Laparoscopy in the Surgical Treatment of Disorders of Sexual Development

Abstract

Introduction: Disorder of sexual development (DSD) is a rare condition. The surgical treatment of these patients includes investigation of the internal genitalia, evaluation of the gonads, and if necessary gonadectomy. The prevention of germ cell tumors is the most important issue in the surgical treatment of this varied and special group of patients. This study aimed to evaluate the role of laparoscopy in the surgical treatment of patients with DSD.

Materials and methods: Over a 4-year-period, all patients presenting with DSD who underwent laparoscopic surgery at our institution were retrospectively reviewed. Operative procedure, age at the time of surgery, and histopathological results were evaluated. In addition, karyotypes and phenotypes were investigated.

Results: Altogether, 12 patients undergoing 14 laparoscopic procedures were included. Median age at the time of surgery was 6 years with a range from 9 months to 17 years. Explorative laparoscopy was performed in all patients. In seven children, laparoscopic gonadectomy was necessary. Histopathologic examination revealed germ cell tumors in four children. In two patients, a gonadoblastoma was identified; in two patients, a dysgerminoma was found. Inguinal exploration was performed in four patients and led to removal of gonadal remnants in one case and gonadopexy in three cases. In two patients presenting with repeated urinary tract infections, laparoscopic removal of an utriculus was performed.

Conclusions: Laparoscopic gonadal biopsy, gonadopexy, and gonadectomy can be performed successfully, even in patients with germ cell tumors. To define guidelines for the surgical treatment of patients with DSD, further prospective and multicenter studies are necessary.