Diphallia or penile duplication is an extremely rare congenital anomaly. It occurs once in every 5.5 million live births. The extent of penile duplication and the number of associated anomalies vary greatly, ranging from a double glans from a penis with no associated anomaly up to complete penile duplication associated with multiple anomalies. Here, we report a 12-year-old boy with complete bifid diphallia associated with bifid scrotum, epispadia, and pubic symphysis diastasis along with a review of the articles pertaining to this anomaly.
Diphallia or penile duplication is an extremely rare congenital anomaly. It occurs once in every 5.5 million live births [
A 12-year-old boy was referred as a case of penile duplication and bifid scrotum. The patient belonged to a low socioeconomic stratum. His complaint was unable to hold to urinate and the urine was always leaking from both of his penises.
Examination of the genitalia revealed complete and separate penises of equal size, each with an epispadia urethral meatus. The scrotum was bifid, and each side contained a testicle. Over the region of the pubis, a bowel loop-like structure was seen (Figure
Complete and separate diphallia. Over the region of the pubis, a bowel loop-like structure was seen.
Both urethral orifices were catheterized easily and ended up in a single bladder. On urethrocystoscopy done on the left penis, the bladder neck was directly seen without urethral mucosa prior to it. The bladder seemed hyperemic. On the right penis, there was fibrotic tissue along the urethra to the bladder. Retrograde pyelography revealed normal ureter and no vesicoureteral reflux. Plain photo showed normal vertebrae with pubic symphysis diastasis (Figure
At surgery, the exteriorized intestinal loop-like structure was excised. The structure had no communication with neither the peritoneal cavity and bladder nor other structures. Each penis was found to have only one corpus cavernosum. Epispadia repair and penis reconstruction were done by joining the corporal bodies in each penis (Figure
Final result after surgery.
Plain photo showed pubic symphysis diastasis (a); retrograde pyelography showed normal ureter and no ureterovesical reflux (b) and (c).
Diphallia is a rare anomaly, and it is believed that no cases are identical. It varies from a small accessory penis or duplication of the glans to complete penile duplication. The duplication may be orthotopic or ectopic. Division of the penis may be sagittal or frontal and symmetric or asymmetric, in shape and size [
Diphallia is usually associated with other malformations, such as bladder and urethra duplication, exstrophy of the cloaca, exstrophy of the bladder, anorectal malformation, colon and rectosigmoid duplication, ventral hernia, pubic symphysis diastasis, abnormality of skeletal and heart muscles, and vertebral anomalies [
The degree of erectile function in cases of diphallia varies significantly. Usually, one or both penises are capable of erection. In cases of true, complete diphallia presented at advanced age, simultaneous erection and, occasionally, ejaculation have been reported. In contrast, pseudodiphallia is rarely associated with normal function of the rudimentary phallus. Several studies reported normal erection of partial bifid phallus and true partial diphallia even though erectile function after surgery is still unclear [
Diphallia is believed to take place during the embryonic development of the penis around the 3rd to 6th week of gestation [
Detailed studies of the developmental anatomy of the genital tubercle have shed light onto the understanding of diphallia. The normal development of the penis begins with the coalescence of bilateral cloacal tubercles at the anterior end of the pars phallica of the urogenital sinus. Columns of mesoderm growing rapidly around the lateral margins of the cloacal plate form the genital tubercle. These bands of mesoderm arise from more than one area and their failure to fuse would lead expectantly to bladder exstrophy and epispadia with a split penis, instead of true diphallia. Consequently, another step is necessary for complete penile duplication to occur. An embryologic explanation of collateral urethral duplication includes the possibility of a longitudinal duplication of the cloacal membrane that would allow three or four columns of primitive streak mesoderm to migrate ventrally around the two cloacal membranes to eventually form two genital tubercles. Duplication of cloacal membrane in this fashion could also explain the frequent concomitant bladder, colon, anal, and spinal anomalies [
Penile duplication poses a difficult treatment problem in terms of medical, ethical, and aesthetic decision making. Detailed study of the external genitalia anatomy not only helps to classify the degree of penile duplication but also assists in the excision or reconstruction of the duplicate penis, by delineating the corporal development and urethral anatomy [
The treatment of diphallia is by excision of the duplicated noncommunicating penis. Excision of ectopic penis was reported in many bodies of literature [
Treatment principally depends on the type of accompanying congenital abnormalities as well as preserving continence and erectile function, which means individualizing each case [
The authors declare that there is no conflict of interests regarding the publication of this paper.