[Retroperitoneal fibrosis at the Ibn Sina University Hospital of Rabat: about 18 cases]. / Fibrose rétropéritonéale au CHU Ibn Sina de Rabat: à propos de 18 cas.

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the formation of a fibro-inflammatory plaque in the retroperitoneal space in front of the abdominal aorta. It is responsible for the sheathing of the ureters. It is characterized by unspecific clinical signs, and it is often detected based on obstructive uropathy. We conducted a retrospective, descriptive study in the Department of Urology B and in the Department of Nephrology of the Ibn Sina University Hospital over a period of 10 years from January 2006 to December 2016. The study enrolled 18 patients, including 11 men and 7 women, with an average age of 51.4 years ± 11.2. Diagnosis was based on lumbar pain in 14 patients. Obstructive renal failure was reported in 15 patients and the diagnosis of RPF was based on uroscanner. Etiological assessment revealed 2 cases with a history of neoplasia, 2 cases of inflammatory disease and 1 case of retroperitoneal surgery; chronic drug intake was noted in more than half of the patients. In all patients, treatment was based on double J stent placement, while systemic treatment with corticosteroids and immunosuppressants was used on the basis of the evolutionary profile. Ureterolysis was immediately performed in 3 patients. Outcome was favorable, with significant improvement in renal function in 12 patients. A relapse occurred in 2 patients after 2 years of follow-up. Retroperitoneal fibrosis (RPF) should be suspected in patients with general signs associated with obstructive renal disease. Secondary causes should be systematically investigated, with particular emphasis on hyper IgG4 disease and neoplastic diseases.

[Malignant renal epithelioid angiomyolipoma (EAML): about a rare case]. / Angiomyolipome rénal épithélioïde malin (AREM): à propos d'un cas rare.

Malignant renal epithelioid angiomyolipoma (EAML) is a rare disease which has long been considered a hamartomatous lesion. Positive diagnosis is purely based on immunohistochemical investigation. Microscopically, it is characterized by mononuclear epithelioid cells showing a clear cytoplasm with severe cytonuclear atypies and expressing smooth muscle cell markers and melanocytic markers (HMB 45). We here report a rare case of EAML diagnosed after anatomopathological examination of a retroperitoneal tumor whose origin wasn't predetermined by CT scan.

[Favorable evolution after medical treatment in three cases of emphysematous pyelonephritis: about 3 cases]. / La pyélonéphrite emphysémateuse à évolution favorable après traitement médical: à propos de 3 observations.

Emphysematous pyelonephritis is a necrotic infection of the kidney characterized by the presence of gas within the renal parenchyma, the secretory cavities or the perirenal spaces. It is a severe condition that could be life-threatening. Patient's management remains controversial and is based either on surgery or on medical treatment. Percutaneous drainage can be also used for the treatment of these patients. We report 3 cases with EPN treated with only antibiotics. Patients' evolution was favorable. These results show that medical treatment could be sufficient.

[Non-secreting paraganglioma retroperitoneal]. / Paragangliome non sécrétant rétropéritonéal: à propos d'une observation.

Non-functional retroperitoneal paragangliomas are rare tumors. They are often asymptomatic and can reach very large sizes. We report the case of a 49-year old woman with retroperitoneal tumor detected during CT scan examination performed to find the cause of non-specific abdominal pain. Malignant forms, more frequent than benign forms, show locoregional invasion and are characterized by delayed-onset metastases. The treatment of these tumors is based on the most complete degree of surgical resection, since prognosis depends on it. There is no consensus on the usefulness of complementary therapies which may nevertheless constitute a supportive treatment.

Complete androgen insensitivity syndrome or testicular feminization: review of literature based on a case report.

Testicular feminization, or the androgen insensitivity syndrome, is a rare disease. Because of various abnormalities of the X chromosome, a male, genetically XY, has some physical characteristics of a woman or a full female phenotype. Indeed the androgen insensitivity syndrome occurs because of a resistance to the actions of the androgen hormones, which in turn switches the development towards the aspect of a woman. We report a case of complete androgen insensitivity syndrome in a 30 years old woman who presented primary amenorrhea. We aim to improve our knowledge of this illness from the data that provides us this study, and a review of the literature.

Bilateral kidney matrix stones: a rare case.

Kedney matrix stones are a rare form of calculi. Flank pain and urinary tract infections (UTI) are the most common presentations of matrix calculi. The diagnosis is usually made at surgery, but some preoperative radiographic findings might be suggestive. Open surgery was the method of choice for treatment. However, combination of ureterorenoscopy and percutaneous nephrolithotomy (PCNL) was found to be safe and effective. We report a rare case of renal and ureteral matrix stones that were diagnosed and treated by open surgery. We also describe its clinical, radiological and therapeutic features through a review of the literature.

Partial substitution of the ureter using a double short segments of the ileum following the Monti procedure.

The partial substitution of the ureter using a pediculated double short segments of the ileum is a technique used to re-establish ureteral transit and preserve the renal unit, following the resection of extensive ureteral lesions. Standard surgical procedure for an ileoureteroplasty consists of isolating an ileal duct of equal or greater length than the ureteral defect and interposing it in the urinary tract in an isoperistaltic direction. Monti described a surgical technique that allows for the creation of catheterizable stomas in continent urinary diversions, using the Mitrofanoff principle. These passageways were created from one or several 2.5 cm long ileal sections by means of their detubulization and transverse retubulization.

Cavernous hemangioma of the bladder: an additional case managed by partial cystectomy and augmentation cystoplasty.

Cavernous Hemangioma of the Bladder (CHB) is benign and rare lesions. Clinical presentation has no pathognomonic signs although gross painless hematuria is the most frequent complain. CHB is suspected by cystoscopy and radiologic findings and confirmed by pathologic examinations. Management is controversial due to the bleeding risk of this highly vascularized lesion. Partial cystectomy is the treatment of choice for surgically accessible lesions. However, it appears that small lesions could be treated using transurethral resection. Since CHB is a rare case, we report another case treated successfully with a partial cystectomy associated with an augmentation cystoplasy.