羅東博愛醫院 外科部 泌尿科
One stage reconstruction in ambigeous genitalia in mixed gonadal dysgenesis：case report
Hueih-Shing Hsu, Shyh-Chyi Chang, Hsu-Hsiang Wang, Heng-Chang Chuang, I-Hung Shao
Division of Urology, Department of Surgery, Lotung Pohai Hospital Yilan, Taiwan
Case : This is a 17 months old child, physical examination showed asymmetry of scrotum with severe hypospadias. The child received bilateral hernia repair at 2 months old. The chromosome study showed 45XO and 46XY. Hormone study revealed 17-OH progesterone is 10.8 ng/ml (0.12-8.4), cortisol is 2.38 ug/ml (8-28), testosterone is 1.81 ng/ml (<0.2) renin is 117 IU/ml（7-76）, ACTH is 38.6 pg/ml (9-52). Image showed low entry of urogenital sinus. We performed clitoplasty and vaginoplaty in one stage. Laparoscopic gonadectomy for ovotestis and streak gonad was also done at the same time.
Discussion: The incidence of malignancy in streak gonad is 15-25% especially with Y chromosome exists. The child will be infertile and female gender is preferred. One stage reconstruction included gonadectomy, clitoplasty and vaginoplasty.