Question

In which of the following is the Mullerian system present?

• A. Mayer-Rokitansky-Kuster-Hauser syndrome
• B. Turner syndrome
• C. Testicular feminization syndrome
• D. Klinefelter syndrome

Hint:

Ask which option has no testes and therefore no anti-Mullerian hormone.

Answer 1

The Correct Option is B

Step 1: Recall what decides Mullerian survival.
The uterus and tubes come from the Mullerian ducts. They survive unless a testis is present and pumps out anti-Mullerian hormone (AMH), which wipes them out early in fetal life.

Step 2: Check each option for testes and AMH.
Mayer-Rokitansky-Kuster-Hauser syndrome has ovaries, not testes, yet the uterus is still missing, because this condition is a primary failure of the Mullerian ducts themselves rather than an AMH problem.
Testicular feminization and Klinefelter syndrome both have testes present, so AMH is made normally and the Mullerian ducts regress; both end up with no uterus.
Turner syndrome has streak gonads, never true testes, so AMH is never produced.

Step 3: Apply the logic to Turner syndrome.
With no AMH to stop them, the Mullerian ducts in Turner syndrome go ahead and form a uterus and tubes. They just end up small and underactive because there is no estrogen from the failed ovaries to help them grow.

Step 4: Conclude.
So among the four options, only Turner syndrome keeps a Mullerian system, even if it is hypoplastic. The answer is Turner syndrome.