International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics · 2026
Prevalence, clinical profile, and associated anomalies with women with Mayer-Rokitansky-Küster-Hauser syndrome in a tertiary care center: A cross-sectional study.
СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Vency Benedict, Kumari Minakshi, Ebenezer Emily Divya, Varghese Lilly, Kumari Pushplata
Аннотация
BACKGROUND: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital anomaly of the Müllerian ducts and represents the second most common cause of primary amenorrhea, accounting for 10%-15% of cases. Despite its significance, limited data exist regarding its clinical profile and associated anomalies in the Indian population.
OBJECTIVE: This study aims to determine the prevalence of MRKH syndrome among women presenting with primary amenorrhea at a tertiary care center in South India and to describe their clinical profiles and associated anomalies using the Vagina Cervix Uterus Adnexa-associated Malformation (VCUAM) classification system.
METHODS: A retrospective study was conducted over 15 years (January 2008 to December 2022) including women diagnosed with MRKH syndrome based on inclusion criteria: primary amenorrhea, normal secondary sexual characteristics, 46-XX karyotype, and normal serum follicle-stimulating hormone levels. Data were extracted from medical records and analyzed using SPSS v25.0.
RESULTS: Out of 340 women with primary amenorrhea, 181 (60%) were diagnosed with MRKH syndrome. The mean age at presentation was 21 years. The predominant complaint was non-attainment of menarche (66.8%), with 16.5% reporting cyclical abdominal pain. Type 1 MRKH was most common (78.9%), followed by Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia (MURCS) association (16.5%). Renal anomalies (15.5%) were the most frequent extragenital malformations. All women had vaginal and cervical agenesis (V5bC2b). Uterine anomalies included bilateral aplasia (89.1%), unilateral aplasia (0.6%), and hypoplasia (10.5%).
CONCLUSION: MRKH syndrome is a significant cause of primary amenorrhea, with notable extragenital anomalies, especially renal. Systematic evaluation using the VCUAM classification enables comprehensive assessment, aiding in individualized and multidisciplinary care strategies.
OBJECTIVE: This study aims to determine the prevalence of MRKH syndrome among women presenting with primary amenorrhea at a tertiary care center in South India and to describe their clinical profiles and associated anomalies using the Vagina Cervix Uterus Adnexa-associated Malformation (VCUAM) classification system.
METHODS: A retrospective study was conducted over 15 years (January 2008 to December 2022) including women diagnosed with MRKH syndrome based on inclusion criteria: primary amenorrhea, normal secondary sexual characteristics, 46-XX karyotype, and normal serum follicle-stimulating hormone levels. Data were extracted from medical records and analyzed using SPSS v25.0.
RESULTS: Out of 340 women with primary amenorrhea, 181 (60%) were diagnosed with MRKH syndrome. The mean age at presentation was 21 years. The predominant complaint was non-attainment of menarche (66.8%), with 16.5% reporting cyclical abdominal pain. Type 1 MRKH was most common (78.9%), followed by Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia (MURCS) association (16.5%). Renal anomalies (15.5%) were the most frequent extragenital malformations. All women had vaginal and cervical agenesis (V5bC2b). Uterine anomalies included bilateral aplasia (89.1%), unilateral aplasia (0.6%), and hypoplasia (10.5%).
CONCLUSION: MRKH syndrome is a significant cause of primary amenorrhea, with notable extragenital anomalies, especially renal. Systematic evaluation using the VCUAM classification enables comprehensive assessment, aiding in individualized and multidisciplinary care strategies.