Gynaecological endocrinology: Swyer syndrome: practice question

A OBG question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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OBG · Gynaecological endocrinology: Swyer syndrome

Sample item

A 17-year-old with primary amenorrhoea has no breast development, normal female external genitalia, a small uterus on ultrasound, and elevated FSH and LH. Karyotype is 46,XY. What is the most important management step?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

A. Bilateral gonadectomy, followed by hormone replacement

Swyer syndrome is 46,XY pure gonadal dysgenesis with streak gonads, a normal Mullerian system and absent puberty. The streak gonads carry a high risk of gonadoblastoma and dysgerminoma, so gonadectomy is advised soon after diagnosis. Oestrogen and progestogen replacement induces puberty and menstruation, and pregnancy is possible with donor oocytes.

Option by option

  • A. Bilateral gonadectomy, followed by hormone replacement (correct)

    Correct. Gonadectomy removes the malignancy risk and oestrogen replacement follows.

  • B. Observation alone

    Observation leaves a high risk of germ cell tumour.

  • C. Testosterone therapy

    Testosterone would not induce female puberty.

  • D. Clomiphene ovulation induction

    Streak gonads contain no follicles, so ovulation induction cannot work.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this OBG question

What is the answer to this OBG question?

The best answer is A, Bilateral gonadectomy, followed by hormone replacement. Swyer syndrome is 46,XY pure gonadal dysgenesis with streak gonads, a normal Mullerian system and absent puberty.

Why is "Observation alone" not the answer?

Observation leaves a high risk of germ cell tumour.

Why is "Testosterone therapy" not the answer?

Testosterone would not induce female puberty.

Why is "Clomiphene ovulation induction" not the answer?

Streak gonads contain no follicles, so ovulation induction cannot work.

What concept does this question test?

It tests Gynaecological endocrinology: Swyer syndrome in OBG. The streak gonads carry a high risk of gonadoblastoma and dysgerminoma, so gonadectomy is advised soon after diagnosis. Oestrogen and progestogen replacement induces puberty and menstruation, and pregnancy is possible with donor oocytes.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Gynaecological endocrinology: Swyer syndrome?

Learn management algorithms as steps: PPH, eclampsia, ectopic pregnancy. Then use the flashcards and the other OBG questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.