diphallus , or duplication of the penis ( or clitoris ) , is a rare anomaly with an incidence of 1 in 5,500,000 live births . the extent of duplication varies greatly from a double glans arising from a common shaft to complete duplication of the phallus . a 1-year - old male child presented with straining and poor urinary stream along with abnormal appearance of the external genitalia noticed since early infancy . clinical examination revealed duplication of glans in the sagittal plane ; the dorsal glans was better in size than the ventral one and had a stenotic meatus at its tip , whereas the ventral glans was hyposplastic with its meatus at the coronal sulcus [ figures 1a and 2a ] . the patient used to dribble from the dorsal meatus and had a thin stream from the ventral meatus . he also had pre - axial polydactyly in the left hand and deep first web space on the left foot . further evaluation revealed solitary kidney and atrial septal defect which did not require any intervention . urethral anatomy was delineated using ascending urethrography and micturating cystourethrography and the findings were suggestive of effmann iia2 duplication [ figure 1c ] with a single , good capacity bladder without vesicoureteric reflux [ figures 1c and 2a ] . the dorsal glandular size was good but had hypoplastic distal ( 2 cm ) urethra which admitted only guide wire . the ventral glans was hypoplastic and had a meatus at the corona with associated hypoplastic entire ventral urethra . two urethral channels , a second ( ventral ) channel arising from the first channel and coursing independently through the meatus in the sagittal plane . ( d ) post - operative mcug the patient underwent byars staged genital reconstruction . in view of hypoplastic ventral glans with its associated narrow ( hypoplastic ) entire ventral urethra the well - developed dorsal glans was preserved and its hypoplastic distal urethra was laid open and incorporated into the byars flaps . byars first stage urethroplasty [ figure 2b ] consisted of penile degloving , creating and transferring byars flaps ventrally and suturing to the edges of the laid open dorsal urethra . the post - operative period was uneventful and he had good functional and cosmetic outcome at 2.5 years of follow - up [ figure 1b and d ] . ( b ) byars procedure stage 1  yellow arrow indicates distal laid open dorsal urethra . the extent of duplication in diphallus varies from a double glans arising from a common shaft to complete duplication of the phallus . scheneider classified diphallus in three groups : diphallus of glans alone , bifid diphallus and complete diphallus . the most widely followed classification for urethral duplication anomalies is described by effmann et al . almost all authors agree that the anomaly is due to a defective fusion of the genital tubercle . johnson et al . classified the anomaly based on the number of corpus cavernosal bodies in each penis . true diphallia has two corpus cavernosal bodies ; pseudodiphallia has a single corpus cavernosum in each penis . in our case the glans was duplicated along with penile urethra but the corporal bodies were not duplicated . there is no standard operative procedure for urethral duplication anomalies and most authors recommend excision of the non - dominant ( dorsal ) urethra in our patient we have retained the dorsal urethra for reconstruction as it was of better caliber . to the best of our knowledge , retaining the dorsal urethra was unusual and has not been reported so far in the literature . described the technique of padua ( progressive augmentation by dilating the urethra anterior ) procedure for the treatment of severe urethral hypoplasia by using gradually progressive indwelling soft catheter or stent dilation . this technique was not attempted in our patient as the patient required excision of the ventral hypoplastic glans . reported that treatment of diphallus usually includes excision of the duplicated penile structure and its urethra . in our patient the ventral glans and its urethra were excised retaining the dorsal glans and its urethra for staged reconstruction . the literature suggests that the best outcomes are seen using the ventral urethra for the reconstruction , but in our patient we have used the dorsal urethra for reconstruction with good results . management of glandular diphallus requires proper evaluation and tailoring the reconstruction to the individual patient anatomy .
diphallus is a rare anomaly and its association with urethral duplication is extremely rare . 
 numerous associated genitourinary and gastrointestinal anomalies have been reported with this condition . 
 challenges in the management are incorporation of the glans and the dominant urethra during reconstruction . 
 we report the successful management of a case of glandular diphallus with complete urethral duplication retaining the dorsal urethra .