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Embryonic Testicular Regression Syndrome : A Case Report

MANEE RATTANACHAIYANONT, M.D.*, KITIRAT TECHA TRAISAK, M.D., Ph.D.*, PHAIBUL JITPRAPHAI, M.D.*** PHAKPHUM PHOPHONG, M.D.* PICHAI CHAROENPANICH, M.D.**,

Affiliation : * Division of Reproductive Endocrinology, * * Division of Pathology, Department of Obstetrics and Gynaecology, *** Division of Urosurgery, Department of Surgery, Faculty of Medicine, Siriraj Hospital, Mahidol University. Bangkok 10700, Thailand.

Abstract
A case of testicular regression syndrome was reported. The patient was an 18 year old girl presenting with primary amenorrhoea. Physical examination revealed normal female external genitalia and underdeveloped secondary sexual characteristics. Hormonal profile indicated gonadal failure. Chromosome analysis revealed 46,XY karyotype. Diagnostic laparoscopy demomstrated undeveloped internal genital organs. Remnants of epididymis, vas deferens and seminiferous tubule were uncovered during exploratory laparotomy. Ontogeny of sexual differentiation and pathoge- nesis of testicular regression syndrome were reviewed and discussed.

Keywords : Hermaphrodite, Testicular Regression


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