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Case report: Bladder neurofibromas causing ureteric obstruction in Von Recklinghausen's disease
Chnical Radiology (1985) 36, 537-538 © 1985 Royal College of Radiologists
000%9260/85/458537502 00
Case Report" Bladder Neurofibromas Causing Ureteric Obstruction in Von Recklinghausen's Disease P. T. B R O O K S a n d J. K. S C A L L Y
The Countess of Chester Hospital, Chester
A case of bladder involvement by Von Recklinghausen's disease with consequent hydronephrosis and hydroureters is presented. The intravenous urographic and ultrasonic appearances are correlated.
B l a d d e r i n v o l v e m e n t with n e u r o f i b r o m a s in V o n R e c k l i n g h a u s e n ' s disease is a rare complication. F o r t y two cases have b e e n d e s c r i b e d , 13 of which were in children (Gerhardt, 1878; Jensen and Nissen, 1976; B a r o n e , 1979). W e p r e s e n t such a case, correlating the u r o g r a p h i c a n d u l t r a s o n i c a p p e a r a n c e s . W e believe this is the first case in which the u l t r a s o u n d features of b l a d d e r n e u r o f i b r o m a s have b e e n described.
CASE REPORT A 37-year-old Caucasian male presented in September 1983 for the removal of two cutaneous lesions of the scrotum and upper thigh. However, examination also revealed numerous truncal cafd-au-lalt
spots. A diagnosis of Von Reckhnghausen's disease was made for the first time, although histological confirmation was not obtained One year later, he was referred for an intravenous urogram (IVU) following sevcral episodes of haematuna and cystitis Blochemlcally, renal function was normal. Eschertchia coh was cultured from his urine. The IVU showed bilateral hydronephrosis and hydroureter (Fig 1) and a small bladder with several large smooth filling defects (Fig. 2). An ultrasound exammanon was then performed. This confirmed the presencc of the hydronephroses and demonstrated numerous intramural masses. These were well dcmarcated ovoid lesions, up to 6 cm in diameter, with a uniform hyperechoic pattern (Figs 3, 4). A number of these indented the bladder cavity, corresponding to the filling defects seen on the IVUs. These were considered to be neuroflbromas. Subsequent cystoscopy revealed an intact bladder cavity, corresponding to the submucosal infiltration, giving the bladder wall a nodular appearance. The right ureteric orifice was partially obstructed by a polypold lesion Histological examination confirmed this to be a neurofibroma. The left ureteric orifice could not be visuahsed due to bladder wall distortion
DISCUSSION B l a d d e r n e u r o f i b r o m a s are t h o u g h t to be derived from the vesicoprostatic plexus in males a n d from the u r e t h r o v a g i n a l plexus m females. As these are a n a t o m i c a l l y c o n t i g u o u s with the distal u r e t e r s and vesical neck, u r e t e r i c and vesical o b s t r u c t i o n are recognised c o m p l i c a t i o n s (Pessin and B o d i a n , 1964; Spence and D a n e , 1981). T h e u r i n a r y tract can also be i n v o l v e d by extrinsic n e u r o f i b r o m a s arising from s u r r o u n d i n g viscera and
Fig 1 - Bilateral hydronephrosis and hydroureter Two opacities shown on the right side of the pelvis are ureteric calculi.
Fig. 2 - Small bladder showing large filling defects.
538
CLINICAL RADIOLOGY
F~g 3 - Transverse scan 'lesions arrowed).
and in one case there was subsequent sarcomatous degeneration (Jensen and Nissen, 1976). Together, these represent 14.3% of the cases with bladder involvement. Phaeochromocytomas are also known to occur in the bladder in Von Recklinghausen's disease, but there is nothing to suggest this in this patient. Bladder involvement with the disease in this patient was suspected following the IVU, the ultrasound examination revealing the exact site and nature of the lesions. The filling defects seen on the IVU correlate well with the masses demonstrated on ultrasound and the nodularity seen at cystoscopy. This is consistent with the cystoscopic appearances in the majority of described cases (Cameron, 1964). There was no evidence of sarcomatous degeneration, either clinically or ultrasonographically, but the patient will need regular follow-up to exclude such change. Ultrasound, possibly coupled with cystoscopy, is the most practical method of monitoring the condition due to its accuracy and ready availability. It is likely that this patient will eventually come to surgery to relieve his ureteric obstruction. Acknowledgements. Our thanks to Mr J Kane lor hxs permission to report this case.
REFERENCES Barone, D
O
(1979)
Neurofibromatosxs
a chmcal overview
Postgraduate Medicine, 66, 73. Cameron, K. (1964). Brit;sh Journal of" Urology, 36, 77.
Fig. 4 - Longitudinal scan (lesmns arrowed).
nerves (Ghrist, 1963; Hyland and Taylor, 1979). Sarcomatous degeneration occurs in up to 13% o~ those afflicted with the condition (Hosoi, 1931). Among the reported cases of bladder involvement, five were histologically malignant without evidence of metastasis,
Gerhardt, C. (1878). Zur dxagnosnk mult@er Neuroblldung Deutsches Arc/re' fitr khms'che Medizm, 21,268. Ghrist, T. D. (t963) Gastrointestinal involvement m neurofibromatosls. Arch;yes of Internal Medicine, 112, 357. Hosol, K. (1931). Multiple neurofibromatosls (Von Reckhnghausen's disease) with special reference to malignant transformauon Arch;yes of Surgery, Chtcago, 22, 258. Hyland, J. M. & Taylor, I. (1979) Unusual comphcations of pelvic neurofibromatosls. British Journal of Chntcal Practice, 33, 183184. Jensen, A. & Ntssen, H. M. (1976) Neurofibromatosis of bladder Scandinavian Journal of Urology and Nephrology, 10, 157 159 Pessin, J. I. & Bodian, M (1964) Neurofibromatosis of the pelvic autonomic plexuses. Brittsh Journal of Urology, 36, 510 Spence, R A. J & Dane, T. E B (1981) Von Reckhnghausen's disease presentmg with acute retention of urine lrtsh Journal o[ Medical Sc;ence, 150, 124.
000%9260/85/458537502 00
Case Report" Bladder Neurofibromas Causing Ureteric Obstruction in Von Recklinghausen's Disease P. T. B R O O K S a n d J. K. S C A L L Y
The Countess of Chester Hospital, Chester
A case of bladder involvement by Von Recklinghausen's disease with consequent hydronephrosis and hydroureters is presented. The intravenous urographic and ultrasonic appearances are correlated.
B l a d d e r i n v o l v e m e n t with n e u r o f i b r o m a s in V o n R e c k l i n g h a u s e n ' s disease is a rare complication. F o r t y two cases have b e e n d e s c r i b e d , 13 of which were in children (Gerhardt, 1878; Jensen and Nissen, 1976; B a r o n e , 1979). W e p r e s e n t such a case, correlating the u r o g r a p h i c a n d u l t r a s o n i c a p p e a r a n c e s . W e believe this is the first case in which the u l t r a s o u n d features of b l a d d e r n e u r o f i b r o m a s have b e e n described.
CASE REPORT A 37-year-old Caucasian male presented in September 1983 for the removal of two cutaneous lesions of the scrotum and upper thigh. However, examination also revealed numerous truncal cafd-au-lalt
spots. A diagnosis of Von Reckhnghausen's disease was made for the first time, although histological confirmation was not obtained One year later, he was referred for an intravenous urogram (IVU) following sevcral episodes of haematuna and cystitis Blochemlcally, renal function was normal. Eschertchia coh was cultured from his urine. The IVU showed bilateral hydronephrosis and hydroureter (Fig 1) and a small bladder with several large smooth filling defects (Fig. 2). An ultrasound exammanon was then performed. This confirmed the presencc of the hydronephroses and demonstrated numerous intramural masses. These were well dcmarcated ovoid lesions, up to 6 cm in diameter, with a uniform hyperechoic pattern (Figs 3, 4). A number of these indented the bladder cavity, corresponding to the filling defects seen on the IVUs. These were considered to be neuroflbromas. Subsequent cystoscopy revealed an intact bladder cavity, corresponding to the submucosal infiltration, giving the bladder wall a nodular appearance. The right ureteric orifice was partially obstructed by a polypold lesion Histological examination confirmed this to be a neurofibroma. The left ureteric orifice could not be visuahsed due to bladder wall distortion
DISCUSSION B l a d d e r n e u r o f i b r o m a s are t h o u g h t to be derived from the vesicoprostatic plexus in males a n d from the u r e t h r o v a g i n a l plexus m females. As these are a n a t o m i c a l l y c o n t i g u o u s with the distal u r e t e r s and vesical neck, u r e t e r i c and vesical o b s t r u c t i o n are recognised c o m p l i c a t i o n s (Pessin and B o d i a n , 1964; Spence and D a n e , 1981). T h e u r i n a r y tract can also be i n v o l v e d by extrinsic n e u r o f i b r o m a s arising from s u r r o u n d i n g viscera and
Fig 1 - Bilateral hydronephrosis and hydroureter Two opacities shown on the right side of the pelvis are ureteric calculi.
Fig. 2 - Small bladder showing large filling defects.
538
CLINICAL RADIOLOGY
F~g 3 - Transverse scan 'lesions arrowed).
and in one case there was subsequent sarcomatous degeneration (Jensen and Nissen, 1976). Together, these represent 14.3% of the cases with bladder involvement. Phaeochromocytomas are also known to occur in the bladder in Von Recklinghausen's disease, but there is nothing to suggest this in this patient. Bladder involvement with the disease in this patient was suspected following the IVU, the ultrasound examination revealing the exact site and nature of the lesions. The filling defects seen on the IVU correlate well with the masses demonstrated on ultrasound and the nodularity seen at cystoscopy. This is consistent with the cystoscopic appearances in the majority of described cases (Cameron, 1964). There was no evidence of sarcomatous degeneration, either clinically or ultrasonographically, but the patient will need regular follow-up to exclude such change. Ultrasound, possibly coupled with cystoscopy, is the most practical method of monitoring the condition due to its accuracy and ready availability. It is likely that this patient will eventually come to surgery to relieve his ureteric obstruction. Acknowledgements. Our thanks to Mr J Kane lor hxs permission to report this case.
REFERENCES Barone, D
O
(1979)
Neurofibromatosxs
a chmcal overview
Postgraduate Medicine, 66, 73. Cameron, K. (1964). Brit;sh Journal of" Urology, 36, 77.
Fig. 4 - Longitudinal scan (lesmns arrowed).
nerves (Ghrist, 1963; Hyland and Taylor, 1979). Sarcomatous degeneration occurs in up to 13% o~ those afflicted with the condition (Hosoi, 1931). Among the reported cases of bladder involvement, five were histologically malignant without evidence of metastasis,
Gerhardt, C. (1878). Zur dxagnosnk mult@er Neuroblldung Deutsches Arc/re' fitr khms'che Medizm, 21,268. Ghrist, T. D. (t963) Gastrointestinal involvement m neurofibromatosls. Arch;yes of Internal Medicine, 112, 357. Hosol, K. (1931). Multiple neurofibromatosls (Von Reckhnghausen's disease) with special reference to malignant transformauon Arch;yes of Surgery, Chtcago, 22, 258. Hyland, J. M. & Taylor, I. (1979) Unusual comphcations of pelvic neurofibromatosls. British Journal of Chntcal Practice, 33, 183184. Jensen, A. & Ntssen, H. M. (1976) Neurofibromatosis of bladder Scandinavian Journal of Urology and Nephrology, 10, 157 159 Pessin, J. I. & Bodian, M (1964) Neurofibromatosis of the pelvic autonomic plexuses. Brittsh Journal of Urology, 36, 510 Spence, R A. J & Dane, T. E B (1981) Von Reckhnghausen's disease presentmg with acute retention of urine lrtsh Journal o[ Medical Sc;ence, 150, 124.