Mayer-Rokitansky-Kuster-Hauser Syndrome: A Unique Case Presentation.
Mil Med
; 183(5-6): e266-e269, 2018 05 01.
Article
em En
| MEDLINE
| ID: mdl-29415121
ABSTRACT
Background:
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital condition characterized by aplasia of the vagina with or without concurrent uterine and/or cervical aplasia. Type II (MURCS) is a rare form involving MUllerian agenesis, Renal agenesis, and Cervicothoracic Somite anomalies. Case A 17-yr-old virginal female presented for evaluation of primary amenorrhea and pelvic pain. Her medical history was significant for a bicuspid aortic valve and right radial dysplasia. She demonstrated normal secondary sexual development and a normal karyotype. Pelvic magnetic resonance imaging revealed an aplastic vaginal, no identifiable cervix or uterus, and normal ovaries. A laparoscopy was performed for the evaluation of pain and findings were significant for bilateral uterine horn and fallopian tube remnants noted along the pelvic sidewalls. This patient evaluation suggests a unique presentation of MURCS association.Conclusion:
To our knowledge, this is the first case of MRKH presenting with a bicuspid aortic valve and radial dysplasia. A review of the literature reveals no other cases of MRKH with these unique anomalies.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Anormalidades Congênitas
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Transtornos 46, XX do Desenvolvimento Sexual
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Ductos Paramesonéfricos
Tipo de estudo:
Etiology_studies
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Prognostic_studies
Limite:
Adolescent
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Female
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Humans
Idioma:
En
Revista:
Mil Med
Ano de publicação:
2018
Tipo de documento:
Article