Re: Editorial: It is (sort of) a boy and (sort of) a girl. You have (sort of) a say and you (sort of) don't? The uneasiness of genital restoration surgery [JPEM 2006(11); 19: 1285-1289].

To the Editor: As a physician and mother of two young adult daughters with complete androgen insensitivity syndrome (AIS), I have counseled a group of 50 parents of children with AIS and related disorders of sex development (DSDs) for 8 years. These parents consistently express frustration over the lack of evidence from which to make choices for the treatment of their children. Decisions like the advisability of genitoplasty are fraught with anxiety because reliable outcome data are scant. The recent international consensus statement detailing a new patient-centered standard of care for DSDs laments this lack of research 1,2. In his editorial, Dr. Rivkees advocates for urogenital sinus repair as " necessary to open up a covered vaginal orifice for menstruation and sexual intercourse ". Genitoplasty is a separate, elective procedure that is not part of routine urogenital sinus repair. Dr. Rivkees describes operations performed on children with cleft palate and clubfoot as analogous to genitoplasty. Ambiguous genitalia, unlike facial and orthopedic anomalies, are not constantly visible differences. While craniofacial and orthopedic surgery can improve feeding and ambulation, Dr. Rivkees does not reveal what function is restored by genitoplasty. In supporting the performance of genitoplasty, Dr. Rivkees cites the development of neuro-vascular-sparing techniques, based on principles identical to those used to preserve potency following prostatectomy. The sexual function of men who have undergone nerve-sparing prostatec-tomy is well-studied. Despite continued surgical refinements, results consistently fail to meet expectations. Recent data show that even when skilled surgeons at a large tertiary-care academic center performed anatomically-based, nerve-sparing prostatectomy, only 62% of patients regained full potency at 2 years of follow-up 3. Similar scientific evidence regarding the retention of adult sexual function after genitoplasty is absent. Conversely, techniques purporting to preserve the neurovascular structures during genitoplasty have unknown effects on at least two components of sexual function, genital sensitivity and erectile tissues. While genital sensitivity is subjectively evaluated during follow-up of genitoplasty, function of the erectile tissue is not. Reduced vascularity of erectile tissue is a cause of disorders of arousal and orgasm in women. Recent functional MRI studies show a previously unsuspected inter-connectedness among the clitoris, labia minora, urethra, and clitoral bulb, including the trabecular tissue, during sexual arousal 4. Feminizing genito-plasty inevitably disrupts the connections between these sexually-responsive erectile tissues. The functional consequence of these disruptions can be assessed using MRI of patients post-genitoplasty to determine whether actual blood flow in the genital …