March 2018 · Volume 7 · Issue 3 Page 1251 International Journal of Reproduction, Contraception, Obstetrics and Gynecology Singh N et al. Int J Reprod Contracept Obstet Gynecol. 2018 Mar;7(3):1251-1254 www.ijrcog.org pISSN 2320-1770 | eISSN 2320-1789 Case Report Prenatal diagnosis of congenital megalourethra: a rare anomaly Neha Singh 1 , Vandana Bansal 1 , Kaizad R. Damania 2 INTRODUCTION Congenital megalourethra is a rare congenital anomaly causing functional obstructive uropathy due to dysgenesis of the penile corpora cavernosa and spongiosa resulting into extensive dilatation of penile urethra. 1 Benacerraf et al reported first time this condition antenatally in year 1989. Exact embryological etiopathogenesis is not clearly understood yet. Most common accepted theory about genesis of this condition suggest defects in migration, differentiation and development of mesenchymal tissues of phallus. 1 Dorairajan has classified it into two types based on severity of maldevelopment of penile erectile tissue; scaphoid type and fusiform type. 2 Scaphoid variant is found to associated with hypoplasia of the corpus spongiosum and bulging of ventral urethra whereas fusiform variant is known to have deficient corpora spongiosa and cavernosa with circumferentially distended 1 Department of Fetal Medicine, Nowrosjee Wadia Maternity Hospital, Mumbai, Maharashtra, India 2 Department of Obstetrics and Gynecology, Seth GS Medical College and Nowrosjee Wadia Maternity Hospital, Mumbai, Maharashtra, India Received: 30 December 2018 Accepted: 31 January 2018 *Correspondence: Dr. Vandana Bansal, E-mail: drvandana_bansal@yahoo.co.in Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Congenital megalourethra is a rare urogenital malformation characterised by pathological elongation and dilatation of penile urethra. It is a form of functional obstructive uropathy which is associated with significant urological and sexual dysfunction secondary to hypoplasia or absence of corpora cavernosa and spongiosa. We report a case of megalourethra in one of the dichorionic diamniotic twin diagnosed prenatally in a young primigravida. Initial ultrasound report suggested two different diagnoses i.e. omphalocoele and gastroschisis. Later, diagnosis of congenital megalourethra was made on detailed ultrasound examination by our fetal medicine consultant. Postnatal gross and ultrasonographic examination confirmed the prenatal diagnosis. In the present case report, we have emphasized proper perineal anatomical ultrasound examination which revealed a megalourethra. Although this anomaly is not lethal in isolation, but it has worse prognosis in presence of other associated structural anomalies. This anomaly also raises the controversy regarding disclosure of sex of foetus in countries like India where sex disclosure is prohibited by law. As this condition typically affects male foetuses and significantly affects sexual function, it may be necessary to reveal fetal sex for appropriate counselling and prognostication to prospective parents. Ethical dilemma arises for practising clinicians while dealing with isolated forms of megalourethra whether to offer termination of pregnancy on the grounds of having significant impact on sexual life due to erectile dysfunction. Anorectal malformation is often associated with megalourethra, although suspected in the present case, can be difficult to identify antenatally prior to 20 weeks (the time limit up to which legal termination can be performed in India). These issues have been highlighted in the present report. Keywords: Congenital urogenital anomaly, Hydronephrosis, Lower urinary tract obstruction, Megacystis, Megalourethra, Megapenis, Sexual dysfunction, Urethral cyst, Vesico-amniotic shunting DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20180931