Preferred Label : Mullerian agenesis;
ICD-11 definition : Absent vagina, absent or hypoplastic uterus (sometimes with areas of functional endometrium),
and normal or hypoplastic fallopian tubes, mostly associated with normal ovaries and
urologic abnormalities and skeletal malformations. Presenting sympoms and signs typically
include primary amenorrhea, absent vagina, and normal breast and pubic hair development.;
ICD-11 synonym : Mayer-Rokitansky-Küster-Hauser syndrome;
ICD-11 inclusion : Mayer-Rokitansky-Küster-Hauser syndrome;
Origin ID : 1098660435;
Automatic exact mappings (from CISMeF team)
Currated CISMeF NLP mapping
Validated automatic mappings to BTNT
Absent vagina, absent or hypoplastic uterus (sometimes with areas of functional endometrium),
and normal or hypoplastic fallopian tubes, mostly associated with normal ovaries and
urologic abnormalities and skeletal malformations. Presenting sympoms and signs typically
include primary amenorrhea, absent vagina, and normal breast and pubic hair development.