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: Start from the definition - an ovotesticular DSD patient possesses both ovarian and testicular elements, classically as a combined ovotestis. We need the genotype most often associated with this.
Step 2: Epidemiologic data show the dominant karyotype is 46,XX, accounting for about 90% of cases; mosaicism (46,XX/46,XY) and pure 46,XY forms are far less frequent.
Step 3: The option pairing 46 XX with an ovotestis therefore captures both the prevailing chromosome complement and the diagnostic gonad, identifying it as the right choice.
Step 4: Excluding the rest: 45,X0 with streak gonads is Turner syndrome, 47,XY +9 is a trisomy unrelated to this entity, and 47,XX is an aneuploid pattern that is not the hallmark of true hermaphroditism.
\[\boxed{\text{46 XX, ovotestis}}\]