腹膜后纤维化诊治1例

Diagnosis and treatment of retroperitoneal fibrosis:A case report

目的提高对腹膜后纤维化罕见疾病的认识水平。方法报告并分析1例腹膜后纤维化IgG4相关硬化性疾病特征及诊治经过。患者左腰部疼痛1年,左下肢麻木3个月来诊。腹部CT考虑左侧腹膜后髂窝处占位性病变,左肾显示不清。行腹膜后肿物切除,受侵乙状结肠切除,降结肠造瘘,受侵左侧输尿管部分切除术。结果患者术后顺利恢复出院,病理为乙状结肠及肿块结肠黏膜慢性炎,结肠浆膜层及壁外见增生之纤维组织玻璃样变伴多量慢性炎细胞浸润结节。结论病因不明的腹膜后广泛进行性纤维组织增生是全身性免疫系统疾病的一种局部表现,手术切除病变解除压迫是治疗的可靠手段,后续治疗可尝试糖皮质激素、免疫抑制剂等。

Objective To improve the understanding of retroperitoneal fibrosis. Methods A patient with retroperitoneal fibrosis, an IgG4-related sclerosing disease was reported. Its clinical characteristics, diagnosis and treatment were analyzed. The patient visited our department due to left lumbar pain for 1 year and left lower extremity numbness for 3 months. Abdominal CT showed an occupying disease in retroperitoneal iliac fossa and unclear left kidney. The retroperitoneal tumor and the involved sigmoid colon were excised. Fistulization was performed for descending colon with the involved left ureter partly removed. Results The patient was recovered and discharged. The patient was diagnosed with chronic inflammation of sigmoid colon tumor and mucositis with fibrous tissue hyaline degeneration in colon serous layer accompanying massive inflammatory cell infiltrative nodules. Conclusion Progressive retroperitoneal fibrous tissue proliferation due to unknown causes is a local manifestation of systemic immune system diseases. Surgical excision is a reliable procedure for the removal of compression caused by the lesion, followed by glucocorticoid hormone and therapy