From Journal of American College Health 49 no6 275-80 My 2001

ABSTRACT

More than 130 million women are subjected to genital mutilation. Despite increasing efforts to reduce the practice, there are many obstacles to eliminating this 2,000-year-old practice, which is based on strong cultural traditions. As college health clinicians provide care to more international students from countries where female genital mutilation is performed, increased awareness and knowledge of the procedure will enable clinicians to understand and manage its complications. We report a case of obstructive uropathy resulting in hydronephrosis secondary to female genital mutilation and review the medical literature regarding this and other complications of genital mutilation "surgery."

THE CASE

A 23-year-old Somalian woman whose chief complaint was lower abdominal pain was seen in a student health clinic. She also had a 3-year history of severe dysmenorrhea and heavy bleeding with her menses, which were regular and lasted about 4 to 5 days. Other symptoms included dysuria and prolonged micturation (about 15 minutes). She had no other medical problems, and her past medical history was significant for including genital mutilation when she was 5 years old.

On physical examination, she had mild lower abdominal tenderness, but no masses were palpable, and the medical examiner physician found no guarding or rebound. Inspection of her external genitalia revealed a midline fusion of the labia minora that completely obscured visualization of the vagina (Figure 1). Superiorly, a 5-mm tissue swelling was noted and identified as the clitoral bulb. The patient's urethral meatus was not visible. Two small openings were noted in the midline, and a 2-mm opening was identified below the clitoral bulb. Inferiorly, a 5-mm opening was identified at the forchette. Because of the labial fusion, a speculum and bimanual exam could not be done.

The rest of her physical exam was unremarkable. Laboratory evaluation revealed a microcytic, hypochromic anemia consistent with iron deficiency and normal urinalysis. An ultrasound examination demonstrated prevoid bladder enlargement (10 cm x 5.2 cm x 12 cm) and bilateral hydronephrosis (Figures 2 and 3). We discussed the results of the ultrasonography with the patient and recommended reconstruction of her perineal anatomy.

After discussing the matter with her fianc,, she declined to have the surgery. In her country, she explained, it is customary for the husband to "break" the scar during intercourse. Two weeks later, she married.

After several unsuccessful attempts at intercourse, she returned to the clinic and said that she and her husband had been unable to have intercourse because of her severe pain. Again, she was advised that it would be best to have the scar removed under general anesthesia. We discussed the situation with both the patient and her husband, scheduled surgery, and performed a perineoplasty without complications. The fused labia minora were separated and her external genitalia reconstructed (Figure 4). There was some residual shortening of the labia minora and the remaining structures appeared normal. We noted that she had an intact clitoris, which accounted for the 5-mm superiorly placed tissue swelling the physician identified on initial exam. Her original perineal anatomy was defined as type I female genital mutilation.

The patient's follow-up examination revealed well-healed, reconstructed perineal anatomy, and the follow-up ultrasound of the kidneys and bladder 5 months after the surgery showed resolution of the calicectasis in both kidneys (Figure 5 and 6). The patient was quite pleased with the results of the corrective procedure. Her micturation time was greatly reduced and she experienced no dysuria or dyspareunia.

COMMENT

An estimated 130 million women worldwide are subjected to female genital mutilation or "circumcision," with an additional 2 million new cases each year.(FN1) Most American physicians rarely encounter genital mutilation because most circumcised women live in Africa, the Middle East, and Southeast Asia.(FN2) However, because of the increased immigration that has occurred over the last 20 years, American physicians can expect to be exposed to this procedure with increasing frequency, and we need to be aware of it the associated complications.

Genital mutilation is classified into various subtypes (Figure 7).(FN3) Type I or sunna ("tradition" in Arabic), is the least extreme form of this procedure and consists of a subtotal clitoridectomy of varying degrees. It involves excision of the skin surrounding the clitoris with or without excision of part of or the entire clitoris.

Type II, referred to as excision, consists of clitoridectomy and, sometimes, removal of all or parts of the labia minora.

Type III, the most extreme form, is referred to as infibulation or pharaonic circumcision and consists of the removal of the entire clitoris and some or all of the labia minora. Incisions are made in the labia majora to create raw surfaces that are then stitched together to cover the urethra and vaginal introitus, leaving only a small posterior opening for urinary and menstrual flow. In Type III genital mutilation, the patient will have a firm band of tissue replacing the labia and obliteration of the urethra and vaginal openings. Medical complications are most commonly seen in women who have had infibulation.

Type IV genital mutilation includes different practices of variable severity, including pricking, piercing, or incision of the clitoris and/or labia; stretching of the clitoris and/or labia; cauterization of the clitoris; and scraping or introduction of corrosive substances into the vagina.(FN3-5)

Genital mutilation typically occurs when the female child is around the age of 7 years, but may be done at any time between infancy and puberty. For the family, the young girl's infibulation ceremony is considered a time for celebration. A midwife, village woman, or gypsy often performs the procedure for a fee. Anesthesia is rarely provided and the instruments that are used to perform the procedure may include razor blades, kitchen knives, scissors, broken glass, a burning piece of wood or coal, or other crude tools.

Once the procedure is completed, the raw edges of the vulva may be pasted together with gum arabic, sugar, or egg. Sometimes the vulvar edges are pinned together with long acacia thorns, but surgical materials such as silk or catgut sutures are rarely used.

In type III genital mutilation, the small opening is kept patent by inserting a small piece of wood or matchstick to allow the passage of urine and menstrual blood. In this most severe form, the young girl is often bound from her hip to her ankle for up to 40 days so that scar tissue will form.(FN4,6) This ordeal was graphically described by Waris Dirie, who is now a model living in the United States. Dirie was born in Somalia and was approximately 7 years old when a "gypsy woman" or killer woman, as she is referred to in the author's description, performed the infibulation. The following is an excerpt from her book, Desert Flower: The Extraordinary Journey of a Desert Nomad:

"The next thing I felt was my flesh... being cut away. I heard the sound of the dull blade sawing back and forth through my skin. When I think back, I honestly can't believe that this happened to me. I just sat there as if I were made of stone, telling myself the more I moved around, the longer the torture would take. Unfortunately, my legs began to quiver of their own accord, and shake uncontrollably, and I prayed, Please, God, let it be over quickly. Soon it was, because I passed out.

When I woke up, I thought we were finished, but now the worst of it had just begun. My blindfold was off and I saw the Killer Woman had piled next to her a stack of thorns from an acacia tree. She used these to puncture holes in my skin, then poked a strong white thread through the holes to sew me up. My legs were completely numb, but the pain between them was so intense that I wished I would die.

My memory ends at that instant, until I opened my eyes and the woman was gone. My legs had been tied together with strips of cloth binding me from my ankles to my hips so I couldn't move. I turned my head toward the rock; it was drenched with blood as if an animal had been slaughtered there. Pieces of my meat... lay on top, drying undisturbed in the sun."(FN7(pp 42-43)*)

Although some upper-class families may have the opportunity to have the procedure performed in a hospital with sophisticated medical equipment, the vast majority of women are subject to primitive tools and nonsterile technique. Religious and cultural factors seem to propagate the tradition of female genital mutilation, despite the suffering and risks associated with the procedure.

Many religious and cultural issues foster female genital mutilation. Although some proponents of the practice claim that it is required by Islam, there is no specific support for this claim in the Koran.(FN1) From a cultural perspective, women look at the operation as something they do "for" their daughters instead of "to" them, as a means of securing their economic and social future through marriage.(FN4) The genital scar provides proof for the prospective husband that a woman has been trained in moral and practical responsibilities and has maintained her virginity.(FN6)

Uncircumcised women are stigmatized and considered unsuitable for marriage.(FN1) Therefore, parents are willing to incur great expense for the procedure to ensure their daughters' desirability as a marriage partner. Parents are often unaware of the potential medical risks, physiologic consequences, and psychological effects of the procedure.(FN3)

The psychological consequences are varied and not well understood. Many of the adverse emotional effects relate to the woman's traumatic memories of the procedure, especially during intercourse.(FN8) Women often experience painful menses and micturation; fear of the first intercourse is common among infibulated women. Dyspareunia may occur from difficult penetration, scarring, and mental trauma. Anxiety and phobic behavior may occur as some women psychologically experience the pain and trauma of the procedure and its complications over again and again during intercourse.(FN8)

In Waris Dirie's book, she relays the uncomfortable experiences of prolonged urination and excruciating menses that would incapacitate her for 7 to 10 days each month. She also shares the feeling of freedom that she experienced after she was surgically deinfibulated.(FN6)

Although many suffer the severe psychological consequences of the experience, some women say that experiencing the procedure is a source of pride and belonging. Thus, the victims of the practice are sometimes its strongest proponents.(FN2) It is difficult to make generalizations concerning the psychological impact of the procedure.

Whether or not adverse psychological consequences are evident, the medical risks and consequences cannot be ignored. The medical complications can be categorized into acute, intermediate, and chronic levels. Approximately 30% of the women with type II or III genital mutilation suffer from some form of intermediate or chronic complication, and approximately 15% of those with type I genital mutilation experience recurrent urinary tract infections and pelvic inflammatory disease.(FN5) Immediately after the procedure, the young girl is at risk for infection, hemorrhagic shock, septicemia, tetanus, gangrene, or death. Later complications include delayed wound healing, anemia, malnutrition, necrotizing fasciitis, pelvic inflammatory disease, dysmenorrhea, vulvar cysts and abscesses, keloid formation, neuromas, adhesions, dermoid cysts, and a number of other sequelae.(FN3-6,8)

The chronic complications include slow urinary stream, dribbling, incontinence, and urinary calculi. When a blind pouch is created around the urethra and vagina by infibulation or another subtype that involves labial fusion, frequent vaginal and urinary tract infections may occur. In addition, a narrow introitus may obstruct passage of menstrual blood and result in dysmenorrhea and hematocolpos. Rectovaginal fistulas, dyspareunia, and vaginismus also occur. For the victims, the risk of infertility is increased, in part as a result of chronic pelvic infections and obstruction to intercourse.

Furthermore, a number of accounts of reduced sensitivity as a result of the destruction of the vulvar nerve endings have been reported. This seems to reduce the female libido and ability to achieve orgasm. When the woman does become pregnant, obstetrical complications are common and morbidity and mortality are increased for both the mother and child during childbirth.(FN3-6,8)

Transmission of the human immunodeficiency virus (HIV) during female genital mutilation is another serious concern. The use of nonsterile and, possibly, shared instruments for the procedure is a definite possibility, given the circumstances under which the procedure is carried out. The connection between transmission of HIV and female genital mutilation has never been documented, yet the risk exists in a region such as sub-Saharan Africa, where the prevalence of HIV in the population is 20% in some areas. In 1998, 1.8 million people died from HIV infection in this region, a number that is almost double the number of deaths from malaria and eight times the deaths from tuberculosis.(FN9) Because of the high prevalence of HIV in adults and, subsequently, congenital or acquired infection during childhood, the risk and concern for transmission of HIV is real.

Our patient, with type I genital mutilation that included labial fusion, experienced a number of the complications often associated with infibulation, including dysmenorrhea, dysuria, prolonged micturation, and dyspareunia. In addition to these symptoms, our patient had radiologic evidence of urinary outflow obstruction (hydronephrosis). Although hydronephrosis occurs frequently in children as a consequence of vesicoureteroreflux or posterior uretheral valves, it is uncommon in adolescents and young adults.

In a review of the literature, we found observations of urinary retention and outflow obstruction in women with genital mutilation, but no reports of hydronephrosis resulting from infibulation. After relief of the obstruction, our patient's hydronephrosis resolved without any apparent permanent sequelae. However, the problem may be more common than we recognize, and the long-term complications of hydronephrosis resulting from genital mutilation are yet to be determined.

Although education, public awareness, and legal action have begun to reduce the number of women subjected to genital mutilation, the prevalence is still high and many girls continue to suffer the consequences of such "surgery."(FN10,11) Eradicating a practice that is based on cultural and traditional patterns that have existed for more than 2,000 years is a complex and delicate process.(FN2) Until young girls are no longer subjected to this procedure, all physicians must be informed about the various types of genital mutilation so that they can manage the psychologic and physiologic consequences of the procedure, including hydronephrosis.

NOTE

The patient whose case is outlined in this article gave her permission for use of the pictures and description of her examination and surgery. Some details have been changed to protect her anonymity.

FIGURES

FIGURE 1 Female Genital Mutilation, Showing a Midline Fusion of the Labia Minora That Obscures Visualization of the Urethral and Vaginal Introitus
FIGURE 2 Renal Ultrasound of the Left Kidney Prior to Surgery, Showing Mild Dilatation of the Upper Pole Calix (Arrow)
FIGURE 3 Renal Ultrasound of the Right Kidney Prior to Surgery, Showing Mild Calicectasis (Arrow)
FIGURE 4 Following Perineoplasty, the Urethral (Thick Arrow) and Vaginal (Thin Arrow) Introitus Are Now Discernible
FIGURE 5 Renal Ultrasound of the Left Kidney 5 Months After Perineoplasty, Showing No Calicectasis (Normal Kidney)
FIGURE 6 Renal Ultrasound of the Right Kidney 5 Months After Perineoplasty, Showing No Calicectasis (Normal Kidney)
FIGURE 7 Female Genital Anatomy, Showing Types of Genital Mutilation

FOOTNOTES

1. Douglas JH, Female circumcision: Persistence amid conflict. Health Care for Women International. 1998; 19:477-479.
2. World Health Organization, International Federation of Gynecology and Obstetrics. Female circumcision: Female genital mutilation. International Journal of Gynaecology & Obstetrics. 1992;37:149.
3. American Academy of Pediatrics: Committee on Bioethics. Female genital mutilation. Pediatrics. 1998;102(1):153-156.
4. Council on Scientific Affairs, American Medical Association. Female genital mutilation. JAMA. 1995;274:1714-1716.
5. Walker LR, Morgan MC. Female circumcision: A report of four adolescents. J Adolesc Health. 1995;17:128-132.
6. Elchalal U, Ben-Ami B, Brzezinski A. Female circumcision: The peril remains. BJU International. 1999;83 (suppl 1):103-108.
7. Dirie W, Miller C. Desert Flower: The Extraordinary Journey of a Desert Nomad. New York: William Morrow; 1998.
8. Horowitz CR, Jackson JC. Female "circumcision": African women confront American medicine. J Gen Intern Med. 1997; 12:491-499.
9. Watts C, Kumaranayake L. Thinking big: Scaling-up HIV-1 interventions in sub-Saharan Africa [Commentary]. Lancet. 1999;354:1492.
10. Research Action and Information Network for Bodily Integrity of Women (RAINB[Graphic Character Omitted]) at www.rainbo.org (includes access to a "Quick Reference Chart" on female genital mutilation, in addition to other references concerning the procedure). Accessed Feb 2001.
11. American College of Obsteticians and Gynecologists (ACOG) and the Society of Obstetricians and Gynaecologists of Canada (SOGC). Female Circumcision/Female Genital Mutilation: Clinical Management of Circumcised Women (Slide Lecture Kit). For further information, contact ACOG at (800) 673-8444 or www.acog.org.