Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome has a prevalence of 1 in 4000–5000 female patients.5 MRKH syndrome, a Mullerian duct anomaly, is the congenital aplasia of uterus and upper two-thirds of vagina in a female with normal ovaries, fallopian tube, secondary sexual characteristics and 46XX karyotype.6 It is inherited as an autosomal dominant trait with an incidence of 1 in 4500 female births.5,7
There are two forms of MRKH syndrome. Type 1, accounting for 44% of MRKH syndromes, is the isolated Mullerian abnormality, i.e., congenital absence of uterus and vagina. Type 2, accounting for 56% of MRKHS, has Mullerian duct agenesis with renal, cardiac, muscular & vertebral defects. MURCS (Mullerian agenesis, Renal agenesis, Cervico-thoracic Somite abnormalities) is a subtype of MRKH type 2, with predefined multi-organ abnormalities. It accounts for 16% of MRKH type 2 cases.8 Such individuals have normal female body pattern with normal thelarche and adrenarche.
Inguinal hernia containing ovary is generally found in paediatric population as groin swellings, that accounts for 6–7% of inguinal hernias in this age group.9,10,11 In one series by Ein et al, the incidence in more than 1000 females was 15% but 76% were younger than 2 years.12 Almost 30% of all reported cases are related to adolescents or women of reproductive age.13 In another series of 1,950 groin hernia patients, the overall incidence of inguinal hernia containing ovary as a content was seen in < 2.9%.3 Association between MRKH type I syndrome and inguinal hernia with ovary as a content has been reported in few isolated case reports however delayed presentation in adult age with MURCS is very rare.4
Such cases also have abnormal genital development. Such presentations are found uncommonly in adult females.10 Ovarian torsion and infarction are seen in 2–33% of patients presenting as ovarian hernia.14 The incidence of incarceration (acute or chronic) was 9%.12 Early diagnosis and management are of prime importance to restore fertility in such patients as these ovaries are more prone to torsion and infarction.
Female adnexa might get entrapped in the inguinal canal and present as inguinal hernia. Various anatomical reasons exist for its occurrence, like, very short & less oblique inguinal canal or presence of a peritoneal pouch, similar to processus vaginalis, called as canal of nuck.14
Various theories were given to explain the development of ovarian hernia in females. According to a hypothesis given by Thomson, non-fusion of mullerian ducts lead to hypermobility of ovaries, thereby increasing the chances of entrapment of adnexa in the inguinal canal.15 Fowler, on the other hand, hypothesised that the congenitally elongated ovarian ligaments were the main cause of inguinal hernia with ovary in the canal.16 Another theory suggested that the weakness of broad ligament or ovarian ligament along with increase in the intra-abdominal pressure, contributed to herniation of ovary in inguinal canal.17 Multiparity can cause lengthening of the broad ligament, ovarian or suspensory ligaments, increasing the chance of entrapment of adnexa in inguinal canal.17
Normal ovary in a hernial sac is seen as a hypoechoic structure with variable sized sonolucent cysts on ultrasonography. Transabdominal ultrasound reveals the absence of ovary in the pelvic cavity. MRI pelvis on T2 weighted sequences demonstrates anatomy of uterus. Uterine aplasia is best diagnosed on T2 sagittal image. Normal vagina is seen as a structure of intermediate signal intensity between the base of bladder and urethra anteriorly and anal canal posteriorly. Vaginal agenesis is best demonstrated on axial images of MRI.18
In our patient, MRI revealed right side ovary as a content of right inguinal hernia sac with absence of uterus and normal left ovary within the pelvis. A battery of investigations was done to look for other congenital malformations, in order to formulate the diagnosis of MURCS syndrome. According to a recent systematic search of literature of women aged > 18 years for ovarian hernia found a total 17 cases, 15 of which presented as groin swelling as tender irreducible lump requiring urgent intervention. 14 cases underwent open surgical repair, 2 had laparoscopic repair and 1 didn’t undergo surgery.4 The surgery was repositioning of structures (in 11), oophorectomy (in 2), and salpingo-oophorectomy (in 2) with repair of the hernia.6
In our case, we performed modified open Bassini’s technique without mesh placement. Intraoperative findings included right ovary within the hernial sac along with right fallopian tube. The right ovary with fallopian tube was repositioned back into the pelvic cavity. Treatment of MRKH/MURCS syndrome requires a multipronged approach. Psychological counselling is required to counter the emotional aspects of patients. Healthy sexual relationship with in-vitro fertilization and surrogacy is possible. Manual dilatation of vagina using dilators and surgical creation of neo-vagina are options given to patients for performance of sexual intercourse.