Pediat Radiology
Pediatr. Radiol. 8, 54-55 (1979)
9 by Springer-Verlag1979
Congenital Ureteral Valve P. M. Fitzer Department of Radiology, Riverside Hospital, Newport News, Virginia, USA
Abstract. A o n e year old b o y with u r e t e r a l o b s t r u c tion d u e to c o n g e n i t a l u r e t e r a l valve is p r e s e n t e d . This rare lesion is usually d i a g n o s e d p r e - o p e r a t i v e l y as u r e t e r a l stricture or p r i m a r y m e g a u r e t e r .
Discussion A r e c e n t review f o u n d a total of 17 cases of u r e t e r a l valve in the E n g l i s h s p e a k i n g l i t e r a t u r e [1]. O n l y four valves were d i a g n o s e d prior to surgery. U r e t e r a l o b -
Key words: C o n g e n i t a l u r e t e r a l valve - U r e t e r a l obstruction - U r e t e r a l stricture - P r i m a r y m e g a u r e t e r Ureter
C o n g e n i t a l u r e t e r a l valve is a rarely d i a g n o s e d cause of u r e t e r a l o b s t r u c t i o n . Since this c o n d i t i o n can b e surgically corrected, it should b e i n c l u d e d in the differential diagnosis of u n u s u a l stenotic lesions of the ureter. This r e p o r t describes a case with typical clinical a n d r a d i o g r a p h i c findings.
Case Report A one year old boy was admitted to Riverside Hospital for evaluation of persistent left back pain. Four months earlier, he had complained of left flank pain and urinary tract infection was diagnosed. An intravenous urogram and retrograde pyelogram showed mild left hydronephrosis and hydroureter with an apparent normal caliber left ureter distally. He was treated with appropriate antibiotic therapy, but the back pain persisted to the present. The child had been otherwise well. The admission physical examination, CBC, SMA-14, and urinalysis were normal. The urine culture showed no growth. An intravenous urogram again showed left hydronephrosis and hydroureter (Fig. 1). A cystogram showed no ureteral reflux. A retrograde pyelogram showed dilatation of the ureter with sudden transition to a normal small caliber ureter distally, and a distal ureteral stricture was diagnosed (Fig. 2). At surgery, the narrow distal ureteral segment was excised and a ureteroneocystostorey with distal ureteral tailoring was performed. The gross specimen showed a filamentous web across the ureteral lumen at the junction of the dilated and normal caliber ureteral segment. The final pathological diagnosis was congenital ureteral valve. The child did well post-operatively.
0301-0449/79/0008/0054/$01.00
Fig, L Intravenous urogram: the mild left hydronephrosis and hydroureter, especially distally, are evident. Note the normal caliber right collecting system
P. M. Fitzer: Congenital Ureteral Valve
55
nite etiology of ureteral valve has been substantiated to the present [1]. A true ureteral valve must have anatomically demonstrable transverse folds of transitional epithelium containing smooth muscle bundles. There must be changes of obstructive disease above the valve, with a normal ureter distally, and no other evidence of mechanical or functional obstruction may be present [2]. Other causes of distal ureteral obstruction will not fulfill these three criteria. The differential diagnosis principally includes distal ureteral stricture and primary megaureter. Due to previous ureteral insult, a stricture usually causes dilatation of the ureter and intrarenal collecting structures. Primary megaureter usually shows ureteral dilatation only. The correct diagnosis of congenital ureteral valve is important. No previous trauma or infection need to be etiologically implicated to account for the ureteral obstruction, as in ureteral stricture. As in primary megaureter, the ureteral obstruction of congenital ureteral valve is amenable to surgical correction. Acknowledgements. The author thanks Paula Kornegay and Peggy Rogers. The kind assistance of Richard Sallade, M. D., in preparation of the manuscript is gratefully acknowledged.
References Fig. 2. Left retrograde pyelogram: the abrupt change from the dilated lower left ureter to the small caliber distal segment is clear
(~ow) struction from ureteral valve may present clinically at any age. There is no sex predilection. The condition is usually unilateral, and about half are found in the distal ureter [1, 2]. The etiology of the lesions is not certain. Up to five percent of newborns have vestigial transverse mucosal folds in the distal ureter; these folds normally later disappear [3]. It has been suggested that persistence of these folds is the cause of distal ureteral valve [2]. A similar mechanism may account for proximal ureteral valves [4]. Other theories have been proposed, but no deft-
1. Albertson, K. W., Talner, L. B.: Valves of the ureter. Radiology 103, 91 (1972) 2. Wall, B., Wachter, H. E.: Congenital ureteral valve: its role as a primary obstructive lesion: classification of the literature and report of an authentic case. J. Urol. 68, 684 (1952) 3. Chwalle, R.: Process of formation of cystic dilation of the vesical end of the ureter and of diverticula at the ureteral ostium. Urol. Cutan. Rev. 31, 499 (1927) 4. Ostling, K.: The genesis of hydronephrosis particularly with regard to the changes at the ureteropelvic junction. Acta Chir. Scand. 86, 1 (1942) Date of final acceptance: May 30, 1978 P. M. Fitzer, M. D. 501 Riverside Drive Newport News, V A 23 606 USA
Pediatr. Radiol. 8, 54-55 (1979)
9 by Springer-Verlag1979
Congenital Ureteral Valve P. M. Fitzer Department of Radiology, Riverside Hospital, Newport News, Virginia, USA
Abstract. A o n e year old b o y with u r e t e r a l o b s t r u c tion d u e to c o n g e n i t a l u r e t e r a l valve is p r e s e n t e d . This rare lesion is usually d i a g n o s e d p r e - o p e r a t i v e l y as u r e t e r a l stricture or p r i m a r y m e g a u r e t e r .
Discussion A r e c e n t review f o u n d a total of 17 cases of u r e t e r a l valve in the E n g l i s h s p e a k i n g l i t e r a t u r e [1]. O n l y four valves were d i a g n o s e d prior to surgery. U r e t e r a l o b -
Key words: C o n g e n i t a l u r e t e r a l valve - U r e t e r a l obstruction - U r e t e r a l stricture - P r i m a r y m e g a u r e t e r Ureter
C o n g e n i t a l u r e t e r a l valve is a rarely d i a g n o s e d cause of u r e t e r a l o b s t r u c t i o n . Since this c o n d i t i o n can b e surgically corrected, it should b e i n c l u d e d in the differential diagnosis of u n u s u a l stenotic lesions of the ureter. This r e p o r t describes a case with typical clinical a n d r a d i o g r a p h i c findings.
Case Report A one year old boy was admitted to Riverside Hospital for evaluation of persistent left back pain. Four months earlier, he had complained of left flank pain and urinary tract infection was diagnosed. An intravenous urogram and retrograde pyelogram showed mild left hydronephrosis and hydroureter with an apparent normal caliber left ureter distally. He was treated with appropriate antibiotic therapy, but the back pain persisted to the present. The child had been otherwise well. The admission physical examination, CBC, SMA-14, and urinalysis were normal. The urine culture showed no growth. An intravenous urogram again showed left hydronephrosis and hydroureter (Fig. 1). A cystogram showed no ureteral reflux. A retrograde pyelogram showed dilatation of the ureter with sudden transition to a normal small caliber ureter distally, and a distal ureteral stricture was diagnosed (Fig. 2). At surgery, the narrow distal ureteral segment was excised and a ureteroneocystostorey with distal ureteral tailoring was performed. The gross specimen showed a filamentous web across the ureteral lumen at the junction of the dilated and normal caliber ureteral segment. The final pathological diagnosis was congenital ureteral valve. The child did well post-operatively.
0301-0449/79/0008/0054/$01.00
Fig, L Intravenous urogram: the mild left hydronephrosis and hydroureter, especially distally, are evident. Note the normal caliber right collecting system
P. M. Fitzer: Congenital Ureteral Valve
55
nite etiology of ureteral valve has been substantiated to the present [1]. A true ureteral valve must have anatomically demonstrable transverse folds of transitional epithelium containing smooth muscle bundles. There must be changes of obstructive disease above the valve, with a normal ureter distally, and no other evidence of mechanical or functional obstruction may be present [2]. Other causes of distal ureteral obstruction will not fulfill these three criteria. The differential diagnosis principally includes distal ureteral stricture and primary megaureter. Due to previous ureteral insult, a stricture usually causes dilatation of the ureter and intrarenal collecting structures. Primary megaureter usually shows ureteral dilatation only. The correct diagnosis of congenital ureteral valve is important. No previous trauma or infection need to be etiologically implicated to account for the ureteral obstruction, as in ureteral stricture. As in primary megaureter, the ureteral obstruction of congenital ureteral valve is amenable to surgical correction. Acknowledgements. The author thanks Paula Kornegay and Peggy Rogers. The kind assistance of Richard Sallade, M. D., in preparation of the manuscript is gratefully acknowledged.
References Fig. 2. Left retrograde pyelogram: the abrupt change from the dilated lower left ureter to the small caliber distal segment is clear
(~ow) struction from ureteral valve may present clinically at any age. There is no sex predilection. The condition is usually unilateral, and about half are found in the distal ureter [1, 2]. The etiology of the lesions is not certain. Up to five percent of newborns have vestigial transverse mucosal folds in the distal ureter; these folds normally later disappear [3]. It has been suggested that persistence of these folds is the cause of distal ureteral valve [2]. A similar mechanism may account for proximal ureteral valves [4]. Other theories have been proposed, but no deft-
1. Albertson, K. W., Talner, L. B.: Valves of the ureter. Radiology 103, 91 (1972) 2. Wall, B., Wachter, H. E.: Congenital ureteral valve: its role as a primary obstructive lesion: classification of the literature and report of an authentic case. J. Urol. 68, 684 (1952) 3. Chwalle, R.: Process of formation of cystic dilation of the vesical end of the ureter and of diverticula at the ureteral ostium. Urol. Cutan. Rev. 31, 499 (1927) 4. Ostling, K.: The genesis of hydronephrosis particularly with regard to the changes at the ureteropelvic junction. Acta Chir. Scand. 86, 1 (1942) Date of final acceptance: May 30, 1978 P. M. Fitzer, M. D. 501 Riverside Drive Newport News, V A 23 606 USA
