Introduction

Uterine inguinale hernias are a rare entity, reaching 1% of incidence in the female population. However, in the male population, it is directly associated with abnormalities of sexual differentiation or intersex conditions such as male pseudohermaphroditism or also called Type I Müllerian duct persistence syndrome, with no more than 150 cases described worldwide. Intersex states usually have an early manifestation that allows multidisciplinary intervention, but cases of silent courses are reported in phenotypically male patients whose only clinical signs are infertility or cryptorchidism and whose diagnosis is made accidentally during an inguinal hernioplasty or an orchidopexy¹,². The following is the case of an adult male patient, infertile, with right homolateral cryptorchidism, who was scheduled on an outpatient basis for a right inguinal hernioplasty and whose intraoperative findings described the presence of macroscopically Müllerian structures such as uterus, fallopian tube and ovaries running inside the operated inguinal hernial sac.

Case report

We present the case of a 64-year-old male patient, infertile, with a long-standing right inguinoscrotal hernia. He had a soft tissue ultrasound where an inguinal and umbilical hernial defect was documented with no additional findings. The patient also presented a diagnosis of right homolateral cryptorchidism and nodular images in the right testicle with normal Doppler analysis. A right inguinal hernioplasty was scheduled on an outpatient basis. The surgical approach was performed through an oblique inguinotomy incision. An indirect inguinoscrotal hernia with sliding of contents into the hernia sac compatible with structures macroscopically compatible with the uterus, fallopian tubes, and involuted ovaries was observed (Fig. 1).

Figure 1 Inguinotomy and content of hernial sac compatible with Müllerian structures (uterus, fallopian tubes, and ovaries). Caudally male reproductive structures, testicles, and penis (*). 

Reduction of the contents of the inguinal hernial sac and hernioplasty with the placement of prosthetic mesh with the Lichtenstein technique was performed. In the post-operative period, the patient had no complications and underwent an axial tomography of the abdomen and total pelvis which confirmed the intraoperative findings described above (Fig. 2A and B).

Figure 2 Contrast abdominal computed tomography in arterial phase in axial and sagittal planes A and B: respectively showing evidence of muscular soft tissue density mass (30 HU) in retrovesical space in contact with pelvic structures (yellow star). 

Discussion

Persistent Müllerian duct syndrome (PMDS) is a very rare form of pseudohermaphroditism characterized by the presence of structures deriving from persistent Müllerian duct in a patient phenotypically and occasionally genotypically expressed as a normal male³-⁵. It was initially described by Nilson in 1939, and since then approximately 150 related cases have been published⁶,⁷. To have an appropriate male differentiation, the production of anti-Müllerian Hormone is needed, which induces the regression of the Müllerian structures, allowing the differentiation of the Wolf's ducts and the formation of the vas deferens, epididymis, and seminal vesicles, which together with testosterone allow the adequate organization of the male reproductive system⁸,⁹. Its etiology is not entirely clarified; however, it is related to a lack of synthesis and release of Müllerian inhibitory factor (MIF) or its receptor (MIFr). MIF is secreted by Sertoli cells in fetal tissue from the 7^th gestational week and is responsible for the regression of the Müllerian ducts in male fetuses. These patients are usually of 46 XY karyotype and besides presenting infertility or cryptorchidism, they can be asymptomatic until they are taken for surgical intervention and accidentally realize the recognition of organs macroscopically similar to the uterus and fallopian tubes, exceptionally during an inguinal herniorrhaphy or an orchidopexy¹⁰-¹². The presence of macroscopically uterine-like structures, with an ovary, in an inguinal hernial sac is due to the persistence of the paramesonephric duct in the male also called as PMDS type I¹³-¹⁵. In patients with true hermaphroditism, both ovarian and testicular tissue will be found in one or both gonads. In female patients classified as pseudohermaphrodites, the gonads are ovarian, but the reproductive organ has a masculine tendency. Pseudohermaphroditism may not be identified until puberty and even, as in the current clinical case, in the context of an elderly patient with an active sexual life, sometimes even with preserved reproductive capacities¹⁶. Currently, there are reports of laparoscopic management of male pseudohermaphroditism in pediatric patients with exeresis of the Müllerian structures, justified by the risk of malignization and infertility¹⁷,¹⁸. In cases of concomitant cryptorchidism, a division of the intra-abdominal uterine tissue attached to the testicle is considered to allow an adequate downward trajectory into the tunica vaginalis and then the remaining Müllerian structures are resected by an anterior inguinotomy¹⁹.

Conclusion

Müllerian duct persistence syndrome Type I is described as an extremely rare pathology that is generally diagnosed intraoperatively when performing orchidopexy or inguinal hernioplasty in patients with phenotypically male characteristics. Given that preoperative findings of physical examination and ultrasonography of the inguinal region, whose ability to discriminate the structures contained in the hernial sac is precarious, it is unlikely that a preoperative identification of the pathology is performed in the patient with silent intersex clinic²⁰. In the current case, through outpatient programming of a right inguinal hernioplasty, intraoperative identification of the Müllerian structures, which were not excised in the first surgical stage, was carried out. The defect of the indirect inguinal hernia was repaired by inserting a prosthetic mesh through the Lichtenstein technique without complications and was sent to complement studies with the genetics and endocrinology service, as well as to follow-up controls by the general surgery service. We consider of great importance to take into account that, in spite of being a very rare pathology, it can mean a therapeutic challenge for the multidisciplinary group in charge during the performance of an inguinal hernioplasty in men.