Shahid A. Khan ( Departments of Urology and Radiology, Royal Sussex County Hospital, Brighton BN2 5ES, United Kingdom )
George John ( Departments of Urology and Radiology, Royal Sussex County Hospital, Brighton BN2 5ES, United Kingdom )
Jonathan L. Richenberg ( Departments of Urology and Radiology, Royal Sussex County Hospital, Brighton BN2 5ES, United Kingdom )
Polyorchidism is a rare congenital anomaly, which is defined as the presence of more than two testes. We report a case of polyorchidism in a 22-year-old man that was detected by ultrasonography of the scrotum
A 22-year-old man presented in clinic with a 8-year history of a painless lump in his right hemi-scrotum. Physical examination was unremarkable with normal secondary sexual characteristics. The left side of the scrotum contained a normal testis, epididymis and vas deferens. On the right a discrete, non-tender, non-transilluminating lump adherent to the testicle was palpable.
Testiclar tumour markers were normal. Scrotal ultrasound confirmed the presence of two oval shaped homogenously echogenic structures on the right appearing as normal testes with similar echogenicity to the left testicle having a common epididymis (Figure). As the sonographic appearances were characteristic of polyorchidism and the patient was asymptomatic, no further action was deemed necessary.
Polyorchidism is a rare anomaly of the genitourinary tract with less than 100 cases reported in the literature. The first accepted case was a post-mortem rport by Ashfield in 1880.1 However, Lane reported the first
Figure. Sonogram showing two homogeneous testicles on the right having identical echogenicity to the larger normal left testicle.
case of polyorchidism confirmed by excision of the extra testicle in 1895.2
Triorchidism is the most common presentation but a patient with 5 testes in the scrotum has been described.3 The left side is affected predominantly and nearly half of the cases are seen between 15 and 25 years of age.4 The scrotum is the location of supernumerary testicle in most of the reported cases but other sites include the inguinal canal and the retroperitoneal space.5 Polyorchidism seldom produces symptoms and is usually discovered incidentally. Associated pathologies or anomalies reported are maldescent (15%), inguinal hernia (20%), hydrocele (9%), varicocele (1%), inguinal mass (66%), torsion (7%), cancer (7%) and infertility (20%).6
Embryologically, the testis develops from a thickening along with the medial edge of the mesonephros called the urogenital ridge. Epididymis and the vas deferens are formed from ducts and tubules in the degenerating mesonephros. As the testis descends, the cephalad ducts and tubules degenerate while new ones develop caudally.7
The exact explanation of polyorchidism is not known but the possible aetiology is thought to be due to either an accidental longitudinal duplication of the urogenital ridge or a transverse division or disappearance of the mesonephric tubules during descent, with upper and lower tubules remaining and the middle section disappearing. Peritoneal bands or a local accident may be responsible for separation of the mesonephric tubules into two groups.5,8 Given that in our case both the testicles had a common epididymis, a transverse division seems more likely but this cannot be accurately substantiated, as our patient was not explored.
Recent improvements in ultrasonography and the provision of high-resolution images have made it easy to differentiate a normal testis from a tumour mass making it possible to diagnose polyorchidism on sonography alone. Colour Doppler can provide further information about the blood flow and vascularity. In some cases MRI may provide confirmation and reassurance when the results of sonography are inconclusive.9
The management of polyorchidism is not well established, as the condition is rare. In cases where polyorchidism is associated with cryptorchidism some clinicians recommend an orchidopexy, surgical exploration with or without biopsy or an excision.10 However, as high-resolution sonography can provide an accurate diagnosis of polyorchidism and in the absence of any concomitant disorder or anomaly, surgical exploration and/or excision seems unnecessary.11
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