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编号:11307639
True Hermaphroditism
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     A 17-day-old black neonate presented with ambiguous genitalia. A sharp line separated hypopigmented skin on the left side of the abdomen from normally pigmented skin on the right side. A pendulous, wrinkled labioscrotal fold contained a gonad on the right side. In contrast, the hypoplastic, flat, left side was empty. A palpable left inguinal mass could not be clearly characterized with the use of ultrasonography. The phallus was 3 cm in length, with perineal hypospadias (inset) and 30-degree chordee. Testing revealed 46,XX/46,XY mosaicism and normal levels of male hormones. Laparoscopic surgery was performed; on the left side a hemiuterus, a fallopian tube, fimbriae, and an ovary were found and resected. These structures were absent on the right side. A normal vas exited the right internal ring, leading to a well-formed epididymis and a scrotal, biopsy-proven ovotestis with dotted islands of ovarian tissue capping the lower pole of the testicle. The ovarian tissue was removed, and left inguinal herniorrhaphy, placement of a left testicular prosthesis, and repair of the hypospadias were subsequently completed. At 21 months, the child has an excellent cosmetic result.

    Jose A. Karam, M.D.

    Linda A. Baker, M.D.

    University of Texas Southwestern Medical Center at Dallas

    Dallas, TX 75390