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KMID : 0358819890160040653
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Journal of Korean Society of Plastic and Reconstructive Surgeons
1989 Volume.16 No. 4 p.653 ~ p.660
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CASES OF FEMALE PSEUDOHER-MAPHRODITISM DUE TO CONGENITAL ADRENOGENITAL SYNDROME
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Abstract
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Congenital adrenogenital syndrome, first described by (undefined) in 1865. is transmitted as an autosomal recessive trait with variable clinical penetrance. There is variety of enzymatic defects which result in decreased or absent production of cortisol and accumulation of androgeny steroid precurssors. The treatment is endocrinologic and surgical. The ideal goal in the surgical procedures is to establish a cosmetic appearance indistinguishable from that of a normal female subject and to preserve the sexually sensitive glands. We have experienced three cases of female pseudohermaphroditism combined with congenital adrenogenital syndrome in 2, 4 and 6 years old patients. We performed reduction clitoplasty consisted of subtotal resection of the gland preserving the dorsal nevrovascular bundle satisfactorily in all cases. There was no specific operative complication.
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KEYWORD
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