Regarding the Consensus Statement on 21-Hydroxylase Deficiency from the Lawson Wilkins Pediatric Endocrine Society and The European Society for Paediatric Endocrinology

Sarah Creighton, Philip Ransley, Patrick Duffy, Duncan Wilcox, Imran Mushtaq, Peter Cuckow, Christopher Woodhouse, Catherine Minto, Naomi Crouch, Richard Stanhope, Ieuan Hughes, Mehul Dattani, Peter Hindmarsh, Caroline Brain, John Achermann, Gerard Conway, Lih Mei Liao, Angela Barnicoat and Les Perry

Clinicians from the Multidisciplinary Intersex Clinic at Great Ormond Street Hospital and University College London Hospitals, London, United Kingdom

Address correspondence to: Sarah Creighton, M.D., FRCOG, University College London Hospitals, Gynaecology Services, Elizabeth Garrett Anderson Hospital, Huntley Street, London WC1E 6DH, United Kingdom.

To the editor:

This consensus statement is a comprehensive review of a range of issues involving the management of 21-hydroxylase deficiency from before birth until adulthood. Many sections of this article are constructive and helpful, but the section on "surgical management and psychology" may be misleading and potentially detrimental to patient care.

The surgical management of ambiguous genitalia is controversial because few long-term follow-up data are available on the effects of surgery on sexual function and psychological outcome. There is increasing concern from intersex consumer groups about possible detrimental effects of genital surgery. Adult patients and parents of affected children should have a central role in this debate.

The authors list three goals of surgery (page 4050, first paragraph): 1) genital appearance compatible with gender; 2) unobstructed urinary emptying without incontinence or infections; and 3) good adult sexual and reproductive function.

1) The authors use the word gender but presumably mean sex of rearing as decided by the clinicians and parents. There is, to date, no evidence that surgery to render the genital appearance compatible with sex of rearing improves psychological or psychosexual outcome or promotes a stable gender identity.

2) Unobstructed urinary emptying without incontinence or infection is an important quality of life issue for the child. Until now clinical instinct that surgery is beneficial has led to early surgery, although there are no data to support this. Surgery itself can result in urinary infections and fistulae (1).

3) The final goal is good adult sexual and reproductive function. There is no evidence that reconstructive surgery gives a better outcome if performed in an infant rather than an adolescent. Clitoral surgery may not promote good sexual and reproductive function, and some studies suggest there may be damage to sexual function (2). Surgery performed in an infant may require revision in adolescence in a significant number of patients (3). An additional major advantage of surgery in an adolescent or adult is that informed consent can be obtained.

Early surgery may be the appropriate course of action and may or may not be supported by long-term outcome data in due course. For the moment, the apparently rigid guidelines in the consensus statement remove flexibility and potentially prejudice the possibility of constructive debate between specialties and with patient groups.

The only consensus attainable at the present time is that of a dedicated multidisciplinary team addressing an individual case including full participation of the affected family who will be responsible for the nurture of the child in the modern world.

Received January 27, 2003.

References

  1. Passerini Glazel G 1989 A new 1-stage procedure for clitorovaginoplasty in severely masculinized female pseudohermaphrodites. J Urol 142:565–568[Medline]
  2. May B, Boyle M, Grant D 1996 A comparitive study of sexual experiences. J Health Psychol 1:479–492
  3. Creighton SM, Minto CL, Steele SJ 2001 Objective cosmetic and anatomical outcomes at adolescence of feminising surgery for ambiguous genitalia done in childhood. Lancet 358:124–125[CrossRef][Medline]