Miller Oren F, Smith Loren J, Ferrara Elizabeth X, McAleer Irene M, Kaplan George W
Children's Hospital and Health Center, San Diego, CA, USA.
J Pediatr Surg. 2003 Nov;38(11):1685-8. doi: 10.1016/s0022-3468(03)00590-6.
Idiopathic fibrosis of the retroperitoneum is rare in childhood. The authors describe an 11-year-old boy who presented with progressive renal failure, bilateral hydronephrosis, hypertension, and elevated erythrocyte sedimentation rate (ESR) owing to retroperitoneal fibrosis. Ureterolysis was performed with improvement in his creatinine level and blood pressure. The soft tissue mass consisted of dense collagenous fibers consistent with retroperitoneal fibrosis. Postoperatively, he received steroids and azathioprine. Retroperitoneal fibrosis in the pediatric population is rare with only 23 cases reported in the English-language literature. Treatment includes pulsed steroid regimens, ureteral catheterization, and retroperitoneal exploration with ureterolysis. If allowed to progress, renal failure can result and lead to death. The etiology of retroperitoneal fibrosis in the pediatric patient may include autoimmune diseases, infection, and neoplasm, but most cases are idiopathic. Retroperitoneal fibrosis should be considered in patients with an elevated ESR, hypertension, renal failure, and hydronephrosis. Evaluation also should include a search for autoimmune diseases and malignancy.
小儿特发性腹膜后纤维化罕见。作者描述了一名11岁男孩，因腹膜后纤维化出现进行性肾衰竭、双侧肾积水、高血压及红细胞沉降率（ESR）升高。行输尿管松解术后，其肌酐水平和血压有所改善。软组织肿块由符合腹膜后纤维化的致密胶原纤维组成。术后，他接受了类固醇和硫唑嘌呤治疗。小儿腹膜后纤维化罕见，英文文献仅报道过23例。治疗包括脉冲类固醇疗法、输尿管插管以及行输尿管松解术的腹膜后探查。若任其发展，可导致肾衰竭并致死。小儿患者腹膜后纤维化的病因可能包括自身免疫性疾病、感染和肿瘤，但大多数病例为特发性。ESR升高、高血压、肾衰竭及肾积水患者应考虑腹膜后纤维化。评估还应包括排查自身免疫性疾病和恶性肿瘤。
