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Retroperitoneal fibrosis in children
RETROPERITONEAL JEFF JOHN DEBRA
WACKSMAN, L. WEINERTH, KREDICH,
FIBROSIS
IN CHILDREN
M.D. M.D. M.D.
From the Department of Urology and Children’s Diagnostic Center, Cleveland Clinic Foundation, Cleveland, Ohio, and Departments of Urology and Pediatrics, Duke University Medical Center, Durham, North Carolina
ABSTRACT $rst msociuted discussed.
1Ve report the seventh documented with juwnile rheumatoid wthritis.
Retroperitoneal fibrosis in children is rare. The following case report is of a girl with retroperitoneal fibrosis associated with rheumatoid arthritis. Case Report A seven-year-old white girl with a past history of severe juvenile rheumatoid arthritis had been treated with large doses of aspirin. She was ad1975, with severe hemamitted in August, temesis and melena. Physical examination revealed a pale girl with limited joint motion and venous congestion over the neck and anterior chest wall. Abdominal examination revealed hematosplenomegaly and hyperactive bowel sounds. After hospitalization the following laboratory data were obtained: hemoglobin 8.2 Gm. per 100 ml., hematocrit 24, white blood cell count 15,700; urinalysis showed pH 5, negative for protein, microscopic trace of sugar, blood urea nitrogen 11 mg. per 100 ml., prothrombin time 12112 sec., partial thromboplastin time 35134 sec. The patient received adequate blood transfusion and gastrointestinal bleeding gradually was resolved. Upper gastrointestinal series showed a prepyloric ulcer. Because of the dilated neck and anterior chest wall veins, the diagnosis of superior vena caval obstruction secondary to sclerosing mediastinitis was suspected. A superior vena cavogram was ob-
cuw of r~troperiton~alJibrosis in a child clntl the> The literature is reciewctl and current therqxy is
tained and confirmed the superior alid inferior vena caval obstruction. Follow-up plain film illustrated bilateral ureteral obstruction and complete intravenous pyelogram confirmed this impression (Fig. 1A). A cystogram was negative. The patient was eventually discharged and enteric-coated aspirin and antacid therapy was In one month, a follow-up intraprescribed. venous pyelogram showed no change. At this point the patient was prescribed prednisone therapy (2 mg./Kg. body weight) and continual antacids. Three months after therap), the ureteral obstruction markedly decreased (Fig. 1B). Currently the patient is doing well on antacids and decreasing prednisone therapy. Comment Farrer and Peterson in 1962l reported the first case of idiopathic retroperitoneal fibrosis in an eight-year-old child with left ureteral obstruction. Since that time, there have been five additional case reports.“” Cerny and Scott” and Hache, Woolner, and Bernatz3 reported cases both of which were associated with Henoch-Schiinlein purpura. Lloyd rt al. 4 subsequently reported on a seventeen-yearold Oriental female with retroperitoneal fibrosis and systemic lupus erythematosis with marked resolution of hydronephrosis after steroid therapy. The association of retroperitoneal fibrosis and sclerosing mediastinitis
in adults is ~vell recognized. 7-J” and tht> associutioll of retroperitoneal fibrosis with other c*onntbcti\v tissue diseases in aclrdts has t,cYn rqx”~tf?‘1. Thib c’ilsc is the, first reported of’ retropc’ritoneal fibrosis associated with juvenile rhcunintoicl arthritis and sclerosing niediastinitis. The s~~ssf~ll 11s~ of steroid therapy to manage ~c.tro~~~~l.itoll~~~ll filxosis is ~vell documented. ‘l” and our case further illrlstrates thch \duca of antiAlinflaniniator~agents to treat this distbase. though this diseasfis rare ill children, xve l)c~lie\;t~ l.etroperiton~al fibrosis should 1x2 suspetted in childrrn \vith systemic connective tissue tlisease and 1,ilateral diffuse rlreteral obstruction. \I’rl also lxlieve that steroid therap can he helpful in the n~anag~~ment of this tlisr~asc~ pt-ocess.
WACKSMAN, L. WEINERTH, KREDICH,
FIBROSIS
IN CHILDREN
M.D. M.D. M.D.
From the Department of Urology and Children’s Diagnostic Center, Cleveland Clinic Foundation, Cleveland, Ohio, and Departments of Urology and Pediatrics, Duke University Medical Center, Durham, North Carolina
ABSTRACT $rst msociuted discussed.
1Ve report the seventh documented with juwnile rheumatoid wthritis.
Retroperitoneal fibrosis in children is rare. The following case report is of a girl with retroperitoneal fibrosis associated with rheumatoid arthritis. Case Report A seven-year-old white girl with a past history of severe juvenile rheumatoid arthritis had been treated with large doses of aspirin. She was ad1975, with severe hemamitted in August, temesis and melena. Physical examination revealed a pale girl with limited joint motion and venous congestion over the neck and anterior chest wall. Abdominal examination revealed hematosplenomegaly and hyperactive bowel sounds. After hospitalization the following laboratory data were obtained: hemoglobin 8.2 Gm. per 100 ml., hematocrit 24, white blood cell count 15,700; urinalysis showed pH 5, negative for protein, microscopic trace of sugar, blood urea nitrogen 11 mg. per 100 ml., prothrombin time 12112 sec., partial thromboplastin time 35134 sec. The patient received adequate blood transfusion and gastrointestinal bleeding gradually was resolved. Upper gastrointestinal series showed a prepyloric ulcer. Because of the dilated neck and anterior chest wall veins, the diagnosis of superior vena caval obstruction secondary to sclerosing mediastinitis was suspected. A superior vena cavogram was ob-
cuw of r~troperiton~alJibrosis in a child clntl the> The literature is reciewctl and current therqxy is
tained and confirmed the superior alid inferior vena caval obstruction. Follow-up plain film illustrated bilateral ureteral obstruction and complete intravenous pyelogram confirmed this impression (Fig. 1A). A cystogram was negative. The patient was eventually discharged and enteric-coated aspirin and antacid therapy was In one month, a follow-up intraprescribed. venous pyelogram showed no change. At this point the patient was prescribed prednisone therapy (2 mg./Kg. body weight) and continual antacids. Three months after therap), the ureteral obstruction markedly decreased (Fig. 1B). Currently the patient is doing well on antacids and decreasing prednisone therapy. Comment Farrer and Peterson in 1962l reported the first case of idiopathic retroperitoneal fibrosis in an eight-year-old child with left ureteral obstruction. Since that time, there have been five additional case reports.“” Cerny and Scott” and Hache, Woolner, and Bernatz3 reported cases both of which were associated with Henoch-Schiinlein purpura. Lloyd rt al. 4 subsequently reported on a seventeen-yearold Oriental female with retroperitoneal fibrosis and systemic lupus erythematosis with marked resolution of hydronephrosis after steroid therapy. The association of retroperitoneal fibrosis and sclerosing mediastinitis
in adults is ~vell recognized. 7-J” and tht> associutioll of retroperitoneal fibrosis with other c*onntbcti\v tissue diseases in aclrdts has t,cYn rqx”~tf?‘1. Thib c’ilsc is the, first reported of’ retropc’ritoneal fibrosis associated with juvenile rhcunintoicl arthritis and sclerosing niediastinitis. The s~~ssf~ll 11s~ of steroid therapy to manage ~c.tro~~~~l.itoll~~~ll filxosis is ~vell documented. ‘l” and our case further illrlstrates thch \duca of antiAlinflaniniator~agents to treat this distbase. though this diseasfis rare ill children, xve l)c~lie\;t~ l.etroperiton~al fibrosis should 1x2 suspetted in childrrn \vith systemic connective tissue tlisease and 1,ilateral diffuse rlreteral obstruction. \I’rl also lxlieve that steroid therap can he helpful in the n~anag~~ment of this tlisr~asc~ pt-ocess.