RESUMO
INTRODUCTION: Persistent Müllerian duct syndrome (PMDS), a rare genetic aberration, is characterized by the presence of Müllerian duct (MD) features in males. PMDS is usually caused by a defect in the Müllerian inhibitory system and is discovered during surgical interventions. CASE REPORT: We present the case of a 14-year-old Afghan boy with severe abdominal pain who was initially diagnosed with bilateral undescended testicles and abdominal complex cysts. He was supposed to undergo a cystectomy and orchiopexy surgery. During the surgical intervention, an unexpected finding was made whereby fibrotic-like ovaries, fallopian tubes, and a segment of the uterus were identified, ultimately leading to the diagnosis of PMDS. The MD was carefully removed, and the testicles were delicately repositioned during an orchiopexy procedure. DISCUSSION: In our case, the patient exhibited bilateral undescended testicles along with fibrotic-like ovaries, fallopian tubes, and a portion of the uterus, representing the presence of the female type of PMDS. To safeguard fertility, orchidopexy is recommended for pediatric patients. Conversely, in the older age group, orchidectomy is advised as a precautionary measure against the heightened susceptibility to testicular carcinoma. CONCLUSION: PMDS can be associated with an undescended testicle and abdominal pain. Hence, it is crucial to thoroughly evaluate patients who have undescended testes for the presence of PMDS, and surgeons must maintain a heightened sense of awareness for PMDS while exploring individuals who present with bilateral undescended testes, as exemplified in our case.
