Study of cases of 46, XX disorder of sex development [46, XX DSD] with assessment after their early surgical intervention

Background: Congenital Adrenal Hyperplasia [CAH] is the commonest cause of disorders of sex development [DSD] in children. The surgical management of children with CAH has always been difficult, subject to evolving attitudes and techniques, and at times controversial. The timing of surgery is one of the many factors that influence outcomes and is currently controversial

---

Patients and methods: Our study was conducted on patients of 46, XX DSD referred to pediatric endocrinology clinic in Alexandria University Children's Hospital. We studied the age of these patients at presentation, degree of civilization, presence of adrenal crisis, hormonal assay and radiological findings. We compared the early [done before the age of 2 years] versus late [done after the age of 2 years] surgical intervention done to these patients. We assessed these patients after at least six months of feminizing surgery, the assessment included the genital anatomical assessment, overall cosmetic results and further treatment recommendations

---

Results: In our study, there were 35 patients with early intervention and 26 patients with late intervention. Good cosmetic results were found more significantly in the early group [94.3%] in comparison to only 19.2% in the late group. Good cosmetic results [97.4%] are found more with the one stage operations

---

Conclusions: After doing the anatomical assessments, cosmetic results and assessing the need for further treatment recommendations, we can state that early feminizing genitoplasty has better anatomical and cosmetic results than late intervention