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Radiology · Musculoskeletal imaging: mucopolysaccharidosis
Sample itemA child with coarse facies, corneal clouding and hepatosplenomegaly has radiographs showing oar-shaped ribs, beaked inferior vertebral bodies and a J-shaped sella. Which condition does this pattern indicate?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Mucopolysaccharidosis (dysostosis multiplex)
Dysostosis multiplex combines oar-shaped ribs, beaked vertebrae, a J-shaped sella and short broad metacarpals, typical of the mucopolysaccharidoses such as Hurler syndrome. Visceral and corneal involvement fit the metabolic cause.
Option by option
A. Rickets
Rickets shows metaphyseal cupping and widened physes, not oar ribs and beaked vertebrae.
B. Osteopetrosis
Osteopetrosis shows uniformly dense bones and bone-in-bone appearance.
C. Mucopolysaccharidosis (dysostosis multiplex) (correct)
Correct. Oar-shaped ribs, beaked vertebrae and a J-shaped sella with coarse features indicate a mucopolysaccharidosis.
D. Achondroplasia
Achondroplasia has rhizomelic shortening and narrowing interpedicular distances, without corneal or visceral involvement.
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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