Transverse Testicular Ectopia with Fused Vas Deferens: A Case Series.

Transverse testicular ectopia (TTE) is a rare anomaly where both gonads descend towards the same side of the hemiscrotum. Although more than 100 cases of TTE are reported, TTE with fused vas are extremely rare. We present our series of 4 TTE patients with fused vas [4, 7, 9 and 26 months old]. Three cases were diagnosed at the time of inguinal exploration and one during a planned laparoscopic inguinal hernia repair. An indirect inguinal hernia with TTE was noted with a fused proximal vas deferens in all 4 cases [Figure 1]. Inguinal herniotomy, ipsilateral orchidopexy along with contralateral transeptal orchidopexy (testis brought down through the inguinal canal on the same side of hernia) [Figure 2] was performed in all patients without any complication. None of the patients had uterine tissue. Testicular biopsies, Array and karyotyping (46XY) were normal in all 4 patients. Follow-up ultrasound doppler scans revealed normal testes with good vascularity in all patients. Fused vas with TTE is rare but transeptal orchidopexy is feasible with excellent outcome. Copyright:

INTRODUCTION

Transverse testicular ectopia (TTE), also called as crossed testicular ectopia, is a rare anomaly first described by Von Lenhossek in 1886.[1] More than 100 cases have been reported in the English literature so far, but TTE with fused vas has rarely been reported. TTE was initially classified into three types [Table 1] based on the associated findings.[2] Type 1 is the most common type (40%–50%) of TTE associated with inguinal hernia, followed by Type 2 (30%) TTE associated with persistent Mullerian ducts (PMDs) and the least common Type 3 (20%) TTE associated with other anomalies such as hypospadias.[3] Preoperative ultrasound and magnetic resonance imaging can help diagnose TTE.[4] However, many cases are often diagnosed intraoperatively during inguinal exploration for herniotomy or laparoscopy.[5] We present a series of four cases with TTE with proximal fused vas, diagnosed intraoperatively and managed successfully with transeptal orchidopexy.

Table 1

Classification of (crossed) transverse testicular ectopia (Esteeves et al., Sau Paulo Med J, 1995)

Classification of (Crossed) TTE
Type	Anomaly
Type 1	Simple TTE associated with inguinal hernia only
Type 2	TTE associated with persistent Mullerian remnants
Type 3	TTE associated with other anomalies

TTE: Transverse testicular ectopia

METHODS

This was a retrospective review of medical records of children with TTE and fused vas deferens managed between 2000 and 2015 at our institution. Data were collected on age at diagnosis, intraoperative findings, type of TTE, and follow-up.

CASE SERIES

Four patients were identified (aged 4, 7, 9, and 26 months) during the study period of 5 years. All four patients presented with unilateral inguinal hernia; palpable ipsilateral undescended testis and impalpable contralateral testis. Preoperative karyotyping (46XY) and array were normal in all four patients. Preoperative ultrasound examination showed left-sided inguinal hernia in two patients and right-sided inguinal hernia in two patients with ipsilateral testis seen but failed to identify the contralateral impalpable testis in all four patients. Inguinal exploration was performed in three patients and the TTE diagnosed at the time of inguinal exploration. The last case was diagnosed during a planned laparoscopic inguinal hernia repair.

An indirect inguinal hernia with TTE was noted with an intraperitoneal contralateral testis and a fused proximal vas deferens in the pelvis [Figure 1] in all four patients. Biopsy of the impalpable testis was done for the first case which showed no evidence of malignancy and was reported as normal testicular tissue. All three patients underwent inguinal exploration, inguinal herniotomy, and ipsilateral orchidopexy along with contralateral trans-septal orchidopexy (testis brought down through the inguinal canal on the side of hernia but placed into the corresponding hemiscrotum) through an extended herniotomy incision [Figure 2]. In the last case, after diagnosis of TTE by laparoscopy, inguinal exploration was done and a similar procedure to previous three cases was performed. The patient characteristics of all four patients are presented in Table 2. Follow-up with Doppler ultrasound scans revealed normal testes with good vascularity in all four patients at 18 months, 3 years, 10 years, and 12 years.

Figure 1

Intraoperative picture of case 1 during transverse testicular ectopia repair

Figure 2

Intraoperative picture of case 1 after trans-septal orchidopexy

Table 2

Patient characteristics in our series

Cases	Age at presentation (months)	Diagnosis made	Findings (nonpalpable testis)	TTE type	Follow-up	Doppler at follow-up
Case 1	4	Intra-operative	TTE (left) with fused VAS	Type 1	3 years	Both testis good flow
Case 2	7	Intra-operative	TTE (right) with fused VAS	Type 1	10 years	Both testis good flow
Case 3	9	Intra-operative	TTE (left) with fused VAS	Type 1	12 years	Both testis good flow
Case 4	26	Intra-operative	TTE (right) with fused VAS	Type 1	18 months	Both testis good flow

TTE: Transverse testicular ectopia, VAS: Visual analog scale

DISCUSSION

Several theories have been postulated regarding the etiology of TTE. Berg suggested that both testes may arise from the same genital ridge and this leads to TTE.[6] However, Gupta and Das suggested that mesonephric and Mullerian ducts adhere and fail to separate.[7] Defective or ruptured gubernaculum was also suggested as a cause of TTE.[8] Familial incidence of TTE has been reported between two brothers, suggesting a possible genetic etiology as well.[9] The exact etiology of TTE still remains unclear. Karyotyping is essential, as XX maleness, ovotesticular disorders of sex development, and TTE are reported together.[10]

Laparoscopy helps to establish the anatomy including identifying PMD remnants and fused vas, which could also be done by open inguinal exploration. Mobilization of fused cord or vas and excising PMD remnants are challenging, and extended inguinal exploration with trans-septal orchidopexy (Ombredanne or modified procedure) [Figure 3] is often necessary; however, this has been managed laparoscopically as well.[1112] Laparoscopy is now being increasingly used for diagnosis and correction of TTE and PMD remnants excision. Frequently, the vas and the PMD remnants are joined together, but laparoscopic dissection of these structures is feasible and can minimize the damage to vas.[12] PMD remnants should always be excised as there is a risk of malignancy,[13] and the testes should be monitored regularly as at least 6% of TTE is reportedly associated with testicular tumors, usually seminomas.[14]

Figure 3

(a) Illustration showing left testicular ectopia with right inguinal hernia, (b) Illustration showing left transverse testicular ectopia fixed by trans-septal (modified Ombredanne procedure) orchidopexy

Usually, the cords of both testis are fused, which includes both the vas and testicular vessels. However, in our cases, the testicular vessels were separate, and only the proximal vas was noted fused. Only five cases of proximal fused vas have been reported so far (one of them not associated with TTE),[15] and our case series reports four such cases of Type 1 TTE with proximal fused vas being seen in all four patients. Separation of the fused cord or vas is not advised, and a conservative approach is suggested with trans-septal orchidopexy alone. This has resulted in all our four cases with good vascularity seen on Doppler scan at follow-up even after 12 years.

CONCLUSION

Fused vas with TTE is rare, but trans-septal orchidopexy is feasible with excellent outcome. Long-term follow-up is essential as there is a small but known risk of malignancy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.