It is rare that surgeries will be needed in the neonatal period. Naturally, operations performed to end an imminent threat to the patient’s health should be scheduled as quickly as possible, and explained to the parents and, if of an appropriate age, to the child. Examples include operations to create a urinary opening where none exists in a newborn, or to remove malignant tissue.
Genital cosmetic surgeries are sometimes offered to relieve parental distress,[Crouch2004b] but parental distress should instead be addressed directly through peer support and competent mental health care. In providing this care for parents, teams show respect for parents and their children.[ Lee2002 Howe1998 ] A combination of reassurance and education will help to reduce the family’s early negative reactions to the condition while allowing them to honestly discuss their concerns and questions.[ Schober1998 Howe1998 ]
Past practice favored the use of surgery to reinforce initial gender assignment.[ Money1955 Money1972 Glassberg1998 Conte1989 Grumbach1992 ] This included operations aimed at making genitalia look more cosmetically normal and the removal of gonadal tissue at odds with the initial gender assignment. For the following reasons, the emerging approach[ Daaboul2001 Frader2004 Creighton2001 ] calls for delaying elective surgeries until the patients themselves can participate in decision-making:
Gender assignment is an imperfect art;[ Cohen-Kettenis2005 Meyer-Bahlburg1998 Zucker1999 Zucker2002 Dittmann1998 ] a small but significant number of patients with DSDs will develop a gender identity at odds with their initial gender assignment, and some will grow to feel and express nontraditional gender identities.[ Meyer-Bahlburg2005 Dessens2005 Mazur2005 ] It is best to let patients decide for themselves what anatomical features accord with their self identities. Professional counseling by a mental health professional can help patients make these decisions.[Zucker2005]
In both autobiographical accounts and outcome studies, a significant number of former patients have reported diminished sexual sensation, sexual dysfunction, or chronic pain following genital operations, including operations (e.g., “nerve-sparing”) thought by their advocates to be low risk.[ Zucker2004 Meyer-Bahlburg2004a Minto2003 Crouch2004 Creighton2004a Schober2004b Stikkelbroeck2003 Chase1996 Dreger1999 Chase1997 Randolph1981 Schober1998a Alizai1999 ] Surgery to construct a neovagina carries a risk of neoplasia.[Steiner2002 SchoberOutcomes] Because all surgeries carry risk, and because sexual sensation and function is vital not only to an individual’s enjoyment of sexuality but to his/her capacity for forming and maintaining intimate relationships and pair-bonds, it is preferable to allow patients to decide for themselves which risks to assume.
Operations designed to normalize genital appearance may undermine the multidisciplinary team’s central message to the parents that the child is unconditionally acceptable and lovable.[ Dreger1998 Chase1999 ]
There is a consistent and growing body of evidence that children raised with “ambiguous” sex anatomy are at no greater risk for psychosocial problems than the general population.[ Reilly1989 Young1937 Money1952 vanSeters1988 Dreger2002 Hawbecker1999 Chase1997 Reiner2003 Kim1999 Dreger1999b ] Meanwhile, there is surprisingly little published evidence to the contrary.[Beh2000] As a consequence, there is a lack of demonstrated need for early cosmetic genital surgeries. Interventions have tended to be based on fears about “worst case scenarios,” not demonstration of medical need.
Allowing a patient to make decisions about elective care signals to the patient a fundamental valuing of his or her autonomy and personhood.[AAPBioethics1995]
Healthy, functioning gonadal tissue should remain in place unless the patient, fully advised of risks and options, requests it be removed. Improving reproductive technologies may make it possible for patients now considered infertile (e.g., women with CAIS) someday to contribute to procreation (e.g., through sperm aspiration, IVF, and surrogacy). Removal of healthy gonadal tissue leads to loss of potential fertility and the loss of the benefits of endogenous hormones (e.g., prevention of osteoporosis; many women with CAIS report a loss of libido and sense of well-being after gonadectomy), and should therefore happen at the will of the fully informed patient. Note that gonadal tumor risk varies with etiology; the risk is highest in PAIS, and lowest (< 5%) in CAIS and ovotestes.[Cools2005 Ramani1993 Hannema2006]
There is a lack of agreement on the recommended age for various treatments and this inevitably influences the ability of affected children to participate in decision-making. A formal assessment of the child’s cognitive status by a child psychologist or psychiatrist can assist in determining the extent to which the child is capable of participating in the decision-making process.