CAS CLINIQUE / CASE REPORT IDIOPATHIC RETROPERITONEAL FIBROSIS PRESENTING AS RECURRENT SCROTAL EDEMA Report of a Case and Review of the Literature

Primary or idiopathic retroperitoneal fibrosis, first described by Ormond in 1948, is a rare and elusive diagnosis, requiring a high level of suspi- cion. Patients usually present with entrapment of re- troperitoneal organs, the most common being the ureters, causing hydronephrosis and acute kidney injury. Here, we present the case of a 56-year-old male presenting for recurrent and intermittent scrotal edema. Upon routine laboratory workup, he was found to have an elevated creatinine level. Imaging showed encasement of bilateral ureters. Bilateral ureteral stents were placed with relief of his obstruc- tive uropathy, followed by normalization of creati- nine. The patient later underwent laparoscopic release of retroperitoneal adhesions. Biopsies taken from the operative site showed fibroblast proliferation and elements of acute and chronic inflammation. With further workup of etiologies being negative, he was diagnosed with idiopathic retroperitoneal fibrosis. RESUME : La fibrose retroperitoneale idiopathique, d'abord decrite par Ormond en 1948, est un diagnos- tic rare et difficile, necessitant un haut niveau de sus- picion. Les patients se presentent habituellement avec une compression des organes retroperitoneaux, les plus communs etant les ureteres, resultant en une hydronephrose, et une insuffisance renale aigue. Nous decrivons ici le cas d'un patient de 56 ans qui s'est presente pour un œdeme scrotal recurrent et in- termittent. Le bilan de routine revela une insuffisance renale aigue. Un CT scan abdominal a montre l'ob- struction des deux ureteres. L'uropathie obstructive disparut apres l'insertion de deux sondes double J entrainant la normalisation de la creatinine. Le patient a subi plus tard une ureterolyse lapa- roscopique. Les biopsies prelevees ont montre une proliferation de fibroblastes et d'elements resultant d'une inflammation aigue et chronique. Un bilan plus approfondi pour exclure d'autres etiologies s'etant revele negatif, le diagnostic fut celui d'une fibrose retroperitoneale idiopathique. Mots-cles : insuffisance renale aigue, œdeme scrotal, syndrome inflammatoire, ureterolyse