True Duplicate Bladder Exstrophy: A Rare Lesion

Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute, (JayKayLon Hospital) S.M.S. Medical College, Jaipur 302004, Rajasthan, India.
The Indian Journal of Pediatrics (Impact Factor: 0.87). 08/2009; 76(8):852-3. DOI: 10.1007/s12098-009-0172-4
Source: PubMed


A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe another one and briefly review the literature.

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Available from: Basant Kumar, Mar 01, 2014
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    ABSTRACT: Agenesis of the bladder is an extremely rare congenital anomaly with approximately 65 cases described with only 22 cases of live birth reported to date in the literature written in English. The cause of agenesis of the bladder is uncertain, but it may be the result of secondary loss of the anterior division of the cloaca; it could be induced in experimental animals after Adriamycin exposure. Almost all patients had other associated congenital anomalies, especially orthopedic, neurological, or other urological anomalies like renal agenesis, dysplastic or ectopic kidneys. Bladder duplication is defined as the presence of two urinary bladder cavities with full-thickness muscular walls, separated by a septum, usually as a part of caudal duplication (Dipygus) anomaly; complete duplication of the bladder and urethra was first reported in a stillborn infant in 1871. This anomaly is classified as complete and incomplete duplication; complete duplication is subclassified according to the orientation of the septum intervening between the two bladders into sagittal or coronal, which is the rarest type. Variants of bladder exstrophy may be presented as pseudoexstrophy, covered exstrophy, duplicate exstrophy, superior vesical fistula and fissure, visceral sequestration, or OEIS syndrome (omphalocele, exstrophy, imperforate anus, spinal defect). Congenital megacystis without other anomalies is extremely rare; it should be considered in the differential diagnosis of any intraabdominal cystic lesion demonstrated in a prenatal sonogram. Although reports suggest that this syndrome has a 4:1 female-to-male ratio, the megaureter-megacystis syndrome occurs as often in girls as in boys. Urachal anomalies were associated with other genitourinary conditions such as meatal stenosis, hypospadias, umbilical and inguinal hernias, cryptorchidism, anal atresia, omphalocele, crossed renal ectopia; those anomalies may be presented as patent urachus, which was reported for the first time by Bartholomaeus Cabrolius in 1550. Urachal cyst had an incidence of 1 in 5,000 live births. Urachal diverticulum frequently coexists with congenital obstruction of the lower urinary tract. Urachal sinus is a noncommunicating dilatation of the urachus at the umbilical end. Although adenocarcinoma of the urachus is rare, it has been reported in some children.
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