Laparoscopic gonadectomy of streak gonads within the inguinal canals in a patient with XY partial gonadal dysgenesis

Abstract

A 33-year-old phenotypic female presented with primary amenorrhea. The physical examination revealed no breast development, scanty axillary and pubic hair, normal female external genitalia, and a short blind vagina. The findings of magnetic resonance imaging, karyotyping, and hormonal examinations supported the diagnosis of XY partial gonadal dysgenesis. Laparoscopic surgery showed that spermatic cords, including the bilateral streak gonads and epididymes, were within the inguinal canals. After opening the spermatic cords and identifying the lower borders of the streak gonads, the spermatic cords were transected at the level of the vas deferens. A prophylactic herniorrhaphy using polyprophylene mesh was performed due to weakening of the internal inguinal ring. Pathologic evaluation of the gonads confi rmed the diagnosis of partial gonadal dysgenesis without evidence of malignancy. A laparoscopic approach is feasible for treatment of XY gonadal dysgenesis, even if the streak gonads are present within the inguinal canal. Th is is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Keywords Copyright © 2012. Korean Society of Obstetrics and GynecologyThe condition termed 46,XY complete gonadal dysgenesis, also known as 'Swyer's syndrome', is characterized by the lack of testicular determination with resulting streak gonads, normal Mullerian structures, and female external genitalia. Because of incomplete testicular determination in the patient with 46,XY partial gonadal dysgenesis, mullerian structures may be present or absent, and the external genitalia may be female, ambiguous, or male [1]. The increased potential for gonadal malignancy in patients with XY gonadal dysgenesis is well-documented [2]. Therefore, prophylactic gonadectomy is indicated soon after diagnosis. In Swyer's syndrome, because the streak gonads are located in the normal adnexal location adjacent to the uterus, a laparoscopic gonadectomy can be easily performed by experienced gynecologists [3][4][5]. However, if streak gonads are located in unusual sites, especially in inguinal canals, it is not easy to identify the streak gonads because the gonads are thin and small. We present our experience with the laparoscopic removal of streak gonads within the inguinal canals in a patient with XY partial gonadal dysgenesis. Case ReportA 33-year-old woman (height, 168 cm; weight, 60 kg) presented with primary amenorrhea. The physical examination revealed no breast development (Tanner stage I), scanty axillary and pubic hair (Tanner stage II), and normal female external genitalia. The gynecologic examination revealed a short blind vaginal pouch CASE REPORT Korean J Obstet Gynecol 2012;55(3):209-212 http://dx