An extremely rare case of diphallia with high anorectal malformation with perineal lipoma

Diphallia is a very rare congenital anomalies occurs 1: 5-6 Million live birth. Associated anomalies like imperforate anus, vertebral deformities, double bladder, exstrophy of the cloacae, and duplication of the recto sigmoid may occurs in association with this. Amongst all anorectal malformations is a common congenital anomaly that can occur in association with diphallia. Here we are presenting a neonate with double functional penis, partial scrotal dysraphism, high anorectal malformations, presence of spina bifida occulta and a perineal lipoma successfully managed by us at our center.


Introduction
Diphallia is a very rare congenital anomalies occurs 1: 5-6 Million live birth in the United States [1]. In India the exact incidence is not known. The first case was reported in 1609 by Wecker, in Bologna, Italy [2]. Since then upto 110 cases have been reported in the literature. This condition has existed in humans since ancient times. Based on the presence of one or two corpora cavernosa in each phallus, diphallia is respectively defined as bifid phallus and true diphallia. Here we are reporting a case of true diphallia with high anorectal malformations, perineal lipoma and spina bifida occulta. In this case successful staged reconstruction was done.

Case history
A neonate presented to us on day two of life with complaints of not passing stool, abdominal distention, had two penises and passing urine from both. The baby delivered from 30 years old prime mother with no antenatal complains, with no history of consanguinity of the parents, antenatal checkup and ultrasound was normal. On examination of baby mild hypothermia, tachycardia and dehydration was present. Abdomen was distended and soft, on perineal examination anal opening was absent, no perineal meconium stain or fistula found.
A small 2.5 × 2.5 cm perineal lipoma was found. Though the scrotum was single with well-developed rouge and two testes palpable in it however there were two median raphes (Fig 1). There was no urinary dribbling. There were two well form penis present side by side and on passing the feeding tube both penises had patent urethra with urine coming out from it. On examination of spine though externally looking normal but on palpation lower lumber spinous process not palpable, suggested features

Results and discussion
In diphallia the two external genitalia may vary in size and shape, either lying beside each other in a sagittal plane or one above the other, no two cases have been identical. According to Schneider classification, double penis is classified into three groups: glans diphallia, bifid diphallia and complete diphallia (double penis) [1][2][3]. According to Vilanora and Raventos, a fourth group called pseudodiphallia is added in which small accessory penile-like tissue is found [4]. The current widely accepted classification, introduced by Aleem, classifies double penis into two groups: true diphallia and bifid phallus [5]. True diphallia is caused by cleavage of pubic tubercle; bifid phallus is caused by separation of pubic tubercle. Each of these two groups is further subdivided into partial or complete. True diphallia is where each phallus has two corpora cavernosa and a single corpus spongiosum containing a urethra. In bifid phallus, each phallus has only one corpus cavernosum and one corpus spongiosum containing a urethra. Diphallia occurs in the fetus in fourth week of gestation by various possible embryological causes suggested are incompletely fused bilateral anlagen or a form of atavism, due to inhibited growth of the dorsal inferior wall of the urogenital sinus underlay accessory urethral development [6][7][8].
Another theory suggest that abnormal Mullerian duct termination was responsible for urethral duplication [9].

Theory suggested by Hollowell et. al. diphallia results
from the failure of fusion of mesodermal bands [10].
Another theory attributed it to an ischemic process in embryogenesis [11]. In Troyer theory, diphallia is due to mal development of the distal portion of the urethra is because of the uncoordinated growth of the urogenital sinus and the urethral plate [12]. Other systemic anomalies occurs in association with diphallia is due to a defect in connecting the genital tubercle. Urine may pass by one or both penises, as in our case coming from both penises and baby was continent. Scrotum may be normal or bifid. In our case bladder and rectum are single, the duplication of urethra occurred just below the bladder neck as seen in contrast study (Fig 2). Complete diphallus  Amputation of accessory penis along with urethral endto-side anastomosis in anterior urethra can be an option as done by some, we only remove the accessory penis with closer of urethral stump close to its bifurcation from main right urethra as in Fig 2, we can see that the course of right urethra is straight, left urethra is looking as originate as a branching from right main urethra below bladder neck [13,14]. On review of the literature, we did not come across any other case of this variety of the penile duplication with anorectal malformations.

Conclusion
Diphallia is a very rare congenital anomaly can occurs in association with anorectal malformations.
Managements of such cases should be individualized on the basis of the severity and spectrum of anomalies.

Compliance with ethical statements
Conflicts of Interest: None.