Abstract
A small subset of males presenting with cryptorchidism are found to have ectopic testes. The most common location for an ectopic testicle is the superficial inguinal pouch, but there are several other possibilities, necessitating a thorough physical exam.
A 4-month-old term male presented for evaluation of a nonpalpable right testicle. Examination revealed an ectopic right testicle located in the perineum. The patient underwent successful right orchiopexy.
Ectopic testes are rare but remain in the differential diagnoses for patients with cryptorchidism. Patients should undergo prompt orchiopexy to avoid potential complications and allow maximal preservation of testicular function.
Keywords: Ectopic, Perineal, Testicle, Cryptorchidism
1. Introduction
Cryptorchidism is a common condition affecting 2–3% of males at birth, with a higher prevalence in preterm males.1 In the majority of cases, the testicle is located along the normal course of descent. However, a small percentage of cryptorchid males are found to have testicles in ectopic positions. The most common ectopic location is the superficial inguinal pouch.2 Due to risks associated with undescended testes, prompt diagnosis is critical and surgical management is recommended in the first 6–18 months of life.
2. Case report
A 4-month-old term male presented to the urology clinic for evaluation of a nonpalpable right testicle. The patient's prenatal history was unremarkable. The parents reported that they had never been able to feel the right testicle. On physical exam, the left testicle was palpated in the left hemiscrotum and the right testicle was palpated in the perineum (Fig. 1). The patient subsequently underwent an open right orchiopexy at 9 months of age, which was performed through an inguinal approach. Once the testicle was delivered through the incision, the patient was found to have an incompletely fused epididymis and closed processus vaginalis. The gubernaculum was noted to be attached to the perineum and not the scrotum (Fig. 2). The testicle was placed within a dartos pouch and secured with Monocryl suture. The patient recovered appropriately from the procedure without complication. At the 8 month follow up visit, both testicles were palpated in the scrotum and equal in size.
Fig. 1.
Examination revealing the perineal location of the right testicle.
Fig. 2.
Demonstration of the gubernacular attachment to the perineum.
3. Discussion
Cryptorchidism is defined as the absence of one or both testes in the scrotum.2 Spontaneous descent may occur during the first 6 months of life and only 0.8% of boys are affected by 12 months of age.1 In most cases of cryptorchidism, the testes have incompletely traversed the normal pathway of descent. However, they can also migrate to an ectopic position or have vanished as a result of torsion or a vascular accident.2
The indifferent gonad develops adjacent to the rudimentary kidney and descends transabdominally beginning at 5 weeks gestation. By 10 weeks, it is in close proximity to the internal inguinal ring, and by 12 weeks the gubernaculum is visible as it begins its swelling phase. The swelling of the gubernaculum, in addition to development of the cremaster muscle and migration of the processus vaginalis, are thought to produce widening of the inguinal canal necessary for testicular passage. Between 20 and 28 weeks the testis passes through the inguinal canal, with further caudal descent into the scrotum occurring up until birth. This is accompanied by regression of the gubernaculum.2
The testicle becomes ectopic when it deviates from the normal pathway of descent. Possible ectopic locations include the superficial inguinal pouch, perineum, penis, lateral to the scrotum, pubic region, thigh or contralateral scrotum. The most common location is the superficial inguinal pouch, and the perineum is the second most common location.1 However, perineal ectopic testes are rare and have been estimated to comprise only 1% of all cases of undescended testes.3 There is no statistically significant difference in the rates of patent processus vaginalis and epididymal abnormalities in ectopic testes compared to undescended testes irrespective of location. The testicular histopathology of these two groups has also been shown to be similar. These findings suggest they may have a similar etiology, which is thought to be multifactorial but has not been fully elucidated.1 Risks associated with both ectopic and undescended testes include torsion, as well as an increased incidence of infertility, trauma and malignancy. Associated anomalies include testis-epididymis dissociation and inguinal hernias.2
The assessment of a neonate with an undescended testis should begin with a thorough history, including risk factors such as prematurity and low birth weight, followed by a physical exam. Findings on exam, particularly unilateral versus bilateral undescended testes and whether an undescended testis is palpable or non-palpable, guide any additional workup and treatment. The treatment for ectopic testes is surgical and, as opposed to testes located in the normal pathway of descent, there is no need to wait for descent. Open orchiopexy is performed through an inguinal incision to allow for concomitant treatment of an inguinal hernia if present.4 This remains the optimal choice for management, even in the setting of a delayed diagnosis.5 Patients and their parents should be counseled regarding the increased risk of subfertility and malignancy and taught to perform self-examinations after puberty.2
4. Conclusion
In patients with cryptorchidism, all possible ectopic sites should be evaluated. Ectopic testes are best managed with surgical intervention.
Approval of the research protocol by an institutional reviewer board
The Institutional Review Board of Loma Linda University Health determined this case report does not meet the definitions of human subject research and does not require IRB review or approval. These findings were communicated in a notice of determination of human subject research, IRB #5220049.
Informed consent
Written consent for publication of this case report, including the use of photographs, was obtained from the patient's legal guardian.
Registry and the registration no. of the study/trial
N/A.
Funding statement
No external funding was received.
Declaration of competing interest
The authors declare no conflict of interest.
References
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