The November 2006 editorial about treatment of children born with ambiguous genitalia proceeds from a problematic premise, and goes on to imbue questions about elective genitoplasty with a rhetoric of optimism that could be misleading to distraught parents.
Dr. Rivkees argues that surgical ‘gender reversal’ no longer occurs because 46,XX infants with CAH are typically assigned as female regardless of virilization, and 46,XY infants with testes and microphallus are typically assigned as male. However, gender identity development in children born with atypical genitalia is poorly understood1. It is certain that neither chromosomes nor gonads decisively determine gender. Patients with complete or partial androgen insensitivity syndrome (AIS), for example, commonly develop a female gender identity; but not always2. Parents who are considering irreversibly altering their child’s genitals need to understand this uncertainty in order to make an informed decision.
Whether or not gender assignment carries a degree of certainty, however, the larger issue is whether it is acceptable, given the lack of evidence of any benefit, to subject infants to the loss of autonomy and degree of risk involved in any imposed surgeries. A significant number of people born with genital anomalies say it is not. This persistent critique distinguishes infant genitoplasty from surgeries to correct clubfoot or cleft palate.
While acknowledging that “we do not have long-term data related to contemporary genital restorative surgical techniques”, Dr. Rivkees asserts that “we are now in a bright era” in which it is “possible to achieve both favorable functional and favorable cosmetic outcome”. This assessment is not uniformly accepted, and cannot be taken for granted. As recently as 2006 the NIH stated that “there is currently a crisis in clinical management” of children with ambiguous genitalia due to uncertainly about both sex assignment and surgical outcomes1. Principles of informed consent require that parents know if the outcome of treatment is uncertain, yet many parents who consent to infant genitoplasty later report feeling that they were under-informed. Parents need to know that questions about the effects of surgery are more than ‘lingering’; that many experts now recommend against elective infant genitoplasty due to concerns that any incisions in the genital region may damage sensitivity; that modern surgical techniques were not developed in adherence to principles of evidence-based medicine; and that evidence of the likelihood of complications necessitating repeat surgeries is plentiful3. Particularly troubling is orchiectomy that often accompanies the management of those with AIS. While it is true that there is a potential for gonadal tumors the likelihood is less than that of breast cancer for woman, yet no one recommends prophylactic mastectomy. Watchful waiting is preferred4.
Parents also need to know that assumptions that surgery will prevent psychological harm from childhood teasing are unsupported by evidence, and that adults who have experienced genitoplasty in childhood have complained that multiple surgeries and the frequent genital examinations they necessitate also cause psychological harm3. Parents who discover such matters after the fact are likely to be dissatisfied.
Some points in the editorial’s legal analysis warrant clarification. While U.S. courts do generally “assume that parents know what is best for their children”, this presumption is not the final word. Many legal experts now believe that elective genitoplasty is a decision that only the patient can make5,6. The Colombian cases referenced in the editorial did not turn on the genotype of the children, nor did they affirm the traditional rights of parents to decide in such cases. Rather, the cases turned on the children’s age; and actually imposed new limitations on parental consent, intended to ensure that decisions are not made on the basis of parental discomfort or ignorance of alternate treatment models6. In America, too, parental discomfort is not a valid basis for surgery on a child. While a desire to relieve this discomfort is understandable, such relief should not come at the expense of giving complete information to the parents. Ultimately, this kind of surgical decision should involve the individual most concerned: the child, when mature enough to express an informed opinion.