The choice of feminizing genitoplasty tactic in girls with congenital adrenal hyperplasya

The choice of tactics for feminizing plastic surgery (one- or two-stage) remains an actually issue. To operate early, in the first months of a child’s life, when endocrinological stabilization is achieved, or to postpone the intervention until puberty? Despite the large number of approaches to surgical treatment, still not all results can be recognized as positive. There are complications that affect the quality of life of patients, not only of a surgical but also of a psychological nature. The advantages of one or another approach are currently impossible to prove, since this will require a prospective multicenter multidisciplinary study of long-term results with each of the approaches. Therefore, pediatric surgeons for a long time in the choice of tactics will be based on accumulated experience and logical reasoning. Given the extensive experience of multidisciplinary rehabilitation, the positive long-term results of two-stage feminization remains the preferred tactic for surgical treatment of girls with CAH. The increased risk of complications from the genitourinary system in the form of infections of the urinary tract, hematocolpos and hematometra in girls with a narrow common urogenital canal, which is inherent in virilization of 3, 4 and 5 degrees according to Prader, can be considered as a reason for an early one-stage feminizing plasty.

Long-term outcomes of the surgical treatment of girls with a classical form of congenital adrenal hyperplasia

Introduction. Currently, to study long-term outcomes of feminizing genitoplasty, which is performed to girls with congenital adrenal hyperplasia, is an important step due to the limited data in literature on this topic. The purpose was to assess long-term outcomes of feminizing genitoplasty in congenital adrenal hyperplasia in girls depending on their gender identity, sexual orientation and the anatomy of external genitalia and vagina.Material and methods. A retrospective analysis of 8 case–histories of girls who were operated on at the Arkhangelsk Regional Children’s Hospital in 2001-2004 was made. In 7 of them, who had low urogenital sinus, feminizing genitoplasty included vaginoplasty with a perineal flap; in one patient, who had a high urogenital sinus, the shortened vagina was advanced down to create a missing part with posterior skin (Fortunoff) and anterior mucous (Passerini) flaps. The Prader scores ranged from III to V. Mean age at the time of surgery was 5.6 y.o. (0.5-14). Mean follow-up period was 17.6 (16-18.5) years.Results. All patients had female gender identity. All four sexually active women reported heterosexual orientation. Cosmetic results were good as stated by physicians, parents and patients themselves. Vaginal stenosis was noted only in one patient.Conclusion. The results obtained have shown that the one-stage reconstruction (clitoroplasty and vaginoplasty) for congenital adrenal hyperplasia can be successfully performed in childhood; thus, there is no any reason to refuse of the accepted tactics in treating this group of patients.

New method for early evaluation of clitoris innervation using clitoro-perineal reflex after feminizing genitoplasty in early childhood: a pilot-study

A major complication of feminizing genitoplasty in children is the loss of clitoral sensation with serious impact at adult life. We suggest a new method to evaluate the surgical results during childhood based on the bulbocavernosus or clitoro-perineal reflex (CPR). The afferent pathway of CPR implies the intact sensory receptors on the clitoral glans. Girls with congenital adrenal hyperplasia who were followed-up medically without surgery or who underwent feminizing genitoplasty with or without clitoroplasty were included (2002–2018). All clitoroplasties were standardized reduction clitoroplasty with preservation of neurovascular bundles associated with vaginoplasty and vestibuloplasty. Standardized examinations were prospectively performed including the CPR starting at one year postoperatively. The reflex was triggered by gentle touch of the glans by a cotton swab. Contraction of the perineal muscles was considered positive. Thirty-two children were operated at a median age of 8.6 months (5.8–12.1). Median follow-up (FU) was 3.9 years (1.3–6.4). Twenty-four patients had clitoroplasties: 17 were tested for CPR at one-year FU, and all had a positive test. Eight girls had genitoplasty without clitoral surgery, two of them were tested and were positive. Ten patients were managed without surgery, two of them were tested for the CPR and were positive. The reflex was always triggered easily and repeated at least twice during the FU. The clitoro-perineal reflex is a simple, non-invasive and reproducible test in early childhood and may serve as an early evaluation tool of clitoral innervation after feminizing genitoplasty. These results need to be confirmed at long term and completed at adult life.