Discussion
Although inguinal hernia is less frequently seen in young female infants
as compared to males, the most often stumbled upon structures in female
infants inguinal hernia sacs are the ovaries, with or without fallopian
tubes.6 However, it is exceedingly uncommon for
ovaries to be found in the inguinal areas during adolescence and
maturity. In these situations, Muller anomalies, which result from
Muller aplasia and MRKH syndrome, are part of the etiopathogenesis.6,7
Initially situated inside the abdominal cavity, the reproductive glands
subsequently descend. By the end of the eighth month of pregnancy,
testes have become localized in the scrotum, and the processus vaginalis
obliterates following birth. The non-obliterated processus vaginalis in
females is known as the canal of Nuck and is a contributing factor to
ovarian herniation, sometimes in conjunction with the uterine
tubes.2,8
The incidence of MRKH syndrome is 1 in 4000-5000 live births and has
autosomal dominant mode of inheritance with incomplete penetrance and
variable expressivity. 9 The aberrant development of
the paramesonephric ducts is the primary cause of MRKH syndrome. Since
the ovaries emanate from genital ridges, these patients have normal
gonadal function. Upon impact on genitalia, the condition is defined as
type 1 MRKH syndrome. whereas, type 2 МRKH or MURCS association syndrome
includes renal anomalies (unilateral agenesis, ectopic kidney, horseshoe
kidney), skeletal anomalies (fused cervical vertebrae, scoliosis,
klippel-feil syndrome), and hearing loss or cardiac
defects.2,3,5,8,9
Individuals with MRKH syndrome have a normal female habitus, including a
typical thelarche and adrenarche. Primary amenorrhea is the very first
symptom of MRKH syndrome in young women who otherwise present with
normal development of secondary sexual traits, karyotype 46, XX, and no
discernible chromosomal aberration.5,9 In our case,
the patient presented with swelling in bilateral groin of recent onset
with a background history of
primary amenorrhea.
It is obscure how genital organ herniation occurs in cases of groin
hernia. According to Ozkan et al. herniation may be spurred on by
weakening of the ovarian suspensory ligament and broad ligament, which
may worsen under conditions of increased abdominal
pressure.10 Various theories are formulated by gallant
researchers. Thomson’s theory states that there is a greater likelihood
of the entire uterus, ovary, and fallopian tube herniating into the
inguinal canal when the Mullerian ducts fail to fuse, which causes the
ovaries to move overly.11 Conversely, Fowler
postulated that the fundamental cause of ovarian inguinal hernia was
lengthened ovarian suspensory ligaments.12 Some also
contend that the inguinal canal herniation of the ovaries was caused by
ovarian or broad ligament weakening and an elevation in intra-abdominal
pressure.13
Kumar et. al reported a case of bilateral ovarian inguinal hernia with
left sided irreducibility in a 21 years old female with mullerian
agenesis. Similarly, Samantroy et al. reported twisted gangrenous ovary
with fallopian tube and underdeveloped uterus as the content of indirect
inguinal hernia in 34 years old female with mullerian agenesis in
2021.1
The patient had a successful emergency hernia exploration with a left
oophorectomy, fallopian tube uterus relocation, and herniorrhaphy.
Moreover, irrespective of congenital genitourinary conditions, multiple
publications have recorded inguinal herniation of the ovary with or
without the fallopian tube and uterus.14 Such as Okada
et al. description of a case of 1 year old female girl with an
irreducible inguinal hernia that covered whole uterus, fallopian tube,
and entire ovaries and Fowler’s explanation of sliding inguinal hernia
in 1 year infant comprising bilateral ovaries.12,13
There are exceptionally few published circumstances involving ovarian
herniation in MRKH syndrome, either with or without the uterus and
fallopian tube. In 1980, Riggall and Cantor reported the first
illustration of an inguinal hernia containing a uterus in a woman with a
female karyotype, phenotypic traits, and
infertility.1,5
Mohanty et al. and Bazi et al. reported a case of bilateral inguinal
hernia containing ovaries in a 20 and 12 year old female with features
of MRKH syndrome.5,15 Likewise, Saini et al. also
published a case report of 20 year lady with mullerian defect who
developed a right indirect inguinal hernia containing the right
ovary.3 In addition, case reports of MRKH syndrome
with inguinal hernia having ovaries as content are described in
literature by various authors, namely Shumarova et al., Khan et al., and
Yao et al.1 While delving more into a piece of
published work, a young woman with type 2 MRKH syndrome and a right
utero-ovarian inguinal herniation who lacked a left kidney came across
which was described by Al Omary et al..4 Type 2 MRKH
syndrome has also been described by Jafari et al. in a 12 year old girl
who developed inguinal herniation of left ovary, fallopian tube and left
horn of underdeveloped bicornuate uterus along with ectopic
kidney.4
Ovarian torsion, blockage, irreducibility, strangulation, and gangrene
are the common repercussions of ovarian inguinal hernia. About 4 to 37%
of complex cases of inguinal hernia have an irreducible ovary, whereas 2
to 33% of cases have torsion or infarction.1 The most
important steps to prevent complications and restore fertility are early
diagnosis and treatment. Independent of müllerian status, relocation and
herniorrhaphy are recommended as soon as the ovarian tissue in hernial
sac is identified.1,2,6,7 Reduction and repair were
carried out employing the open method on our patient. This repositioning
can also be done laparoscopically.
Patients with MRKH syndrome require sophisticated care that involves a
multidisciplinary approach centered on the patient, careful discussion,
and treatment of all gynecological, sexual, mental, emotional , and
reproductive difficulties. Through in vitro fertilization and surrogacy,
it is possible for couples to establish genetic offspring. Two
alternatives exist for performing sexual intercourse: surgically
creating a neo-vagina or manually dilating the vagina with
dilators.2,3,4,5,14