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CASE REPORT Table of Contents  
Ahead of print publication
A rare form of inguinal hernia complicated with Mayer-Rokitansky-Küster-Hauser syndrome: A case report


 Department of General Surgery, The Sixth Affiliated Hospital of South China University of Technology, Foshan, Guangdong, China

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Date of Submission17-Feb-2022
Date of Decision08-Mar-2022
Date of Acceptance10-Mar-2022
Date of Web Publication14-Apr-2022
 

  Abstract 

Herein, we reported a rare case of the herniation of the uterus, ovaries, and fallopian tubes into the inguinal canal complicated with Mayer-Rokitansky-Küster-Hauser syndrome in adolescents and provided anatomic, diagnostic, and therapeutic considerations for this rare phenomenon. The malformed uterus, ovaries, and fallopian tubes were returned to the abdominal cavity by using the Bassini technique for inguinal hernia. No intraoperative or postoperative complications were reported, and the patient recovered well after surgery. Collectively, our study provided evidence for the coexistence of this rare phenomenon with sex development disorders and suggests that precise and timely detection is a key for the successful outcome of the therapeutic intervention.

Keywords: Inguinal hernia, Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, mullerian agenesis, ovaries, rudimentary uterus


How to cite this URL:
Liang W, Zhu Y, Chen Q, Tan M. A rare form of inguinal hernia complicated with Mayer-Rokitansky-Küster-Hauser syndrome: A case report. Int J Abdom Wall Hernia Surg [Epub ahead of print] [cited 2022 Jul 1]. Available from: http://www.herniasurgeryjournal.org/preprintarticle.asp?id=343317





  Key Messages: Top


  • This study reported the importance of preoperative diagnosis, especially for premenstruating adolescents by determining the relationship between a rare condition (inguinal hernia complicated with MRKH syndrome) and congenital malformations.


  • Preoperative ultrasound examination should be considered for patients with this rare condition; otherwise, intraoperative misdiagnosis can lead to an ovarian injury and even removal of the uterus and uterine adnexa.



  •   Introduction Top


    Inguinal hernias containing the entire uterus and appendages ( Fallopian tube More Detailss and ovaries) are a very rare phenomenon,[1] presenting approximately 0.4% of cases.[2],[3],[4],[5],[6],[7],[8] However, in the present case, the patient was diagnosed with inguinal hernias complicated with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a very unlikely and even more rare condition with an incidence of about 1/5,000.[9] This rare condition is attributed to the female inguinal hernias combined with a malformed uterus and vaginal agenesis. Although MRKH is characterized by a malformed uterus and vaginal agenesis,[10] however, patients manifest normal female phenotype, vaginal hypoplasia, uterine hypoplasia, and normal ovaries with the typical female phenotype.[11] Therefore, precise diagnosis is a major challenge. Accordingly, here, we presented a case report of this rare phenomenon by highlighting the importance of preoperative diagnosis, especially for premenstruating adolescents to avoid the intraoperative misdiagnosis that can lead to ovarian injury and even removal of the uterus and uterine adnexa.


      Case Report and Technique Description Top


    A 13-year-old female patient was presented at the gynecologic clinic of our hospital on May 13, 2016, with a left inguinal mass that was developed 2 months earlier. The case history indicated that this patient has undergone a laparoscopic high ligation of the hernia sac for the treatment of inguinal hernia about 3 years ago on May 20, 2013. At the time of first surgery (May, 2013), hernial contents were returned to their normal location, the blood vessels in the opening of the internal ring of the inguinal canal were relatively thick, and no intraoperative or postoperative complications were reported at that time (laparoscopic view after the internal ring was closed during the first operation, [Figure 1]A and B). Three years later (May, 2016), when the patient was about 13 years old, physical examination revealed a mass of about 30 × 20 mm in size at the left inguinal area. The clinical examination indicated that the mass was moderately hard with poorly defined edges, irreducible, and exhibited light pressure pain. Color Doppler ultrasound revealed that in addition to an irregular mass of about 62 × 18 × 25 mm in size with a clear boundary interconnecting the abdominal cavity, there was a liquid dark area with a depth of about 13 mm above this irregular mass. Ultrasound images demonstrated the abundant blood flow signals in the mass. Furthermore, laparoscopic exploration and left inguinal hernia repair were performed, and the preoperative diagnosis reveals it as the recurrence of inguinal hernia after the first surgery.
    Figure 1: A, Laparoscopic view of the thick vessel at the left internal ring of the inguinal canal. B, Laparoscopic view after the internal ring was closed during the first operation. C, Laparoscopic view of the closed original internal ring during the second operation

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    During the second surgical operation, no significant defect was found at the left internal ring of the inguinal canal, and no bowel protrusion into the hernial sac was observed during the laparoscopic examination (laparoscopic view of the closed original internal ring during the second operation, [Figure 1]C). However, to expose a mass of about 40 × 25 mm in size (protruding from the outer ring), a 40-mm incision was made in the left inguinal region. Furthermore, the mass protruding from the lateral side of the inferior abdominal artery was revealed by cutting the aponeurosis of the abdominal external oblique muscle. Following that, the sac was cut open and we found the presence of the left ovary and fallopian tube, as well as a dysplastic uterus in the hernia. Further examination indicated that the size of the ovary was normal with a regular structure containing an oviduct umbrella (the contents of the hernia at the left ovary, fallopian tube, and a dysplastic uterus, [Figure 2]A). Besides, an underdeveloped uterine floor with a transverse diameter of about 2.5 cm was also observed [Figure 2]B.
    Figure 2: A, A photo taken during the second operation indicating the contents of the hernia at the left ovary, fallopian tube, and a dysplastic uterus. B, A photo was taken during the second operation showing a normal ovary, fallopian tube, and a dysplastic uterus

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    The investigation of the inner ring orifice through the inguinal canal revealed that the inguinal canal was not completely closed ([Figure 3]A indicating that the internal ring of the inguinal canal was not completely closed during the first surgery), the uterine body was found to have a stripe-like appearance with a transverse diameter of about 15 mm (laparoscopic view of the abdominal uterus, [Figure 3]B), the analysis of the vulva indicated the absence of a vagina. During the second surgical intervention, the right ovary was fixed to the right pelvic wall (laparoscopic view of the right ovary fixed to the pelvic wall, [Figure 3]C), the malformed uterus, ovaries, and fallopian tubes were returned to the abdominal cavity by using the Bassini technique for inguinal hernia. Genetic karyotyping showed 46XX chromosomes, while no other congenital malformations were observed. The patient recovered well and was discharged the day after surgery.
    Figure 3: A, A photo taken during the second operation showing the result of finger exploration through the inguinal tube, indicating that the internal ring of the inguinal canal was not completely closed during the first surgery. B, Laparoscopic view of the abdominal uterus. C, Laparoscopic view of the right ovary fixed to the pelvic wall

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    The final evaluation of this case indicated it as a left inguinal hernia containing the uterus, ovary, and fallopian tube complicated with MRKH syndrome. A follow-up evaluation was performed every 3 months after the surgical intervention, and the follow-up study of about 3 years after the surgery indicated no inguinal mass recurrence or abdominal pain. The patient’s physical development was according to the standard of female secondary sexual characteristics except for the lack of menarche.


      Discussion Top


    This case study indicated the importance of preoperative diagnosis because inguinal hernia containing the uterus, ovaries, and fallopian tubes was complicated with MRKH syndrome (which is a rare condition), and slight negligence could not only result in ovarian injury but also mistakenly cause ovariectomy during surgery, thus highlighting the importance of preoperative diagnosis, especially for premenstruating adolescents. Moreover, a preoperative ultrasound examination should be considered for patients with this rare condition; otherwise, intraoperative misdiagnosis can lead to ovarian injury and even removal of the uterus and uterine adnexa. Since there is a relationship between this rare condition and congenital malformations, attention should be paid to the exclusion of musculoskeletal and renal abnormalities, while chromosome examination and postoperative ultrasound and gynecological follow-up are highly recommended.

    Declaration of patient consent

    The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

    Ethics committee approval

    This study was approved by the ethical committee of the Sixth Affiliated Hospital of South China University of Technology, Foshan, Guangdong, China.

    Financial support and sponsorship

    Nil.

    Conflicts of interest

    There are no conflicts of interest.



     
      References Top

    1.
    Esposito C, Escolino M, Turrà F, Roberti A, Cerulo M, Farina A, et al. Current concepts in the management of inguinal hernia and hydrocele in pediatric patients in laparoscopic era. Semin Pediatr Surg 2016;25:232-40.  Back to cited text no. 1
        
    2.
    Cascini V, Lisi G, Di Renzo D, Pappalepore N, Lelli Chiesa P. Irreducible indirect inguinal hernia containing uterus and bilateral adnexa in a premature female infant: Report of an exceptional case and review of the literature. J Pediatr Surg 2013;48:e17-9.  Back to cited text no. 2
        
    3.
    Ibrahim H, Khaled M, Gates T, Trice H, Sangster G. Irreducible indirect inguinal hernia in premature infant with ectopic uterus and bilateral adnexa. J La State Med Soc 2016;168:194-5.  Back to cited text no. 3
        
    4.
    Okada T, Sasaki S, Honda S, Miyagi H, Minato M, Todo S. Irreducible indirect inguinal hernia containing uterus, ovaries, and fallopian tubes. Hernia 2012;16:471-3.  Back to cited text no. 4
        
    5.
    George EK, Oudesluys-Murphy AM, Madern GC, Cleyndert P, Blomjous JG. Inguinal hernias containing the uterus, fallopian tube, and ovary in premature female infants. J Pediatr 2000;136:696-8.  Back to cited text no. 5
        
    6.
    Ming YC, Luo CC, Chao HC, Chu SM. Inguinal hernia containing uterus and uterine adnexa in female infants: Report of two cases. Pediatr Neonatol 2011;52:103-5.  Back to cited text no. 6
        
    7.
    Turk E, Karagulle E, Oguz H, Toprak E. Indirect hernial sac containing the uterus, ovary, and fallopian tube in association with a giant intraabdominal lipoma: Report of a case. Hernia 2012;16:593-5.  Back to cited text no. 7
        
    8.
    Liu YB, Yan L, Zhou Y, Bi SY, Du YF, Zhang L, et al. [Female reproductive system dysplasia: A clinical study of 924 cases]. Zhonghua Fu Chan Ke Za Zhi 2019;54:166-72.  Back to cited text no. 8
        
    9.
    Griffin JE, Edwards C, Madden JD, Harrod MJ, Wilson JD. Congenital absence of the vagina. The Mayer-Rokitansky-Küster-Hauser syndrome. Ann Intern Med 1976;85:224-36.  Back to cited text no. 9
        
    10.
    Laufer MR. Congenital absence of the vagina: In search of the perfect solution. When, and by what technique, should a vagina be created? Curr Opin Obstet Gynecol 2002;14:441-4.  Back to cited text no. 10
        
    11.
    Rescorla FJ, Grosfeld JL. Inguinal hernia repair in the perinatal period and early infancy: Clinical considerations. J Pediatr Surg 1984;19:832-7.  Back to cited text no. 11
        

    Top
    Correspondence Address:
    Yingchang Zhu,
    Department of General Surgery, The Sixth Affiliated Hospital of South China University of Technology, 120 Guidance Road, Foshan 52800, Guangdong
    China
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    Source of Support: None, Conflict of Interest: None



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