Images Of Pseudohermaphrodites

Figure 5.19. Micropenis with a normal scrotum in an infant who presented with severe hypoglycemia. This combination should alert one to the diagnosis of hypopituitarism. There is a higher incidence of hypopituitarism in patients with a variety of midline defects. Hypopituitarism was confirmed in this infant.

Figure 5.20. This female infant has ambiguous genitalia. The karyotype was XX. Difficulties in determining the sex of an infant may arise from abnormalities of the external genitalia. Ambiguous genitalia encompass a wide range of abnormalities having their origin before the 12th week of gestation. The phallus commonly shows hypospadias with chordee formation and appears large in proportion to the persisting labioscro-tal folds which may or may not contain gonads (testis, ovotestis, or rarely a well-defined ovary). In females, the labia may be fused and the clitoris hypertrophied. This condition is known as pseudohermaphroditism.

Figure 5.21. Lateral view of the same infant as in Figure 5.20 with ambiguous genitalia showing the marked clitoromegaly. Hermaphroditism (intersex) includes 1) true hermaphroditism, 2) female pseudo-hermaphroditism (virilizing adrenal hyperplasia), 3) male pseudohermaphroditism (the syndrome of incomplete testicular feminization; masculinization with 3-B-hydroxysteroid dehydrogenase deficiency), 4) pseudohermaphroditism in syndromes (feminizing adrenal tumors).

Images Pseudohermaphrodites
Figure 5.22. Ambiguous genitalia in a male pseudohermaphrodite widi a karyotype of XY. Note the rugae in the labioscrotal folds. Gonads were not palpable.

Figure 5.23. Another example of ambiguous genitalia in a male pseudohermaphrodite. Note the marked labioscrotal folds, absence of testes and presence of hypospadias.

Figure 5.24. This infant with ambiguous genitalia is an example of incomplete testicular feminization in that there are normal-appearing female genitalia except for clitoral hypertrophy and a sinus urogenitalis. Because of the swelling of both labia majora an inguinal hernia may be suspected. At surgery the hernial sac was found to include both testes; no uterus was present. Karyotype was XY.

Figure 5.24. This infant with ambiguous genitalia is an example of incomplete testicular feminization in that there are normal-appearing female genitalia except for clitoral hypertrophy and a sinus urogenitalis. Because of the swelling of both labia majora an inguinal hernia may be suspected. At surgery the hernial sac was found to include both testes; no uterus was present. Karyotype was XY.

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