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Presentation of idiopathic retroperitoneal fibrosis in the pediatric population
Oren F Miller1, Loren J Smith, Elizabeth X Ferrara
1Children's Hospital and Health Center, San Diego, CA, USA.
Insights
Idiopathic retroperitoneal fibrosis is a rare childhood condition causing kidney failure and high blood pressure. Surgical ureterolysis and medical treatment can improve outcomes in pediatric patients.
Area of Science:
- Pediatric Nephrology
- Urology
- Pathology
Background:
- Idiopathic retroperitoneal fibrosis is exceptionally rare in children.
- It can lead to severe complications including renal failure and hypertension.
Observation:
- An 11-year-old boy presented with progressive renal failure, bilateral hydronephrosis, and hypertension.
- Elevated erythrocyte sedimentation rate (ESR) was noted, indicating inflammation.
Findings:
- Retroperitoneal fibrosis was diagnosed, characterized by a dense collagenous tissue mass.
- Surgical ureterolysis resulted in improved creatinine levels and blood pressure.
- Histopathology confirmed dense collagenous fibers consistent with fibrosis.
Implications:
- Early consideration of retroperitoneal fibrosis is crucial in pediatric patients with unexplained renal failure, hypertension, and hydronephrosis.
- Treatment involves surgical intervention (ureterolysis) and immunosuppressive therapy (steroids, azathioprine).
- Prompt diagnosis and management are vital to prevent irreversible renal damage and mortality.
Abstract:
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.