Transverse Testicular Ectopia: A Report of Five Cases and Review of Literature.

Transverse testicular ectopia (TTE) is an uncommon anatomical abnormality where both the gonads migrate toward the same hemiscrotum. Embryologically, several theories regarding the origin of TTE have been suggested including adhesion and fusion of developing Wolffian canals, aberrant gubernaculum, testicular adhesions, defective formation of the internal inguinal ring, and traction on the testis by persistent Mullerian structures. To date, about 100 cases of TTE have been reported in the literature. Herein, we report five cases of TTE operated in the Department of Paediatric Surgery, Sardar Patel Medical College, Bikaner, over a period of 5 years. All cases were in the age group of 3 months to 4 years, out of which four were diagnosed preoperatively by clinical examination and ultrasonography (USG). Two of these four cases presented with inguinoscrotal swellings and contralateral undescended testes and other two presented with absence of testes in the scrotum. One case presented with a scrotal abscess which revealed both the testes in the abscess cavity. All five cases were operated upon with herniotomy and transseptal orchidopexy and discharged successfully. TTE, although rare, has a spectrum of presentations and should be kept in mind as a possibility in cases of inguinoscrotal disorders in young age group. A preoperative USG may be helpful in conformation of diagnosis. Copyright:

INTRODUCTION

Transverse testicular ectopia (TTE) is a rare congenital anomaly. Associated abnormalities may include persistent Mullerian duct syndrome (PMDS), true hermaphroditism, inguinal hernia, hypospadias, pseudohermaphroditism, and scrotal anomalies. Variations in the anatomical position of the vasa deferentia and abnormalities of insertion of the vas into the testis can occur. The mean age of presentation is 4 years.[12]

The normal development of testes starts at 3 weeks of gestation when germ cells start migrating from the yolk sac to the genital ridge.[23] The scrotal development occurs by fusion of labioscrotal folds by 9th week. Now, the testicular descent occurs in which various factors play role including hormonal, processus vaginalis, and intra-abdominal pressure. Testicular descent is complete between 37 and 40 weeks.

At any one of these developmental stages, anomalies can occur which result in problems after birth.

About hundred cases of TTE have been reported in literature in which both the gonads migrate toward the same side. The ectopic testis may lie in opposite hemiscrotum, in the inguinal canal or at the deep inguinal ring. An inguinal hernia is invariably present on the side to which the ectopic testis has migrated.[2]

Most cases of TTE present with a complaint of empty scrotum on one side with an inguinal swelling on the other side; however, rarely cases can present in emergency as a result of torsion or infection.

This is a report of five cases of TTE which presented to the Department of Paediatric Surgery, Sardar Patel Medical College, Bikaner, in the last 5 years. Furthermore, this study emphasizes on avoiding excessive dissection during surgical management of TTE solely to gain length for transseptal orchidopexy. It helps preserve the blood supply to the testes and gives better results. In our experience, adequate length can be achieved in most cases for a tension-free transseptal orchidopexy. All the cases have been tabulated in [Table 1].

Table 1

Details in tabulated form

Name	Age at presentation	Clinical findings	USG findings	Treatment	Follow up
A	3 years	Bilateral undescended testis	Both testes visualized in right inguinal canal	Transseptal orchidopexy with herniotomy	6 months. No abnormality detected
B	3 months	Right large inguinoscrotal hernia with left undescended testis	Both testes located on right side	Transseptal orchidopexy with herniotomy	6 months. No abnormality detected
C	9 months	Left hemiscrotal abscess	USG not done	Exploration with drainage of pus followed by herniotomy and transseptal orchidopexy	3 months. Wound sepsis
D	4 years	Bilateral undescended testis	Both testes located on left side	Transseptal orchidopexy with herniotomy	4 months. No abnormality detected
E	4 months	Left inguinal hernia with right undescended testis	Both testes located on left side	Transseptal orchidopexy with herniotomy	6 months. No abnormality detected

USG: Ultrasonography

CASE REPORT

Case 1

A 3-year-old male child presented with bilateral undescended testes (UDT). Both the testes were detected on bilateral inguinoscrotal ultrasonography (USG) in the right inguinal canal and confirmed intraoperatively. Both the testes had separate vas deferens. The patient was operated upon through a right inguinal incision. Both the testes were brought down after herniotomy. Right orchidopexy was done and the left testis was placed in corresponding to hemiscrotum through transseptal route. The patient's postoperative recovery was uneventful.

Case 2

A 3-month-old boy presented with large right inguinoscrotal hernia and left UDT [Figure 1]. The right testis was in place. An USG of bilateral inguinoscrotal region was conducted, revealing both testes on the right side which was confirmed intraoperatively [Figure 2]. A transseptal orchidopexy was performed on the left side after herniotomy. The patient's postoperative recovery was uneventful.

Figure 1

Right inguinal hernia with left-sided undescended testis

Figure 2

Both the testes in the right inguinal region of a patient with right inguinal hernia on inguinal exploration

Case 3

A 9-month-old male child presented to the emergency with high-grade fever and left hemiscrotal abscess. Clinical examination revealed a large scrotal abscess and both the gonads were not separately palpable. USG was not done in this case. On exploration, around 50 ml of frank pus was drained and both the testes were found to be present in the left hemiscrotum in the abscess cavity. Transseptal orchidopexy was done with herniotomy [Figure 3]. After thorough debridement and irrigation of abscess cavity, primary closure of wound was done. In postoperative period, wound sepsis was noted.

Figure 3

Both the testes in the same hemiscrotum (transverse testicular ectopia) in case of scrotal abscess

Case 4

A 4-year-old boy presented with bilateral UDT. An USG of bilateral inguinoscrotal region was performed revealing both testes in left inguinal canal. The USG findings were confirmed intraoperatively. Both the testes were brought down and transseptal orchidopexy after herniotomy was performed. Postoperative recovery was uneventful.

Case 5

A 4-month-old patient came with a large left inguinal hernia and right UDT. An USG of bilateral inguinoscrotal region was done revealing both testes in left inguinal canal. Findings were confirmed intraoperatively and transseptal orchidopexy with herniotomy was done.

DISCUSSION

TTE is a rare condition presenting with UDT and contralateral inguinal hernia. TTE was first described by Von Lenhossek in 1886.[2] The average age of presentation is around 4 years.[24] Mostly, the cases are diagnosed during surgery. Preoperative diagnosis can be made by USG or magnetic resonance imaging (MRI). Some cases can be diagnosed clinically when both the testes are palpable on the same side with an empty scrotum on the opposite side.

Many explanations have been postulated to explain the entity of TTE. Berg postulated that the two testes arise from the same germinal ridge [5] whereas Josso suggested various anatomical factors that may result in TTE which may include abnormal adhesions between the testes and adjacent structures or a narrow inguinal ring causing obstruction.[6] Gupta and Das postulated that adherence and fusion of the developing Wolffian ducts took place early and that descent of one testis caused the second one to follow.[7] Paltii suggested a defective implantation of the gubernaculum testis or an obstruction of the inguinal ring preventing testicular descent on the ipsilateral side.[8] Even though numerous theories have been postulated to explain TTE, no definitive conclusion can be drawn.

Depending on the presence of various associated anomalies, TTE has been classified into three types:[24]

Type 1 – Accompanied only by hernia (40% to 50%)

Type 2 – Accompanied by persistent or rudimentary Mullerian duct structures (30%)

Type 3 – Associated with disorders other than persistent Mullerian remnants (inguinal hernia, hypospadias, disorders of sexual differentiation, and scrotal abnormalities) (20%).

TTE in association with PMDS is a rare entity and it was first described by Jordan in 1895 and was thought to be due to insufficient amount of anti-Mullerian hormone or due to insensitivity of the target organ to this hormone.[29] Whenever PMDS is suspected on USG or MRI, it has to be confirmed with a diagnostic laparoscopy, testicular biopsy, and chromosomal studies.[10] TTE is at increased risk of malignant transformation with an overall incidence of 18%, which is more than that of UDT. The common malignancies include embryonal carcinoma, seminoma, yolk sac tumor, and teratoma.[11] There are not many reports of malignancy arising from the Mullerian remnants, so there is currently no indication for need to remove the remnants which may injure the vas and blood supply to testes.[10] The available management options include inguinal exploration and orchidopexy, diagnostic laparoscopy and orchidopexy, diagnostic laparoscopy and transseptal orchidopexy, diagnostic laparoscopy, and transseptal contralateral orchidopexy.[12] The finding on exploration dictates the management strategy and should be individualized. An algorithm has also been postulated for the management of TTE, which was given by Bascuna et al.[13] who performed extensive dissection in order to gain length of vas and vessels.

Vinod et al.[2] proposed a modification of their algorithm and proposed not to perform extensive dissection to gain length because of the intricate blood supply which may be shared between the testes and their vas, and instead perform bilateral orchidopexy on the same side if transseptal orchidopexy is not possible.

In our study, all the cases were managed by transseptal orchidopexy with herniotomy as adequate length could be achieved in each case. No patient had any complaints in a 3–6-month follow-up period.

CONCLUSION

TTE is a rare anomaly with little literature and research to provide guidance regarding its thorough management, long-term effects, and possible complications. Its pathogenesis still remains unclear which means an embryologically and anatomically correct management cannot be definitely defined. Any surgeon operating inguinal hernias and UDT should be aware of this condition and its surgical management in case it is encountered during surgery. It is thought that it will be beneficial to keep this rare clinical entity in mind in cases of bilateral UDT. A preoperative USG and MRI can be the useful tools in diagnosis of suspected cases.

We suggest that all the cases of inguinoscrotal swellings and UDT should at least be subjected to USG. MRI and diagnostic laparoscopy should be reserved when diagnosis is in doubt or other congenital malformations are suspected. We also observed that sufficient length can usually be achieved without extensive mobilization for a tension free transseptal orchidopexy in most of the cases.

Further studies to assess its pathogenesis may help in selecting the ideal treatment for each type of TTE.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.