Laparoscopic hysterectomy with bilateral orchidectomy for Persistent Mullerian duct syndrome with seminoma testes: Case report

Abstract

Persistent Mullerian duct syndrome (PMDS) is one of the three rare intersex disorders caused by defective anti-mullerian hormone or its receptor, characterized by undescended testes with presence of underdeveloped derivatives of mullerian duct in genetically male infant or adult with normal external genitals and virilization. This population will essentially have normal, 46(XY), phenotype. We hereby present a case of PMDS, presented with incarcerated left inguinal hernia associated with cryptorchidism and seminoma of right testes. Patient underwent laparoscopic hernia repair with bilateral orchidectomy and hysterectomy with uneventful postoperative recovery. Here we highlight the importance of minimal access approach for this scenario in terms of better visualization, less blood loss, combining multiple procedures along with early return to work and excellent cosmetic outcome.

Intersex Studies: A Systematic Review of International Health Literature

“…The sources included a broad array of participant numbers, ranging from sources with unclear numbers of people with intersex variations (Dwyer et al, 2015;Howard, 2015;Kim et al, 2015;Rios et al, 2015) through to a source with 272 participants specifically from the group (Jones, 2016). Higher numbers were rare, however, as over a third of the sources focused on only one participant (Amanda, 2015; Bonanni et al, 2015;Brasileiro et al, 2016;Ceci et al, 2015;Cynthia, 2015;Davis, 2015b;Garcia, 2015;Grimbly et al, 2016;Grover et al, 2015;Gül et al, 2015;Inter, 2015;Kalra et al, 2016;Long, 2015;Mannaerts et al, 2015;Mutlu et al, 2015;Pagonis, 2015;Palanisamy et al, 2015;Paula et al, 2015;Petersen, 2015;Quinn, 2015;Simon, 2015;Truffer, 2015;Viloria, 2015;von Klan, 2015;Walsh, 2015;Zieselman, 2015). Almost as many had no direct participants with intersex variations and, instead, used the general existence of the group, other participants, or secondary sources to make key arguments about intersex variations (Achermann et al, 2015;Anderson, 2015;Anonymous, 2016;Baratz & Karkazis, 2015;Dreger, 2015;Frader, 2015;Grimes, 2016;…”

“…A smaller group (four of 61) exclusively used the specific diagnosis or condition in focus only, framing the group in terms of particular condition-based traits and risks (Bonanni et al, 2015;Lin-Su et al, 2015;Lucas-Herald et al, 2015;Palanisamy et al, 2015). These included patients with persistent mullerian duct syndrome (PMDS; Palanisamy et al, 2015), patients with CAH (Lin-Su et al, 2015), patients with Turner syndrome (TS; Bonanni et al, 2015), and children with suspected disorders of steroid/hormone synthesis (Lucas-Herald et al, 2015). The sources using group-specific nomenclature were mostly affirming and unquestioning about the need for early analysis and intervention, although one considered the need for greater consultation with the patient group (Lin-Su et al, 2015).…”

“…Over half (31) of the studies were based in the United States (Amanda, 2015;Amarillo et al, 2016;Anderson, 2015;Baratz & Karkazis, 2015;Cynthia, 2015;Davis, 2015b;Dreger, 2015;Frader, 2015;Garcia, 2015;Grimes, 2016;Grover et al, 2015;Howard, 2015;Inter, 2015;Kelsey et al, 2016;Kraus, 2015;Lin-Su et al, 2015;Long, 2015;Miller, 2015;Pagonis, 2015;Petersen, 2015;Quinn, 2015;Rubin, 2015;Saraswat et al, 2015;Simon, 2015;Topcu et al, 2015;Truffer, 2015;Viloria, 2015;von Klan, 2015;Wall, 2015;Walsh, 2015;Zieselman, 2015). Four studies were based in India (Kalra et al, 2016;Khadilkar et al, 2015;Palanisamy et al, 2015;Vasundhera et al, 2016), three in Australia (Dwyer et al, 2015;Jones, 2016;Tinney et al, 2015), and three in Brazil (Brasileiro et al, 2016;Oliveira et al, 2015;Paula et al, 2015). Two studies were based in China (Dong et al, 2016;Wang &...…”

“…These journals historically appeared to have evidenced regular past attention to intersex themes over time outside of the pieces represented in this literature review. Of the remaining sources, most (15) came from individual journals/publishing series in areas of the medical or broader health sciences that have had some association with intersex themes in the past-in genetics (Amarillo et al, 2016;Dong et al, 2016;Kim et al, 2015), endocrinology (Achermann et al, 2015;Khadilkar et al, 2015;Latrech et al, 2015), biology (Ahmed & Fadl-Elmula, 2016;Rios et al, 2015), nursing (Anderson, 2016;Anonymous, 2016), pediatric or other surgery (Ekenze et al, 2015;Palanisamy et al, 2015), psychogeriatrics (Tinney et al, 2015), neuropsychiatry (Gül et al, 2015), and urology (Ceci et al, 2015). Only nine sources came from nonmedical journals-in education (Jones, 2016;Miller, 2015), science ethics (Dreger, 2015;Mohamed & Noor, 2015), religion (Grimes, 2016), rural society (Dwyer et al, 2015;Howard, 2015), and feminist legal studies (Lalor, 2015;Rubin, 2015).…”

Persistent M�llerian duct syndrome: A case report and review

“…PMDS was first described by Nilson ( 11 ) in 1939 as a rare type of male pseudohermaphroditism characterized by the presence of MD derivatives in genotypic and phenotypic males with a 46,XY chromosome pattern ( 1 , 2 , 5 ). It is caused by a deficiency in AMH or a defect in AMHR-II ( 12 – 14 ). The patterns of PMDS inheritance include X linkage, autosomal dominant and autosomal recessive inheritance ( 14 ).…”