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Ectopia Vesicae and Intrauterine Pregnancy


Author Information

Mahajan JS*, Samant PY**,  Parulekar SV***
(* Third Year Resident, ** Additional Professor, *** Professor and Head of Department
Department of Obstetrics and Gynaecology, Seth G.S. Medical College and K.E.M Hospital, Mumbai, India.)
Abstract
A rare case of ectopia vesicae and intrauterine pregnancy in a 25 years old, unmarried woman is presented. A medical termination of pregnancy was performed by dilatation and evacuation.
Introduction
Ectopia vesicae is a congenital anomaly in which there is failure of development of lower anterior abdominal wall. It occurs in 1 in 25000-50,000 people, the incidence in males being twice  as in females. It is seven times more common in babies conceived by in vitro fertilization. [1] The lower anterior abdominal wall and the anterior wall of the bladder are absent. The posterior bladder wall and the ureteric orifices are exposed. The bladder neck and urethra are poorly defined. The pubic symphysis  is widely separated.[2,3] There are reports of intrauterine pregnancy  and successful deliveries in patients with uncorrected ectopia vesicae. Cesarean section  at term gestation has always been preferred in such cases. A case of ectopia vesicae in a primigravida with 11 weeks of gestation managed by medical termination of pregnancy is presented.
Case report
 A 25  years  old unmarried woman presented with chief complaints of  amenorrhea for  3      months, pain in abdomen and generalized weakness for 1 week. She had an ultrasonographic scan showing an intrauterine gestation of 11 weeks. She gave a history of urinary incontinence from childhood and only spotting monthly from the orifice below the bladder from the age of puberty at the age of 14 years. There was a history of interfemoral sexual intercourse. On  abdominal examination, the umbilicus was absent. The open bladder base was at the lower end of the abdominal wall. An  opening was  present in the area of the mons pubis which was extremely tender to digital palpation,. The clitoris was absent, the labia minora were widely separated, and the pubic rami were absent. On per rectal examination, a bulky uterus was palpable anterior to rectum. One finger examination was done through the introital orifice. It was extremely painful. The uterus could be felt posteriorly, but its size could not be determined. The cervix  could not be felt.
Figure. 1. Ectopia vesicae. The pubic bones and labia minora  appear to be parts of lower abdominal wall. Pubic hair is seen on either side of midline. Introitus appears to be very small.

Her serum beta-hCG level was 97218 mIU/ml. Ultrasonography showed an intrauterine fetus of 11 weeks of gestation. Magnetic resonance imaging showed a single intrauterine gestation. The connection between the gravid uterus and the external orifice could not be defined.  It also could not be determined if the opening present on mons was vaginal or urethral.
Examination under general anesthesia showed that the  introit opening led to a cavity which had a normal appearing cervix. It was dilated to 9.5 mm. The products of conception were evacuated with ovum forceps. A blunt curettage was done. The patient made an uneventful recovery. She was given contraceptive advice and referred to a urologist for repair of the ectopia vesicae.
Discussion
 Ectopia vesicae or bladder extrophy occurs due to a failure of development of the lower anterior abdominal wall. That leaves the posterior wall of the bladder bulging like soft red swelling with multiple sites of excoriation on it. The condition should be surgically treated in childhood. A neglect may lead to its persistence in adult life, with increased risk of  ascending infection as openings of ureters are on the surface, and of development of malignancy in the exposed mucus membranes. In a combined series of 22 patients, there were 32 pregnancies, of which two aborted spontaneously and two were terminated medically. There were 27 live births and one intrauterine death of twins.[4] There were two case reports of continuation of the pregnancy to the third trimester and successful obstetric outcome was obtained by caesarean section.[5]  In the case presented, the pregnancy termination was performed for social reason (out of wedlock pregnancy) rather than ectopia vesicae.
References
1.      Wood  HM,  Trock  BJ,  Gearhart  JP:  In  vitro  fertilization  and  the cloacal-bladder exstrophy-epispadias complex: Is there an association? J Urol 2003;69:1512-1515.
2.      Gearhart JP, Ben-Chaim J, Jeffs RD, Sanders RC. Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol 1994; 85:961-964.
3.      Woodhouse CRJ. The gynaecology of exstrophy. BJU International 1999; 83(S3):34-38.
4.      Woodhouse CRJ. Long term results of bladder exstrophy. In Gearhart JP, Mathews R (editors). The Exstrophy-Epispadias Complex: Research Concepts and Clinical Applications. First Edition. New York.  Kluwer Academic/Plenum Publishers. 1999; p 182.
5.      Mandal A, Chaudhuri S, Manna SS, Jana SK, et al. Successful pregnancy outcome in a woman with untreated ectopia vesicae: A case report and review of literature. J Obstet Gynecol Res 2013;39:868–871.

Citation
Mahajan J, Samant PY,  Parulekar SV. Ectopia  vesicae  and   intrauterine  pregnancy. JPGO 2014 Volume 1 Number 1 Available from: http://jpgyob.blogspot.in/2014/01/ectopia-vesicae-and-intrauterine.html