Laparoscopic gonad-sparing procedure in Ovotesticular disorder of sex development – Case video technique

Introduction

Ovotesticular disorder of sex development (OTD) is a rare condition. There’s a lack of literature addressing gonad-sparing surgery for OTD.

Objective

Report the laparoscopic partial gonadectomy technique – gonad-sparing surgery – in an 11-year-old child, 46, XX karyotype with OTD with atypical genitalia.

Material and Methods

After a complete diagnostic evaluation the patient underwent feminizing followed by laparoscopic partial gonadectomy (gonad-sparing surgery). The patient is positioned on supine position and Trendelenburg. One 5mm port is placed on the umbilicus and two 3mm ports in both flanks. A gonadal wedge biopsy was performed to achieve histopathological confirmation before resection. The testicular component of the ovotestis is clearly identified based on macroscopic aspects, and resected with laparoscopic scissors and limited use of electrocautery.

Discussion

This case was classified as bipolar or terminal ovotestis. At the 5-month follow-up, the patient attained menarche. No adverse outcomes recorded. Postoperative third year follow-up hormone evaluation revealing female pattern and ultrasound demonstrating uterine volume as well as bilateral ovarian tissue development with follicles.

Conclusions

Gonad-sparing procedure is feasible and desirable whenever possible, especially in 46, XX patients with female sex of rearing, since it preserves the fertility potential. The risk of malignancy must be monitored.

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