Okpii Emmanuel, Okpii Kingsley, Adamu-Biu Fatima
Urology, North West Anglia NHS Foundation Trust, Peterborough, GBR.
Respiratory Medicine, University Hospitals of Leicester NHS Trust, Leicester, GBR.
Cureus. 2022 Aug 19;14(8):e28187. doi: 10.7759/cureus.28187. eCollection 2022 Aug.
Retroperitoneal fibrosis is a rare disease with a largely unknown aetiology, varying presentation, and is characterized by fibrous tissue formation in the retroperitoneal region. It causes entrapment and obstruction of retroperitoneal tubular structures, notably the ureters, and has been associated with autoimmune disorders. We report a 52-year-old male who was admitted to the emergency department with a seven-day history of lower abdominal pain, anorexia, and unintentional weight loss. Routine blood work revealed the patient to have acute kidney injury, and an unenhanced computed tomography scan of the abdomen showed bilateral hydronephrosis (grades 1 and 3 on the right and left, respectively) caused by a soft tissue mass in the retroperitoneal region. This mass was investigated with further imaging and a core biopsy, which confirmed retroperitoneal fibrosis. He is currently being planned for ureterolysis after a poor response to steroid therapy under the nephrology team. Urinary diversion was achieved with bilateral nephrostomies following unsatisfactory drainage with bilateral ureteric stents. This case highlights some of the difficulties that may be encountered in the management of retroperitoneal fibrosis.
腹膜后纤维化是一种罕见疾病，病因大多不明，临床表现各异，其特征是腹膜后区域形成纤维组织。它会导致腹膜后管状结构（尤其是输尿管）受压和梗阻，并与自身免疫性疾病有关。我们报告一名52岁男性，因下腹痛、厌食和非故意体重减轻7天而入住急诊科。常规血液检查显示该患者患有急性肾损伤，腹部非增强计算机断层扫描显示腹膜后区域的软组织肿块导致双侧肾积水（右侧和左侧分别为1级和3级）。通过进一步的影像学检查和核心活检对该肿块进行了评估，确诊为腹膜后纤维化。在肾病团队的治疗下，他对类固醇治疗反应不佳，目前正计划进行输尿管松解术。在双侧输尿管支架引流效果不佳后，通过双侧肾造瘘实现了尿液改道。该病例突出了腹膜后纤维化管理中可能遇到的一些困难。
