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KMID : 0377619640060040445
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Korean Jungang Medical Journal
1964 Volume.6 No. 4 p.445 ~ p.449
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A case of female pseudohermaphroditism
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裵炳胄/Bai, Byoung Choo
尹惠淑/白鏞舜/이진무/Yoon, Hae Sook/Paik, Yong Soon/Lee, Jin Moo
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Abstract
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An 18 years old young woman was visited to the Obs, & Gyn. department of the Seoul Red-Cross Hospital on June 7, 1963 with the chief complaints of a protruded penis like mass on her external genitalia and to determine her real sex. At birth, there was nothing to noticeable change on her external genitalia but clitoris was began to protrude from 5 years of her age, and then it grew up to 6 cm, in length and 2cm, in thickness, It seems just like a penis.
She hasn´t ever experienced menstruation and the breast was not developed, There was nothing to be noted on her past history and family history.
The patient was admitted on June 14, 1963 and physical examinations at admission revealed virilization, enlarged clitoris, urogenital sinus under the enlarged clitoris instead of vaginal and urethral orifice, and fused labia majora, By rectal examination a finger tip sized round mass was palpated at the site of uterus.
Urinary excretion of 17-ketosteroid was 22~23mg daily, Sex chromatin of oral mucous membrane was about 10%, Drumsticks of neutrophiles on peripheral blood smears were 7 to 9%. Oncystography the conical lumen was visible possibly indicating vaginal cavity which is communicating with triangular shadow.
Skull X-rays showed nomal shaped and sized Sella turcica indicating no evidence of pituitary tumor. According to the above findings exploratory laparotomy was performed on June 25, There are bilateral small ovaries with salpinx and a small uterus, but no tissues suggesting for testis were observed.
Biopsies of both ovaries and adrenal glands were done for pathological studies. Both ovaries revealed moderate number of premordal follicles and a few growing follicles, however no mature follicle with ovum was observed, and the histological findings of both ovaries were concluded as hypoplastic. The cortex of Rt. adrenal gland showed moderate degree of fatty degeneration and suggested to be atrophic.
The Lt. adrenal gland showed "diffuse cortical hyperplasia and was suggestion for adrenogenital syndrome".
Finally the patient was diagnosed as "a case of female pseudohermaphroditism with adrenogenital syndrome".
Plastic surgery of external genitalia was performed on July 24, and then steroid therapy was started on november 20. Oral administration of 10mg prednisolone per day was continued for 45 days.
The patient shows feminizing tendencies in general statue with developping Aping breasts and softer voice. We are planning to continue the steroid therapy for several months until the cessation of virilization and the achievement of feminization.
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KEYWORD
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