The recent consensus conference addressed several aspects of the care of patients with Disorders of Sex Development (DSD). Among these were proposals regarding a new terminology to classify patients, medical, surgical and psychological care and the decision regarding sex of rearing or gender assignment for those with the most severe forms of genital ambiguity. However, a basis was found to be lacking for the assignment of sex of rearing for many diagnoses as well as for some for whom no diagnosis could be made. Also, no consensus could be made concerning genital surgery, particularly in response to the call to defer all genital surgery until the age a child could decide. This in part involved the lack of a clear indication of the point when a person has arrived at a state of maturity enabling such a decision. However, consensus was reached that genital surgery should be performed for the infant with significant ambiguity, in spite of the lack of outcome data, when the parents unequivocally desire. It was clear that desired outcome data will not be available for decades, if ever. Also, blatantly lacking are adequate data concerning the impact of prenatal androgen on the fetal central nervous system regarding subsequent development of gender and sexual issues.

1.
Baskin LS: Anatomical studies of the female genitalia: surgical reconstructive implications. J Pediatr Endocrinol Metab 17:581–587 (2004).
2.
Berenbaum SA, Bailey JM: Effects on gender identity of prenatal androgens and genital appearance: evidence from girls with congenital adrenal hyperplasia. J Clin Endocrinol Metab 88:1102–1106 (2003).
3.
Cohen-Kettenis PT: Gender change in 46,XY persons with 5 alpha-reductase-2 deficiency and 17 beta-hydroxysteroid dehydrogenase-3 deficiency. Arch Sex Behav 34:399–410 (2005).
4.
Colombia Court Decision: Text at website of The Intersex Society of North America, www.isna.org (1999).
5.
Consortium on the Management of Disorders of Sex Differentiation: Clinical guidelines for the management of disorders of sex development in childhood; www.dsdguidelines.org (2006).
6.
Creighton SM: Long-term outcome of feminization surgery: the London experience. BJU Int 93:44–46 (2004).
7.
Crouch NS, Minto CL, Laio LM, Woodhouse CR, Creighton SM: Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: a pilot study. BJU Int 93:135–138 (2004).
8.
Damiani D, Guerra-Júnior G: New definitions and classifications of the intersexual states: What the Chicago Consensus has contributed to the state of the art? Arq Bras Endocrinol Metabol 51:1013–1017 (2007).
9.
Dasgupta R, Schnitzer JJ, Hendren WH, Donahoe PK: Congenital adrenal hyperplasia: surgical considerations required to repair a 46,XX patient raised as a boy. J Pediatr Surg 38:1269–1273 (2003).
10.
Dreger AD, Chase C, Sousa A, Gruppuso PA, Frader J: Changing the nomenclature/taxonomy for intersex: A scientific and clinical rationale. J Ped Endocrinol Metab 18:729–733 (2005).
11.
Frader J, Alderson P, Asch A, Aspinall C, Davis D, et al: Health care professionals and intersex conditions. Arch Pediatr Adolesc Med 158:426–429 (2004).
12.
Himes M, Brook C, Conway GS: Androgen and psychosexual development: core gender identity, sexual orientation and recalled childhood gender role behavior in women and men with congenital adrenal hyperplasia (CAH). J Sex Res 41:75–81 (2004).
13.
Honecker F, Stoop H, de Krijger RR, Chris Lau YF, Bokemeyer C, Looijenga LH: Pathobiological implications of the expression of markers of testicular carcinoma in situ by fetal germ cells. J Pathol 203:849–857 (2004).
14.
Houk CP, Hughes IA, Ahmed SF, Lee PA, et al: Summary of consensus statement on intersex disorders and their management. International Intersex Consensus Conference. Pediatrics 118:753–757 (2006).
15.
Lee PA, Houk CP: Outcomes studies among men with micropenis. J Pediatr Endocrinol Metab 17:1043–1053 (2004).
16.
Lee PA, Houk CP, Ahmed SF, Hughes IA, et al: Consensus statement on management of intersex disorders. International Consensus Conference on Intersex. Pediatrics 118:e488–500 (2006), also Arch Dis Child 91:554–563 (2006).
17.
Long DN, Wisniewski AB, Migeon CJ: Gender role across development in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. J Pediatr Endocrinol Metab 17:1367–1373 (2004).
18.
Lumen N, Monstrey S, Selvaggi G, Ceulemans P, De Cuypere G, et al: Phalloplasty: a valuable treatment for males with penile insufficiency. Urology 71:272–276 (2008).
19.
Mazur T: Gender dysphoria and gender change in androgen insensitivity or micropenis. Arch Sex Behav 34:411–421 (2005).
20.
Mendonca BB, Inacio M, Costa EMF, Arnhold IJP, Russell DW, et al: Male pseudohermaphroditism due to 5alpha-reductase deficiency: outcome of a Brazilian cohort. Endocrinologist 13:201–204 (2003).
21.
Meyer-Bahlburg HF: Gender and sexuality in classic congenital adrenal hyperplasia. Endocrinol Metab Clin North Am 30:155–171 (2001).
22.
Meyer-Bahlburg HF: Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation. Arch Sex Behav 34:423–438 (2005).
23.
Meyer-Bahlburg HF, Dolezal C, Baker SW, Carlson AD, Obeid JS, New MI: Prenatal androgenization affects gender-related behavior but not gender identity in 5–12-year-old girls with congenital adrenal hyperplasia. Arch Sex Behav 33:97–104 (2004).
24.
Minto CL, Liao LM, Woodhouse CR, Ransley PG, Creighton SM: The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study. Lancet 361:1252–1257 (2003).
25.
Money J, Hampson JG, Hampson JL: Hermaphroditism: Recommendations concerning assignment of sex, change of sex, and psychologic management. Bull Johns Hopkins Hosp 97:284–300 (1955).
26.
Nordenstrom A, Servin A, Bohlin G, Larsson A, Wedell A: Sex-typed toy behavior correlates with the degree of prenatal androgen exposure assessed by CYP21 genotype in girls with congenital adrenal hyperplasia. J Clin Endocrinol Metab 87:5119–5124 (2002).
27.
Reiner WG, Gearhart JP: Discordant sexual identity in some genetic males with cloacal exstrophy assigned to female sex at birth. N Engl J Med 350:333–341 (2004).
28.
Reis E: Divergence or disorder? The politics of naming intersex. Perspect Biol Med 50:535–543 (2007).
29.
Woelfle J, Hoepffner W, Sippell WG, Brämswig JH, Heidemann P, et al: Complete virilization in congenital adrenal hyperplasia: clinical course, medical management and disease-related complications. Clin Endocrinol 56:231–238 (2002).
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.