A rare case of an appendiceal mass masquerading as a pelvic tumour and causing bilateral hydronephrosis

Diagnosing acute appendicitis in children can be difficult due to atypical presenting symptoms. While there are reported cases of acute appendicitis or appendiceal masses causing unilateral hydronephrosis, bilateral hydronephrosis as a complication of appendiceal mass is very rare. We report a case of a child who presented with cardinal symptomatology associated with the urogenital tract. Ultrasound (US) investigation showed a pelvic mass causing bilateral hydronephrosis. An initial diagnosis of a pelvic teratoma was made based on the US and computed tomography (CT) scan findings. The final diagnosis of an appendiceal mass causing bilateral hydronephrosis was established intraoperatively.

Congenital Nephrogenic Diabetes Insipidus Presented with Bilateral Hydronephrosis: Genetic Analysis of V2R Gene Mutations

Most cases of hydronephrosis are caused by urinary tract obstruction. However, excessive polyuric syndrome rarely gives rise to non-obstructive hydronephrosis, megaureter, and a distended bladder. The authors report here on two cases of congenital nephrogenic diabetes insipidus (NDI) with severe bilateral hydronephrosis and megaureter. It is Interesting that the patients were symptomless except for their polyuria, and they both presented with bilateral hydronephrosis. Fluid deprivation testing revealed the presence of AVP resistant NDI. Gene analysis for these patients showed the AVP receptor 2 (V2R) missense mutations (Q225X and S126F), which have previously been reported on in other studies. We made the diagnosis of NDI by using a physiologic test, and we confirmed it by mutation analysis of the V2R gene.

A Case of Congenital Nephrogenic Diabetes Insipidus with Bilateral Hydronephrosis and Hydroureter / 대한신장학회잡지

We describe a case of congenital nephrogenic diabetes insipidus with severe dilatation of bilateral urinary tracts without anatomical obstructions. Functional obstruction can be occurred when polyuria surpasses the transporting ability of urine in the urinary tract. The patient was admitted to our hospital due to decreased mentality developed after traffic accident. On radiologic study, bilateral hydronephrosis and hydroureter were noted. Because the patient excreted copious dilute urine, we performed water deprivation test and the result was consistent with nephrogenic diabetes insipidus. We are presenting this case in an attempt to describe strong association between congenital diabetes insipidus and nonobstructive hydronephrosis in which polyuria is responsible for the hydronephrosis.

A Case of Erdheim-Chester Disease with Bilateral Hydronephrosis / 대한비뇨기과학회지

Erdheim-Chester disease (ECD) is a disseminated xanthogranulomatous infiltrative disease of unknown origin which reveals the infiltration of different organs and bones by foamy histiocytes. Patients with ECD have specific skeletal X-ray findings, with characteristic osteosclerosis of long bones, and histiocytes stained negative for S-100 antibody and electron microscopy of their cytoplasm does not reveal Birbeck granules. ECD represents variable clinical manifestations due to systemic involvement including the liver, spleen, pancreas, peritoneum, lung, pericardium, retroperitoneum and kidney. We report a case of ECD with bilateral hydronephrosis treated with the insertion of double J catheter following to the steroid based chemotherapy with a brief review of the literature.