Case of the Fall Season H. S. Myers.
A
M.B
trations). A firm nontender nodular mass extended from deep in the pelvis up to the umbilicus; per anus, this mass was felt to be fixed and encircling the rectum. His blood pressure was 160/95 mm Hg. The urine contained pus, and a heavy growth of Gram-positive and Gram-negative bacteria was cultured from it. The excretory urogram and chest roentgenogram are shown in Figures 1 and 2. The patient died a few days after admission.
N AFRICAN male youth was brought to Groote Schuur Hospital from a remote primitive community; a missionary’s letter provided the only history available: “This boy has been deaf and dumb since he was a small child. His belly has been swelling for about 2 years.” Examination revealed that the patient was pyrexial and toxic. His skin had the blackish pigmentation normal for his race, with a few small brownish patches and many septic lacerations (thought to be due to witch doctors’ minis-
Fig. 1.
H. S. Myers, M.B.: Department of Radiology, Groote Schuur Hospital and Universiiy of Cape Town, Republic of South Africa. 0 1977 by Grune & Stratton, Inc. ISSN: 0037-198X. Seminars
in Roentgenology,
Vol. XII. No. 4 (October),
1977
263
264
H. S. MYERS
Fig. 2.
DIAGNOSIS: DIFFUSE NEUROFIBROMATOSIS WITH URINARY OBSTRUCTION, SACRAL INVASION, AND HYPERTENSION
The urogram shows a dilated, corrugated bladder; it is pushed to the left by a large mass that also displaces the right kidney and ureter. The kidneys are hydronephrotic. The sacrum appears expanded and has a soap bubble pattern. On the chest radiograph, there are lobulated mediastinal mass shadows and a scalloped contour at the periphery of the left lung suggesting pleural or extrapleural lesions. The radiologic diagnostic possibilities include lymphoma, neoplasia such as .sarcomatosis, and neurofibromatosis. The latter was supported by tomograms that showed widened internal auditory canals, most likely due to acoustic neuromas. Autopsy confirmed the presence of diffuse neurofibromatosis. A dysplastic neural mass encased the pelvic organs and invaded the sacrum. The bladder wall was studded with submucosal neurofibromas and was hypertrophied due to outlet obstruction. The proximal urinary tracts
were dilated. There were renal cortical abscesses and terminal pyemia. The middle and posterior mediastinum contained neurofibromatous masses, and there were several intercostal neurofibromas. The wall of the left ventricle was hypertrophied. The radiologic spectrum of neurofibromatosis was comprehensively reviewed in a recent issue The present case of these Seminars.’ demonstrates (1) urinary tract involvement, which is rare; (2) bone defect due to contiguous mass invasion, not primary osseous dysplasia or intrinsic bone tumor; (3) posterior mediastinal neurofibromas, which are less common than lateral thoracic meningocele in von Recklinghausen disease; and (4) the hypertension that may occur in this disease in children, usually due to renovascular lesions.
REFERENCE 1. Klatte spectrum 11:17-33,
EC, Franken EA, in neurofibromatosis. 1976
Smith
JA: Semin
Radiographic Roentgen01
A
M.B
trations). A firm nontender nodular mass extended from deep in the pelvis up to the umbilicus; per anus, this mass was felt to be fixed and encircling the rectum. His blood pressure was 160/95 mm Hg. The urine contained pus, and a heavy growth of Gram-positive and Gram-negative bacteria was cultured from it. The excretory urogram and chest roentgenogram are shown in Figures 1 and 2. The patient died a few days after admission.
N AFRICAN male youth was brought to Groote Schuur Hospital from a remote primitive community; a missionary’s letter provided the only history available: “This boy has been deaf and dumb since he was a small child. His belly has been swelling for about 2 years.” Examination revealed that the patient was pyrexial and toxic. His skin had the blackish pigmentation normal for his race, with a few small brownish patches and many septic lacerations (thought to be due to witch doctors’ minis-
Fig. 1.
H. S. Myers, M.B.: Department of Radiology, Groote Schuur Hospital and Universiiy of Cape Town, Republic of South Africa. 0 1977 by Grune & Stratton, Inc. ISSN: 0037-198X. Seminars
in Roentgenology,
Vol. XII. No. 4 (October),
1977
263
264
H. S. MYERS
Fig. 2.
DIAGNOSIS: DIFFUSE NEUROFIBROMATOSIS WITH URINARY OBSTRUCTION, SACRAL INVASION, AND HYPERTENSION
The urogram shows a dilated, corrugated bladder; it is pushed to the left by a large mass that also displaces the right kidney and ureter. The kidneys are hydronephrotic. The sacrum appears expanded and has a soap bubble pattern. On the chest radiograph, there are lobulated mediastinal mass shadows and a scalloped contour at the periphery of the left lung suggesting pleural or extrapleural lesions. The radiologic diagnostic possibilities include lymphoma, neoplasia such as .sarcomatosis, and neurofibromatosis. The latter was supported by tomograms that showed widened internal auditory canals, most likely due to acoustic neuromas. Autopsy confirmed the presence of diffuse neurofibromatosis. A dysplastic neural mass encased the pelvic organs and invaded the sacrum. The bladder wall was studded with submucosal neurofibromas and was hypertrophied due to outlet obstruction. The proximal urinary tracts
were dilated. There were renal cortical abscesses and terminal pyemia. The middle and posterior mediastinum contained neurofibromatous masses, and there were several intercostal neurofibromas. The wall of the left ventricle was hypertrophied. The radiologic spectrum of neurofibromatosis was comprehensively reviewed in a recent issue The present case of these Seminars.’ demonstrates (1) urinary tract involvement, which is rare; (2) bone defect due to contiguous mass invasion, not primary osseous dysplasia or intrinsic bone tumor; (3) posterior mediastinal neurofibromas, which are less common than lateral thoracic meningocele in von Recklinghausen disease; and (4) the hypertension that may occur in this disease in children, usually due to renovascular lesions.
REFERENCE 1. Klatte spectrum 11:17-33,
EC, Franken EA, in neurofibromatosis. 1976
Smith
JA: Semin
Radiographic Roentgen01
