Case presentation of isolated Mayer -Rokitansky -Kuster -Hauser (MRKH) Syndrome- A Short Review of Two Cases

Abstract

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is a congenital condition with an unclear cause that is characterized by mullerian agenesis and may be linked to cardiac, skeletal, or renal malformations. Most incidents are infrequent. Two typical cases of MRKH syndrome are presented here. When a patient presents with primary amenorrhea, a comprehensive diagnostic workup should encompass hormonal screening, physical examination, chromosomal analysis,.and history collection. A healthy ovary is indicated by normal levels of hormones , follicle stimulating hormone (FSH) and luteinizing hormone (LH). The precise anatomical deficiencies, however, are highlighted by imaging modalities like MRI and USG. In conclusion, the absence of a regular menstrual cycle in a young, healthy-looking adolescent girl should raise the possibility of Mayer-Rokitansky-Kuster-Hauser syndrome. Regarding her potential for conception in the future and the various treatment options (both surgical and non-surgical), the patient should get appropriate counseling.