Male hermaphroditism may be defined as a process of incomplete masculinization of the internal or external genital organs or both in subjects with male gonads and male sex chromatic distribution.
In the differential diagnosis of genital ambiguity, the important points include history, physical examination. sex chromatin pattern, and determination of the urinary steriod excretion pattern, and also, of help in selected cases are endoscopy, X-ray contrast studies, laparotomy with gonadal biopsy and chromosomal analysis.
Cases of male hermaphroditism with ambiguous external genitals can be devided into those with absence of breast development which are usually called PPSH(Pseudovaginal Perineoscrotal Hypospadia) and those with breast development which are usually called incomplete or partial testicular feminization. The former is characterized by a phallus of intermediate size and a perineal urethral meatus, and blindly ending "vagina" which may poen either into the urethra or the perineum. A labia majora-like cleft scrotum frequently contains normally developed testes, and Wolffian-duct derivatives show normal male differentiation. Masculinization at puberty results in masculine body build, axillary, facial and pubic hair, and deepening of the vioce. Breast development is minimal or absent.
We have experienced recently a case of male hermaphroditism which was concerned to be a PPSH with the characteristics of external genitals, sex-chromatin pattern(sex-chromatin mass positive), chromosome study(karyotype 46XY) urinary steriod excretion (17-KS; normal value), laparoscopy(no Mullerian-duct structure), IVP (normal), and gonadal biopsy (atrophy of seminiferous tubule and decreased spermatogensis). Extragenital malformations were not detected and he was of normal intelligence.
His sex was assigned male, and treatment consists of urethroplasty and resection of urogenital sinus.
|