Tooth development and dental anatomy: Ellis-van Creveld syndrome: practice question

A Dentistry 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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Dentistry · Tooth development and dental anatomy: Ellis-van Creveld syndrome

Sample item

A newborn has natal teeth, postaxial polydactyly, short limbs and a heart murmur from an atrial septal defect. Which syndrome is this?

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

The answer and the reasoning

D. Ellis-van Creveld syndrome

Ellis-van Creveld is an autosomal recessive chondroectodermal dysplasia with short limbs, polydactyly, nail and tooth anomalies including natal teeth, and congenital heart disease, usually a common atrium. A short upper lip with fused frenula is typical. It is common in the Amish.

Option by option

  • A. Down syndrome

    Down syndrome shows trisomy 21 features such as macroglossia and delayed eruption.

  • B. Cleidocranial dysplasia

    Cleidocranial dysplasia shows absent clavicles and supernumerary teeth.

  • C. Treacher Collins syndrome

    Treacher Collins shows malar hypoplasia and ear defects without polydactyly.

  • D. Ellis-van Creveld syndrome (correct)

    Correct. Polydactyly, short limbs, natal teeth and heart defect point to this syndrome.

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 Dentistry question

What is the answer to this Dentistry question?

The best answer is D, Ellis-van Creveld syndrome. Ellis-van Creveld is an autosomal recessive chondroectodermal dysplasia with short limbs, polydactyly, nail and tooth anomalies including natal teeth, and congenital heart disease, usually a common atrium.

Why is "Down syndrome" not the answer?

Down syndrome shows trisomy 21 features such as macroglossia and delayed eruption.

Why is "Cleidocranial dysplasia" not the answer?

Cleidocranial dysplasia shows absent clavicles and supernumerary teeth.

Why is "Treacher Collins syndrome" not the answer?

Treacher Collins shows malar hypoplasia and ear defects without polydactyly.

What concept does this question test?

It tests Tooth development and dental anatomy: Ellis-van Creveld syndrome in Dentistry. A short upper lip with fused frenula is typical. It is common in the Amish.

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 Tooth development and dental anatomy: Ellis-van Creveld syndrome?

Learn the dental formula, eruption ages and tooth structure once, as tables. Then use the flashcards and the other Dentistry 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.