Paediatric orthopaedics: osteogenesis imperfecta type II: 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.

Try it

Orthopaedics · Paediatric orthopaedics: osteogenesis imperfecta type II

Sample item

A newborn has a deformed chest, markedly short and crumpled long bones with continuously beaded ribs on radiograph, and dark blue sclerae. The baby dies of respiratory failure in the first days of life. Which type of osteogenesis imperfecta (Sillence) is this?

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

The answer and the reasoning

C. Type II

Sillence type II is the perinatal lethal form, usually from severe COL1A1 or COL1A2 mutations. Radiographs show crumpled femora and beaded ribs, and death is from pulmonary hypoplasia. Type I is mild with blue sclerae, type III is progressive and deforming and type IV is moderate with normal sclerae.

Option by option

  • A. Type III

    Type III is severe and progressively deforming but children survive the neonatal period.

  • B. Type IV

    Type IV is moderate, with normal or greyish sclerae and variable fractures.

  • C. Type II (correct)

    Correct. Beaded ribs, crumpled long bones and early death define type II.

  • D. Type I

    Type I is mild, compatible with a normal lifespan, with blue sclerae and few fractures.

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.

More Orthopaedics questions

Questions about this Orthopaedics question

What is the answer to this Orthopaedics question?

The best answer is C, Type II. Sillence type II is the perinatal lethal form, usually from severe COL1A1 or COL1A2 mutations.

Why is "Type III" not the answer?

Type III is severe and progressively deforming but children survive the neonatal period.

Why is "Type IV" not the answer?

Type IV is moderate, with normal or greyish sclerae and variable fractures.

Why is "Type I" not the answer?

Type I is mild, compatible with a normal lifespan, with blue sclerae and few fractures.

What concept does this question test?

It tests Paediatric orthopaedics: osteogenesis imperfecta type II in Orthopaedics. Radiographs show crumpled femora and beaded ribs, and death is from pulmonary hypoplasia. Type I is mild with blue sclerae, type III is progressive and deforming and type IV is moderate with normal sclerae.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. 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 Paediatric orthopaedics: osteogenesis imperfecta type II?

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.