Introduction
Inguinal hernia is exclusively common in males and relatively uncommon in females. Mostly the small intestine and omentum protrude into the hernial sac. In extremely rare instances, adult females develop inguinal hernias encompassing the uterus, fallopian tubes, and ovaries. As many as 31% of inguinal hernia sacs in young girls involve uterine adnexa; bilateral ovarian sliding hernias are unusual, and their incidence declines with age, making it infrequent in adult women. A substantial number of reported cases in adult females have been linked to additional genital tract congenital abnormalities. 1,2,3,4
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a moniker assigned to the occurrence of mullerian dysgenesis in the ovaries, uterus, and fallopian tube. The absence of paramesonephric ducts leads to halt in normal development of uterus, cervix, and vagina. However, the ovaries and fallopian tube have normal development; karyotyping reveals normal 46,XX and females exhibit signs of normal thelarche and pubarche.2,5
There are two reported subtypes of this syndrome: Patients with type 1 have malformations pertaining to genital system; meanwhile, patients with type 2 have an aberrant MURCS (Müllerian, Renal, Cervicothoracic Somite) association abnormality.2,3
In this article, we document the case of a 25 years old female with type 1 MRKH syndrome. She initially presented to our outpatient department with a complaint of swelling in the bilateral inguinal region.
The majority of clinicians are not familiar with MRKH syndrome because it is seldom seen in clinical practice. Introducing this infrequent case is our primary objective.