CONGENITAL ABSENCE OF UTERUS

Sadia Anwar

Abstract


These are two case reports of females with primary amenorrhea, well developed secondary sexual characteristics and congenital absence of uterus. The incidence of Mayer-Rokitansky-Kuester-Hauser syndrome was not clearly established, but studies indicate a variation of 1/4,000 and 1/5,000 live births of the female sex. The syndrome is characterized by aplasia of the Müllerian duct (uterus and upper two-thirds of the vagina) on a person who has karyotype 46, XX with female phenotype characteristic of primary amenorrhea in adolescence. Treatment is usually delayed until the patient is ready to begin sexual activity.

Keywords


Rokitansky syndrome; Mullerian duct; Karyotype; Primary amenorrhea.

Full Text:

PDF

References


Guerrier D, Mouchel T, Pasquier L, Pellerin I. The Mayer-Rokitansky- Küster-Hauser syndrome (congenital absence of uterus and vagina) - phenotyp manifestations and genetic approaches. J Negat Results in Biomed 2006;5:1.

Karine Morcel, Laure Camborieux, Programme de Recherches sur les Aplasies Müllériennes (PRAM), Daniel Guerrier. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Orphanet J Rare Dis 2007; 2:13. doi:10.1186/1750-1172-2-13

Dornelas MT, Arruda FR, Anna LLS, Nett GM, Souza RG, Machado DC, et al. Vaginal reconstruction by neurovascular pudendal thigh flap in Rokitansky’s syndrome. Rev Bras Cir Plást 2010;25:525-31.

Folch M, Pigem I, Konje JC. Müllerian agenesis: etiology, diagnosis, and management. Obstet Gynecol Surv 2000;55:644-9.

Sharma BB, Sharma S, Singh Y, Balesa J. Mayer-Rokitansky-Kuester- Hauser Type I Syndrome - A Case Report. Indian J Case Reports 2015;1:8-10.

Sultan C, Biason-Lauber A, Philibert P. Mayer-Rokitansky-Kuester- Hauser syndrome: Recent clinical and genetic findings. Gynecol Endocrinol 2009;25:8-11.

Chandiramani M, Gardiner CA, Padfield CJ, Ikhena SE. Mayer - Rokitansky - Kuester - Hauser syndrome. J Obstet Gynaecol 2006;26:603-6.

Oppelt P, Renner SP, Kellerman A, Brucker S, Hauser GA, Ludwig KS, et al. Clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: recommendations for clinical diagnosis and staging. Human Reprod 2006;21:792-7.

Edmonds DK. Dewhurst’s Textbook of Obstetrics & Gynaecology, 8th ed. New Jersey; John Wiley: 2012. DOI:10.1002/9781119979449




DOI: https://doi.org/10.46903/gjms/17.01.2015

Refbacks

  • There are currently no refbacks.


Copyright (c) 2019 Gomal Journal of Medical Sciences



© 2011 Gomal Journal of Medical Sciences