Retroperitoneal fibrosis in children (Eosinophilic and idiopathic)

Diagnosis and Management of Eosinophilic Cystitis

“…In some patients a mass is palpable during examination of the lower abdomen or digital rectal examination. This was the case in 15 patients [11, 31, 32, 33, 42, 43, 45, 50, 54, 58, 62, 63, 65, 66, 76]. In 2 patients a previously unknown prostatic enlargement was detected [46, 59].…”

“…We collected 81 articles from the international literature containing one or more cases of patients with EC [2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82]. A total of 153 patients were mentioned in these articles.…”

“…One enterovesical fistula developed in a patient with EC due to an allergic reaction to chromic catgut used in a previous abdominal operation [23]. Retroperitoneal fibrosis which contained an eosinophilic inflammation obstructing the ureter was described in 1 patient [63]. Two patients died of EC: 1 patient died of complications of cytotoxic therapy [50]and the other patient also had multiple eosinophilic granulomata in the bowel, lymph nodes, mesenterium, ovaries, uterus and heart and died of bowel complications [28].…”

“…One patient went into a uremic coma due to renal insufficiency [32]. Vesicoureteral reflux was present in 6 patients, but this may have been preexistent [18, 29, 32, 42, 63, 73]. Four patients needed blood transfusions because of severe hematuria; 1 patient had a bleeding disorder (Glanzmann’s thrombasthenia) [12]; 2 other patients eventually needed a cystectomy because of hematuria unresponsive to conservative therapy [62].…”

Retroperitoneal fibrosis – an unsolved mystery

“…There is a male preponderance (3:1) (7) and it is typically found in the age group of 40-60 years (8). There had been a few cases presenting below the age of 18 as well (9). Almost 70% of RPF cases are Idiopathic with 8 to 10% being malignant and the rest due to inflammation or medication.…”