Question

Which of the following conditions presents with absence of both Mullerian and Wolffian duct structures?

• A. Antimullerian hormone deficiency
• B. Androgen insensitivity syndrome
• C. FSH receptor defect
• D. Ovotesticular syndrome

Hint:

AMH removes Mullerian ducts; a broken androgen receptor stops Wolffian ducts from forming.

Answer 1

The Correct Option is B

Step 1: Recall the two internal duct systems. The Mullerian (paramesonephric) ducts form the uterus, fallopian tubes and upper vagina. The Wolffian (mesonephric) ducts form the epididymis, vas deferens and seminal vesicles. For both to be absent, the embryo must have signals that suppress Mullerian ducts AND a failure of signals that maintain Wolffian ducts.
Step 2: In androgen insensitivity syndrome (AIS), the karyotype is 46,XY with functioning testes. The testes secrete anti-Mullerian hormone (AMH), so Mullerian structures regress and are absent. The testes also secrete testosterone, but because the androgen receptor is defective, the Wolffian ducts cannot respond and therefore fail to develop. So both duct systems are missing.
Step 3: Why the other options are wrong.
AMH deficiency (option A): testes still make androgens, so Wolffian ducts develop normally and Mullerian ducts persist. Wolffian structures are present, so it does not fit.
FSH receptor defect (option C): affects gonadal follicular function, not duct formation; ducts are not both absent.
Ovotesticular syndrome (option D): both ovarian and testicular tissue coexist, so duct development is mixed, not absent.
Step 4: Only AIS removes both Mullerian (via AMH) and Wolffian (via androgen receptor failure) structures. The answer is option B, Androgen insensitivity syndrome.