Question

True hermaphroditism (ovotesticular DSD) most commonly has which karyotype?

• A. 45 X0 with streak gonads
• B. 46 XX with ovotestis
• C. 47 XY +9
• D. 47 XX

Hint:

Both gonadal tissues plus the commonest 46,XX complement.

Answer 1

The Correct Option is B

Step 1: True hermaphroditism, now termed ovotesticular disorder of sexual differentiation (DSD), is defined by the presence of both ovarian and testicular tissue (an ovotestis) in the same individual.
Step 2: The question asks for the most common karyotype. In roughly 90% of cases the karyotype is 46,XX. Rarely there is 46,XX/46,XY mosaicism or a 46,XY pattern.
Step 3: Option b states 46 XX with ovotestis, which matches both the commonest karyotype and the defining gonadal finding. So option b is correct.
Step 4: Option a (45,X0 with streak gonads) describes Turner syndrome. Option c (47,XY +9) and option d (47,XX) are abnormal aneuploidies not characteristic of true hermaphroditism. Hence 46,XX ovotestis is the answer.