotence.
Area of Science:
- Pediatric Hematology
- Urology
- Genetics
Background:
- Priapism is a known complication of sickle cell disease (SCD).
- Prolonged priapism can lead to severe penile complications, including fibrosis and erectile dysfunction.
- The pathophysiology of priapism in SCD remains incompletely understood, with both high and low flow states observed.
Purpose of the Study:
- To evaluate the management and outcomes of priapism in children with sickle cell disease.
- To review treatment principles and long-term follow-up for SCD-related priapism.
Main Methods:
- Retrospective case series of six children with SCD experiencing priapism.
- Review of treatment modalities including medical management, corpus cavernosum puncture, and spongiocavernous anastomosis.
- Literature review on priapism pathophysiology and management in SCD.
Main Results:
- Two of six cases resolved with initial medical treatment.
- Four cases underwent corpus cavernosum puncture, with successful detumescence in three.
- One case required spongiocavernous anastomosis due to persistent priapism; one patient developed a cerebrovascular accident.
- Long-term follow-up showed no recurrence and normal erections post-puberty in the examined patients.
Conclusions:
- Prompt surgical intervention, including corpus cavernosum puncture or spongiocavernous anastomosis, is crucial for managing priapism in SCD.
- Transfusional exchange carries risks and should not delay surgical consideration.
- Long-term follow-up is essential to monitor for recurrences, which can lead to fibrosis and impotence.
Abstract:
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.
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