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.