Urol Radiol 12:106-108 (1990)
Urologic Radiology © Springer-VerlagNewYorkInc. 1990
Ultrasound Findings in Diffuse Granulomatous Cystitis in Chronic Granulomatous Disease of Childhood R.T. Speirs, B.N. Raghavendra, A. Rausen, N.B. Geneiser, and M. Ambrosino Department of Radiology, Bellevue Hospital, New York, New York, USA
Abstract. Chronic granulomatous disease (CGD) is a genetically inherited disease of childhood, which is characterized by repeated bacterial and/or fungal infections. The genitourinary tract is rarely involved. We report a case of CGD with involvement of the urinary bladder. Pertinent clinical and sonographic findings of this disease are described. Key words: Chronic granulomatous disease -Cystitis, ultrasound -- Granulomatous cystitis.
of the mucosal surface. A cystogram (Fig. 2) showed an irregular bladder contour with nodularity of the dome, and no evidence of vesicoureteral reflux. Cystoscopy demonstrated patchy areas of nodularity throughout the bladder. Biopsies performed from multiple sites showed chronic inflammation with multiple intramural granulomata. The patient was put on an oral regimen of cortisone. Substantial clinical improvement was noted at the end of 2 weeks. A repeat sonogram of the bladder (Fig. 3) showed marked resolution of the previously noted findings in the bladder wall. The bladder volume was also markedly increased since the earlier study.
Discussion Case Report A 3-year-old boy was admitted to the hospital because of urinary urgency, frequency, dysuria, enuresis, and incontinence of 3 to 4 weeks' duration. The diagnosis of CGD had been made at the age of 2 weeks by nitroblue tetrazolium test, which was performed because of a family history of CGD, including a brother with the disease. The patient has received daily trimethoprim-sulfamethoxazole since that time. At the age of 7 months, evaluation for guaiac-positive stool revealed lymphoid hyperplasia of the colon, as well as generalized lymph node enlargement, and retroperitoneal lymph node node enlargement with abscesses had been documented at the age of 13 months. Repeated skin infections due to Staphylococcus aureus had occurred between the ages of 18 and 24 months. On admission, the patient was afebrile, and physical examination was unremarkable. The white blood cell count was 14,400. Urinalysis revealed a pH of 8, specific gravity of 1.020, 2+ proteinuria, with 0-4 white blood cells and 0-4 red blood cells per high-power field. Urine cultures were negative. An ultrasound examination of the pelvis (Fig. 1) showed diffuse, irregular thickening of the bladder wall, with nodularity
Address reprint requests to: R. T. Speirs, M.D., Department of Radiology, Bellevue Hospital, 27th Street at First Avenue, New York, NY 10016, USA
CGD is an X-linked recessively inherited disorder ofneutrophil function. Phagocytosis remains intact, but ingested bacteria cannot be killed. The disease usually presents in the first year of life, and common manifestations include hepatosplenomegaly, lymphadenopathy, gastrointestinal and respiratory tract infections, osteomyelitis, and skin infections, especially around body orifices. Staphylococci are the most common infectious agent [1, 2]. The diagnosis is generally established by the nitroblue tetrazolium test [2]. Genitourinary involvement is rare. Cystitis and pyelonephritis may be seen when the genitourinary tract is involved [3, 4]. Patients with granulomatous cystitis often present with hematuria, dysuria, frequency, and enuresis [3-7]. Physical examination may reveal a suprapubic mass, representing the inflamed and thickened bladder, but it is generally unremarkable. Cystography will show a small bladder capacity with an irregular mucosal surface in the involved area. Vesicoureteral reflux may be seen and may cause hydronephrosis [3, 6]. Cystoscopy
R.T. Speirs et al.: Granulomatous Cystitis
107
Fig. 1. Longitudinal (A) and transverse (B) views from a pelvic sonogram reveal diffuse thickening of the bladder wall, with irregular mucosal surface.
an
Fig. 2. An anteroposterior view from a cystogram shows marked nodularity of the bladder dome, Fig. 3. Transverse view from a pelvic sonogram following therapy shows a return of bladder wall thickness to normal, as well as an increase in bladder capacity compared to the prior scan.
generally reveals erythematous, inflamed mucosa, and biopsies show acute and chronic inflammation associated with intramural granulomata [5]. Therapy includes antibiotics to treat an identified or suspected infectious agent, as well as steroids to suppress the inflammation [4, 5, 7]. In our case, ultrasound clearly demonstrated diffuse thickening with nodularity of the bladder wall. The involved areas in cases of focal granulomatous cystitis described by Hassel et al. [7] and by Haller
and Schneider [8] demonstrated similar sonographic findings. Ultrasound was also of use in monitoring the response to therapy, which was quite remarkable in this case. We conclude that ultrasonography is an effective noninvasive modality for the diagnosis of granulomatous cystitis. Ultrasonography is a painless procedure and is not associated with ionizing radiation. It can be used as often as necessary and is particularly valuable in monitoring the response to treatment.
108 References
1. Berendes H, Bridges RA, Good RA: A fatal granulomatous disease of childhood: Clinical study of a new syndrome. Minn Med 40:309, 1957 2. Good RA, Quie PG, Windhorst DB, et al.: Fatal (chronic) granulomatous disease of childhood: A hereditary defect of leukocyte function. Semin Hematol 5:215, 1968 3. Forbes GS, Hartman GW, Burke EC, et al.: Genitourinary involvement in chronic granulomatous disease of childhood. AJR 127:683, 1976
R.T. Speirs et al.: Granulomatous Cystitis 4. Young AK, Middleton RG: Urologic manifestationsof chronic granulomatous disease of infancy. J Urol 123:119, 1980 5. Kontras SB, Bodenbender JG, McClave CR, etaL: Interstitial cystitis in chronic granulomatous disease. J Urol 105:575, 1971 6. Cyr WL, Johnson H, Balfour J: Granulomatous cystitis as a manifestationof chronic granulomatous disease of childhood. J Urol 110:357, 1973 7. Hassel DR, Glasier CM, McConneU JR: Granulomatous cystitis in chronic granulomatous disease: Ultrasound diagnosis. Pediatr Radiol 17:254, 1987 8. Haller JO, Schneider M: Pediatric Ultrasound. Chicago: Year Book Medical Publishers, 1980, pp 125, 158-159
Urologic Radiology © Springer-VerlagNewYorkInc. 1990
Ultrasound Findings in Diffuse Granulomatous Cystitis in Chronic Granulomatous Disease of Childhood R.T. Speirs, B.N. Raghavendra, A. Rausen, N.B. Geneiser, and M. Ambrosino Department of Radiology, Bellevue Hospital, New York, New York, USA
Abstract. Chronic granulomatous disease (CGD) is a genetically inherited disease of childhood, which is characterized by repeated bacterial and/or fungal infections. The genitourinary tract is rarely involved. We report a case of CGD with involvement of the urinary bladder. Pertinent clinical and sonographic findings of this disease are described. Key words: Chronic granulomatous disease -Cystitis, ultrasound -- Granulomatous cystitis.
of the mucosal surface. A cystogram (Fig. 2) showed an irregular bladder contour with nodularity of the dome, and no evidence of vesicoureteral reflux. Cystoscopy demonstrated patchy areas of nodularity throughout the bladder. Biopsies performed from multiple sites showed chronic inflammation with multiple intramural granulomata. The patient was put on an oral regimen of cortisone. Substantial clinical improvement was noted at the end of 2 weeks. A repeat sonogram of the bladder (Fig. 3) showed marked resolution of the previously noted findings in the bladder wall. The bladder volume was also markedly increased since the earlier study.
Discussion Case Report A 3-year-old boy was admitted to the hospital because of urinary urgency, frequency, dysuria, enuresis, and incontinence of 3 to 4 weeks' duration. The diagnosis of CGD had been made at the age of 2 weeks by nitroblue tetrazolium test, which was performed because of a family history of CGD, including a brother with the disease. The patient has received daily trimethoprim-sulfamethoxazole since that time. At the age of 7 months, evaluation for guaiac-positive stool revealed lymphoid hyperplasia of the colon, as well as generalized lymph node enlargement, and retroperitoneal lymph node node enlargement with abscesses had been documented at the age of 13 months. Repeated skin infections due to Staphylococcus aureus had occurred between the ages of 18 and 24 months. On admission, the patient was afebrile, and physical examination was unremarkable. The white blood cell count was 14,400. Urinalysis revealed a pH of 8, specific gravity of 1.020, 2+ proteinuria, with 0-4 white blood cells and 0-4 red blood cells per high-power field. Urine cultures were negative. An ultrasound examination of the pelvis (Fig. 1) showed diffuse, irregular thickening of the bladder wall, with nodularity
Address reprint requests to: R. T. Speirs, M.D., Department of Radiology, Bellevue Hospital, 27th Street at First Avenue, New York, NY 10016, USA
CGD is an X-linked recessively inherited disorder ofneutrophil function. Phagocytosis remains intact, but ingested bacteria cannot be killed. The disease usually presents in the first year of life, and common manifestations include hepatosplenomegaly, lymphadenopathy, gastrointestinal and respiratory tract infections, osteomyelitis, and skin infections, especially around body orifices. Staphylococci are the most common infectious agent [1, 2]. The diagnosis is generally established by the nitroblue tetrazolium test [2]. Genitourinary involvement is rare. Cystitis and pyelonephritis may be seen when the genitourinary tract is involved [3, 4]. Patients with granulomatous cystitis often present with hematuria, dysuria, frequency, and enuresis [3-7]. Physical examination may reveal a suprapubic mass, representing the inflamed and thickened bladder, but it is generally unremarkable. Cystography will show a small bladder capacity with an irregular mucosal surface in the involved area. Vesicoureteral reflux may be seen and may cause hydronephrosis [3, 6]. Cystoscopy
R.T. Speirs et al.: Granulomatous Cystitis
107
Fig. 1. Longitudinal (A) and transverse (B) views from a pelvic sonogram reveal diffuse thickening of the bladder wall, with irregular mucosal surface.
an
Fig. 2. An anteroposterior view from a cystogram shows marked nodularity of the bladder dome, Fig. 3. Transverse view from a pelvic sonogram following therapy shows a return of bladder wall thickness to normal, as well as an increase in bladder capacity compared to the prior scan.
generally reveals erythematous, inflamed mucosa, and biopsies show acute and chronic inflammation associated with intramural granulomata [5]. Therapy includes antibiotics to treat an identified or suspected infectious agent, as well as steroids to suppress the inflammation [4, 5, 7]. In our case, ultrasound clearly demonstrated diffuse thickening with nodularity of the bladder wall. The involved areas in cases of focal granulomatous cystitis described by Hassel et al. [7] and by Haller
and Schneider [8] demonstrated similar sonographic findings. Ultrasound was also of use in monitoring the response to therapy, which was quite remarkable in this case. We conclude that ultrasonography is an effective noninvasive modality for the diagnosis of granulomatous cystitis. Ultrasonography is a painless procedure and is not associated with ionizing radiation. It can be used as often as necessary and is particularly valuable in monitoring the response to treatment.
108 References
1. Berendes H, Bridges RA, Good RA: A fatal granulomatous disease of childhood: Clinical study of a new syndrome. Minn Med 40:309, 1957 2. Good RA, Quie PG, Windhorst DB, et al.: Fatal (chronic) granulomatous disease of childhood: A hereditary defect of leukocyte function. Semin Hematol 5:215, 1968 3. Forbes GS, Hartman GW, Burke EC, et al.: Genitourinary involvement in chronic granulomatous disease of childhood. AJR 127:683, 1976
R.T. Speirs et al.: Granulomatous Cystitis 4. Young AK, Middleton RG: Urologic manifestationsof chronic granulomatous disease of infancy. J Urol 123:119, 1980 5. Kontras SB, Bodenbender JG, McClave CR, etaL: Interstitial cystitis in chronic granulomatous disease. J Urol 105:575, 1971 6. Cyr WL, Johnson H, Balfour J: Granulomatous cystitis as a manifestationof chronic granulomatous disease of childhood. J Urol 110:357, 1973 7. Hassel DR, Glasier CM, McConneU JR: Granulomatous cystitis in chronic granulomatous disease: Ultrasound diagnosis. Pediatr Radiol 17:254, 1987 8. Haller JO, Schneider M: Pediatric Ultrasound. Chicago: Year Book Medical Publishers, 1980, pp 125, 158-159
