Mayer-Rokitansky-Kuster-Hauser Syndrome: A rare case report from Nepal

Ray, Umesh; Adhikari, Subodh; Dhital, Roman; Shrestha, Sushant; Shah, Sangam; Poudel, Sunil; Sah, Sanjit; Gami, Roshan; Adhikari, Abhishek; Gautam, Bishnu

doi:10.1016/j.amsu.2022.104725

Cited by 4 publications

(5 citation statements)

References 7 publications

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“…First identified in 1961, MRKH syndrome often leads to absence of vaginal development which is a major congenital anomaly that affects the female reproductive system [ 1 ]. Despite maintaining typical female genetic, physical, and hormonal characteristics, individuals with this syndrome commonly experience abnormalities in the renal and skeletal systems.…”

Section: Discussionmentioning

confidence: 99%

“…One way to cure vaginal agenesis is to use the present mucous membrane-lined canal for neovagina, lining the surgical cavity with an autologous graft from the buccal mucosa or an allograft, like amnion [ 8 ]. The best and most recommended technique for this is still the 1888-introduced Abbe McIndoe approach [ 1 ]. Even if it is inconvenient, patient commitment to stent wear is essential since non-compliance can greatly contribute to treatment failure [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, alternatively referred to as Mullerian agenesis or congenital absence of the uterus and vagina (CAUV), is a congenital condition marked by the partial or complete lack of the uterus and the upper portion of vagina in individuals assigned female at birth. This condition typically manifests as primary amenorrhea during adolescence due to absence of menstruation [ 1 ]. MRKH syndrome affects approximately 1 in 4,500 female births and is believed to result from abnormal development of the Mullerian ducts during embryonic development.…”

Section: Introductionmentioning

confidence: 99%

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Fabrication of Hollow Acrylic Vaginal Stents Using Frozen Coconut Oil for Vaginal Agenesis Management

Gorripati,

Dubey,

Nimonkar

et al. 2024

Cureus

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In a rare condition known as vaginal agenesis, the uterus (womb) may develop only partially or not at all, while the vagina fails to develop altogether. It is common to diagnose vaginal agenesis, when a female does not start menstruation at puberty. This is a prenatal disorder that may also be linked to bone or kidney issues. Mullerian agenesis, Mullerian aplasia, and Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome are other names for the illness. Treatment modalities encompass surgical and nonsurgical interventions, including the utilization of prefabricated or customized vaginal stents for neovagina reconstruction and maintenance. This case report describes the development of a neovagina in a 27-year-old female diagnosed with vaginal agenesis, a characteristic of MRKH syndrome. A customized clear acrylic stent, designed to provide a resilient surface, serves as a straightforward and cost-efficient alternative for managing this condition. Significantly, it enhances patient's compliance and comfort during treatment, addressing both the physical and psychological ramifications of this congenital anomaly. This customized vaginal stent not only provides a practical solution but also contributes to enhancing the quality of life for individuals grappling with vaginal agenesis, thereby offering a promising avenue for addressing the multifaceted challenges associated with this condition.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Fabrication of Hollow Acrylic Vaginal Stents Using Frozen Coconut Oil for Vaginal Agenesis Management

Gorripati,

Dubey,

Nimonkar

et al. 2024

Cureus

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“…They are of two types: Type 1 having only uterovaginal agenesis and type 2 having uterovaginal agenesis with anomalies in the fallopian tube, kidney, spine, heart and other organ amenorrhea and painful sexual intercourse [3]. The exact etiology of MRKH syndrome is not known.…”

Section: Introductionmentioning

confidence: 99%

Mayer rokitansky kuster hauser syndrome with pituitary hypoplasia A rare case report from Pakistan

Asad Mehdi Rizvi

2023

JCIMCR

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“…Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKHS) is a very rare congenital malformation of mullareanducts. Its incidence is 1 in 5000 females 1 . It has two types; type 1 is characterized by uterovaginal aplasia with normal secondary sexual characters and karyotype 46XX.…”

Section: Introductionmentioning

confidence: 99%

A Rare Case of Mullerian Agenesis (Mayer-Rokitansky-Kuster-Hauser Syndrome)

Nisa¹,

Aleena²,

Iqbal³

et al. 2022

PJMHS

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A 21 years old girl presented in Gyne OPD of Lady Aitchison Hospital Lahore with complaint of primary amenorrhea. There was no complaint of cyclical dysmenorrhea, urinary retention or feeling of abdomino pelvic mass. Her mother had menarche at 14 years of age. Her height was 5 feet and one inch. Her secondary sexual characteristics were of normal (female) and well developed, external genitalia were of female with rudimentary vagina.Ultrasound showed normal size ovaries and hypoplasticuterus and rudimentary vagina.Hormones (FSH, LH, Prolactin) were normal. HerKaryotype was 46XX.On the basis of these findings, diagnosis of Mullerianagenesis (Maye-Rokitansky-Kuster Hauser Syndrome) was made. Family was counselled about the case. They were informed the patient is unable to menstruate and reproduce. However, for sexual function, it is possible to create a new vagina bynon-surgical method using Hegar dilator or surgical by vaginoplasty.

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Mayer-Rokitansky-Kuster-Hauser Syndrome: A rare case report from Nepal

Cited by 4 publications

References 7 publications

Fabrication of Hollow Acrylic Vaginal Stents Using Frozen Coconut Oil for Vaginal Agenesis Management

Fabrication of Hollow Acrylic Vaginal Stents Using Frozen Coconut Oil for Vaginal Agenesis Management

Mayer rokitansky kuster hauser syndrome with pituitary hypoplasia A rare case report from Pakistan

A Rare Case of Mullerian Agenesis (Mayer-Rokitansky-Kuster-Hauser Syndrome)

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