A female infant with rectovestibular fistula and imperforate hymen
Kiran Khedkar, Yashwant R Lamture, Sham Lohia, Harshal Atul TayadeJournal of Indian Association of Pediatric Surgeons 2023 28(2):177-178
In a female neonate with anorectal malformation (ARM), the diagnosis is usually simple. But when there are two openings in the introitus and absent anal opening at the normal site, the scenario poses a diagnostic challenge. Careful and detailed evaluation of anomaly is therefore necessary before planning definitive correction. Imperforate hymen, although is not commonly associated with ARM should always be kept in mind as a differential diagnosis and other vaginal anomalies like Mayer&#x...
Source: Journal of Indian Association of Pediatric Surgeons - March 3, 2023 Category: Surgery Authors: Kiran Khedkar Yashwant R Lamture Sham Lohia Harshal Atul Tayade Source Type: research
Hemi-hysterectomy for mrkh syndrome
The objective of this video is to illustrate surgical techniques and identify critical anatomic structures to complete a laparoscopic hemi-hysterectomy for a patient with Mayer-Rokitansky-K üster-Hauser (MRKH) Syndrome. (Source: American Journal of Obstetrics and Gynecology)
Source: American Journal of Obstetrics and Gynecology - March 1, 2023 Category: OBGYN Authors: A. Nimz, T. Baker, C. Ramirez, A. Truehart Tags: Videofests Source Type: research
Rare structural variants, aneuploidies, and mosaicism in individuals with Mullerian aplasia detected by optical genome mapping
AbstractThe molecular basis of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome remains largely unknown. Pathogenic variants inWNT4 andHNF1B have been confirmed in a small percent of individuals. A variety of copy number variants have been reported, but causal gene(s) remain to be identified. We hypothesized that rare structural variants (SVs) would be present in some individuals with MRKH, which could explain the genetic basis of the syndrome. Large molecular weight DNA was extracted from lymphoblastoid cells from 87 individuals with MRKH and available parents. Optical genome mapping (OGM) was performed to identify SVs, whi...
Source: Human Genetics - February 17, 2023 Category: Genetics & Stem Cells Source Type: research
The long-term outcomes of vaginoplasty using acellular porcine small intestinal submucosa grafts in patients with Mayer-Rokitansky-K üster-Hauser syndrome: A case series
CONCLUSIONS: Nearly half of MRKH patients after SIS vaginoplasty in our study had good long-term sexual function. Sexual function and psychological status improved as the postoperative time increased. In addition, reducing the diagnosis to surgery interval was associated with improved psychological function.PMID:36660801 | DOI:10.1111/1471-0528.17388 (Source: BJOG : An International Journal of Obstetrics and Gynaecology)
Source: BJOG : An International Journal of Obstetrics and Gynaecology - January 20, 2023 Category: OBGYN Authors: Zhi-Yang Xu Ling-Xia Li Xing-Guo Wang Meng-Xin Wang Gai-Jing Cao Bi-Liang Chen Shu-Juan Liu Source Type: research
The long-term outcomes of vaginoplasty using acellular porcine small intestinal submucosa grafts in patients with Mayer-Rokitansky-K üster-Hauser syndrome: A case series
CONCLUSIONS: Nearly half of MRKH patients after SIS vaginoplasty in our study had good long-term sexual function. Sexual function and psychological status improved as the postoperative time increased. In addition, reducing the diagnosis to surgery interval was associated with improved psychological function.PMID:36660801 | DOI:10.1111/1471-0528.17388 (Source: BJOG : An International Journal of Obstetrics and Gynaecology)
Source: BJOG : An International Journal of Obstetrics and Gynaecology - January 20, 2023 Category: OBGYN Authors: Zhi-Yang Xu Ling-Xia Li Xing-Guo Wang Meng-Xin Wang Gai-Jing Cao Bi-Liang Chen Shu-Juan Liu Source Type: research
The long-term outcomes of vaginoplasty using acellular porcine small intestinal submucosa grafts in patients with Mayer-Rokitansky-K üster-Hauser syndrome: A case series
CONCLUSIONS: Nearly half of MRKH patients after SIS vaginoplasty in our study had good long-term sexual function. Sexual function and psychological status improved as the postoperative time increased. In addition, reducing the diagnosis to surgery interval was associated with improved psychological function.PMID:36660801 | DOI:10.1111/1471-0528.17388 (Source: BJOG : An International Journal of Obstetrics and Gynaecology)
Source: BJOG : An International Journal of Obstetrics and Gynaecology - January 20, 2023 Category: OBGYN Authors: Zhi-Yang Xu Ling-Xia Li Xing-Guo Wang Meng-Xin Wang Gai-Jing Cao Bi-Liang Chen Shu-Juan Liu Source Type: research
Neovagina in Mayer-Rokitansky-K üster-Hauser (MRKH) Syndrome: Vaginoplasty Using Ileal Flap
CONCLUSION: Ileal vaginoplasty without a vascular pedicle is an option that can be used to reconstruct the vagina, because it provides an excellent tissue for vaginal replacement. This technique can be used in patients with genital malformations of the genital tract with absence or vaginal hypoplasia.PMID:36599171 | DOI:10.20344/amp.18563 (Source: Acta Medica Portuguesa)
Source: Acta Medica Portuguesa - January 4, 2023 Category: General Medicine Authors: S ílvia Serrano In ês Pereira Alexandra Henriques Alexandre Valentim Louren ço Source Type: research
Update on Mayer —Rokitansky—Küster—Hauser syndrome
AbstractThis review presents an update of Mayer —Rokitansky—Küster—Hauser (MRKH) syndrome on its etiologic, clinical, diagnostic, psychological, therapeutic, and reproductive aspects. The etiology of MRKH syndrome remains unclear due to its intrinsic heterogeneity. Nongenetic and genetic causes that may interact during the embryonic develo pment have been proposed with no definitive etiopathogenesis identified. The proportion of concomitant extragenital malformations varies in different studies, and the discrepancies may be explained by ethnic differences. In addition to physical examination and pelvic ultrasound, t...
Source: Frontiers of Medicine - December 23, 2022 Category: General Medicine Source Type: research
Oestrogen-induced epithelial-mesenchymal transition (EMT) in endometriosis: Aetiology of vaginal agenesis in Mayer-Rokitansky-K üster-Hauser (MRKH) syndrome
Endometriosis occurs when endometrial-like tissue forms and grows outside the uterus due to oestrogen-induced epithelial-mesenchymal transition in the female reproductive tract. Factors that suppress this event could become potential therapeutic agents against disease occurrence and progression. However, an overview of these studies is still lacking. This review assessed the impact of a number factors on oestrogen-mediated epithelial-mesenchymal transition in the emergence of several diseases in the female reproductive tract, primarily endometriosis. The association between epithelial-mesenchymal transition and Mayer-Rokit...
Source: Frontiers in Physiology - December 13, 2022 Category: Physiology Source Type: research
Unusual Torsion of Hematosalpinx in a Case of MRKH Syndrome
Mayer-Rokitansky-K üster-Hauser (MRKH) syndrome is a rare congenital anomaly of the female reproductive tract characterized by the absence of the upper two-thirds of the vagina and the uterine cervix and hypoplasia or complete aplasia of the uterus [1]. Typically, patients with MRKH syndrome present with primary amen orrhea while exhibiting normal secondary sexual characteristics and a normal female karyotype (46, XX) [2]. In fewer cases, they experience cyclic pelvic pain owing to active endometrium in the hypoplastic uterine horns [3]. (Source: The Journal of Minimally Invasive Gynecology)
Source: The Journal of Minimally Invasive Gynecology - November 24, 2022 Category: OBGYN Authors: Antoine Naem, Zaki Sleiman Tags: Images in Gynecologic Surgery Source Type: research
Unusual Torsion of Hematosalpinx in a Case of MRKH Syndrome.
Mayer-Rokitansky-K üster-Hauser (MRKH) syndrome is a rare congenital anomaly of the female reproductive tract characterized by the absence of the upper two thirds of the vagina and the uterine cervix, and hypoplasia or complete aplasia of the uterus [1]. Typically, patients with MRKH syndrome present with primary ame norrhea while exhibiting normal secondary sexual characteristics and a normal female karyotype (46, XX) [2]. In fewer cases, they suffer from cyclic pelvic pain due to active endometrium in the hypoplastic uterine horns [3]. (Source: The Journal of Minimally Invasive Gynecology)
Source: The Journal of Minimally Invasive Gynecology - November 24, 2022 Category: OBGYN Authors: Antoine Naem, Zaki Sleiman Tags: Images in Gynecological Surgery Source Type: research
Laparoscopic Vaginoplasty by Peritoneal Pull through Technique
Laparoscopic management of MRKH syndrome and use of near ideal vaginal mold made up from locally readily available materials in a rural settings. (Source: The Journal of Minimally Invasive Gynecology)
Source: The Journal of Minimally Invasive Gynecology - November 1, 2022 Category: OBGYN Authors: S.S. Dayama, D.D.C. Shirsath Tags: VIDEO SESSION 4 - LAPAROSCOPY & Tissue Containment Vaginal Surgery (11:30 AM — 12:35 PM) 12:09 PM Source Type: research
8697 Vaginoplasty in MRKH: A Single Center Experience
The aim is to compare the efficacy of MacIndoe and laparoscopic Davydov's vaginoplasty to create neovagina in MRKH patients. (Source: The Journal of Minimally Invasive Gynecology)
Source: The Journal of Minimally Invasive Gynecology - November 1, 2022 Category: OBGYN Authors: G. Kachhawa, R. Mahey, N. Varun, D.M. Kamalakar, R. Rakhi, J.B. Sharma, N. Bhatla Source Type: research
