Although the rates of forced genital cutting have been on the decline in North America since the 1970s, it does persist, with an estimate of one to two intersex infants or children per 1,000 live births receiving non-therapeutic genital surgery [Blackless et al., 2000].
While a wealth of medical, legal, and ethical discussion from a North American perspective has been gathered against the practice of forced female genital cutting, and has shifted one aspect of circumcisionism, we have been slower to tackle forced male and intersex genital cutting practices. In 2008, research was published which concluded that feminizing genital surgery performed on children led to significant sensitivity impairment as well as sexual dysfunction [Crouch, Liao, Woodhouse, Conway, & Creighton, 2008]. Combined with the calls to end forced gender reassignment surgery based on poor outcomes and patient advocacy groups which began in the 1990s, there has been a recent shift in the assumptions about forced intersex genital cutting.
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, [Crouch, 2004b] 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. [Lee, 2002, Howe, 1998] 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.[Schober, 1998, Howe, 1998]
Past practice favored the use of surgery to reinforce initial gender assignment. [Money, 1955, Money, 1972, Glassberg, 1998, Conte, 1989, Grumbach, 1992] 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 [Daaboul, 2001, Frader, 2004, Creighton, 2001] calls for delaying elective surgeries until the patients themselves can participate in decision-making.
Gender assignment is an imperfect art; [Cohen-Kettenis, 2005, Meyer-Bahlburg, 1998, Zucker, 1999, Zucker, 2002, Dittmann, 1998] a small but significant number of patients with DSD will develop a gender identity at odds with their initial gender assignment, and some will grow to feel and express nontraditional gender identities. [Meyer-Bahlburg, 2005, Dessens, 2005, Mazur, 2005] 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. [Zucker, 2005]
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. [Zucker, 2004, Meyer-Bahlburg, 2004a, Minto, 2003, Crouch, 2004, Creighton, 2004a, Schober, 2004b, Stikkelbroeck, 2003, Chase, 1996, Dreger, 1999, Chase, 1997, Randolph, 1981, Schober, 1998a, Alizai, 1999] Surgery to construct a neovagina carries a risk of neoplasia.[Steiner, 2002, Schober Outcomes] 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. [Dreger, 1998, Chase, 1999]
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. [Reilly, 1989, Young, 1937, Money, 1952, vanSeters, 1988, Dreger, 2002, Hawbecker, 1999, Chase, 1997, Reiner, 2003, Kim, 1999, Dreger, 1999b] Meanwhile, there is surprisingly little published evidence to the contrary. [Beh, 2000] 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. [AAP Bioethics, 1995]
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.[Cools,2005, Ramani,1993, Hannema, 2006]
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.
HELPING PARENTS THINK ABOUT ELECTIVE SURGERY
Parents pick up signals from their children’s pediatricians about what they should do, sometimes picking up signals pediatricians don’t intend. [Dayner, 2004, Hester, 2004] The best thing pediatricians can do is to be explicit with parents (and, as age appropriate, patients) about the choices available and the evidence regarding outcomes for each.
If there’s no urgent need for surgery—as is the case for many DSD surgeries—clinicians can make vivid the option of waiting by introducing the family to adults who have lived without the intervention being considered and to other parents who have decided to hold off and let the child decide. So, when working with parents considering clitoral reduction surgery for their infant daughter, one pediatric urologist has introduced those parents to another set of parents who chose against infant clitoral reduction surgery—and to the daughter herself (now an adolescent). This way the parents facing a decision have had the opportunity to meet a child who will make the elective surgery decision for herself, and to learn from her and her parents how to manage the psychosocial challenges of raising a girl with a noticeable clitoris.
Pediatric urologists and surgeons know well that sometimes parents seek early elective surgeries for their child with a DSD because they think it will spare the child the psychological trauma of having them done later. This is the case sometimes, for example, with infant orchidectomies chosen on behalf of girls with Complete Androgen Insensitivity Syndome. But many adults with DSD have said that they were not spared the psychological trauma of the surgery because it hit them full-on when they came to know about the surgery. Some, in fact, have felt the psychological trauma of surgery was amplified by having had surgeries done without their consent. This in turn increased their sense of betrayal and disenfranchisement, an outcome unintended by their parents and doctors, who made the decision with the best of intentions.