Mayer-Rokitansky-Küster-Hauser syndrome as an interdisciplinary problem
| Autor: | Magdalena Liszewska-Kapłon, Maciej Bagłaj, Łukasz Kotarski, Mateusz Strózik, Lidia Hirnle |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
030213 general clinical medicine
MRKH Syndrome medicine.medical_specialty 46 XX Disorders of Sex Development Uterus Medicine (miscellaneous) General Biochemistry Genetics and Molecular Biology Congenital Abnormalities 03 medical and health sciences 0302 clinical medicine Internal Medicine medicine Humans Pharmacology (medical) Mayer-Rokitansky-Kuster-Hauser Syndrome Mullerian Ducts Genetics (clinical) business.industry General surgery Aplasia medicine.disease Müllerian agenesis Sexual intercourse medicine.anatomical_structure Mullerian aplasia Reviews and References (medical) Vagina Female business |
| Zdroj: | Advances in clinical and experimental medicine : official organ Wroclaw Medical University. 29(4) |
| ISSN: | 1899-5276 |
| Popis: | The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, also known as Mullerian agenesis or aplasia, is a congenital disease manifested by the aplasia of the uterus and the upper 2/3 of the vagina; its incidence is 1 in 4,000-5,000 female live births. We can distinguish 2 types of the MRKH syndrome: type I, which is characterized by an isolated absence of 2/3 of the vagina and uterus; and type II or MURCS (Mullerian duct aplasia, unilateral renal agenesis and cervicothoracic somite anomalies), which is also associated with other symptoms. The treatment of the MRKH syndrome patients aims at creating a neovagina and enabling sexual intercourse. Non-surgical techniques are the first-choice methods, and more than 90% of patients notice an anatomical and functional improvement if they are well-prepared emotionally. If non-surgical treatment does not bring about the expected results, a surgical procedure remains an option. The surgical method is mainly determined by the surgeon's experience. There are a few types of operations, though none of them seems superior to others. The next challenge is to provide these patients with a chance to become parents. Nowadays, a uterine transplant, a surrogate or adoption are the available solutions. An interdisciplinary approach is required, and the treatment should consist of medical and psychological support. This review presents the current knowledge about the MRKH syndrome with regard to the current methods of non-surgical and surgical treatment as well as a summary of the associated psychological problems. |
| Databáze: | OpenAIRE |
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