Skeletal dysplasias: fibrodysplasia ossificans progressiva: practice question

A Orthopaedics 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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Orthopaedics · Skeletal dysplasias: fibrodysplasia ossificans progressiva

Sample item

A 4-year-old has short, valgus-deviated great toes and painful neck swellings that later become hard. A surgeon plans a biopsy of a lump. What is the correct advice?

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

The answer and the reasoning

B. Avoid biopsy and intramuscular injections as they can trigger heterotopic bone

Fibrodysplasia ossificans progressiva is due to an ACVR1 mutation and presents with congenital great toe malformation and flare-ups that progress to heterotopic ossification of muscle and tendons. Surgery, biopsy, intramuscular injections and trauma can provoke further ossification and should be avoided. Management is supportive.

Option by option

  • A. Biopsy is essential to exclude sarcoma

    Biopsy can precipitate bone formation and is avoided when the diagnosis is clear.

  • B. Avoid biopsy and intramuscular injections as they can trigger heterotopic bone (correct)

    Correct. Invasive procedures and injections can trigger more heterotopic ossification.

  • C. Give intramuscular steroids for the flare

    Intramuscular injections are avoided because they provoke ossification.

  • D. Excise the lumps early to prevent progression

    Excision usually causes regrowth with more bone.

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 Orthopaedics question

What is the answer to this Orthopaedics question?

The best answer is B, Avoid biopsy and intramuscular injections as they can trigger heterotopic bone. Fibrodysplasia ossificans progressiva is due to an ACVR1 mutation and presents with congenital great toe malformation and flare-ups that progress to heterotopic ossification of muscle and tendons.

Why is "Biopsy is essential to exclude sarcoma" not the answer?

Biopsy can precipitate bone formation and is avoided when the diagnosis is clear.

Why is "Give intramuscular steroids for the flare" not the answer?

Intramuscular injections are avoided because they provoke ossification.

Why is "Excise the lumps early to prevent progression" not the answer?

Excision usually causes regrowth with more bone.

What concept does this question test?

It tests Skeletal dysplasias: fibrodysplasia ossificans progressiva in Orthopaedics. Surgery, biopsy, intramuscular injections and trauma can provoke further ossification and should be avoided. Management is supportive.

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 Skeletal dysplasias: fibrodysplasia ossificans progressiva?

Pair each fracture with its classic complication and nerve injured. Then use the flashcards and the other Orthopaedics 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.