Congenital vesicouterine fistula along with distal vaginal agenesis, solitary kidney and tongue tie: a rarity.
CASE REPORT: A 19 year old female from Bihar, the Eastern Region of India, presented with complaints of painful cyclical hematuria with clot, ammenorhea, and pain abdomen for the past seven years. Pain abdomen started with cyclical hematuria and subsided with cessation of hematuria. Patient was continent for urine and faeces.
There was no history of urinary tract infection or faecaluria. Patient had undergone operation for tongue tie at the age of four years. Physical examination revealed absence of vagina, although labia minora and majora were adequately developed. Secondary sexual growth such as axillary and pubic hair, breast development were normal. Laboratory examination revealed normal hemogram, liver function tests and kidney function tests. Abdominal Ultrasonography showed normal uterus and ovaries, urinary bladder with solitary left kidney. Intravenous pyelography further documented solitary functioning right kidney.
Contrast enhanced CT scan revealed solitary functioning right kidney along with vaginal agenesis and an abnormal communication between uterus and bladder. Cystoscopy performed during the period of menouria revealed an orifice of size about 8mm in the supratrigonal area in midline. Blood clots were seen coming from the orifice into the bladder. Laparotomy in supine position by low transverse incision revealed well-developed uterus and ovary. Fistulous tract was identified and excised along with closure of uterus and bladder.
A segment of sigmoid colon was isolated on its mesentry and its proximal end was anastomosed to the uterus. The distal end of bowel was brought out via the vaginal introitus and sutured to the skin to create a neovagina. Now she is doing well as per her last followup at one year. She has been menstruating per neovagina.
DISCUSSION: Vesicouterine fistula has been sparsely described in literature. Peak incidence occurs in young woman between 25 and 33 years of age. (3,6,9) The important causes of vesicouterine fistula can be classified as obstetrical, surgical, radiation necrosis, malignancy, inflammatory bowel disease, endometriosis, intrauterine device migration, bladder tuberculosis and congenital lesion. We have reported congenital vesicouterine fistula along with distal vaginal agenesis, which is quite rare.
A case of primary menouria due to congenital vesicouterine fistula with distal vaginal agenesis was reported by Singh V. Sinha R J et al., in 2011. (10) Another case of congenital vesicouterine fistula with vaginal agenesis and left renal agenesis was reported by Erman-Akar M, Ozkan O, et al. (11) Our case was also associated with left renal agenesis along with tongue tie.
Commonest presentation of congenital vesicouterine fistula is cyclical menouria and same was seen in our case. The presence of vaginal agenesis paved the way for the egress of menstrual blood through the bladder. Posteriorly, the tough Denonvilliers fascias limited its way to rectum. Accurate and early diagnosis of vesicouterine fistula is difficult, since there are many different clinical pictures. In our case, patient was asymptomatic till the age of menarche when she developed pain abdomen and cyclical hematuria.
The mainstay of diagnosis is typical history, physical examination, cystoscopy, and urinary tract imaging. (7) A cystoscopy is necessary to detect fistula, its size, location, proximity to ureteral orifices and inflammatory state of tissue surrounding the fistula. Dye test and hysterosalpingography, which is used in detection of urogenital fistula in noncongenital cause could not be used in this case due to vaginal agenesis. Ultrasound and intravenous pyelography detected solitary right kidney, but fails to detect vesicouterine fistula. Micturating cystourethrogram revealed good capacity bladder without any reflux, but failed to detect vesicouterine fistula.
Additional diagnostic procedure include contrast enhanced CT, MRI, Vaginal ultrasound. (1,2,8) Our patient underwent contrast enhanced CT scan, which was readily available and affordable to patient. CT scan revealed abnormal communication between uterus and bladder along with left renal agenesis.
Treatment options for vesicouterine fistula include conservative management, medical and surgical therapy. (3,13,14) Surgery is the treatment of choice in most cases. (3,15) In our case of congenital vesicouerine fistula, repair of fistula with vaginoplasty was the aim. Patient had not given consent for hysterectomy, so we did repair of vesicouterine fistula with preservation of uterus. We did sigmoid colon vaginoplasty to create neovagina.
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(10.) Singh V, Sinha R J, Mehrotra S, Primary menouria due to congenital vesicouterine fistula with vaginal agenesis: A rarity Int. urogynecol J.2011 Aug; 22(8):1031-3.
(11.) Erman-akar M, Ozkan O, Yucel S, Dolay K Ertugrul F, Bektas G. Uterine preservation and vaginal reconstruction in a patient with congenital vaginal agenesis presenting with cyclic menouria. J Minim Invasive Gynacol 2011 Sep-Oct;18(5):682-5.
(12.) Hadzi -Djokic JB, Pejcic TP, Colovic VC, Vesicouterine fistula: Report of 14 cases. BJU Int 2007;100:1361-3.
(13.) Jozwick M. Spontaneous closure of vesicouterine fistula, Account for hormonal treatment. Uro int 1999 ; 62:1837.
(14.) Eogan, McKenna P, Conservative management of traumatic vesicouterine fistula Eur J Obstet Gynecol Reprod Bio 2003;110:114-6.
(15.) Tarhan F et al., Minimal invasive treatment of vesicouterine fistula, A case report. Int Urol Nephrol 2007;39:791-3.
Khalid Mahmood (1), Amit Kumar (2), Rana Pratap Singh (3), Ahsan Ahmad (4), Takallum Khatoon (5)
(1) Assistant Professor, Department of Urology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar.
(2) Junior Resident, Department of Anatomy, Indira Gandhi Institute of Medical Sciences, Patna, Bihar.
(3) MCh 3rd Year, Department of Anatomy, Indira Gandhi Institute of Medical Sciences, Patna, Bihar.
(4) Assistant Professor, Department of Urology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar.
(5) Senior Resident, Department of Anaesthesia, Indira Gandhi Institute of Medical Sciences, Patna, Bihar.
Financial or Other, Competing Interest: None.
Submission 03-11-2015, Peer Review 04-11-2015, Acceptance 13-11-2015, Published 23-11-2015.
Dr. Khalid Mahmood, Shams Manzil Block-B, Shahganj Mahendru, Patna-800006, Bihar.
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|Title Annotation:||Case Report|
|Author:||Mahmood, Khalid; Kumar, Amit; Singh, Rana Pratap; Ahmad, Ahsan; Khatoon, Takallum|
|Publication:||Journal of Evolution of Medical and Dental Sciences|
|Date:||Nov 23, 2015|
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