Diphallia

Also called Diphallasparatus, penile duplication (PD) or diphallic terata is a rare developmental disorder where a male infant is born with two or more penises. The first reported case was in Bologna; Italy (1609), by Johannes Jacob Wecker, a Swiss female physician and philosopher.

It occurs in 1 out of every 5.5 million males worldwide, and so far only about 100 cases of diphallia have been recorded.

 

Diphallia, Picture credit mamamia.

Diphallia is usually accompanied by other congenital abnormalities like Vertebral (spinal bifida), renal (exstrophy of the bladder, bladder/urethra duplication), hindgut (exstrophy of the cloaca), anorectal (colon and rectosigmoid duplication), skeletal (pubic symphysis diastasis) skeletal muscle abnormalities (ventral hernia) heart muscles abnormalities, etc.

Risk factors/Causes

It is thought diphallia occurs in the fetus between the 23^rd to 35^th day  (3rd to 6th week) of gestation(pregnancy) when an injury; chemical stress, or malfunctioning “homeobox genes” hamper proper function of the caudal cell mass of the foetal mesoderm as the urogenital sinus separates from the genital tubercle and rectum to form the penis.

The embryologic explanation of diphallia is quite obscure, because at no time during normal development is the genital tubercle a paired structure. Reptiles such as snakes and lizards possess a double penis. Moreover, diphallia could represent a teratoid structure or a form of incomplete twinning. Still, none of these explanations is adequate and the wide spectrum of the anomalies cannot be explained by a single hypothesis.

Signs and symptoms

It is believed that no cases are identical as signs and symptoms vary for each patient. It varies from a small accessory penis or duplication of the glans to complete penile duplication.

Generally, it is characterized with two or more penis, both are situated side by side and has equal size, urine passes through both penis, some patient in one penis only, other patient neither of the penis. Some patient is sterile or incapable to produce semen, but the penis will erect if stimulated.

The duplication may be orthotopic or ectopic. Division of the penis may be sagittal or frontal and symmetric or asymmetric, in shape and size.

The urethra shows a range of variations, from functioning double urethras to complete absence of the urethra in each penis. The majority have a single corpus cavernosum in each organ. The meatus may be normal, hypospadias, or epispadia, and the scrotum may be normal or bifid. The testes are normal, athropic, or undescended.

Types

Various classifications for diphallia exist. One classifies it into four types while a recent classification is into two

Classifying Diphallia into four types:

• ·         Diphallia of the glans alone,
• ·         bifid diphallia,
• ·         Complete diphallia.
• ·         Pseudodiphallia.

Classifying diphallia into two types (widely accepted):

• ·         True diphallia; partial duplication or complete duplication.
• ·         Bifid phallus; partial duplication or complete duplication.

True complete diphallia refers to complete penile duplication, each with two corpora cavernosa (body of the penis) and a corpus spongiosum (spongy tissue surrounding the male urethra within the penis). Here both penises are capable of simultaneous erection and ejaculation.

True Partial diphallia; here the duplicate penis is smaller or rudimentary, it corresponds to the term “pseudodiphallia,”. Here the rudimentary or smaller penis may not be capable of erection and ejaculation)

Bifid phallus; when only one corpus cavernosum (body of the penis) is present in each penis. Normal erection in both penises occurs here).

         Complete bifid phallus; when the degree of separation is complete to the base of the shaft

         Partial bifid phallus; when the separation is just to the glans (Tip/cap of the penis).

Diagnosis

Ultrasound is used to help confirm the diagnosis. It detects the number of corpora cavernosum or corpora spongiosum and their accompanying abnormalities. Improved interpretation of anatomical structures has been made possible with the advent of magnetic resonance. Imaging also help in decision making during treatment.

Treatment

Several factors are considered during the treatment of diphallia, these are; medical, ethical and aesthetic factors.

Treatment of diphallia depends on the type of diphallia and the associated anomalies. During treatment of diphallia, it is necessary to individualise each case and also aim at preserving or achieving erectile function, urinary continence, urinary stream and cosmesis.

Surgical Excision of the duplicated noncommunicating penis is reported in many bodies of literature. Penis reconstruction by joining the corporal bodies in each penis in a patient with true complete diphallia has also been reported in some literatures.

Complications

Congenital defects, sterility, erectile dysfunctions, prostatitis, etc.

Prognosis

Infants born with diphallia and its related conditions have a higher death rate from various infections associated with their more complex renal or colorectal systems.

References

R. Mukunda, P. S. Bendre, R. G. Redkar, and S. Hambarde, “Diphallus with anorectal malformation-case report,” Journal of Pediatric Surgery, vol. 45, no. 3, pp. 632–634, 2010

V. C. Varghese and T. U. Sukumaran, “Penile duplication,” Indian Pediatrics, vol. 41, no. 11, p. 1166, 2004

H. S. Bhat, S. Sukumar, T. B. Nair, and C. S. M. Saheed, “Successful surgical correction of true diphallia, scrotal duplication, and associated hypospadias,” Journal of Pediatric Surgery, vol. 41, no. 10, pp. e13–e14, 2006.

M. M. Aboodi and H. A. Al-Jadeed, “Accessory pseudophallus with accessory pseudoscrotum detected during antenatal sonographic scanning,” Journal of Ultrasound in Medicine, vol. 24, no. 8, pp. 1163–1166, 2005.

A. Mirshemirani, F. Roshanzamir, S. Shayeghi, and L. Mohajerzadeh, “Diphallus with imperforate anus and complete duplication of recto-sigmoid colon and lower urinary tract,” Iran Journal of Pediatric, vol. 20, no. 2, pp. 229–232, 2010

S. Priyadarshi, “Diphallus with ectopic bowel segment: a case report,” Pediatric Surgery International, vol. 21, no. 8, pp. 681–683, 2005.

K. Gyftopoulos, K. P. Wolffenbuttel, and R. J. M. Nijman, “Clinical and embryologic aspects of penile duplication and associated anomalies,” Urology, vol. 60, no. 4, pp. 675–679, 2002.

S. Acimi, “Complete diphallia,” Scandinavian Journal of Urology and Nephrology, vol. 38, no. 5, pp. 446–447, 2004.

M. E. Torres, P. J. C. Sanchez, T. A. Aragon, T. V. Camacho, and G. A. Colorado, “Diphalia,” Revista Mexicana de Urología, vol. 69, no. 1, pp. 32–35, 2009.

Wikipedia “Diphallia” Wikipedia 2016.

M. C. de Oliveira, R. Ramires, J. Soares, A. P. Carvalho, and F. Marcelo, “Surgical treatment of penile duplication,” Journal of Pediatric Urology, vol. 6, no. 3, pp. 257-e1–257-e3, 2010

P. Tirtayasa, R. Prasetyo, and A. Rodjani “Diphallia with Associated Anomalies: A Case Report and Literature Review,” Case Reports in Urology Vol. 2013, Article ID 192960, 2013.