Remnant mullerian structure with transverse testicular ectopia

Abstract

29-year-old male patient with infertility, presented to emergency room with recurrent testicular pain. Patient had MRI done with suspicion of TTE. Laparoscopic exploration showed right spermatic vessels crossing the midline toward the left side with normal course of left vas. Inguinal exploration was carried out and showed common cord. Biopsy was sent and identified the presence of sperm in left testis only.

Introduction

Transverse Testicular Ectopia (TTE) is a rare type of undescended testes. It is characterized by migration of one testis to the contralateral route of testicular descent.¹ TTE is associated with a multitude of abnormalities including hernia, hypospadias, and persistent Mullerian structures.1, 2, 3 Our case is unique in terms of age of presentation and the full development of external genitalia.

Case presentation

Twenty-nine year-old, medically free male, with history of primary infertility for 4 years, visited emergency department multiple times due to severe left testicular pain that starts always after ejaculation. During every visit to the emergency department, scrotal ultrasound was done by emergency physicians with no signs of torsion. Diagnosed as chronic orchialgia, patient repeatedly was discharged with analgesics. Past surgical history includes left open inguinal hernia repair on 2009, repeated laparoscopically on 2013, and left sub inguinal microscopic varicocelectomy. Physical examination revealed normal male genitalia. Both testes were palpable within the scrotum. MRI pelvis showed a tubal structure with smooth wall muscle extending from the midline of the seminal vesicle until the left hemipelvis, with both cords extending to the left inguinal canal (Fig. 1). All previous histopathology slides from the previous surgeries were reviewed. The hernial sac showed endometrial cavity surrounded by smooth muscle layer consistent with infantile uterus, the histological picture suggestive of mixed gonadal dysgenesis (hernia uteri inguinalis). Chromosomal analysis was done for him showing a normal male karyotype (46XY). Hormonal profile was all within normal limits. The decision was to proceed for diagnostic Laparoscopic exploration with inguinal exploration. Exploration showed abnormal course of right spermatic vessels crossing the midline toward the left side with normal course of left vas (Fig. 2). Left Inguinal exploration was carried out and the cord was identified which was huge in size during manipulation of the left testis. The right testis was followed and retracted toward the left inguinal canal (Fig. 3). Diagnosis of right testicular ectopia was confirmed clinically with 2 spermatic cords joining to become a common cord. Bilateral testicular fresh frozen section was taken confirming normal testicular tissue with no malignancy. The biopsy identified the presence of sperm in left testis only. The final diagnosis was type 2 Transverse testicular ectopia i.e. Persistent Mullerian Duct syndrome (PMD) with trans testicular ectopia.

Fig. 1.

MRI showing both testes in same hemiscrotum.

Fig. 2.

Laparoscopic view of both cords passing together toward the same inguinal ring.

Fig. 3.

Both testes delivered through a left inguinal incision.

Discussion

Transverse testicular ectopia is considered to be a rare variant of undescended testis where one testis ends up in any position along the route of testicular descent. Classical presentation is an undescended testis with contralateral hernia.¹ The first descriptions were documented by Von Lenhossek in 1886.²

Transverse testicular ectopia has been classified into three different types. Type 1 is characterized by the presence of hernia only. This type is the most common among all types of TTE (40%–50%). The presence of Mullerian duct structures is a feature of type 2 which can be seen in 30% of TTE cases. Type 3 is associated with genitourinary disorders other than persistent Mullerian remnants such as inguinal hernia, hypospadias, disorders of sexual differentiation and scrotal abnormalities and can be identifies in 20% of TTE patients.³

Persistent Mullerian Duct syndrome (PMDS), is a rare disorder of sexual differentiation. Due to decreased production of Mullerian inhibiting factor from Sertoli cells or lack of sensitivity from end organs, Mullerian structures will fail to regress in a phenotypic male with a XY karyotype. The coexistence of TTE with PMDS is an extremely rare entity.^4,5

Conclusion

TTE is a rare finding to be seen in literature. To our knowledge, this is the only case of adult discovered TTE as a cause of chronic orchialgia, fully descended with bilaterally developed scrotum. Physical examination and radiological as well are important tools to diagnose such cases specially if the spermatic cord course is questionable. TTE diagnosis should be considered in un-explained cases of chronic orchialgia.