Clinical case with Persistent Müllerian Duct Syndrome
Keywords:
cryptorchidism, disorders of sex development, mullerian ducts.Abstract
Introduction: Persistent Müllerian Duct Syndrome is a rare inherited disorder characterized by incomplete regression of Müllerian duct structures in an individual with male physical and genetic traits.
Objective: To describe the presentation and surgical treatment in a patient with persistent Müllerian duct syndrome.
Clinical Case: An 18-year-old male patient who attended the general surgery outpatient clinic due to left cryptorchidism, right hydrocele and a synechiae from the penis to the scrotum. During the surgical procedure, the presence of an ovary with a fallopian tube on the left side without the presence of the uterus was confirmed. An oophorectomy with salpingectomy was performed, in addition to a hydrocelectomy on the right side and the release of the penis, leaving it in a functional position. The diagnosis was made early in the patient's life, with no evidence of malignancy during follow-up.
Conclusions: The knowledge of general surgeons about persistent Müllerian duct syndrome is limited due to the small number of cases and the variety of presentations. Surgeons should have a high level of suspicion for this syndrome when evaluating individuals with cryptorchidism.
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2. Farikullah J, Ehtisham S, Nappo S, Patel L, Hennayake S. Persistent Müllerian duct syndrome: lessons learned from managing a series of eight patients over a 10-year period and review of literature regarding malignant risk from the Müllerian remnants [Internet]. BJU Int. 2012; 110(11 Pt C):1084-9. DOI: 10.1111/j.1464-410X.2012.11184.x
3. de Faria IM, de Souza AM, Júnior LRP, Leite G. Surgical resection therapy of a rare presentation of persistent Mullerian duct syndrome: a case review [Internet]. Ther Adv Rare Dis. 2023; 4:26330040231184484. DOI: 10.1177/26330040231184484
4. Da Aw L, Zain MM, Esteves SC, Humaidan P. Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management [Internet]. Int Braz J Urol. 2016; 42(6):1237-43. DOI: 10.1590/S1677-5538.IBJU.2016.0225
5. Hamd ZY, Ali MAM, Alorainy AI, Gareeballah A, Hamdoun A, Manssor E, et al. Persistent Mullerian duct syndrome with polycystic ovary in a young adult: A rare case report [Internet]. Radiol Case Rep. 2023; 18(5):1825-9. DOI: 10.1016/j.radcr.2023.02.015
6. Gagliardi F, Lauro A, De Anna L, Tripodi D, Esposito A, Forte F, et al. The Risk of Malignant Degeneration of Müllerian Derivatives in PMDS: A Review of the Literature [Internet]. J Clin Med. 2023; 12(9):3115. DOI: 10.3390/jcm12093115
7. Bahouth Z, Little MW, Badea RD, Musa F, Charlesworth PJS. High-Grade Prostate Cancer Invading the Vagina: A Case Report of an Unusual Prostate Cancer Diagnosed in a Man with Persistent Mullerian Duct Syndrome [Internet]. Case Rep Oncol. 2023; 16(1):49-54. DOI: 10.1159/000529059
8. Umair M, Khan AU, Arruda JB, Lakhani DA, Adelanwa A, Hadi YB, et al. Prostatic adenocarcinoma in a patient with persistent Müllerian duct syndrome [Internet]. Urol Ann. 2022; 14(4):398-402. DOI: 10.4103/ua.ua_74_21
9. McCroskey Z, Koen TM, Lim DJ, Divatia MK, Shen SS, Ayala AG, et al. Prostatic adenocarcinoma in the setting of persistent müllerian duct syndrome: a case report [Internet]. Hum Pathol. 2018; 75:125-31. DOI: 10.1016/j.humpath.2017.10.033
10. Ramzan R, Khan NA, Khalique A, Aziz MA. A rare form of male pseudohermaphroditism-Persistent Mullerian Duct Syndrome [Internet]. J Surg Case Rep. 2022; 2022(12):rjac596. DOI: 10.1093/jscr/rjac596
11. Fatima N, Kiran Z, Shabbir KU, Baloch AA. Persistent Müllerian Duct Syndrome Diagnosed Incidentally: A Case Report [Internet]. J Pak Med Assoc. 2023; 73(11):2280-3. DOI: 10.47391/JPMA.9172
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