Testicular maldescence is the most common anomaly of the genitalia with incidence of 1.5%. It is reported that 5 % of maldescended testes are ectopic¹. Etiopathogenesis of perineal ectopic testis (PET) is controversial. The role of the gubernaculum is not well elucidated. For some authors this ectopy results from a gubernaculum failure and it's due to an anomaly of fixation of the distal extremity of the gubernaculum testis resulting in an abnormal position of the testis^2,3.

The five major sites of ectopic testes are the superficial inguinal pouch, femoral, suprapubic, transverse (contralateral hemiscrotum), and perineal³. PET is a rare congenital anomaly in which the testis is abnormally situated between the penoscrotal raphe and the genitofemoral fold⁴. For some authors this ectopic testis may be due to a mechanic obstruction of scrotal inlet or in the entrance of the testis in the scrotum (which become fibrous) that leads the gonad to an abnormal position⁵.

A 21-year-old man was admitted to the urology clinic because of perineal pain while he is walking and sitting and right empty scrotum. General and systemic examination revealed nothing abnormal. Perineal examination showed a right empty and less developed scrotum. The left testis was present in the scrotum but right testis was not present in the scrotum and inguinal region. An oval shaped soft mass was found in the perineal region. The diagnosis of right ectopic testis was made (Figure 1). By ultrasonography, a right PET that is 3.8x2.0x2.2 cm in diameter and normally echogenicity was demonstrated. Transrectal and urinary system ultrasound showed no pathology. His medical and family histories were unremarkable. The semen parameters, sex hormones and biochemically parameters were normal. Surgical exploration through inguinal incision revealed gubernaculum testis attached with perineal tissues. The testis was mobilized and gently delivered into the inguinal wound. It was found to be small in size with adequate length of the vas deferens and spermatic vessels; the testis was fixed in the ipsilateral scrotum using the standard dartos pouch technique (Figure 2). Follow-up revealed right testis present inside the scrotum (Figure 3).

Figure 1: The right perineal testis that was observed by perineal examination.

Figure 2: The right testis that fixed in the ipsilateral scrotum using the standard dartos pouch technique.

Figure 3: The right testis that replaced inside the scrotum

The cause of testicular ectopia is unknown but it is thought to be due to abnormal position of genito-femoral nerve which leads to an abnormal migration of gubernaculum and thus takes testis to abnormal position⁶. Ectopic testis can be found at opposite side of the scrotum, suprapubic region, femoral canal, perineum, and anterior abdominal wall⁷. Perineal testicular ectopia is seen very rarely and bilaterality is even more scarce⁸. The perineal ectopic testes have been reported in the literature and 80% of these cases are unilateral⁴. An empty scrotum with a soft perineal mass on ipsilateral side is very suggestive of perineal testis as was the case in this patient⁹. As this case, perineal pain may be a symptom of PET, too. Some cases of ectopic testis diagnosed on ultrasound, antenataly at 38 weeks and confirmed in postnatal period by clinical examination, are also reported².

Ectopic location of the testes is associated with a number of complications like trauma, torsion and infertility⁴. It is generally accepted that undescended testis should not be operated before 6 months of age, but surgery for ectopic testes should be carried out before the age of 6-months even if not associated with inguinal hernia¹.

Perineal ectopic testes are usually explored through standard inguinal skin-crease incision; some surgeons use a low scrotal approach due to the low incidence of concomitant hernia⁹. Perineal ectopic testis is frequently associated an inguinal hernia and can sometimes be associated with other disorder such as hypospadias and scrotal anomalies¹⁰. The hernia sac and other anomalies weren't noted in this patient. Gubernaculum is usually found fixed to the perineum as was noted in this case. The testes can be placed in ipsilateral hemiscrotum easily because the spermatic cord and vessels will be sufficiently long¹. The functional outcome of ectopic testis is difficult to define, but has been found to be similar to other forms of maldescended testis⁹. Histopathologically, the course of perineal ectopic testis is better than undescended testis⁵. Consequently, PET is certainly kept in mind in differential diagnosis of perineal pain and empty scrotum and also all of the cases should be researched in terms of PET.

Conflict of Interest
No conflict of interest was declared by the authors