Pseudoexstrophy associated with penile duplication and hypospadias: A case report and literature review
Journal Title: Pediatric Urology Case Reports - Year 2018, Vol 5, Issue 3
Abstract
Bladder exstrophy is a rare developmental anomaly. Four principle variants of bladder exstrophy have been described and they themselves are rarer than the bladder exstrophy. Authors describe the management of a case of pseudoexstrophy type of variant in a 9 month old male child with penile duplication with torsion and coronal hypospadias. The rectal fascial defect was repaired without osteotomy as the distance between two pubic bones was <4 cm (3.1 cm). Genital reconstruction with excision of duplicate atrophic penile shaft and repair of coronal hypospadias with detorsion of the functional penile shaft could be accomplished. The patient had good outcome in terms of cosmesis and urinary stream. Total of 18 cases of the pseudoexstrophy have been described till date. Pseudoexstrophy of bladder is a very rare condition and can simultaneously present with other defects like omphalocele, anorectal malformations, pouch colon, multiple or solitary urogenital anomalies. The principles of correction though remain same with correction of abdominal wall defect with or without osteotomy depending upon severity of pubic diastasis. Other anomalies can undergo treatment as per standard protocol.
Authors and Affiliations
Gursev Sandlas, Bhushanrao Bhagawan Jadhav
Keywords
Related Articles
- EP ID EP498339
- DOI 10.14534/j-pucr.2018338708
- Views 54
- Downloads 0
Ureteric diverticulum: A diagnostic challenge
Ureteric diverticulum is a rare urological condition with only 47 cases described in the literature till 2013. A full term female infant presented with a huge cystic lump occupying almost the entire right side of the a...
A rare cause of chronic urinary retention in children, Fowler's syndrome: A case report
In 1986, Fowler and colleagues described a condition currently known as Fowler's syndrome in young women with unexplained urinary retention associated with polycystic ovary syndrome. Herein, we present a case of a 13-yea...
Penile hair tourniquet syndrome: A case report and review of literature
Penile hair tourniquet syndrome is a relatively uncommon clinical situation. It involves a thread of hair tied around a penis, causing strangulation which may result in various complications. It is seen mostly in circumc...
Aphallia (Penile agenesis): A preliminary report of three cases
Aphallia (penile agenesis) is an extremely rare abnormality with the reported incidence of 1 in 30 million births. The cause of this anomaly is associated with no genital tubercle formation or its development impairment....
Prostatic utricle cyst presenting with recurrent urethral discharge in the newborn: A case report
A 6 month old boy with normal external genitalia, presented with purulent urethral discharge from the neonatal period and recurrent urinary tract infections. Radiologic and urethrocystoscopic evaluation showed a midline...