Clinical and embryologic aspects of penile duplication and associated anomalies.
OBJECTIVE
To report 2 cases of penile duplication and review the literature in an attempt to categorize the associated anomalies in relation to the degree of penile duplication. Embryologic considerations of this rare anomaly are also reviewed.


METHODS
We report 2 distinct cases of diphallia. In the first case, true complete penile duplication was associated with multiple malformations, including a cloacal anomaly, colon and bladder duplication, a horseshoe kidney, a bifid scrotum with undescended testes, a hypoplastic right leg, and a ventricular septum defect. The second patient presented with true, complete diphallia and bladder and urethral duplication but an absence of other anomalies. The patients were individually treated according to the concomitant malformations. A review of published reports allowed a classification of associated anomalies in 77 cases of diphallia, according to the degree of penile duplication.


RESULTS
The first patient underwent a series of staged surgical repairs, including correction of the congenital heart anomaly, separation of the urogenital and gastrointestinal tract and resection of the duplicate terminal colon, excision of the smaller bladder and underdeveloped duplicate penis, bilateral orchiopexy, and hypospadias correction. The second patient underwent bladder fusion and excision of a urethrorectal fistula. Penile reconstruction was left for a later stage. An analysis of the cases available in published studies suggests that diphallia is often associated with a wide spectrum of anomalies that vary from severe malformations to less significant variations of human anatomy.


CONCLUSIONS
Penile duplication is a rare anomaly. Thorough investigations are mandatory in all cases to reveal underlying congenital malformations that are potentially life threatening and require immediate surgical correction. Treatment should always be individualized according to the degree of penile duplication and the extent of the concomitant anomalies.
Gyftopoulos
Kostis
K
Department of Pediatric Urology, Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands.
Wolffenbuttel
Katja P
KP
Nijman
Rien J M
RJ
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000196:embryology* / Q000453:epidemiology; D005837:Genitalia, Male / Q000002:abnormalities / Q000196:embryology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010413:Penis / Q000002:abnormalities* / Q000196:embryology*; D014564:Urogenital Abnormalities / Q000175:diagnosis / Q000196:embryology