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Pelvis » Female Pelvis (Gynecology)
Intersex syndrome, male pseudo-hermaphroditism
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Author(s) :
S. Manohar, MD, DMRD
Presentation A 15 year old girl presents with primary amenorrhea. Transabdominal and translabial ultrasound scans were performed.
Caption: Transverse translabial ultrasound image
Description: This high frequency side-by-side comparison image shows oval echogenic structures in the superior aspect of the labia on both sides.
Caption: Transabdominal sagittal and transverse image
Description: There was no evidence of a uterus or ovaries in the pelvis.
Caption: Transverse translabial ultrasound image
Description: Side-by-side comparison image through the lower inguinal region/upper labia shows transverse sections of both oval echogenic structures.
Differential Diagnosis Male pseudo-hermaphroditism
Final Diagnosis Male pseudo-hermaphroditism. Patient had bilateral labial testes.
Discussion This is a classical case of male pseudohermaphroditism. Pseudo- hermaphroditism is a condition in which the internal gonads are of one sex, but the external appearance is of the opposite sex. Male pseudohermaphroditism is characterized by the following:
- the genetic sex is XY.
- the gonads are exclusively testes.
- the external genitalia are either ambiguous or completely female. 
Male pseudohermaphroditism can occur due to many reasons, common to all of which is defective virilization of the male embryo. Complete androgen insensitivity syndrome is the most common form, resulting from a mutation in the gene for the androgen receptor. This disorder is inherited as X linked recessive. Its management involves corrective surgery of the genitalia and removal of the testes. Estrogen therapy is begun at the appropriate age to allow development of normal female secondary sex characters.

An article on Intersex syndrome is available [click here to view].
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