Résumé
La prise en charge clinique des anomalies du développement génitosexuel a évolué récemment en relation avec les progrès du suivi médical et les enjeux de nouvelles valeurs sociales. Cet article présente le rôle de plusieurs principes éthiques (neutralité intertemporelle, évitement de malfaisance, rôle du bien de la vie sexuelle, droit à l’avenir ouvert et importance de la justice morale). Il montre que la spécialisation exige un regroupement dans des centres qualifiés adaptés, et l’importance des délibérations participatives et pluridisciplinaires pour appliquer ces principes. Des exemples d’application sont indiqués pour la période anténatale ainsi que pour l’assignation de genre sexe et pour les indications chirurgicales en pédiatrie. Les modalités de décisions en cas d’incertitude complète sont aussi rappelées.
Abstract
The clinical management of children with disorders of sexual development has shown a recent evolution due to the improvement of clinical follow-up and to the pressure of new social values. This paper discusses the role of many ethical principles (intertemporal neutrality, avoidance of maleficence, promotion of future better sexual life, the right to open future and the importance of moral justice). It shows that the treatment of these rare and complex problems must be dealt in a few specialized medical centers and that a shared medical decision must be provided with a multidisciplinary approach. Some examples of application of the ethical principles are provided for three situations: the antenatal period, the attribution of sexual gender and the indications of surgical treatments. The rules of decisions in strict uncertainty are also mentioned.
Références
Bosinski HAG (2005) Psychosexuelle probleme bei Intersexsyndromen. Sexuologie 12:31–59
Sidgwick H (1967) Methods of ethics. Éditions MacMillan, Londres (Grande-Bretagne)
Arpaly N (2003) Unprincipled virtue. An inquiry into moral agency. Oxford University Press. New York, NY, USA
Landman J (1987) Regret: a theoretical and conceptual analysis. J. Theory social behavior. 17:135–60
Looingjenga LH, Hermus JW, Oosterhuis M, et al (2007) Tumor risks in disorders of sex development. Best Practice and Research 21:480–95
Lee P, Schober J, Nordenström A, et al (2012) Review of recent outcome data of disorders of sex development (DSD): emphasis on surgical and sexual outcomes. J Pediatr Urol 8:611–5
Zucker KJ, Bradley SJ (2005) Gender identity and psychosexual disorders. The Journal of Lifelong Learning in Psychiatry 3:598–617
Yang JH, Baskin LS, DiSandro M (2010). Gender identity in disorders of sex development: Review article. Urology 75:153–60
de Vries AL, Doreleijers TA, Cohen-Kettenis PT (2007) Disorders of sex development and gender identity outcome in adolescence and adulthood: understanding gender identity development and its clinical implications. Pediatr Endocrinol Rev 4:343–51
Veale JF, Clarke DE, Lomax TC (2010) Biological and psychosocial correlates of adult gender-variant identities: A review. Personality and individual differences 48:357–66
Meyer-Bahlburg HFL (2011) Gender monitoring and gender reassignment of children and adolscents with a somatic disorder of sex developement. Child Adolesc Psychitric Clin N Am 20:639–49
Reilly JM, Woodhouse CRJ (1989) Small penis and the male sexual role. J Urol 142:569–71
Kuhnle U, Krahl W (2002) The impact of culture on sex assignment and gender developement in intersex patients. Perspect Biol Med 45:85–103
Warne G, Bhatia V (2006) Intersex, east and west. In Systma S (ed). Ethics and intersex. Springer, Dordrecht, pp. 183–205
Slipjer FME, Drop SL, Molenaar JC, de Muinck Keizer-Schrama SM (1998) Long term psychological evaluation of intersex children. Arch Sex Behav 27:125–44
Schützmann K, Brinkmann L, Schacht M, Richter-Appelt H (2009) Psychological distress, self-harming behavior and suicidal tendencies in adults with disorders of sex developement. Arch Sex Behav 38:16–33
Warne G, Grover S, Huston J, et al (2005). A long term outcome study of intersex conditions. J Pediatr Endocrinol Metab 18:555–67
Creighton S, Alderson J, Brown S, Minto CL (2002) Medical photography: ethics, consent and the intersex patient. Br J Urol Int 89:67–72
Crissman HP, Warner L, Gardner M, et al (2011) Children with sex developement: a qualitative study of early parental experience. Int J Pediatr Endocrinol 2011:10
Hullman SE, Fedele DA, Wolfe-Christensen C, et al (2011) Differences in adjustement by child developmental stage among caregivers of children with disorders of sex development. Int J Ped Endocrinol 2011:e16–e22
Kirk KD, Fedele DA, Wolfe-Christensen C, et al (2011) Parenting characterics of female caregivers of children affected by chronic endocrine conditions: a comparison between disorders of sex developement and type 1 diabetes mellitus. J Pediatr Nursing 26:e29–e36
Bauman Z (1998) On postmodern use of sex. Theory, culture society 15:19–33
Kipnis K, Diamond M (1998) Pediatric ethics and the surgical assignment of sex. J Clin Ethics 9:398–404
Arlt W, Willis DS, Wild SH, et al (2010) Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients. J. Clin Endocrinol Metab 95:5110–21
Hagenfelt K, Janson PO, Holmdahl G, et al (2008). Fertility and pregnancy outcome in women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Hum Reprod 23:1607–617
Katz MD, Kligman I, Cai LQ, et al (1997) Paternity in intrauterine insemination with sperm from a man with 5a-reductase-2 deficiency. N Engl J Med 336:994–7
Gerharz EW, Eiser C, Woodhouse CR (2003) Current approaches to assessing the quality of life in children and adolescents. Brit J Urol Int 91:150–4
Johannsen TH, Ripa CPL, Mortensen EL, Main KM (2006). Quality of life in 70 women with disorders of sex development. Europ J Endocrinol 155:877–85
Schaeffer AJ, Yenokyan G, Alcorn K, et al (2012) Health-related quality of life in adolescents with bladder extrophy-epispadias as measured by the child health questionnaire-child form 87. J Urol 188:1924–9
Feinberg J (1992) The child’s right to an open future. In Joel Feinberg Freedom and Fulfillment. Philosphical essays. Princeton University Press, Princeton, New Jersey USA, pp. 76–97
Mills C (2003) The child’s right to an open future? J Social Philosophy 34:499–509
Gillam L, Hewitt JK, Warne GL (2012) Ethical principles for the management of children with disorders of sex development: a systematic approach for individual cases. In: JM Huston et al Disorders of Sex development. Springer-Verlag Berlin Heidelberg pp. 147–57
McCabe MA (1996) Involving children and adolescents in medical decision making: Developmental and clinical considerations. J Pediatr Psychol 21:505–16
Martin CL, Ruble DN (2010) Patterns of gender development. Ann Rev Psychol 61:353–81
McConaghy MJ (1980) The gender understanding of Swedish children. Child Psychiatry Hum Dev 11:19–32
Burnett S, Blakemore SJ (2009) The development of adolescent cognition. Ann New York Acad Science 1167:51–6
Resnick MD, Bearman PS, Blum RW, et al (2010) Protecting adolescents from harm. Findings of the National longitudinal study on adolescent health. JAMA 278: 823–32
Walzer M (1983) Spheres of justice. A defense of pluralism and equality. Basic books, New York, NY, USA pp. 136–46
Huston JM, Warne GL, Grover SR (2012) Short-, medium- and long term outcomes following surgery of disorders of sex development (DSD) at the Royal Children’s Hospital. In: JM Huston et al (editors). Disorders of sex development. Springer-Verlag. Berlin Heidelberg Allemagne pp. 265–77
Lind EA, Tyler TR (1988) The social psychology of procedural justice. Plenum Press, New York, États-Unis
Karkazis K, Tamar-Mattis A, Kon AA (2010) Genital surgery for disorders of sex development: implementing a shared decisionmaking approach. J Pediatr Endocrinol Metab 23:789–805
Mill JS (1990) De la liberté. Éditions Presses Pocket, pp. 47–105
Habermas J (1983) Morale et communication. Éditions du Cerf
Diekema DS (2004) Parental refusals of medical treatment: the harm principle as threshold for state intervention. Theor Med Bioeth 25:243–64
Chitty LS, Chatelain P, Wolffenbuttel KP, Aigrin Y (2012) Prenatal management of disorders of sex developement. J Pediatr Urol 8:576–84
Nihoul-Fekete C (2008) How to deal with congenital disorders of sex development in 2008. Europ J Pediatr Surg 18:364–7
Appiah KA (2005) The ethics of identity. Princeton University Press Princeton, New Jersey, États-Unis
van Ijzendoorn MH, Goldberg S, Kroonenberg PM, Frenkel OJ (1992) The relative effects of maternal and child problems on the quality of attachment: a meta-analysis in clinical samples. Child Development 63:840–58
Spriggs M, Savulescu J (2006) The ethics of surgically assigning sex for intersex children. In: D. Benabar (ed.) Cutting the core: exploring the ethics of contested surgeries. Rowman & Littlefield. Lantham (Grande Bretagne)
Callens N, van der Zwan YG, Drop SL, et al (2012) Do surgical interventions influence psychosocial and cosmetic outcomes in women with disorders of sex development? ISRN Endocrinol e1–e7
Creighton S, Chernausek SD, Romao R, et al (2012) Timing and nature of reconstructive surgery for disorders of sex development–Introduction. J Pediatr Urol 8:602–10
Luce RD, Raiffa H (1957) Games and decisions. John Wiley & Sons, New York, NY, États-Unis
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Sann, L., Et le Conseil d’éthique de l’hôpital Femme-Mère Enfant. Éthique et anomalies de développement génitosexuel. Rev. med. perinat. 7, 179–186 (2015). https://doi.org/10.1007/s12611-015-0328-6
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DOI: https://doi.org/10.1007/s12611-015-0328-6