Mayer-Rokitansky-Kuster-Hauser syndrome: a review

Figures & data

Table 1 Differential diagnosis between MRKH (Mayer-Rokitansky-Kuster-Hauser) and AIS (androgen insensitivity syndrome)

MRKH	AIS
Genotype XX	Genotype XY
Normal female serum T levels	Normal male T levels
Normal female pubic and axillary hair	Decreased pubic and axillary hair
Normal female height	Increased height compared with female counterparts
Normal ovaries located high on the pelvic side wall	Intra-abdominal testicles
No increased risk of gonadal malignancy	Gonads removal recommended after puberty due to increased risk of malignancy

Note: Milder forms of AIS might present a less affected phenotype.

Abbreviation: T, testosterone.

Table 2 Treatment options for the creation of neovagina

Non-surgical treatment	Surgical treatment
Frank method	Traction-based methods
Vaginal dilators with progressive caliber	– Vechietti (laparotomic or laparoscopic)
Ingram method	Graft-based methods
Vaginal dilators attached to a seat	– Abbe-McIndoe (vaginal approach, various tissues used, skin graft most common) – Intestinal (combined vaginal and laparotomic or laparoscopic approach) – Davydov (combined vaginal and laparoscopic approach using peritoneum)

Note: Most surgical methods require the continued use of non-surgical methods or frequent intercourse to maintain results.