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Penile Duplication with Two Anal Opening A Rare Case Report

Mohammad Refaei¹, Mohamed Abdel Al M.Bahkeet², Saleh AL Rowally³

¹Pediatric Surgery Department, Arar Central Hospital
²Pediatrics Department, Sohag University Hospital,
³Pediatrics Department, Arar Central Hospital,
Kingdom of Saudi Arabia

 

Correspondence 

Mohammad Refaei, HOD & Consultant Pediatric Surgeon
Arar Central Hospital,
P.O.Box.1938,Arar City
Northern Region, Kingdom of Saudi Arabia.
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

We encountered a complete phallus or penile duplication (diphallia) in a 21 day old Syrian neonate, with two scrotums and each one carrying two palpable testis. Both penises have normal shaft (size 5 cm each) with normally located me- atus.The voiding urine from two penises was at the same time during micturation. The child has also a fold of redundant skin about 4x5 cm at the anal region in which were placed two separate anal opening. Per rectal examination detected normal ani and both pass feces at the same time during defecation. The perineum was normal. No dysmorphic features and no associated other congenital anomalies. All laboratory results were within normal range for his age. Voiding cystourethrography revealed two penies with two separate meatus and one bladder in which the two urethral open seperatelly. IVP showed two normal kidneys and two ureters. Barium study reveled duplication of rectum and colon, otherwise normal GIT pattern. In our exhaustive review of the literature, we did not come across any other case of this variety of the penile duplication and congenital presence of two ani (anuses).

key words: Diphallia (duplication of penis), ani ( anuses), penile duplications

 

Introduction

Penile duplication (PD) or diphallia, is a medical condition in which a male infant is born with two penises This is an extremely rare disorders with only approximately 1000 cases of diphallia recorded since the first, reported by Jahannes Jacob Wecker in 1609. Its occurrence is one in 5.5 million men in the uinited states [1]. When diphallia is present, it is usually accompanied by other congenital anomalies such as renal, vertebral, hindgut or anorectal duplication [2]. There is also a higher risk of spina bifida. Infants born with PD and its related conditions have a higher death rate from various infections associated with their more complex renal or colorectal systems. It is thought diphallia occurs in the fetus between the 23rd and 25th days of gestation when an injury, chemical stress, or malfunctioning homeobox genes hamper proper function of the caudal cell mass of the fetal mesoderm as the urogenital sinus separates from the genital tubercle and rectum to form the penis [3, 4]. On the other hand anal canal duplication is a very rare congenital malformation: the most distal and least common duplication of the digestive tube. It can be confused with other types of anorectal pathology including hemorrhoids, fistula-in-ano, and perirectal abscess. Anal canal duplications are usually located posterior presenting as a one to 2 mm perineal opening just behind the anus in the midline [5, 6]. The tract runs along the posterior aspect of the anal canal without communication with the anorectum. Simple perineal inspection makes the diagnosis [7, 8]. Most children are asymptomatic. Associated malformations include sacrococcygeal teratomas, dermoid cysts, sacral dysgenesis, hindgut anomalies and lumbosacral myelomeningocele. Non-invasive preoperative investigations consisting of pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved to evaluate the presence of associated anomalies [9].

Case presentation

History: We report a 21 days old Syrian neonate, who presented to our OPD clinics by his parents as duplication of penis and two separate anal opening. A product of normal vaginal delivery to Syrian couple in beginning of their life, mother not exposed to harm during pregnancy, with smooth delivery, they belonging to same extended family.

On examination: The child weight 4.3 Kg, no dysmorphic features and no associated other congenital anomaly, his feeding well. Abdominal, chest and heart examinations were normal. There was a true duplication of penis, both equal in size (5 cm in length), normal located meatus and voiding clear urine from both at same time ( fig. 1, 2). They attached to two scrotums each one carrying two palpable separate normal testes and separate from each other by smooth fold of skin about 2 x 2 cm in width free from any skin rouge. ( fig. 3) Voiding of urine was forcibly and pass from both penises at the same time during micturation.The urine was clear and not mixed with fecal matter, it means no associated fistulae. There was a redundant skin fold over the perineum 4 x 5 cm in which two separate anal opening passed fecal matter at same time ( fig. 4). We were able to pass two separate thermometers in each anal opening freely ( fig. 5). With PR examination of the two anal opening revealed normal ani with double rectum and

 

14 JPSS 8 1 2010-14 -1 14 JPSS 8 1 2010-14 -2
Figure 1: two penises and two scrotums Figure. 2: completely 2 separate normal scrotums and penises

14 JPSS 8 1 2010-14 -3 

Figure. 3: Skin fold separate the 2 scrotums

14 JPSS 8 1 2010-14 -4  14 JPSS 8 1 2010-14 -5
Figure 4: skin fold with two anal openings Figure 5: two thermometers freely enter in two anal openings

 

Discussion

Penile duplication (synonym diphallus, bifid penis, penis duplex) is a rare anomaly with an incidence of 1 in 5,500,000. It is almost always associated with other malformations like double bladder, extrophy of the cloacae, imperforate anus, duplication of the rectosigmoid and vertebral deformities. The duplication may involve whole or only part of the penis. The urine may be passed by one or both penis. It is suggested that the anomaly results from failure of fusion of mesodermal bands. Treatment is by excision of the duplicated non communicating glans. Complete diphallus is best treated by excision of the less well developed penile structure and its urethra [4].

In our current case we found a true duplication of the penis with normal shaft and normally located meatus. They voiding clear forcible urine at same time. They were attached to two scrotums, which are adherent to each other. Both separated from each other by smooth fold of skin 2 x 2 cm in width free from any skin rouge. Each scrotum was carrying two palpable testes. The baby also has a redundant skin fold in which two separate functioning anuses open. By PR examination and with barium study revealed two recti, two colon and two anal openings and normal perineum. In our exhaustive review of the literature, we did not come across any other case of this variety of the penile duplication and congenital presence of two ani with duplication of rectum and colon.

This type of anomaly is a very rare association. The child is health and feeding well. The baby is followed up in our OPD clinic and we have not decided the type of operative corrective surgery. The surgical interference depends on the baby status and parent consent. However we intend to choose the corrective surgery for him after 1 year of age, in order to have one penis and one anus.

Conclusions

Penile duplication is a rare anomaly. Thorough investigations are mandatory in all cases to reveal underlying congenital malformations that is 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.

 

References 

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