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Anatomy · Neuroanatomy: hindbrain malformations
Sample itemAn infant has an enlarging head. MRI shows a large cystic dilatation of the fourth ventricle, an absent or hypoplastic cerebellar vermis and an elevated tentorium. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Dandy-Walker malformation
Dandy-Walker malformation combines cystic enlargement of the fourth ventricle, vermis agenesis or hypoplasia and an elevated tentorium and torcula, often with hydrocephalus. It results from defective development of the roof of the fourth ventricle.
Option by option
A. Aqueduct stenosis
Aqueduct stenosis enlarges the lateral and third ventricles but leaves the fourth ventricle small.
B. Dandy-Walker malformation (correct)
Correct. A cystic fourth ventricle with a deficient vermis is the defining triad with the high tentorium.
C. Chiari II malformation
Chiari II shows a small posterior fossa with the vermis and medulla pushed downward, usually with a myelomeningocele.
D. Holoprosencephaly
Holoprosencephaly is a failure of forebrain cleavage and does not cause a cystic posterior fossa.
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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