Surgical management of persistent müllerian duct syndrome

Vandersteen, David R.; Chaumeton, Amy K.; Ireland, Karen; Tank, Edward S.

doi:10.1016/s0090-4295(97)00104-0

Cited by 84 publications

(70 citation statements)

References 21 publications

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“…Earlier studies [8,12] opined that while removing the müllerian remnants, there is a high probability of damage to vas deferens and its blood supply with no disadvantage of conversion into malignancy, so they should not be removed. Thiel and Erhard reported the death of a 14-year-old boy with PMDS, due to metastasis of adenosarcoma arising from the müllerian remnant [14].…”

Section: Discussionmentioning

confidence: 99%

Persistent Müllerian Duct Syndrome (PMDS): a Rare Anomaly the General Surgeon Must Know About

Agrawal¹,

Kataria²

2014

Indian J Surg

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Persistent Müllerian duct syndrome is a rare condition occasionally encountered in men with normal phenotype but with presence of Müllerian duct structures. In India, owing to neglect and lack of facilities, we encounter this condition in adult males. We encountered on the same day in the operation theatre two phenotypic males aged 40 years and 10 months who had inguinal hernia on one side along with contralateral undescended testis. Both patients intraoperatively had uterus with fallopian tubes and underwent subtotal hysterectomy with preservation of vas. Repair of inguinal hernia with fixation of the testis in the scrotum was done. Though rare, every surgeon operating upon inguinal hernia or undescended testes or cryptorchidism needs to know about the presence of the uterus in a phenotypic male patient at any age. High degree of suspicion and awareness is needed to diagnose this condition. Early treatment is needed to maintain fertility and to prevent the occurrence of malignancy in remnant müllerian structures.

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Section: Discussionmentioning

confidence: 99%

Persistent Müllerian Duct Syndrome (PMDS): a Rare Anomaly the General Surgeon Must Know About

Agrawal¹,

Kataria²

2014

Indian J Surg

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“…2 Clinically, PMDS is most commonly diagnosed as an incidental finding during an operation, but the presence of unilateral or bilateral undescended testes may increase clinical suspicion. [7][8][9][10][11] Imaging modalities for the diagnosis of PMDS have thus far proven unreliable. 12 Most (60%-70%) of patients with PMDS have bilateral undescended testes located in an area analogous to the ovaries in women.…”

Section: Discussionmentioning

confidence: 99%

“…Several authors have asserted that the Mullerian structures should not be removed, as there is minimal risk associated with their retention, and excision of the Mullerian structures risks damaging primary blood supply to the PMDS testis via the internal spermatic and the deferential arteries. 9 However, more recent reports have demonstrated rare malignancies, usually adenocarcinomas of the Mullerian duct, associated with retained Mullerian structures. [20][21][22][23][24][25][26] If resection is attempted, great care must be taken to avoid damage to the vascular supply to the testis, or vas deferens, which run in close proximity to the remnant uterus and proximal vagina.…”

Section: Discussionmentioning

confidence: 99%

“…[20][21][22][23][24][25][26] If resection is attempted, great care must be taken to avoid damage to the vascular supply to the testis, or vas deferens, which run in close proximity to the remnant uterus and proximal vagina. 9 Interestingly, laparoscopic management of PMDS has now also been successfully reported. 27,28 See Table 1 for our key points.…”

Section: Discussionmentioning

confidence: 99%

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Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review

Ahdoot

Qadan

Santa-María

et al. 2013

CUAJ

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A 32-year-old male presenting with a direct inguinal hernia was found to have uterine tissue extending through the inguinal canal, warranting a diagnosis of persistent Mullerian duct syndrome (PMDS). PMDS is an extremely rare form of internal male pseudohermaphroditism in which female internal sex organs, including the uterus, cervix and proximal vagina, persist in a 46XY male with normal external genitalia. The condition results from a congenital insensitivity to anti-Mullerian hormone, or lack of anti-Mullerian hormone, leading to persistence of the female internal sex organs in a male. Clinically, this condition is associated with cryptochoridism. Controversy persists regarding the appropriate treatment of PMDS, since resection of the remnant structures is associated with potential morbidity, but retention risks development of occasional malignancies. We review the literature and discuss various aspects of pathophysiology, diagnosis, and management of PMDS.

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“…1 In situations where the testis cannot be mobilized enough to reach the scrotum, orchidectomy should be performed. 2 Crossorchidopexy is required in transverse testicular ectopia. 6 There have been recent reports of the use of laparoscopy in PMDS.…”

Section: Discussionmentioning

confidence: 99%

Persistent Müllerian Duct Syndrome in a Child: Surgical Management

Crankson

Yahib

2000

Ann Saudi Med

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Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism in which uterus and fallopian tubes are present in a genotypic and phenotypic male. Over 150 cases have been reported in the literature, mostly in adults.1,2 PMDS is likely to be encountered during surgery for undescended testis and inguinal hernia, and surgeons should be aware of this disorder and the options of surgical management. We report a case of PMDS and discuss the etiology, pathogenesis, anatomic types and surgical management. Case ReportA three-month-old Saudi male infant was referred to the Pediatric Surgery clinic for bilateral cryptorchidism. His parents were not related, and there was no family history of undescended testis. On examination, both testes were undescended and impalpable. The penis and scrotum were normal in appearance. A pelvic ultrasound (US) had shown absence of testes and no other abnormality. Human chorionic gonadotrophin stimulation (HCG) test confirmed the presence of testicular tissue.At seven months of age, there was a palpable left testis within an inguinal hernia. A month later at surgery, the uterus, fallopian tubes and both testes were unexpectedly found within the hernia sac (Figure 1). Testicular biopsies were performed and the testes, uterus and fallopian tubes returned into the pelvis, followed by herniorrhaphy. Postoperatively, chromosome analysis confirmed a normal male 46, XY karyotype, and a pelvic US showed a uterus. Histology of the testicular biopsies revealed immature testes consistent with cryptorchid testes.At the age of 15 months, the patient underwent bilateral proximal salpingectomies, leaving the fimbria with each epididymis, corporeal hysterectomy and pedicles of myometrium left with the vasa deferentia. The cervix was split longitudinally in the midline to achieve successful bilateral orchidopexy after mobilizing the internal spermatic vessels. Postoperative follow-up after one year showed both testes in the scrotum to be normal.

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Surgical management of persistent müllerian duct syndrome

Cited by 84 publications

References 21 publications

Persistent Müllerian Duct Syndrome (PMDS): a Rare Anomaly the General Surgeon Must Know About

Persistent Müllerian Duct Syndrome (PMDS): a Rare Anomaly the General Surgeon Must Know About

Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review

Persistent Müllerian Duct Syndrome in a Child: Surgical Management

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