Revista Mexicana de

Camacho-Gutirrez SF, et al. Genitourinary reconstruction in a case of penis duplication. CASO CLNICO Rev Mex Urol 2004; 64(3): 135-138

135 UROLOGIA
Vol.64, Nm. 3 Mayo-Junio 2004 135-138

Genitourinary reconstruction in a case of penis duplication associated to bladder duplication, perineal hypospadias and bowel sequestration
Sergio Francisco Camacho-Gutirrez,* Carlos Olvera-Caballero,** Margarita Barrientos-Prez,*** Juan Manuel Aparicio-Rodrguez**** ABSTRACT Duplication of the genital phallus (the penis or clitoris) is a very uncommon genitourinary malformation. It may present as true diphallia or as a bifid phallus, and is frequently associated to other abnormalities in the genitourinary tract and elsewhere. This report deals with one case of this very rare condition, stressing the need for a multidisciplinary approach to its treatment. Key words: Phallus duplication, bladder duplication, hypospadias, genitourinary reconstruction. RESUMEN La duplicacin del falo (pene o cltoris) es una malformacin genitourinaria poco comn. Puede presentarse como difalia verdadera o falo bfido, y est comnmente asociada a otras anomalas del aparato genitourinario. Presentamos el reporte de un caso de esta rara anomala, enfatizando la necesidad del manejo multidisciplinario. Palabras clave: Duplicacin de pene, duplicacin vesical, hipospadias, reconstruccin genitourinaria. INTRODUCTION Diphallia is a congenital malformation whose incidence has been estimated at approximately one per 5.5 million live newborns. It is usually associated to other genital and extragenital defects, such as hypospadias, epispadias, exstrophy of the bladder, anorectal anomalies, cryptorchidism, absent or duplicated bladder, bifid scrotum, and others.1-3 CASE REPORT This was a 16-year old male patient born in the northern mountainous range of the state of Puebla, Mexico. He had been catalogued as a girl at

* Pediatric Urology Service, ** Plastic Surgery Service, *** Endocrinology Pediatric Service, ****Genetics Service. Hospital para el Nio Poblano, Puebla, Pue. Mexico. Reprint request: Sergio Francisco Camacho-Gutirrez M.D. Calle 3 Poniente # 1309-203, Puebla, Pue., Mxico 72000. Tel./Fax: (222) 242-2814. E-mail: sefrac2000@yahoo.com

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Camacho-Gutirrez SF, et al. Genitourinary reconstruction in a case of penis duplication. Rev Mex Urol 2004; 64(3): 135-138

birth, wore womans clothes and had been confined to the familys home until adolescence. History revealed no other relevant data. The patient initially requested medical care in April, 1995. Physical exploration revealed a morphological alteration of the genitalia, with two phalluses, one on each side of the midline. The right phallus was 6 cm long and the left one, 7 cm long. Both phalluses were well developed; each had a slender chordee in its distal third, a distal glans with a central cleavage, and a urinary meatus at the base. The patient had urinary incontinence and thus used diapers. Besides, a red, two-centimeter tumor was found between the testicles (Figure 1). The karyotype of the patient was found to be 46-XY. The initial imaging studies included a pelvic X-ray, which showed no bony abnormalities. An excretory urogram revealed that each ureter drained to a separate bladder. Urethrocystoscopy revealed two urinary meatuses in a shotgun disposition. Visualization through each one of the urethras revealed two normal-looking bladders, each with a standard ureteral meatus, although the left one was wider. In a first surgical stage, excision of the perineal tumor and the right phallus was carried out. The decision to remove this phallus was based on its smaller size and on the fact that, during artificial erection, the right phallus had a more pronounced chorda and achieved less turgescence than the left one (Figure 2).

Figure 2. Post-operative view, first stage. The bowel sequestration and the right phallus have been removed.

Figure 3. Creation of the neo-urethra, centralization of the phallus and scrotum repair. Histopathologic studies reported that the red tumor was a bowel sequestration, and that the resected phallus was a penis with a corpus cavernosum and no urethra. In a second stage, the left phallus was repositioned more centrally, through Z-plasties in the skin

Figure 1. Pre-operative view. Diphallia and bowel sequestration.

Camacho-Gutirrez SF, et al. Genitourinary reconstruction in a case of penis duplication. Rev Mex Urol 2004; 64(3): 135-138

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erections, and has achieved urinary continence (Figure 4). DISCUSSION Several classifications have been suggested for this malformation. In 1972, Abdel proposed dividing the reported cases into two main groups: true diphallia (complete or partial) and bifid phallus (complete or partial).2 This classification stresses the need for the phalluses to have two corpora cavernosa and one corpus spongiosum in order for the condition to be consider a true diphallia. Schneider pointed out at three anatomical variants: glans diphallia, bifid diphallia and full diphallia or duplicate penis.4 Vilanova added a fourth variety called pseudodiphallia.5 This term is also used in Johnsons classification for cases with a single corpus cavernosum, in contrast to true diphallia, in which each phallus has two corpora cavernosa.6 According to Abdels or Scheniders classifications, our patient could be said to have complete bifid diphallia, since each phallus had only one corpus cavernosum. Most cases reported in literature as diphallia would really correspond to phallus bifidus, with a single corpus cavernosum in each penis and fully split to the base (complete phallus bifidus) or partially split (phallus bifidus or bifid glans).2 The association of other congenital abnormalities with penis duplication has also been described, and the range of these genital and extragenital disorders is very wide.7-10 Marti has mentioned the usefulness of preoperative ultrasound to assess the presence and number of corpora cavernosa or corpus spongiosum.11 Both this author and Abdel2 consider that phallus bifidus should be surgically treated, conjoining the two phalluses to preserve both corpora cavernosa. The patient treated by Abdel for complete bifid diphallia was two months old and the size of both phalluses made it possible to join them uneventfully. A recent report by Landy about a two-year-old patient with true diphallia describes removal of the accessory penis, which had corpora cavernosa and a corpus spongiosum.12

Figure 4. Post-operative view, two years later. Urinary continence and normal urination have been achieved. and removing the chorda. However, during surgery, it was considered necessary to perform a transverse incision on the corpus cavernosum and place a dermal graft to cover the defect due to persistent curving of the distal third of the phallus. In a third stage, a new urethra was formed, using an 8-cm long graft of bladder mucosa and perineal skin for 3 more centimeters. A terminoterminal anastomosis was performed. Initially, the two hypospadial shotgun urethras had been joined into a single one by means of an incision in their medial walls and a V suture (Figure 3). The two bladders were conjoined by removing their dividing wall and performing the marsupialization of their borders, creating a single bladder cavity. An urethrocutaneous fistula that developed after urethroplasty in the site of the proximal anastomosis was closed in an outpatient fashion. The patient has undergone four internal urethrotomies due to stenosis in the site of the neourethras proximal anastomosis and currently has a normal urinary stream. Finally, he underwent a cystolithotomy because of a single intravesical calculus; in the same stage, a replantation of the left ureter was performed, because grade IV vesicoureteral reflux had appeared. During this last surgery, it was decided to excise a portion of the residual intravesical septum and to create a single vesical neck. Nowadays, the patient leads his social life as a male, has normal

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Camacho-Gutirrez SF, et al. Genitourinary reconstruction in a case of penis duplication. Rev Mex Urol 2004; 64(3): 135-138

Early anatomical identification by ultrasound at birth is helpful in deciding whether to remove the accessory phallus or to join both. This malformation is apparently diagnosed at birth; however, there are cases that may go undiagnosed until adulthood because of its variability.13 Several embriological mechanisms may account for this malformation. Most theories state that the common denominator to explain these malformations is an inadequate development of the cloacal membrane and abnormal mesenchymal growth. In his excellent work, Hollowell points out the embriological implications involved in its appearance. He states that it is difficult to explain penis duplication, since in no stage of normal development is the genital tubercle a paired structure. However, it is formed with mesoderm from different sources and, if the process of fusion of its band fails, the expected malformation will be exstrophy of the bladder with a split penis. Furthermore, Hollowell maintains that, in order to explain urethral duplication and the presence of reasonably formed penises, there must be mesoderm around both primitive urethral pads.14 Kapoor published the case of a female newborn with complete duplication of bladder, urethra and external genitalia, emphasizing that duplication of the bladder and urethra are more common in males. He suggests that the malformation may be due to a partial cleavage of the caudal region in the embryo during development of totipotential cells.15 Esham reported the case of a 7-year-old girl with duplication of the bladder, the urethra and the large intestine. He mentions that the posterior primitive intestine is the anlage from which the terminal ileum, colon and cloaca arise, and that an early embriological duplication may have caused such malformation.16 (We mention this report, in spite of the fact that it deals with a female, in order to present a wider view).

Finally, it should be remembered that the management of patients with genital abnormalities needs a multidisciplinary approach in order to offer the best options. REFERENCES
1. Kaufman A, Guia R, Davila H, Kaufman A. Diphallus with third urethra. Urology 1990; 35: 257-60. 2. Abdel A. Diphallia: Report of a case. J Urol 1972; 108: 357-8. 3. Azmy A. Complete duplication of the hindgut and lower urinary tract with diphallus. J Pediatr Surg 1990; 25: 647-9. 4. Schneider P. Citado por Lattimer JK, Uson AC. Male genital tract. En: Ravitch MM, Welch KJ, Benson CD, Aberdeen E, Randolph JG, editores. Pediatric surgery. Philadelphia; Year book medical publishers, inc., 1979: 1338-44. 5. Vilanova X, Raventos A. Pseudodiphallia, a rare anomaly. J Urol 1954; 71: 338-46. 6. Dean JA, Horton CHE. Diphallia and hindgut duplication. Plast Reconstr Surg 1991; 87: 358-61. 7. Pendino J. Diphallus (double penis). J Urol 1950; 64: 156-7. 8. Kode G. Penile duplication. Brit J Plast Surg 1991; 44: 151-2. 9. Savir A, Lurie A, Lazebnik J. Diphallia. Report of a case. Brit J Urol 1970; 42: 498-500. 10. Zolfaghari A, Pourissa M, Hajialilou Sh, Amjadi M. True complete diphallia. Scand J Urol Nephrol 1995; 29: 233-5. 11. Marti L, Menor F, Gomez J, Cortina H, Garca F. Value of sonography in true complete diphallia. J Urol 1989; 142: 356-7. 12. Landy B, Signer R, Oetjen L. A case of diphallia. Urology 1986; 28: 48-9. 13.Fujita K, Tajima A, Suzuki K, Aso Y. Diphallia with a normal and a blind-ending urethra. Eur Urol 1979; 5: 328-9. 14. Hollowell J, Witherington R, Ballagas A, Burt J. Embryologic considerations of diphallus and associated anomalies. J Urol 1977; 117: 728-32. 15. Kapoor R, Saha MM. Complete duplication of the bladder, urethra and external genitalia in a neonate-a case report. J Urol 1987; 137: 1243-4. 16. Esham W, Holt HA. Complete duplication of bladder and urethra: a case report. J Urol 1980; 123: 773-5. 17.Rock SD, Geearhart JP. Complete lower urinary tract duplication with true diphallia presenting as a rare covered exstrophy variant. J Urol 1997; 157: 1907-8.