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

Abstract

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.