Question:
What is the most common karyotype seen in true hermaphroditism (ovotesticular disorder of sex development)?
What is the most common karyotype seen in true hermaphroditism (ovotesticular disorder of sex development)?
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Recall the gonad named ovotestis and its usual chromosome count.
Updated On: Jun 23, 2026
- 45 X0 with streak gonads
- 46 XX with ovotestis
- 47 XY +9
- 47 XX
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The Correct Option is B
Solution and Explanation
Answer: 46 XX with ovotestis.
Step 1: True hermaphroditism, now called ovotesticular disorder of sex development (OVO-DSD), means the same person has both ovarian and testicular tissue, often in a single gonad called an ovotestis.
Step 2: It is one of the rarest intersex anomalies. In about 90% of cases the karyotype is 46 XX. Less often there is 46 XY/46 XX mosaicism or pure 46 XY.
Step 3: 45 X0 with streak gonads is Turner syndrome, not true hermaphroditism. 47 XY +9 and 47 XX are abnormal aneuploidies that do not define this condition.
Step 4: So the most common karyotype with an ovotestis is 46 XX. Ref: Standard textbooks of Endocrinology / Pediatric Surgery.
Step 1: True hermaphroditism, now called ovotesticular disorder of sex development (OVO-DSD), means the same person has both ovarian and testicular tissue, often in a single gonad called an ovotestis.
Step 2: It is one of the rarest intersex anomalies. In about 90% of cases the karyotype is 46 XX. Less often there is 46 XY/46 XX mosaicism or pure 46 XY.
Step 3: 45 X0 with streak gonads is Turner syndrome, not true hermaphroditism. 47 XY +9 and 47 XX are abnormal aneuploidies that do not define this condition.
Step 4: So the most common karyotype with an ovotestis is 46 XX. Ref: Standard textbooks of Endocrinology / Pediatric Surgery.
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