Résumé
Von Hippel-Lindau disease (VHL) is a rare, autosomal dominantly transmitted familial, hereditary disease. This disease genetically predisposes affected people to the development of some types of tumor, such as cerebellar, spinal and medullary hemangioblastomas, and retinal angiomas, renal cell carcinomas and pheochromocytomas. Herein, a case of a cystic renal cell carcinoma, accompanied by Von Hippel-Lindau disease, treated by nephron sparing surgery, is reported. The VHL gene mutation of this case was also identified.
Sujets)
Néphrocarcinome , Maladies génétiques congénitales , Hémangioblastome , Hémangiome , Néphrons , Phéochromocytome , Rétinal , Maladie de von Hippel-LindauRésumé
PURPOSE: Despite the increasing incidence of renal trauma, the management of renal injuries still remains controversial. Because the renal anatomy of children is different to that of adults, the management of their renal injuries will differ. We attempted to clarify the efficacy of conservative management for pediatric renal trauma by comparing our experiences of conservative and operative managements. MATERIALS AND METHODS: The medical records of 46 patients, with blunt renal injuries, were retrospectively reviewed. All patients were evaluated by computed tomographic scanning. Injuries were graded according to the American Association for the Surgery of Trauma Organ Injury Scale. RESULTS: 37 patients were initially managed conservatively and 9 by an early operation. 35 of the 37 patients (94.6%) were successfully managed conservatively, but the other 2 required a delayed operation (1 renorrhaphy, 1 open clot drainage), but a delayed nephrectomy was not required. Two of the 3 patients with urine leakage spontaneously resolved, and the other was resolved with endoscopic ureteral stenting. 7 of the 9 patients having undergone an early operation resulted in a nephrectomy, but kidney preservation was only possible in 2. CONCLUSIONS: Except for the hemodynamically unstable patients and those with a pedicle injury, conservative management was effective from the view point of renal preservation. Major renal lacerations and most of the urine leakages resolved spontaneously. Persistent extravasation can be managed with minimally invasive treatment, such as ureteral stenting. We consider the conservative management of renal injuries, even with abdominal exploration, for the treatment of associated organ injuries.
Sujets)
Adulte , Enfant , Humains , Incidence , Rein , Lacérations , Dossiers médicaux , Néphrectomie , Pédiatrie , Études rétrospectives , Endoprothèses , UretèreRésumé
Crossed testicular ectopia is an uncommon anatomical abnormality in which both gonads migrate toward the same hemiscrotum. We report a case of crossed testicular ectopia associated with bilateral cryptorchism. The crossed ectopic and intra-abdominal testis was fixed in it's own hemiscrotum by laparoscopic orchiopexy.