Persistent mullerian duct syndrome: A 24-year experience

Saleem, Muhammad; Ather, Uzma; Mirza, Bilal; Iqbal, Shahid; Sheikh, Afzal; Shaukat, Mahmood; Sheikh, Muhammad Tahir; Ahmad, Farooq; Rehan, Tariq

doi:10.1016/j.jpedsurg.2016.06.005

Cited by 27 publications

(22 citation statements)

References 22 publications

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“…In the third type, known as "transverse testicular ectopia", both testes are present on the same side, either in the inguinal canal or scrotum. 8 The clinical features in the present case were compatible with the second (female form) type of PMDS.…”

Section: Discussionsupporting

confidence: 71%

“…Germ cell tumors (embryonal carcinoma, seminomas, yolk sac tumors, and teratomas) are the most common types of tumors reported in the testes. 8,9 Tumors of Müllerian ductal derivatives have also been reported. 10 The treatment of PMDS is surgical.…”

Section: Discussionmentioning

confidence: 99%

“…8,9,11 Thus, some authors have proposed the adoption of a more aggressive surgical approach, which involves removing the Müllerian ductal structures. 8,9,11 To conclude, in all cases of bilateral cryptorchidism, the possibility of PMDS should be borne in mind. Genetic counselling should be considered in cases of parental consanguinity.…”

Section: Discussionmentioning

confidence: 99%

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Persistent Müllerian duct syndrome: A novel mutation in the Αnti-Müllerian Ηormone gene

Altıncık

Karaca

Önay

2017

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Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 99%

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Persistent Müllerian duct syndrome: A novel mutation in the Αnti-Müllerian Ηormone gene

Altıncık

Karaca

Önay

2017

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“…[14] Salehi et al [15] partially removed MR in only 2 out of 8 patients, and stated that these patients had a satisfactory overall prognosis; but their fertility potential appeared to be decreased, and risk of testicular malignancy due to cryptorchidism as well as a small risk of malignancy due to retained MR existed. Saleem et al [16] didn't prefer bilateral orchidopexy with retention of MR or removal of MR along with testes and vas. Although their patients whose MRs were removed had not been followed-up in terms of testicular atrophy, to balance the chance of malignancy in the remnants and potential of fertility, removal of Mullerian structures and bilateral orchidopexy were preferred.…”

Section: Discussionmentioning

confidence: 99%

Management of the patients with persistent Müllerian duct syndrome: Is the ultimate goal testicular descent?

Sancar¹,

Ozcakir²,

Kaya³

2018

Turkish Journal of Urology

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Objective: Persistent Müllerian duct syndrome (PMDS) is a rare congenital disease characterized by the presence of rudimentary Müllerian structures within an intra-abdominal or hernial sac in a virilized male, often presenting as undescended testes. In this study, we aim to present a series of the PMDS patients who were managed by orchiopexy without removal of Müllerian remnants (MR). Material and methods:Between May 2010 and June 2017, we treated six cases diagnosed as PMDS in our department. Laparoscopy and gonadal biopsy were performed in all patients, and vessel ligation was done in four patients for the first session of Stephen-Fowler orchiopexy. After initial diagnosis, genetic analyses and endocrine investigations were performed. Demographic and clinical features of the patients, operative methods and follow-up data were analyzed retrospectively.Results: Mean age of the patients was 5.5 years. Three boys had undergone inguinal surgery due to hernia or undescended testis, while others were diagnosed during laparoscopic investigation of nonpalpable testis. As a definitive operation, testes and MR were completely removed in an adult patient, and the remaining patients were treated with laparoscopic or open orchiopexy with or without utero-cervical splitting and the MRs were left in situ. Two testes atrophied during follow-up period. Conclusion:The goal of the approach in PMDS patients is to preserve testes, as well as carry them to their natural location. Leaving the MR in place is a suitable option for blood circulation of the testes but the longterm results are still unknown.

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“…4,5 Persistent mullerian duct syndrome is a rare form of internal male pseudohermaphroditism in which Mullerian duct derivatives are seen in a karyotypically male patients. 6,7 This duct, originated by an anomaly in sexual differentiation, can have large size and to predispose to urinary infection with acute urinary retention. 7…”

Section: Discussionmentioning

confidence: 99%

Operative cystoscopy in the neonatal period

et al. 2016

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Aim of the study is to report the experience in the last three years about endourological treatment of newborns affected by genito-and urinary malformation, implying critical urinary tract dilatation and bladder mass requiring a neonatal operative management. Sevenfour males and three female -patients were treated cystoscopically during the neonatal period. Three newborns with a posterior urethral valves (PUV), underwent a successful ablation of PUV. In a male with infected persistent large mullerian duct and subsequent acute urinary retention, a cystoscopically-assisted catheterization was performed. Two females, one with cloaca and the other with high persistent urogenital sinus and massive associated hydrometrocolpos underwent a cystoscopical drainage of the vagina and a positioning of a balloon catheter into the bladder. Another female with a bladder mass had a endoscopical biopsy. Thanks to modern neonatal operative urethrocystoscope, today is possible to treat early many pathological genito-urinary conditions in newborns.

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Persistent mullerian duct syndrome: A 24-year experience

Cited by 27 publications

References 22 publications

Persistent Müllerian duct syndrome: A novel mutation in the Αnti-Müllerian Ηormone gene

Persistent Müllerian duct syndrome: A novel mutation in the Αnti-Müllerian Ηormone gene

Management of the patients with persistent Müllerian duct syndrome: Is the ultimate goal testicular descent?

Operative cystoscopy in the neonatal period

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