Case of mullerian agenesis presenting as bilateral inguinal hernia with left sided irreducibility in a 21 years old female: A rare case report

Abstract

Introduction and importance

Presence of ovary in an inguinal canal in adult female is a rare presentation. Most of the cases are associated with congenital abnormalities of the female genital tract. The incidence of mullerian agenesis decreases with increasing age. The presence of bilateral inguinal hernia with ovaries as a content in an adult female is extremely rare.

Case presentation

A 21 year old female presented with bilateral groin swelling with associated pain on left side. On evaluation, there was inguinal hernia on both sides, of which left sided was irreducible. The ultrasonography of groin showed uterus and ovary as contents on left sided sac and ovary on the right side. Considering the severe pain and irreducibility on left side, the exploration of bilateral inguinal regions was done. On exploration, the biopsy was taken and the contents were repositioned back into the pelvis.

Clinical discussion

The presence of ovary, uterus or both in the inguinal canal is extremely rare in an adult female. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by normal secondary sexual character with agenesis of uterus and vagina. Karyotype of individual female is essentially 46XX. Early diagnosis is essential to prevent the complications. The primary treatment of irreducible inguinal hernia is exploration and repositioning the contents back to pelvis if grossly normal and repair of inguinal hernia at earliest.

Conclusion

A case of bilateral inguinal ovarian hernia with irreducibility in an adult female requires an urgent exploration. Delaying the intervention may result in obstruction, strangulation, and subsequent infertility.

Highlights

• •Bilateral ovarian inguinal hernias are rare occurrences in adult female.
• •Ovarian hernia is usually associated with MRKH Syndrome.
• •Ultrasonography is the basic adjunct in such case.
• •Early diagnosis and surgical correction are the key factors to prevent the complications.
• •The grossly normal appearing contents can be safely repositioned in to the pelvis.

1. Introduction

Inguinal hernia surgery is one of the most commonly done procedures in the surgical discipline, with majority of the patients presenting to outpatient department. The usual content of inguinal hernia sac is omentum, and small intestine, but other contents like sigmoid colon, caecum, appendix, and urinary bladder are not unusual [1]. Inguinal hernia containing ovaries, fallopian tubes and uterus occurs rarely in adult females [2].The occurrence of uterus, fallopian tube, and ovary with mullerian dysgenesis is also known as Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome and its incidence is 1 in 4000/5000 live birth [3]. This syndrome is transmitted as an autosomal dominant trait. We are presenting a case of bilateral inguinal hernia with ovarian content on both side and uterus on left side with features of irreducibility on left side in an adult female. To the best of our knowledge, bilateral inguinal hernia with left sided irreducibility in a 21 years old female has not been reported in the literature previously.

The aim of this presentation is to report an extremely rare case where clinical diagnosis and early intervention are required to prevent complication. This case report is in line with SCARE criteria 2020 [4].

2. Case report

A 21 years old female presented to our emergency department with left inguinal pain. She had a history of swelling in both inguinal regions since childhood. The swelling disappeared on lying supine but it became prominent during lifting weight, straining, and coughing. She felt that the swelling was reducible for the previous 15 days, and after aching two days prior to being seen. On general physical examination, patient was haemodynamically stable, except for tachycardia which was 110 beats per minute. There was a non-reducible lump of 4 × 3 × 2 cm in left inguinal region with absent impulse on coughing. The right sided inguinal hernia was reducible which was globular in shape with positive impulse on coughing. The ultrasonography of groin revealed atrophied uterus, ovary and fallopian tubes in the left sided sac and ovary in the right side. The ultrasonography of the abdomen revealed no other urogenital abnormalities except absent uterus. She had significant past history of primary amenorrhea, with normal development of secondary sexual characters and had thelarche at normal age. Per vaginal examination revealed blind ending vaginal pouch with absent cervix. However, she had normal sexual exposure with her husband. The peripheral karyotyping showed 46XX, which suggested the diagnosis of MRKH syndrome with bilateral inguinal hernia. The hormonal evaluation suggested serum follicular stimulating hormone (FSH) of 4.7 IU/L, anti mullerian hormone (AMH) of 19.33 ng/ml, serum oestradiol of 42.00 pg/ml, serum prolactin of 15.1 ng/ml, serum luteinizing hormone of 6 IU/L, serum testosterone of 52 ng/dl, and serum progesterone of 0.3 ng/ml. Considering the severe pain and clinical diagnosis of irreducible hernia on left side, exploration of bilateral inguinal regions was done. Intraoperative findings revealed hernia sac with rudimentary uterus adherent to ovary and fallopian tube on the left side (Fig. 1). On the right side the hernia sac contained only ovary (Fig. 2). As left sided ovary was adherent to the rudimentary uterus, intraoperative biopsy was taken from both the masses and repositioned to the pelvis. Lytle's repair of both the side of deep ring was done. Post-operative period was uneventful and patient discharged on 7th post-operative day. Biopsy report showed right sided mass to be ovary and of left sided to be ovary and uterus. Patient was absolutely fine in the follow-up clinic at 3 months after discharge.

Fig. 1.

Showing rudimentary uterus (black arrow) and ovary (green arrow) within the sac on the left side, and repair done on the right side (red arrow).

Fig. 2.

Showing ovary (green arrow) within the sac on the right side.

3. Discussion

The surgical repair of inguinal hernia is one of the most commonly performed procedures in the department of general surgery [5]. The usual contents of the hernia sac are omentum, intestine, appendix and urinary bladder [6]. The hernia sac content as ovary, uterus or both in the inguinal canal is an extremely rare occurrence in adult female [6]^, [7]. Although the content as ovary, fallopian tube or rudimentary uterus in the inguinal canal of an infant or a paediatric age group is very common and usually associated with congenital genitourinary tract anomalies such as Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome [8]^, [9]. The patient of this syndrome typically present with primary amenorrhea with normal female phenotype, vaginal agenesis, rudimentary or absent uterus and normal ovaries. Karyotype of patient is essentially 46XX, which differentiate it from androgen insensitivity syndromes, which has 46XY karyotype [10]^, [11]. There are two types of MRKH syndrome, where Type 1 is characterized by the congenital absence of uterus and vagina and Type 2 has mullerian duct agenesis with renal, cardiac, muscular, and vertebral defect. Such individuals have normal female body pattern with normal thelarche and adrenarche. The usual complications in case of ovarian inguinal hernia are ovarian torsion, infarction, and obstruction [12]. In most of complicated cases of inguinal hernia about 4 to 37 % present with irreducible ovary in inguinal hernia sac, while torsion or infarction are reported in 2 to 33 % cases [12]. The obstructed inguinal hernia irrespective of the content is usually diagnosed clinically but bedside ultrasonography help in augmenting the clinical diagnosis. The early diagnosis and management are the key steps to prevent complications and restore fertility. There are multiple hypotheses for the development of the ovarian hernia in female such as non-fusion of mullerian ducts, congenitally elongated ovarian ligaments, multiparity, weakness of broad ligament or ovarian ligament along with increase in the intra-abdominal pressure [13]^, [14]^, [15]. As per available literature for the inguinal hernia in adult female with content as ovary was found in most of cases (15 cases), as fallopian tube and ovary in 3 cases, and as uterus, ovary, and fallopian tube in 4 cases (Table 1). Out of 16 such cases, most of cases were of left sided (9 cases), 2 cases were of right sided, and 5 cases were bilateral type. Most of the inguinal hernias in female are diagnosed clinically with ultrasonography as an adjunct to diagnose the contents of the sac [16]. The differential diagnosis of the inguinal swelling in adult female may include hernia with variable content (omentum, intestine, bladder, appendix, ovary, fallopian tube, or rudimentary uterus), soft tissue tumours, abscess, enlarged lymph nodes, or hydrocele [17]. The main aim of the treatment is to explore the inguinal canal, examine the content, and assess the viability of the content. The decision of the excision of the contents depends upon the surgeon's judgment regarding the viability and feasibility.

Table 1.

Summary of previously reported cases.

Reported by (authors)	Year	Age (years)	Side	Content	Remarks
Riggall F.C.	1980	16	Left	Ovary, fallopian tube (FT), and rudimentary uterus	Repositioning the content into pelvis and repair of hernia (RC and RH)
Bradshaw KD	1986	28	Left	Ovary & fallopian tube	RC and RH
Suneja A	1996	14	Bilateral	Ovary and fallopian tube	RC and RH
Bazi T	2006	12	Bilateral	Ovaries	RC and RH
Yao. L	2009	28	Left	Ovary	RC and RH
Khairatar R	2015	45	Bilateral	Ovary (right side) & fallopian tube (left side)	RC and RH
Medeiros FC,	2015	15	Bilateral	Ovary, fallopian tube, and uterus.	RC and RH
Junji U.	2016	87	Right	Ovary, uterus, and fallopian tube.	RC and RH
Mohanty H.S.	2017	20	Bilateral	Ovaries	RC and RH
Park Y.H.	2017	22	Left	Fallopian tube	Laparoscopic TEP done to repair the hernia
Verma R.	2018	45	Left	Rudimentary uterus & ovary	RC and RH
Khan W.F.	2019	18	Left	Ovary	TEP for hernia repair.
Jafari R	2020	13	Left	Ovary	RC and RH
Rahul Saini	2021	20	Right	Ovary	RC and RH
Samantroy S.	2021	34	Left	Ovary, tube, and uterus	RC and RH
Shumarova S	2021	18	Left	Ovary	Ovarian cyst

4. Conclusion

There must be high degree of suspicion for the possibility of ovarian hernia in female patient presenting with an irreducible swelling in the inguinal region. The early clinical diagnosis and intervention are the key steps to avoid serious complications. The grossly normal appearing contents can be safely repositioned in to the pelvis after intraoperative biopsy.

Patient's perspective

I am thankful to the operating surgeon for saving me and my ovary and now have a hope that I will conceive.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Ethical approval

N/A.

Funding

N/A.

Guarantor

Dr Anil Kumar.

Research registration number

Our paper is a case report; no registration was done for it.

CRediT authorship contribution statement

• Anurag Kumar: Study concept and operated the case.

• Anil Kumar: Writing the paper

• Majid Anwer: References and drafting the manuscript.

• Deepak Kumar: Review of the literature.

Declaration of competing interest

All authors have nothing to disclose.