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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
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Year : 2020  |  Volume : 25  |  Issue : 4  |  Page : 258-259

Bladder exstrophy with anovestibular fistula: A rare presentation

Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission24-Aug-2019
Date of Decision31-Aug-2019
Date of Acceptance12-Oct-2019
Date of Web Publication24-Jun-2020

Correspondence Address:
Dr. Jai K Mahajan
Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_139_19

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How to cite this article:
Yhoshu E, Mahajan JK. Bladder exstrophy with anovestibular fistula: A rare presentation. J Indian Assoc Pediatr Surg 2020;25:258-9

How to cite this URL:
Yhoshu E, Mahajan JK. Bladder exstrophy with anovestibular fistula: A rare presentation. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2022 Jan 23];25:258-9. Available from: https://www.jiaps.com/text.asp?2020/25/4/258/287653


A full-term, female newborn, weighing 2.1 kg was brought to the pediatric emergency with complaints of a pinkish bulge in the lower part of the abdomen, with absent anal opening. On examination, the neonate was active and healthy looking with mild abdominal distension. There was a defect in the lower part of the abdomen with exstrophied bladder and a pale smooth swelling below it, with absent anal opening [Figure 1]a. The umbilical cord was inserted at the upper part of the exposed bladder. Both the ureteric openings could be visualized in the bladder patch, while the distal pale swelling had a pinpoint opening caudally, which discharged some whitish fluid on compressing the swelling. Another pinpoint opening was seen more distally, which had meconium on being probed with an infant feeding tube, suggestive of an anovestibular fistula (AVF) [Figure 1]b. The labia and the clitoris were divergent and no separate anal opening was seen. X-ray of the abdomen revealed wide pubic diastasis and distended rectal and sigmoid segments with no evidence of pouch colon. Ultrasound abdomen showed mild hydronephrosis of the left kidney. Baseline blood parameters were normal.
Figure 1: (a) Bladder exstrophy with hydrocolpos caudally and cord attached cranially. (b) Infant feeding tube in anovestibular fistula (solid arrow) and another tube in stenotic vagina (Hollow arrow)

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As the patient was not able to pass meconium, a cutback anoplasty was done. However, there was persistent posterior shelving of the rectum, 1 cm above the anal verge, not permitting free passage of Hegar dilators; a transverse colostomy was also performed to ensure proper bowel evacuation. Vaginal opening was enlarged, which allowed the whitish vaginal discharge to flow out freely. At present, the baby is decompressing well by stoma, started on feeds and doing well.

In general, infants with bladder exstrophy have significant widening of the pubic symphysis and anteriorly placed anus. In females, the clitoris is bifid and vagina is anteriorly placed with a stenotic opening.[1] Exstrophy–epispadias sequence is a spectrum of disease of abdominopelvic fusion abnormality, including phallic separation with epispadias, pubic diastasis, exstrophy of the bladder, cloacal exstrophy, and OEIS complex (omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defect).[2] Our case was a variant of this sequence; exstrophy bladder, hydrocolpos with AVF. To our knowledge, this is the first report of bladder exstrophy with AVF in a female; a similar case has been reported by Singh et al. in a male neonate wherein the anus was placed as an anteriorly placed perineal fistula and anoplasty was done.[3]

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   References Top

Woo LL, Thomas JC, Brock JW. Bladder and Cloacal Exstrophy. 7th ed. Philadelphia: Elsevier Saunders; 2012.  Back to cited text no. 1
Smith NM, Chambers HM, Furness ME, Haan EA. The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): Recurrence in sibs. J Med Genet 1992;29:730-2.  Back to cited text no. 2
Singh AP, Mathur V, Tanger R, Gupta AK. Exstrophy bladder with low anorectal malformation-A rare association. J Neonatal Surg 2017;6:69.  Back to cited text no. 3


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