Xanthine urolithiasis.

Hereditary xanthinuria type I, a defect of purine metabolism, results from a genetic deficiency of xanthine oxidase. It is an uncommon cause of stone formation in children. We report here two children with xanthine urolithiasis. The first patient was an 8-year-old boy who presented with repeated episodes of hematuria evaluated with excretory urography, which demonstrated radio-lucent pelvic stone in the right kidney, causing hydronephrosis. He had pyelolithotomy, and the extracted stone consisted of pure xanthine. Family study revealed an asymptomatic xanthinuria in younger brother. The second patient was a 5-year-old boy who had a 2-week history of abdominal pain and gross hematuria. Conventional excretory intravenous urography showed a non-functioning right kidney. Nephrectomy was performed, and histology revealed end-stage pyelonephritis. The calculi consisted of pure xanthine. In both patients, plasma and urinary concentrations of uric acid were low but xanthine and hypoxanthine concentrations were markedly elevated. Xanthine urolithiasis is usually a benign condition, easy to prevent or cure by appropriate alkalinization, forced hydration and restriction of dietary purines. However asymptomatic, and therefore undiagnosed, stones may invade the kidney and urinary tract, resulting in destruction of parenchyma, nephrectomy and renal failure.

Giant condyloma acuminatum in an infant.

We report a 10-month-old boy who presented with a giant perianal condyloma acuminatum, and a similar lesion on the neck. These lesions were treated by surgical excision with satisfactory results. This size, extent, and early age of appearance make this case highly unusual.

[Acute cholangitis revealing a primary pancreatic hydatid cyst in a child]. / Une angiocholite aiguë révélant un kyste hydatique du pancréas chez l'enfant.

Pancreatic hydatid cysts represent 0.1 to 1% of all hydatidoses. A 12 year-old-boy, with a previous history of abdominal pain, was admitted for acute cholangitis. Laboratory investigations showed cholestasis, cytolysis and eosinophilia. Serum amylase levels were normal. Abdominal ultrasound and CT scan revealed a 100 mm hydatid cyst, located in the head of the pancreas. The biliary and pancreatic ducts were markedly dilated. Exploratory laparotomy confirmed the diagnosis. A pancreatic fistula was discovered. Subtotal excision of the cyst and external drainage of the pancreatic fistula were performed. Laboratory tests were normal post-operatively. The pancreatic fistulae persisted for two months. Hypercaloric and hyperprotidic feeding resulted in occlusion in three weeks. The rest of the postoperative course was uneventful and the size of the biliary and pancreatic ducts was normal. The authors discuss the diagnostic features of pancreatic hydatid cyst and discuss therapeutic modalities.

[Glans reimplantation after circumcision accident]. / Réimplantation balanique après accident de circoncision.

INTRODUCTION: Circumcision, the most frequent operation in boys, is performed by various persons and is responsible for a considerable number of documented or non-documented complications. CASE REPORT: The authors report a case of accidental traumatic amputation of the glans during ritual circumcision performed by a nurse on a 5-year-old boy. Reimplantation was successfully performed after suture of the glans. In the light of this case and a review of the literature, the authors describe the treatment and prevention of this type of lesion. CONCLUSION: Circumcision is not a completely benign operation. Iatrogenic lesions are frequent but rarely serious. The best treatment is prevention, which requires education of the population about the importance of medicalized circumcision.

[Congenital mesoblastic nephroma with multiple cysts]. / Le néphrome mésoblastique congénital aux manifestations multikystiques.

Mesoblastic nephroma is a rare, benign congenital disease with a clinical presentation similar to that of Wilms tumour. It was described for the first time by BOLANDE in 1967. The authors report the case of a 6-month-old infant admitted for investigation of an abdominal mass. The radiological assessment (ultrasonography. CT) concluded on a right intrarenal retroperitoneal tumour occupying almost all of the abdominal cavity, predominantly cystic suggesting a diagnosis of non-metastatic Wilms tumour. Radical ureteronephrectomy was performed. The outcome was favourable with a follow-up of 2 years. Histological examination corrected the diagnosis to mesoblastic nephroma by showing a fibrous tumour composed of spindle cells resembling muscle cells, associated with intracystic haemorrhage. The authors emphasize the clinical and radiological features of this tumour, its treatment (exclusively surgical) and its good prognosis (98% survival).

[Neonatal congenital mega urethra]. / Méga-urètre congénital à révélation néonatale.

Congenital megaurethra is a rare mesenchymal anomaly of the male urethra, characterized by severe dilatation of the penile urethra. The authors review the embryogenesis and management of this abnormality in the light of a case of scaphoid megaurethra observed in a new-born infant with no associated congenital anomalies who was treated successfully.