Laparoscopic Gonadectomy in Children with Disorders of Sex Development

Abstract

Abstract Background Gonadectomy is necessary due to the risk of malignancy in children with disorders of sex development (DSD) carrying Y chromosome. We aimed to investigate the efficacy of laparoscopic gonadal surgery in DSD patients. Methods Fourteen female phenotype patients carrying Y chromosomes with DSD who underwent laparoscopic gonadal surgery between February 2015 and December 2022 are retrospectively evaluated. Results The mean age of the patients at the time of surgery was 11.71 ± 6.4 years (1–18 years). The underlying disease was androgen insensitivity syndrome in four, 46,XY gonadal dysgenesis in three patients, lipoid congenital adrenal hyperplasia (CAH) in two, Leydig cell hypoplasia in two, 45,X/46,XY mixed gonadal dysgenesis in one, ovotesticular DSD in one, 46X, dic(Y) gonadal dysgenesis in one patient. The average operation time was 33 ± 2 (25–64) minutes. None of the cases were converted to open surgery. All patients were discharged on the first day. No postoperative complication is reported. The histological evaluation of the patients with lipoid CAH, Leydig cell hypoplasia, and androgen insensitivity syndrome revealed immature testis, ovotesticular DSD patients revealed ovotestis, 45XO/46XY mixed gonadal dysgenesis patients' and 46 X,dic(Y) mixed gonadal dysgenesis patients revealed ovarian tissue, 46,XY gonadal dysgenesis' patients revelead gonadoblastoma, immature testis on the right side and ovary on the left side and bilateral mesonephric remnants. Conclusions In Y chromosome carrying DSD patients, laparoscopic gonadectomy should be the preferred treatment method as an efficient, safe, and well-tolerated procedure, for gonads that are planned to be removed due to the risk of malignancy or non-compliance with the determined gender.