Persistent mullerian duct syndrome and transverse testicular ectopia: embryology, presentation, and management

Abstract

The finding of persistent mullerian duct syndrome (PMDS) with transverse testicular ectopia (TTE) is rare. We present nonidentical triplets with PMDS with TTE. Triplet A presented with a right inguinal hernia and left nonpalpable testis at 4 months of age. Ultrasound demonstrated 1 testis in the right hemiscrotum. Laparoscopy revealed both testes in the right inguinal canal with a thick midline structure. Triplet B presented at 6 months of age in the exact manner with similar intraoperative findings as triplet A. No additional mullerian structures were identified in triplets A and B. Both underwent laparoscopic left orchiopexy and open right inguinal herniorraphy/orchiopexy. A portion of vas was noted in the path specimen of triplet B. Triplet C presented at 7 months of age with a nonpalpable left testis. Laparoscopy demonstrated bilateral fallopian tubes and a midline uterine remnant. Open bilateral orchiopexy was performed, and bilateral biopsies confirmed testes. All 3 were 46-X,Y. Persistent mullerian duct syndrome with TTE may be encountered when performing laparoscopy for patients with nonpalpable testis. The persistent mullerian remnants vary among individuals and alter the normal anatomy, thus may complicate diagnosis and surgical management.