A case of vulval swelling secondary to female circumcision posing a diagnostic dilemma

Abstract

INTRODUCTION

The practice of FGM is most prevalent in the African countries such as Nigeria, Ethiopia, Sudan, Egypt, and some area of the Middle East. It is not restricted to any ethnic, religious or socioeconomic class. There are many reasons for perpetuation of this practice; the most common are cultural and religious beliefs.

The aim of this paper is to highlight the diagnostic dilemma associated with this type of case and the psychological trauma of a patient following her unfortunate genital mutilations.

PRESENTATION OF CASE

We present the case of epidermal inclusion dermoid cyst in an 18-year-old teenage girl referred to us from the gynecologist as a case of hydrocoele of the canal of Nuck involving the left labia majora. Patient was previously seen by general practitioner who diagnosed a left Bartholins cyst.

Excision of the mass, revealed a well encapsulated cystic mass containing serous fluid with no extension to the inguinal area, measured 10 cm × 8 cm. Histology showed epidermoid inclusion dermoid cyst probably related to circumcision (female genital mutilation).

DISCUSSION

Implantation dermoid cyst though a recognized complication of FGM is rare in our environment and a high index of suspicion is required any time a girl presents with a vulval swelling. Cosmesis still remains the watchword to assuage the psychological impact on the patient.

CONCLUSION

There is need for more public health campaigns to educate communities about the harms of circumcision with the goal of eradicating the practice.

1. Introduction

Female genital mutilation (FGM), which is inaccurately referred to as female circumcision by some people, has been practiced for centuries. Egyptian mummies were found to have been circumcised as far back as 200 B.C. In the 19th century it was practiced in Europe and North America as a remedy for ailments like epilepsy, hysteria, and masturbation.¹ The practice of FGM is most prevalent in the African countries such as Nigeria, Ethiopia, Sudan, Egypt, and some area of the Middle East. It is not restricted to any ethnic, religious or socioeconomic class. There are many reasons for perpetuation of this practice; the most common are cultural and religious beliefs. Although often associated with Islam, it is also practiced by other religious groups, including Christians.¹

The World Health Organization (WHO) clinical classifications¹ is as follows:

• •Type I—Clitoridectomy is the removal of prepuce and all or part of the clitoris. Also called Sunna Circumcision.^2–4 This is the least mutilating one.
• •Type II—The clitoris and part of the labia are excised and then sewn together by sutures, thorns, or tying the girl's legs together until the edges have united.
• •Type III—Infibulation (pharaonic) is the most extreme. Here the clitoris, labia minora are excised and incisions made in the labia majora to create raw surfaces that are then either stitched together or kept in close contact until they seal and form a cover for the urethral meatus. A very small orifice is left for the passage of urine and menstrual flow. Because this type is the most mutilating, the medical, obstetrical, and psychological complications are more profound. In many regions it is the most common procedure performed (e.g., in Djibouti and Somalia 98% of FGM are infibulations).^2,3,5

Complications following FGM may be immediate or late. The major immediate complications are hemorrhage from the dorsal artery, shock and then infection, urinary retention and tetanus, which can lead to mortality.^6–8

Some late and long-term complications seen are urinary incontinence, cysts, urogenital tract infections; severe dyspareunia, pelvic inflammatory disease, infertility, and obstetrical problems such as delayed or obstructed second stage labor, trauma, and hemorrhage.

We report a case of a young female adult with a histologically confirmed large vulval epidermal inclusion dermoid cyst following circumcision at birth. The cyst had started causing her discomfort while walking and made her a laughing stock amongst peer group. For this reason she became withdrawn, depressed, unwilling to have friends especially boys.

2. Case report

An 18-year-old teenage girl was referred to us from the gynaecologist as a case of hydrocoele of the canal of Nuck involving the left labia majora. She was previously seen by a general practitioner who diagnosed left Bartholins cyst. She noticed this swelling eight years prior to presentation as a tiny painless swelling, gradually increasing in size to occlude the whole introitus (Fig. 1) making her an object of ridicule by her peers. As a result she became withdrawn and was afraid to have friends especially boys. However she was able to pass urine and had normal menstrual flow.

Fig. 1.

Cyst occluding the introitus.

Further probing revealed she was delivered per vagina in a hospital but was circumcised a week after delivery by traditional birth attendants.

Father was late and mother is illiterate, a farmer. She is the fourth out of six girls and a boy. No similar problems in other girls even though they were all circumcised.

Examination revealed an unhappy but healthy looking teenager. Pulse rate was 76 beats per minute, blood pressure was 110 mmHg systolic and 70 mmHg diastolic. The other systems ere essentially normal except for the urogenital system which showed a left cystic mass involving the left labia majora and overlying the introitus, measured 10 cm × 8 cm, no discoloration of the overlying skin, non-tender, no differential warmth, soft in consistency, fluctuant but did not trans-illuminate light and it was not reducible. The remnant clitoris was stretched by the mass. Urethral orifice was present and there was normal vagina and intact hymen (Fig. 2).

Fig. 2.

A view of the intact urethral orifice and vagina.

A tentative diagnosis of hydrocoele of canal of Nuck was made. Investigations done include an ultrasound of the mass which revealed a left sided labial, non-septate, echorich cystic mass, 85 mm × 42 mm × 32 mm in size. Packed cell volume was 35%, urinalysis, urine culture and serum electrolytes, urea, creatinine were normal.

Operative technique and findings (Figs. 3 and 4).

Fig. 3.

Excised cyst.

Fig. 4.

Apperance of vulva immediately after cyst excision.

Under general anaesthesia and in lithotomy position, using an elliptical longitudinal incision, mass was excised by blunt and sharp dissection and use of diathermy. It was a well-encapsulated cystic mass containing serous fluid with no extension to the inguinal area, measured 10 cm × 8 cm. Redundant skin was trimmed and cavity obliterated. Vulval reconstruction was done using vicryl 3-0. Subcuticular closure of skin was done and a urethral catheter size 16 passed.

Wound dressing was removed after three days and patient commenced on sitz baths.

Postoperative recovery was uneventful and patient was elated at the final appearance of the vulva (Fig. 5). At follow up, her depression had disappeared as she was in very high spirits.

Fig. 5.

Final appearance of vulva few days after surgery.

Histology report confirmed an epidermal inclusion dermoid cyst (Fig. 6) (slide).

Fig. 6.

Histology slide of cyst showing keratinized epithelium.

It was at this point that the diagnosis was changed to epidermal inclusion dermoid cyst secondary to circumcision as there was no other evidence of trauma except during circumcision.

3. Discussion

The practice of female circumcision (female genital mutilation) was condemned by the WHO.⁹ An overwhelming factor for its justification is the cultural influence and traditions, social acceptance within the community ensuring chastity and fidelity by attenuating sexual desire.^10,11 This has made eradication difficult. A study done in Nigeria on the Igbo tribe showed that women believe that FGM makes them more feminine and thus more attractive to men.¹²

The complications of female genital mutilation continue to rear up its head continuously posing a challenge to medical practitioners. Most times, the physical complications are grappled with completely disregarding the psychosocial impact on the victim.

We have presented an 18-year-old girl who came to the hospital unaccompanied because of years of low self-esteem and depression. She was already turning into a social misfit and was unable to make or keep friends especially boys because she felt abnormal due to a discomforting swelling in between her legs.

Vulval epidermal inclusion dermoid cyst is a very rare entity in our environment and none as big as this patient's has ever been reported. This explained why there was a diagnostic dilemma initially. The gynaecologists ruled out a Bartholins cyst and referred to the surgeons as a case of hydrocoele of canal of Nuck. It was the histology report after surgical excision that confirmed the diagnosis of an epidermal inclusion dermoid cyst. Our patient had type I female circumcision in which the prepuce and part of the clitoris was removed but this was not immediately evident on physical examination because of the excessive stretching of the clitoral tissues by the sheer size of the cyst. This contributed to the diagnostic dilemma as it was on invitation of the mother that she volunteered that our patient was circumcised in the first week following birth.

Asante et al.¹³ in USA also reported a case of a 37-year-old female from Guinea with a large clitoral mass of 6 months duration confirmed in retrospect to be postcircumcision epidermal inclusion cyst. Dirie and Lindmark¹⁴ in Somalia in their series of 290 patients reported few with vulval swellings. All had circumcision at 18 years and above.

Hanly and Objeda⁸ in Saudi Arabia also reported six patients who presented with a large painless mass in the infibulation scar pathologically confirmed as implantation dermoid cysts. The only difference with our patient is that hers occurred from type I FGM while theirs was from type III (infibulation which is the most mutilating form of FGM). Circumcision was also done for most of the patients in these series at older ages.

Circumcisers may be skilled in traditional medicine, but their lack of training in surgery, their poor equipment and the fact that the girl may struggle, all suggest that it is difficult to be precise in an excision.¹⁵ For infibulation, the wound edges are closed with thorns or sutures, and the thighs may be bound together to fuse the labial edges, with a matchstick or twig inserted to ensure a patent vaginal foramen.¹⁴ These increase the likelihood of implantation dermoid cysts and other complications.

It is worthy to note that apart from circumcisions, blunt/penetrating trauma to the female genitalia may also be complicated by dermoid cysts. This was pointed out in a study done by Celik et al.¹⁶ who reported dermoid cyst in a 9-year-old girl following a blunt injury to her genitalia.

The aim of treatment in these patients is to excise the cyst and reconstruct a cosmetically acceptable vulva with normal urethral orifice and vagina. This was achieved in our patient as she was elated at the final outcome of the appearance of the vulva (Fig. 5).

4. Conclusion

Implantation dermoid cyst though a recognized complication of FGM is rare in our environment and a high index of suspicion is required any time a girl presents with a vulval swelling. Cosmesis still remains the watchword to assuage the psychological impact on the patient.

There is need for more public health campaigns to educate communities about the harms of circumcision with the goal of eradicating the practice.

Conflict of interest

There is no conflicts of interest.

Funding

None.

Ethical approval

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.

Author contributions

Amu Okwudili C.: lead author, responsible for study design, surgery and writing the paper. Udeh Ihechi E: assisted in the surgery and contributing to literature review. Ugochukwu Anthony I: contributed also in the literature review and proof reading of the manuscript. Madu Chukwudi C: a resident doctor who assisted in the surgery and did all the running around in paper work.