Author/Authors :
mirshemirani, Alireza shahid beheshti university of medical sciences - Pediatric Surgery Research Center, تهران, ايران , Khaleghnejad, Ahmad shahid beheshti university of medical sciences - Pediatric Surgery Research Center, تهران, ايران , Pourang, Hoshang tehran university of medical sciences tums - Department of Pediatric Surgery, تهران, ايران , Sadeghian, Naser shahid beheshti university of medical sciences - Pediatric Surgery Research Center, تهران, ايران , Rouzrokh, Mohsen shahid beheshti university of medical sciences - Pediatric Surgery Research Center, تهران, ايران , Salehpour, Shadab shahid beheshti university of medical sciences - Department of Pediatrics, تهران, ايران
Abstract :
Background: Penile agenesis (PA) is an extremely rare anomaly with profound urological and psychological consequences. The opening of the urethra could be either over the pubis or at any point on perineum or most frequently in anterior wall of the rectum. The aim of treatment is an early female gender assignment and feminizing reconstruction of the perineum. Case(s) Presentation: We report 8 cases of penile agenesis with urination and defecation through the rectum, apparently normal scrotum, bilateral descended testis, normally located anus, urethral opening in anus, 46xy karyotype and associated anomalies. In 2 cases parents refused any surgical interventions, but in 6 cases we did perform different operations (transforming five cases to females and one case to male gender). Conclusion: We recommend feminizing operations in newborns or infants, but in older patients, regarding the child s psychology, it is advised to perform masculinizing operations, and finally, no surgical intervention should be undertaken before counseling the parents.
Keywords :
Aphallia , penile agenesis , reconstruction , Ambiguous genitalia.
