Year : 2006 | Volume
: 17 | Issue : 4 | Page : 572--575
Ectopia Vasa Deferentia Inguinal Hernia, Vesicourteric Reflux, Imperforate Anus, and Recto-Vesical Fistula: A Case Report
Ibrahim Daradka, Issa Hazza
King Hussein Medical Center- Amman, Jordan
P.O. Box 540997, Amman 11937
Urologic congenital anomalies are frequently associated with anorectal abnormalities. Vas deferens anomalies in the general population are estimated to be less than 0.05%. Many of the abnormalities can be explained by events in early fetal life. Urosepsis and epididymitis are the usual presenting signs and symptoms in such patients. We describe an infant who was discovered after birth to have an association of high imperforate anus with recto-vesical fistula, right inguinal hernia, left vesicoureteral reflux, and bilateral dilated vasa deferentia ectopically terminating in the posterior urethra, which caused recurrent epididymo-orchitis.
|How to cite this article:|
Daradka I, Hazza I. Ectopia Vasa Deferentia Inguinal Hernia, Vesicourteric Reflux, Imperforate Anus, and Recto-Vesical Fistula: A Case Report.Saudi J Kidney Dis Transpl 2006;17:572-575
|How to cite this URL:|
Daradka I, Hazza I. Ectopia Vasa Deferentia Inguinal Hernia, Vesicourteric Reflux, Imperforate Anus, and Recto-Vesical Fistula: A Case Report. Saudi J Kidney Dis Transpl [serial online] 2006 [cited 2021 Sep 21 ];17:572-575
Available from: https://www.sjkdt.org/text.asp?2006/17/4/572/32498
Urologic congenital anomalies are frequently associated with anorectal abnormalities.  The higher the anorectal malformation, the higher is the incidence of urologic anomalies. , Vas deferens anomalies in the general population are estimated to be less than 0.05%.  Many of the abnormalities can be explained by an event in early fetal life. 
Ectopic vas deferens is a rare congenital anomaly and is associated with anorectal anomalies. , The first reported case was in 1898 by Friendland.  Patient age at presentation ranges from 3 weeks to 40 years, with multiple variations of intercommunications with the urinary tract. ,
Urosepsis and epididymitis are the usual presenting signs and symptoms in such patients.  Although scrotal pain may occur, many patients are asymptomatic.  Additionally, ectopic vas deferens may be discovered on routine examination or incidentally as an intraoperative finding. ,
An association of high imperforate anus with recto-vesical fistula, right inguinal hernia, left vesicoureteral reflux and bilateral dilated vasa deferentia ectopically terminating in the posterior urethra causing recurrent epididymoorchitis is presented in the following case.
A male neonate, a product of a normal gestation, was noted at birth to have a high imperforate anus and underwent an emergency sigmoid colostomy.
The infant was referred to our hospital in October 2005 at the age of two weeks, for further management. On examination, there was also an inguinal hernia on the right side. Both testes were felt and were normally descended in the scrotum. At subsequent evaluation, renal ultrasound showed normal kidneys and ureters and no renal or collecting system abnormalities were detected.
Two months later, the patient started to suffer several episodes of urinary tract infection; particularly left epididymitis. A voiding cystourethrogram was mistakenly reported as a second right vesicoureteral reflux and rectovesical fistula, but in fact was a bilateral reflux into both vasa deferens [Figure 1].
At the age of three months and during elective repair of the right inguinal hernia, a tubular vas-like thick-walled structure was noted, mimicking small intestine. The spermatic cord was therefore thoroughly dissected and a broad vas deferens was identified, 15 mm in diameter, which entered in its whole course in the inguinal canal. Dissection of the inguinal content showed testicular vessels and hernial sac, which was separated and clamped. The vas deferens was excluded from the clamp prior to division of the hernial sac.
At age of four months, the patient underwent posteriosaggital anorectoplasty and division-repair of recto-vesical fistula.
One month post-operatively, a cystoscopy under general anesthesia was performed after an episode of a greenish discharge from the urethra in association with left epididymoorchitis; recurrence of the recto-vesical fistula was suspected. The cystoscopy revealed a normal anterior urethra, membranous urethra, verumontanum and bladder epithelium. The ureteric orifices were in normal locations and appearance. At the bladder neck there was a depression of previously closed fistula. However, in the posterior urethra proximal to the vermontanum there was a passable orifice at nine o'clock through which the tip of cystoscope was inserted, and another small orifice at 3 o'clock was also noted. Methylene blue was injected through the distal colostomy site that demonstrated no communication between the colostomy and bladder or urethra. However, when a ureteric catheter was inserted in the tiny openings followed by injection of contrast, the dye drained into both vasa deferentia [Figure 2].
Four weeks later, before closure of the colostomy, a distal colostogram confirmed that there was no recurrence of the recto-vesical fistula. At operation, it was noted that the left vas also dilated but to a lesser degree than the right and seemed to lie in a normal course with no obvious connection between the vas deferens and the left ureter or bladder.
Prophylactic antibiotics were continued for another six months. During the following year the child remained free of infection. Followup renal ultrasound showed tubular structure behind the bladder. Intravenous pyelogram showed normal kidneys, ureteres and no collecting system abnormality. Ascending urethrography using Foley's catheter with balloon inflated at position in the distal urethra demonstrated early swift reflux into both vasa deferentia and a grade II reflux into the left ureter [Figure 3].
A week later the patient presented with early testicular pain not associated with bacteruria, and was treated as an outpatient.
Of the children with congenital anorectal anomalies, 25 to 55 percent have urogenital tract abnormalities. ,, There is a strong association of vesicoureteral reflux (VUR) and anorectal malformation.  VUR was noted in 47% of children with high imperforate anus and in 35% of those with low anomalies. ,,,
In many series, a voiding cystourethrogram was not performed routinely or only in children with high anorectal lesion. It is recommended that voiding cystourethrogram and (IVP) be performed in all children with imperforate anus. 
Although rare, ectopic vas deferens has a spectrum of presentations and has been associated with anorectal anomalies. , It has been reported to drain anywhere from the prostatic urethra to the renal pelvis;  the vas orifice seen in the prostatic urethra, at bladder neck or near the bladder neck, proximal or distal to verumontanum.  Normally the ejaculatory duct should pierce the upper part of the posterior surface of the prostate to open into the prostatic urethra at lateral margins of prostatic utricle.
The diagnosis of ectopic vas deferens should be suspected on clinical grounds and can be confirmed by cystourethroscopic catheterization, and retrograde urethrogram in which the dye refluxes into the vas deferens. However, vaso& laphy is preferred for definitive diagnosis. ,,,
In our case, cystourethroscopic catheterization and Foley's injection method were found to be the most productive method of radiologic evaluation of vasal ectopia. The vas deferens is a thick-walled tube variable in length according to age. The average external and luminal diameter is 1.0 and 0.19(mm) from birth up through 11 years.  In our case, it was 10 to 15 folds wider. Due to this abnormal size of the vasa deferens, the reflux of contrast was demonstrated clearly with our radiologic maneuvers. Though reflux commonly occurs into an ectopic vas deferens, the vas deferens does not necessarily have to be ectopic to allow reflux. 
For the patient with imperforate anus, we should evaluate not only anorectal function, but also the genitourinary tract in order to preserve renal function and fertility, since faulty development or agenesis of the seminal vesicles as embryological consequences of an ectopic vas deferens will result in infertility in the patient with bilateral ectopic vas deferens.  This rare condition of vasal ectopia may result from possible underlying defects of the proximal vas precursor and upper mesonephric duct.  Furthermore, congenital malformation of the epididymis and vas deferens are the causes of infertility in up to 80% of sterile adults. ,,
Finally, urologic care of children with anorectal malformation is an important though partly missed aspect in this group of patients.  The goals of treatment in children with ectopic vas deferens are to preserve renal function, prevent epididymitis and preserve fertility. ,
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