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Sickle-cell anaemia is an autosomal recessive hereditary haemoglobinopathy. Patients with sickle-cell anaemia present a high risk of priapism. At least 40% of sickle-cell patients report episodes of priapism. Priapism in patients with sickle-cell tend to start during childhood and rapidly threaten the erectile prognosis. Apart from specific treatment of sickle-cell anaemia, the urologist must be able to recognize forms of intermittent priapism and promote medical prevention. The urologist must also be able to distinguish low-flow priapism from the rarer high-flow priapism, as the treatments differ. Treatment strategies are increasingly well defined, with an increasingly limited place for surgery.

Objectives: The authors propose the use of a pedicle inguinal flap for reconstruction of the scrotal region after wide tissue resection following gangrene of the perineum., Material and Methods: Between 1998 and 2002, the authors treated 14 patients by surgical debridement following Fournier's perineo-perianal cellulitis. The authors used a pedicle inguinal flap in two patients with complete resection of the scrotal sac., Results: Clinical examination at 3 years showed a good functional and aesthetic result. The trophicity and sensitivity of the neo-scrotum and testes were normal., Conclusions: The inguinal flap proposed by that authors allows cover of the scrotal region with relatively thick, sensitive tissue with limited scarring and functional sequelae.

Introduction: Cystine stones is a rare disease, related to a genetic metabolic disease. Its management must treat both the stones and their complications, and prevent recurrences by controlling cystinuria., Objective: The objective of this study was to define the natural history of cystine stones, identify reliable diagnostic criteria and propose a simple treatment regimen., Material and Method: The authors studied the clinical, biochemical and radiological data of a retrospective series of 116 cases, treated by the same urologist between 1953 and 1999., Results: Cystinuria is often diagnosed in young adults on the basis of urine biochemistry (Brand's reaction, urinary cystine assay) or spectrophotometric analysis of the stones or urinary crystals. This assessment, not performed routinely, must be guided by a family history, recurrent stones or following failure of lithotripsy. The appearance of the stones, macroscopically a waxy yellow, radiologically only slightly opaque with a large component in the renal pelvis associated with a small round caliceal stones, is highly suggestive. Radiological assessment by IVU is generally sufficient. Treatment of the disease is medical and surgical: dietary measures, alkalinization of the urine and possibly drug treatments, are associated with extracorporeal lithotripsy or surgical treatment. These modalities eliminate cystine stones in 70% of cases. Recurrent stones are observed in 42% of cases followed for more than 5 years, mainly due to the presence of residual fragments (60% of recurrences). Surveillance and dietary measures must be maintained for life. Surveillance is based on biannual radiographic follow-up (plain x-rays +/- ultrasound) to detect and consequently treat small stones. A test for cystinuria must be proposed to all relatives of affected patients., Conclusion: This disease presents very polymorphic clinical features and clinical course. It can be responsible for impaired renal function and must therefore be investigated in the presence of suggestive clinical signs or history to avoid a delayed diagnosis, as treatment modalities are available to decrease the frequency of recurrence and which can potentiate treatment by surgical or lithotripsy. However, there are no predictive factors of deterioration of the disease, and biannual surveillance, for life, is essential to detect and treat small stones.

Objectives: To define the clinical and laboratory characteristics and natural history of Leydig cell tumours in order to define a general management plan., Material and Methods: The files of 10 patients operated for Leydig cell testicular tumour between 1982 and 1996 were studied retrospectively., Results: In nine out of ten cases, the presenting complaint was gynaecomastia, erectile dysfunction or infertility. In every case, serum testosterone was normal or low and oestradiol was normal or elevated. Eight patients were treated by radical orchidectomy, and two by subcapsular orchidectomy. The course was favourable in 9 out of 10 cases in the absence of any other treatment. Only one patient had an immediately malignant form with a fatal outcome., Conclusion: Although many teams prefer total orchidectomy because of the diagnostic difficulty associated with malignant forms, simple subcapsular orchidectomy should become the first-line treatment, provided it is subsequently followed by close surveillance, as it preserves maximum fertility, and these tumours usually have a favourable prognosis.