Sarah Creighton Consultant Gynaecologist,

Catherine Minto Research Fellow

Department of Gynaecology, University College London Hospitals.

TYPES OF CLITORAL SURGERY

There are three main groups of plastic clitoral procedures:

1. Clitorectomy or Clitoral Amputation

This operation simply removes all that can be seen of the clitoris (ie all of the glans clitoris), and usually involves dissection and partial removal of the corpora. Often the prepuce and clitoral hood will also be removed or used for reconstruction elsewhere. It is believed this procedure is now rarely done in the UK although no data are available.

2. Clitoral Recession

In this procedure, none of the clitoral structures are removed, instead the clitoral structures are dissected out and then folded up and moved in their entirety, backwards under the symphysis pubis. As in the clitoral reduction procedure, the bilateral dorsal clitoral nerves maintain their connection to the clitoral glans. This procedure was found to cause pain on clitoral engorgement and again it is presumed this procedure is no longer performed.

3. Clitoral Reduction

In this procedure the glans clitoris is preserved, and the corpora are dissected and partially or totally removed. Most procedures today will identify the two dorsal clitoral nerves and maintain their connection to the clitoral glans. Sometimes the clitoral glans will be reduced in size by wedge excisions, either laterally, ventrally or dorsally.

AIMS AND BENEFITS OF SURGERY

Once a female sex of rearing has been determined, current practice is to perform appropriate surgery to ensure the genitalia are "normal". The immediate aim is cosmetic. It is believed that normal looking female genitalia encourage a stable gender identity whilst reducing stigma and psychological distress thought to occur in children growing up with ambiguous genitalia. This practice is based on the work of John Money in the 1950s and 1960s. His premise was that infants are psychosexually neutral until the age of 2 years and that what is required for a stable normal gender identity is unambiguous genitalia and unequivocal assurance from parents at to the chosen gender.

There are no long term data to support this although there is a widely held belief that feminising genital surgery is "successful" both cosmetically and functionally. It is however impossible to predict the true gender identity or sexual preferences of any baby without an Intersex condition and even more difficult with Intersex children. It is most likely that gender identity has a multifactorial basis including anatomical, genetic and endocrine factors. The contribution of genital appearance to gender identity is unknown but men diagnosed with micropenis in infancy can remain happy with a male gender identity and have a male sexual role (Reilly and Woodhouse 1989).

There are no comparative data as yet published comparing women who have undergone surgery to women who have not had surgery. In the USA almost all babies with ambiguous genitalia have undergone genital surgery since the 1950s. However in the UK surgeons were initially reluctant to perform feminising surgery and there is existing a cohort of older patients who have not had genital surgery.

WHAT IS A LARGE CLITORIS?

There are standard measurements available for the average clitoris for a baby. However most clinical assessment of the genitalia is very subjective. The genital appearance changes dramatically at puberty with the deposition of labial and pubic fat and the arrival of pubic hair. What may appear as a large clitoris in a baby may look much less prominent in a teenage or adult. The majority of paediatric surgeons spend little time examining adult female genitalia and may misjudge potential clitoral size. There is a huge variation in size and anatomy of normal female genitalia. The patient herself may be entirely happy with a larger clitoris than average.

FOLLOW-UP DATA

While reports of operative techniques to reduce clitoral size have been numerous, there have been correspondingly few studies looking at outcomes of these surgical techniques. Reasons often put forward for this situation are that much of this surgery is performed on young infants by paediatric urologists and paediatric surgeons. These clinicians will not follow up these children regularly and will therefore not see the adult results of their surgery, either cosmetic or functional. The genitalia and sexual functioning are extremely delicate subjects that may be inappropriate or difficult for a paediatrician or paediatric surgeon to discuss with patients.

A literature review to look for functional outcomes of clitoral surgery has shown three groups of studies;

1. Small retrospective cohort studies or case reports performed by the original surgeon, with vague outcome parameters. These usually concentrate on surgical technique and most do not assess sexual function in any detail if at all. None of these studies is well reported, and all are subject to many biases.

2. Objective neurological assessment

Only one study has attempted to evaluate objective clitoral sensory innervation after surgery. Gearhart et al in 1995 performed pudendal evoked potentials in children before and after clitoral reduction. They used stimulation of the dorsal neurovascular bundle with unipolar electromyegraphic electrodes at the base of the clitoris and EMG response recorded at the tip of the clitoris. He demonstrated preservation of nerve conduction and claimed this may permit normal sexual function in adulthood. This is an inaccurate method to study sensation as it measures large myelinated fibres. Clitoral sensory information is carried in non-myelinated (C fibres) and small myelinated nerve fibres (Ad) which can only be assessed by temperature, vibration and light touch. These studies have not been done. In addition Chase (1996) stated that adult women who had undergone genitoplasty as children retained normal pudendal evoked potentials but had impaired sensation and orgasmic response.

3. Psychosexual function

Two studies have looked at psychosexual function in detail. Both have been published in psychology journals and have received little surgical exposure.

a. May et al (1996) looked at 19 women with congenital adrenal hyperplasia and compared this with a control group of 17 women with diabetes. Structured interviews were taped and analysed. The CAH group reported higher levels of penetration difficulties and a pattern of persistent pain during intercourse. They were less likely to masturbate and less likely to attain orgasm compared with the diabetic group (58.3% compared to 88%). The CAH group were doubtful about the success of surgery and worried about their genital appearance not being normal.

b. Dittman et al (1992) looked at 34 women with CAH compared to a control group of 14 sisters. The CAH women were less likely to experience orgasm with masturbation or intercourse. They were also less likely to be sexually active.

REPEAT PROCEDURES

Repeat clitoral reductions are common especially during adolescence. It is likely that if one procedure interferes with sexual function, then more procedures will do more harm. Repeat procedures are common in children with CAH with poor control or compliance.

WHAT DO THE PATIENTS WANT?

The difficulty with feminising surgery on babies is that the decision cannot be made by the patient. All parents want the best for their children and want them to be happy and "normal". Most clinicians at present believe normal looking genitalia are essential and are happy to recommend and advise surgery. Whilst parents want to feel able to make their own decisions, they look to the medical profession for guidance. We should be clear for whom we are doing the surgery i.e. clinicians and parents rather than the baby.

However adult patients are increasingly expressing dissatisfaction with the outcome of surgery. There is a growing campaign - especially in the USA - for a moratorium on feminising genital surgery. This has led to the establishment of NATFI (North American Taskforce on Intersex) and our current working party. Some clinicians are also questioning the need for cosmetic genital surgery (Schober 1998, 1999).

CURRENT WORK

At UCLH we are looking at long term follow up in adult Intersex womenincluding those who have undergone surgery and those who have not. Our study is a retrospective study comprising a standardised sexual function questionnaire (modified Golombok Rust Inventory of Sexual Satisfaction (GRISS)), full genital examination and full review of medical records including operative notes. So far 131 women have completed the questionnaire and 40 have been examined. Initial results comparing women with ambiguous genitalia during childhood who underwent surgery to those who didn't suggest a high level of sexual dysfunction in both groups. However the group who had had clitoral surgery were significantly worse off with 26% unable to achieve orgasm by any means (Minto et al 2001). We have also reviewed anatomical and cosmetic finding in adolescents following childhood surgery and have shown that 77% of children will require further major genital surgery during adolescence and adulthood (Creighton et al 2001).

CONCLUSIONS
1. Most data comes from women with CAH. There is no data looking at long-term result of women with other conditions causing virilisation. Research in all areas of sexual function in Intersex women is very scanty and should be encouraged.
2. Clitoral surgery has a detrimental effect on sexual function particularly on the ability to attain orgasm.
3. There are little long-term data to confirm or refute the benefit of "normalising" the genital appearance. Gender development is multifactorial and impossible to predict
4. The effect on female children of having severely virilised genitalia throughout childhood are unknown.

RECOMMENDATIONS.

1. Consultation with Intersex support groups. Seek opinions from adult patients who have undergone surgery and parents of children who have undergone surgery

2. All data on effects of sexual function should be discussed with the parents. The option to decline surgery must be discussed.

3. Clitoral Surgery should be avoided on mild and moderately virilised children.

4. Clitoral surgery on severely virilised children must be carefully discussed with all involved in the full understanding of effects on future. The possibility of deferring surgery should be discussed with the parents. The possible requirement for further revision surgery must be recognised.

REFERENCES

Chase C 1996 Letter of response J Urol 156:1139

Creighton S, Minto C, Steele S 2001 Cosmetic and anatomical outcomes following feminizing childhood surgery for ambiguous genitalia. Presented to N. American Society for Pediatric and Adolescent Gynaecology 2001. Accepted for publication in the Lancet - publication date July 2001

Dittman RW, Kappes ME, Kappes MH, 1992 Sexual behaviour in adolescent and adult females with congenital adrenal hyperplasia. Psychoneuroendocrinology Vol17 No2/3 pp153-70

Gearhart JP.,Burnett A.,Owens JH., 1995 Measurement of pudendal evoked potentials during feminising genitoplasty:technique and applications J Urol 153:486-7

May B.,Boyle M.,Grant D., 1996 A comparative study of sexual experiences. Journal of Health Psychology vol 1(4) 479-492

Minto C, Creighton S, Woodhouse C 2001 Long term sexual function in intersex conditions with ambiguous genitalia. Presented at British Assosciation of Urological Surgeons 2001 (submitted for publication).

Money J.,Hampson JG.,Hampson JL 1955 Hermaphroditism:recommendations concerning assignment of sex, change of sex and psychologic management. Bull John Hopkins Hosp 97:284-300

Money J.,Hampson JG.,Hampson JL., 1957 Imprinting and the establishment of gender role. Arch.Neurol.Psychiatry 77:333-336

Reilly J.,Woodhouse C., 1989 Small penis and the male sexual role. J.Urol 142:569-572

Schober JM 1998 Early feminizing genitoplasty or watchful waiting. J. Paedaitr. Adolesce.Gynecol. 11(3):154-6

Schober J. 1999 Longterm outcomes and changing attitudes to intersexuality. BJU International 83 Supppl3, 39-50

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