Transverse testicular ectopia associated with persistent Müllerian duct syndrome treated by transseptal orchiopexy

Rationale Persistent Mullerian duct syndrome (PMDS) is rare form of male pseudohermaphroditism characterized by the presence of uterus and fallopian tubes with normal external genitalia and secondary sexual characteristics. Transverse testicular ectopia (TTE) is also a rare form of testicular ectopia that may be associated with PMDS. Patient concerns We present a 2-year-old boy who presented with bilateral non-palpable testes with left inguinal mass. Diagnosis TTE with PMDS. Interventions On exploration, both testes were present in the left inguinal region. Uterus and fallopian tubes were located between the testes. A hysterectomy was perfomed with resection of the underdeveloped fallopian tubes. Bilateral orchiopexy was performed by placing both gonads into subdartos pouches in each scrotum with transseptal approach. Outcomes Both testes were palpable in both the scrotum at 1-year postoperative follow-up and we are planning a regular follow-up. Lessons In case of TTE with PMDS, optimal surgical approach with orchiopexy and excision of Mullerian duct should be needed. A long-term postoperative follow-up is necessary for assessment of malignant transformation and infertility.