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