Abstract
In 1965, a botched circumcision left Bruce Reimer, a healthy, 8-month old XY male, with a disfigured penis. At the recommendation of Dr. John Money and physicians at Johns Hopkins, the infant was reassigned to female sex and underwent an orchiectomy and vaginoplasty. The family renamed the child “Brenda.” Unaware of her history, Brenda struggled with significant gender identity, psychological, and behavioral issues throughout her childhood and adolescence. When made aware of this history, she transitioned to male gender and assumed the name “David.” After years of psychological distress, David Reimer committed suicide in 2004. Despite the myriad lessons gleaned from this tragic story, medical and surgical management of children with atypical genitalia still remains often misguided, as providers continue to assume paternalistic roles in determining sex assignment and surgical interventions. A fifteen year old XY male with Robinow Syndrome presented for evaluation of hypogonadism and urinary incontinence. At birth, the patient was discovered to have a micropenis and perineal hypospadias and was diagnosed with hypogonadotropic hypogonadism. At the recommendation of the medical team, the infant underwent bilateral orchiectomy at eight months of age followed by urethroplasty and vaginoplasty at six years of age. The child was then given a female sex assignment. At twelve years of age, the child felt discordant from the sex of rearing and wished to be identified as male—his natal, genetic sex. He transitioned to male gender and began testosterone injections. He had history of recurrent UTIs and severe incontinence requiring diaper use. He strongly desired neophallus and urethral reconstruction for improved quality of life. The patient endorsed prior depression and desires to self-harm. He had significant concerns regarding his gender presentation and transition. He shared his difficulties in continuing in the same school system with peers who knew him as a female prior to transition and was concerned about peers knowing his medical history. In the years since the famous David Reimer case, the medical system has made tremendous strides in recognizing the need for patient autonomy and shared decision-making in patients with Differences of Sex Development and genital atypia. However, the paternalistic history of this field continues to leave its indelible mark more than 20 years since David Reimer’s case made headlines, as physicians continue to recommend definitive sex assignments and surgical interventions. As with the David Reimer case, the bodily integrity of this XY infant was altered in a permanent fashion with inadequate education of his family and little to no credence given to the autonomy of the child himself. We, as physicians, cannot continue to paternalistically apply John Money’s concept of gender neutrality and rigidly mandate sex assignments and early surgical interventions.
Penoscrotal Transposition to Achieve Urethral Continuity After Long-Segment Urethral Defect: A Case Report