Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH): A Review

Abstract

Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH) is a sporadic disorder that attacks women. It is a congenital abnormality occurring during fetal development characterized by failure of the proper development of the uterus and the vagina in females who have a normal ovarian function and external genitalia. Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH) is characterized by an undeveloped or absent uterus and vagina and females with this condition suffer from Uterine Component Infertility (UCI). There are two types:1) Typical form (Type A)-The syndrome is characterized by the congenital absence of the uterus and upper 2\3 vagina with normal ovaries and fallopian tubes. 2) Atypical form (Type B)-The syndrome includes abnormalities of the ovaries and fallopian tubes and renal disorders. The cause of MRKH is unknown. The signs and symptoms are as follows: Absence of menstruation by age 16 is often one of the first signs of MRKH Syndrome. Other symptoms include: Symptoms of MRKH Type 1 include: Dyspareunia, Decreased vaginal length and thickness. Symptoms of MRKH Type 2 are similar to type 1, but may also include: Renal complications due to abnormal kidney development or position, or a missing kidney, Skeletal abnormalities mostly seen in the spinal vertebrae, Minor hearing disorder, Other symptoms include: Primary amenorrhea, Infertility, Voiding difficulties like dysuria, urinary incontinence or recurrent urinary tract infections (UTIs), Renal fusion and hydronephrosis, Extremity anomalies (polydactyly, syndactyly, club-foot), Patients undergo all bodily changes associated with menstruation as the ovarian function is normal. Diagnostic Evaluation-Detailed patient history, Thorough clinical evaluation, Transabdominal ultrasonography, Magnetic Resonance Imaging (MRI), Diagnostic Laparoscopy Celioscopy, Karyotyping, Spinal radiography, Audiogram, Echocardiography Non-Surgical Treatment-Dilators are used to make a neo-vagina. Surgical Treatment-Vecchietti Procedure, Uterine Transplant. In conclusion, we can say that advancement in medicine can treat girls with MRKH syndrome to a quality sexual life and will also offer different modalities of achieving motherhood.