Objective: Congenital Mullerian duct malformations are a challenging group of conditions for surgeons and need surgical experience and skill. Accordingly, the aim of this study is to present the diagnosis, surgical management, and clinical implications of congenital uterovaginal abnormalities.
Methods: Between 1980 and 2015, 8 patients with congenital uterovaginal abnormalities were diagnosed. In one patient a unique case of an unusual horseshoe shaped double uterus communicating via a transverse canal along with agenesis of the cervix and vagina was noted, and utero-vaginal agenesis was diagnosed in 6 patients. Complete androgen insensitivity syndrome with its female phenotype associated with bilateral testicular tissue in the inguinal canal with an accompanying absence of the ovaries, uterus, uterine tubes, vagina, and an imperforate hymen, was diagnosed in one patient. Clinical examination of all the patients revealed well-developed secondary sexual characteristics. A modified McIndoe vaginoplasty procedure was the surgical treatment common to all patients to treat vaginal agenesis. The surgery was performed by a consultant (Dr. K.G. Paul) using the standardized surgical technique.
Results: An unusual Mullerian duct anomaly, uterus bicornisacollis, was successfully corrected by uteroplasty and a new cervix was constructed. Complete vaginal agenesis was corrected by a modified McIndoe vaginoplasty technique. None of the patients had any significant post-operative complications.
Conclusion: Knowledge of congenital uterovaginal abnormalities diagnosed in this study is essential for surgeons, clinical anatomists, radiologists, and morphologists who may increase the success of their diagnostic evaluations and surgical approaches to the region.
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http://dx.doi.org/10.5468/ogs.20046 | DOI Listing |
Clin Case Rep
December 2024
Department of Obstetrics and Gynecology Tribhuvan University, Institute of Medicine Kathmandu Nepal.
Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital disorder characterized by agenesis of the uterus and upper two-thirds of the vagina. It affects around 1 in 4000-5000 females and is of two types: type 1, also known as isolated Müllerian aplasia or Rokitansky, which involves only uterovaginal agenesis, and type 2, presents as uterovaginal agenesis along with renal, cardiac, and other organ anomalies. Despite the absence of vaginal and uterine structures, individuals with MRKHS typically present with normal secondary sexual characteristics and ovarian functions.
View Article and Find Full Text PDFCureus
November 2024
School of Medicine, Hospital Militar Escuela "Dr. Alejandro Dávila Bolaños", Managua, NIC.
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder affecting the female reproductive system, primarily characterized by the absence or underdevelopment of the uterus and upper two-thirds of the vagina, with preserved ovarian function and normal secondary sexual characteristics. It is a rare disease though prevalence may vary based on genetic and environmental factors. This report details a case of a 26-year-old female patient with a history of smoking, alcohol use, and prior inguinal hernioplasty, presenting with primary amenorrhea and inability to engage in vaginal intercourse.
View Article and Find Full Text PDFInt J Surg Case Rep
October 2024
Faculty of Medicine, Kilimanjaro Christian Medical University College, Moshi, Tanzania; Department of Pathology, Kilimanjaro Christian Medical Center, Moshi, Tanzania; Kilimanjaro Clinical Research Institute, Moshi, Tanzania. Electronic address:
Nat Commun
September 2024
National Clinical Research Center for Obstetric and Gynecologic Diseases, Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, State Key Laboratory for Complex Severe and Rare Diseases, Peking Union Medical College, Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan, 100005, Beijing, China.
Med J Malaysia
August 2024
Universitas Gadjah Mada, Faculty of Medicine, Public Health and Nursing, Department of Obstetric and Gynecology, Yogyakarta, Indonesia.
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital malformation of the female urogenital tract characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. It was formerly known as Herlyn Werner Wunderlich Syndrome (HWWS). The syndrome usually presents with cyclic pelvic pain following menarche.
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