Congenital vas deferens-ureteral connection

Congenital vas deferens-ureteral connection

Congenital V as D eferens-Ureteral Connection By E. Thomas Boles, J r , , T h o m E. Lobe, and Ala H a m o u d i 9 Congenital drainage of the vas defe...

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Congenital V as D eferens-Ureteral Connection By E. Thomas Boles, J r , , T h o m E. Lobe, and Ala H a m o u d i 9 Congenital drainage of the vas deferens into a ureter permits retrograde passage of urine through the vas to the epididymis. If this anomaly is accompanied by urinary tract pathology with vesicoureteral reflux, hydroureter, stasis, and infection, the passage of urine into the vas can very well lead to epididymitis of a chronic or recurring nature and secondary scrotal abscesses. T w o cases of this rare anomalous connection are presented in which such scrotal infections occurred. The diagnosis should be suspected on clinical grounds and can be confirmed by cystograms in which the dye refluxes up the ureter and passes into the vas. The association of rectal anomalies is of embryologic significance and may be additionally important clinically by further suggesting the possibility of the ureteral-vas anomaly. INDEX duct,

WORDS:

Vas (ductus) deferens, Wolffian

H E U N U S U A L A N O M A L Y of a vas deferens inserting into a ureter rather than into the posterior urethra may result in clinical manifestations that are perplexing and not easily diagnosed. This anomalous connection is readily understandable on embryologic grounds, but has been rarely described and with little reference to its clinical significance. Two cases are presented that illustrate the bizarre anatomic possibilities, the association of other anomalies, the confusing clinical picture, and the diagnostic features.

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CASE REPORTS

Case 1 As a newborn male, this patient was evaluated because of abdominal distention. An anal opening was present, but rectal examination could not be done. Abdominal radiographs showed multiple loops of distended intestines, and a barium e n e m a showed rectal stenosis extending up from the anus for about 4 cm with distended sigmoid above this. Intravenous pyelogram showed left hydronephrosis but no function on the right. A right transverse colostomy together with suprapubic insertion of a small plastic catheter into the bladder were done on the second day of life. C y s t o g r a m s done at 2 and 7 days of age showed a narrow, distorted posterior u r e t h r a and free reflux into an ectopic right ureter that was dilated and tortuous and arose from the dome of the bladder. T h e infant voided per u r e t h r a with a weak, dribbling stream. A left loop ureterostomy was done on the 20th day of life because of posterior urethral obstruction together with rising blood urea nitrogen and creatinine levels. Journal of Pediatric Surgery, Vol. 13, No. 1 (February),1978

Subsequently, the infant did well with good drainage from the ureterostomy and return of the blood chemistry values to normal. However, beginning at 4 mo of age, an area of firm swelling adjacent to the right testis became evident, and at 12 mo of age this became an obvious abscess requiring drainage, Recurrent right scrotal abscesses, thought to be secondary to epididymitis, continued during the following year; and because simple drainage procedures failed to control the problem, at 2 yr of age a right orchiectomy was performed. The ductus deferens and spermatic cord showed chronic inflammation and abscesses, the testis chronic inflammation of the tunica albuginea. At 3 yr of age, the rectal stenosis was corrected using an abdominal-perineal pull-through approach. Prior to this operation, a r e t r o s p e c t i v e review of t h e c y s t o g r a m s performed in the newborn period showed the right vas deferens completely outlined by the dye, and demonstrated that it drained into the ectopic right ureter in its midportion. Accordingly, at the s a m e operation, the thickened right ureter was explored and found, indeed, to arise from the dome of the bladder. It ascended in the midline to communicate with a small, dysplastic, multicystie kidney. Five cm from its junction with the bladder, the right vas connected to the ureter and ran retroperitoneally to the right internal inguinal ring (Fig. 1). A right nephroureterectomy together with excision of the vas was done. The boy has had some problems with the rectal reconstruction, but his general health, growth, and development have been normal. He has had no more problems of scrotal abscesses. His ureterostomy functions well, and the hydronephrosis on the left has improved markedly.

Case 2 This 2-yr-old boy was born with a high type of imperforate anus (rectal atresia) for which a transverse colostomy was done as a newborn. Because of failure to thrive, a severe metabolic acidosis, and uremia (blood urea nitrogen-70 rag/100 ml), he was transferred to the Columbus Children's Hospital at 8 wk of age. Intravenous pyelography showed no function on either side; a renal scan demonstrated a left kidney but none on the right. A cystogram showed a large, cone-shaped bladder with marked trabeculation. There was free reflux bilaterally demonstrating severe bilateral hy-

From the Department of Surgery, Division of Pediatric Surgery, and the Department of Pathology, Ohio State University College of Medicine, and the Children's Hospital, Columbus, Ohio. Presented before the X X I V Annual International Congress of the British Association of Paediatric Surgeons, Oslo, Norway, August 2-6, 1977. Address reprint requests to E. Thomas Boles, Jr., M.D., Department of Pediatric Surgery, Children's Hospital, 700 Children's Drive, Columbus, Ohio 43205. 9 1978 by Grune & Stratton, Inc. 0022-3468 / 78/ 1301-0010 $01.00/0 41

VAS-,

/ Fig. 1. (A) Cystogram w i t h reflux into dilated, tortuous right ureter. The ureter arises from the dome of the bladder. Note filling of the right vas deferens that connects to the midportion o! the ureter. (S) Schematic drawing of anatomic situation in case 1.

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Fig. 2. (A) Cystogram with massive bilateral vesicoureteral reflux, Marked bilateral hydroureter and left hydronephrosis. Retrograde filling of right vas from the right ureter. Note marked dilatation of vas in its midportion. (B) Illustration of anatomic findings in case 2.

VAS DEFERENS-URETERAL CONNECTIONS

droureter and hydronephrosis. The posterior urethra was markedly dilated with an abrupt caliber change, but subsequent endoscopy did not show posterior urethral valves. There were deformities of the fifth lumbar and sacral vertebral segments with partial agenesis. A suprapubic cystostomy was performed, and the uremia gradually subsided. A month later this tube was removed, and he was able to void adequately with suprapubic pressure. At 6 mo of age he developed an acute right epididymitis. This responded to antibiotic therapy. Because of this episode plus previous bouts of urinary tract infection, the rectourethral fistula was divided and an end colostomy performed with the purpose of stopping contamination of the urinary tract via the rectal communication. However, episodes of right epididymitis with abscess formation continued. Repeat intravenous pyelography showed hydronephrosis and hydroureter on the left but no function on the right. A repeat cystogram at 1 yr of age was similar to the first, but in addition demonstrated the right vas deferens communicating with the upper third of the right ureter. Accordingly, a right nephroureterectomy along with removal of the vas to the internal ring was performed. At laparotomy, the right kidney was very small with a bifid pelvis and two ureters. These two ureters joined into one about 5 cm from the kidney. The vas arose from the ureter draining the lower pole above the point of junction, and proceeded to the internal ring (Fig. 2). Following this operation therehave been no further bouts of epididymitis or scrotal abscesses. At 16 mo of age, a sacral-abdominal-perineal pullthrough anoplasty was performed with takedown of the colostomy. At the same time, because of no improvement of the left hydronephrosis, a left loop cutaneous ureterostomy was performed. The child has done well. Repeat pyelograms have shown significant improvement of the hydronephrosis on the left. The bladder appearance by cystogram is unchanged and is felt to represent a neurogenic bladder. He is free of urinary tract infection, and renal function is reasonable with normal creatinine and blood urea nitrogen levels and a creatinine clearance of 45 liter/M2/24 hr. His growth in height and weight have both remained on approximately the third percentiles. EMBRYOLOGY

By t h e 4 m m (3 wk) s t a g e o f d e v e l o p m e n t , the Wolffian (mesonephric) duct has elongated caudally and bends sharply, ventrally and m e d i a l l y , to r e a c h t h e side o f t h e c l o a c a at t h e level o f t h e first s a c r a l v e r t e b r a l body. A t this bend, a localized thickening and evagination give rise to t h e u r e t e r a l bud t h a t t u r n s c e p h a l a d toward the undifferentiated nephrogenic mese n c h y m e . T h e Wolffian d u c t cranial to t h e u r e t e r i c bud develops into t h e vas d e f e r e n s (ductus deferens). T h e Wolffian d u c t distal to t h e u r e t e r a l bud is t e r m e d t h e c o m m o n m e s o n e p h r i c duct.',2 A s t h e u r e t e r a l bud grows cranially to m e e t the c a u d a l p o r t i o n of t h e n e p h r o g e n i c b l a s t e m a , t h e c o m m o n m e s o n e p h r i c d u c t is s h o r t e n e d by

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r e s o r p t i o n into t h e wall o f t h e u r o g e n i t a l sinus. T h e exact m e c h a n i s m o f this p r o c e s s is a m a t t e r of d e b a t e ? -5 T h e c l o a c a by this t i m e has divided into t h e u r o g e n i t a l sinus a n t e r i o r l y and t h e r e c t u m p o s t e r i o r l y . A t t h e 14-15 m m stage, t h e c o m m o n m e s o n e p h r i c d u c t is fully r e s o r b e d , resulting in s e p a r a t e o p e n i n g s of the u r e t e r a l bud and t h e Wolffian d u c t into the u r o g e n i t a l sinus. F u r t h e r distal m i g r a t i o n of the Wolffian duct o c c u r s until it u l t i m a t e l y t e r m i n a t e s in t h e posterior urethra. F a i l u r e o f this p r o c e s s o f r e s o r p t i o n o f t h e c o m m o n m e s o n e p h r i c d u c t r e s u l t s in p e r s i s t ing c o m m u n i c a t i o n b e t w e e n t h e u r e t e r and t h e vas d e f e r e n s . PATHOLOGY

T h e g r o s s and m i c r o s c o p i c f e a t u r e s of both o f t h e s e c a s e s a r e of i n t e r e s t . In t h e first case, t h e right u r e t e r was ectopic, inserting into the d o m e of the b l a d d e r . T h e r e is no clear e m b r y o l o g i c e x p l a n a t i o n for such an ectopic position, although this m a y p e r h a p s be an e x t r e m e form o f the " c r a n i o z o n e " location in t h e classification p r o p o s e d by M a c k i e , A w a n g , and S t e p h e n s . 6 T h e r e was free reflux from t h e b l a d d e r into this u r e t e r , which was dilated, t o r t u o u s , and showed chronic i n f l a m m a t i o n . T h e vas d e f e r e n s conn e c t e d to t h e m i d p o r t i o n o f this u r e t e r , and t h e vas was also m a r k e d l y d i l a t e d and thick-walled. P r o x i m a l l y , the u r e t e r was a t t a c b e d to a small, d y s p l a s t i c kidney t h a t lay in t h e midline over t h e g r e a t vessels and was a t t a c h e d on the left side to the m e d i a l a s p e c t o f t h e left kidney. In t h e s e c o n d c a s e , t h e i n v o l v e d u r e t e r d r a i n e d into the b l a d d e r in t h e n o r m a l location, but t h e r e was also free reflux into it from t h e b l a d d e r ; it was also d i l a t e d and tortuous. It bec a m e bifid in its p r o x i m a l portion, and the vas d r a i n e d into t h e lower o f the two p r o x i m a l segm e n t s of u r e t e r . T h e nonfunctioning kidney on the involved side was only a small nubbin of renal tissue t h a t histologically showed chronic i n f l a m m a t i o n , sclerotic glomeruli, i n t e r s t i t i a l t u b u l a r fibrosis, and thyroidization. T h e vas d e f e r e n s on t h i s s i d e w a s a l s o m a r k e d l y t h i c k e n e d and dilated. T h e histologic s t r u c t u r e o f t h e r e m o v e d vas d e f e r e n s in both cases r e s e m b l e d u r e t e r . T h e lining epithelium was of t h e t r a n s i t i o n a l cell type rather than pseudostratified or tall c o l u m n a r as is c h a r a c t e r i s t i c in t h e n o r m a l vas. F u r t h e r m o r e , t h e m u s c u l a r i s showed d e n s e fi-

Fig. 3. (A) Cross-section from ureter (case 2) showing dilated lumen and transitional epithelial cell lining, Note the infiltration of connective tissue between the smooth muscle layers, x 22. (B) Cross-section of vas deferens (case 2) at same magnification ( x 22) as ureter. Histologically similar to ureter with transitional cell epithelium and dense fibroconnective tissue between the muscle bands.

VAS DEFERENS-URETERAL CONNECTIONS

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broconnective tissue between the muscle bands. This is again similar to ureter, rather than to the usual three-coat-of-muscle architecture of the vas (Fig. 3). CLINICAL FEATURES

C h r o n i c e p i d i d y m i t i s with i n t e r m i t t e n t abscess formation was the prominent clinical consequence of this anomaly in both cases. This undoubtedly resulted from free reflux of urine into the vas from the large, hypotonic ureters. Furthermore, in both cases there was stasis of urine, poor drainage, and intermittent infection. The organisms cultured from the scrotal abscesses were the same as from the urine. In all likelihood, the severe accompanying pathology of the urinary tract played an important and necessary role in the clinical picture. It seems doubtful that sterile urine would have resulted in such intense inflammation of the proximal vas deferens, epididymis, and adjacent tissues. Although, in retrospect, the clinical picture should have strongly suggested the diagnosis in both cases, the association was not made in either for rather long periods of time. In both, the anomaly was clearly demonstrated by cystograms, although in the first this was recognized only after a careful review of radiographs taken many months previously. Intravenous pyelography, particularly with the high infusion technique, may also d e m o n s t r a t e the anomaly, as in a case described by Williams, but, of course, would be unlikely to help in instances with no renal function on the side of the lesion, as was the case in both of our patients. 7 DISCUSSION

Communication between the ureter and vas deferens was first noted by Hoffman in 1872 in

two autopsy cases. 8 Since then, a considerable number of such cases have been reported, most of the earlier cases also being found at postm o r t e m examinations. O f 12 clinical cases published m o r e recently, both vasa deferens joined the respective ureters in one case, and in another both vasa joined a blind-ending left ureter. Of the remaining, the anomaly was on the left in one, on the right in three, and not clearly described in the remainder. 7,9-1s Other genitourinary anomalies were found in most of the cases in association with the ureteral-vas anomaly. These included absent, h y p o p l a s t i c , d u p l i c a t e d , m a l r o t a t e d , and horsehoe kidneys; absent or hypoplastic seminal vesicle or prostate; undescended testicle and hypospadias. The vas deferens-ureteral connection has been reported to be a common necropsy finding in cases of vesico-intestinal fissure (exstrophy of cloaca) cases. ~ Rectal atresia or other rectal anomalies have been reported previously in association with the ureteral-vas a n o m a l y ) '12 This is not surprising in view of the close anatomic relationships of the cloaca and Wolffian ducts in early embryonic life, and the similar timing of the Wolffian duct development and of the division of the cloaca into urogenital sinus and rectum. E p i d i d y m i t i s as a c o m p l i c a t i o n o f the anomaly has been previously noted in two reports. 7,12 The possibility of this complication is obvious from the anatomic arrangement, and is probably inevitable if stasis of urine in the affected ureter and urinary tract infections complicate the situation. The possibility of the anomaly should be entertained if epididymitis occurs, particularly when the inflammation is chronic, recurrent, and suppurative. Study of the bladder and ureters by cystography is the procedure most likely to be diagnostic.

REFERENCES

1. Arey LB: Developmental Anatomy (ed 7), Philadelphia, W. B. Saunders, 1974, pp 324-325 2. Gray SW, Skandalakis JE: Embryologyfor Surgeons, Philadelphia, W. B. Saunders, 1972, pp 522, 596 3. Frazer JE: The terminal part of the Wolman duct. J Anat 69:455-468, 1935 4. Wesson MB: Anatomical, embryological and physiological studies of trigone and neck of bladder. J Urol 4:279, 1920 5. Gyllensten L: Contributions to the embryologyof the urinary bladder. Acta Anat 7:305-344, 1949

6. Mackie GG, Awang H, Stephens FD: The ureteric orifice: the embryologic key to radiologic status of duplex kidneys. J Pediatr Surg 10:473-481, 1975 7. Williams DI, Royle M: Ectopic ureter in the male child. Br J Uro141:421-427, 1969 8. Hoffmann CEE: Swei Falle von umwandlung der samenblasen in harnleiter. Arch Heilkunde 13:532-544, 1872 9. Borger JA, Belman AB: Uretero-vas deferens anastomosis associated with imperforate anus: An embryologi-

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cally predictable occurrence. J Pediatr Surg 10:255-257, 1975 10. Alfter H J, Gillenwater JY: Ectopic vas deferens communicating with lower ureter: Embryological considerations. J Urol 108:172 173, 1972 11. Scultety VS, Lelck I: Aspermie infolge einmundung heider samenleiter in einen blind endenden ureter. Z Urol 61:11-13, 1968

BOLES, LOBE, AND HAMOUDI

12. Seitzman DM, Patton JF: U r e t e r a l ectopia: Combined ureteral and vas deferens anomaly. J Urol 84:604 608, 1960 13. Redman JF, Sulieman JS: Bilateral vasal-ureteral communications. J Urol 116:808-809, 1976 14. Soper RT, Kilger K: Vesico-intestinal fissure. J Urol 92:490 501, 1964