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Kaplan Qbank USMLE



Author9 Posts
  #1

38.RI studies reveal the following congenital malformations in the CNS of a 6 month-old baby presenting with intractable vomiting: small posterior fossa, downward displacement of the cerebellar vermis and medulla through the foramen magnum, syringomyelia, and myelomeningocele. Which of the following is the most likely diagnosis?

A. Anencephaly

B. Arnold-Chiari type 1 malformation

C. Arnold-Chiari type 2 malformation

D. Dandy-Walker malformation

E. Holoprosencephaly


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  #2

B

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  #3

its C, arnold chiari type 2.

  #4

Most children with these malformations who do not have spina bifida will have the form known as type I.In general the symptoms of the type I malformations are less severe than that of the type II malformation.
. There are three primary types of CM.
The most common is Type I, which may not cause symptoms and is often found by accident during an examination for another condition.
Type II (also called Arnold-Chiari malformation) is usually accompanied by a myelomeningocele-a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord to protrude through an opening in the back. This can cause partial or complete paralysis below the spinal opening.
Type III is the most serious form of CM, and causes severe neurological defects. Other conditions sometimes associated with CM include hydrocephalus, syringomyelia, and spinal curvature.


  #5

just for the sake of discussion..
Dandy-Walker Malformation is a rare malformation of the brain that is present at birth (congenital). It is characterized by an abnormally enlarged space at the back of the brain (cystic 4th ventricle) that interferes with the normal flow of cerebrospinal fluid through the openings between the ventricle and other parts of the brain (foramina of Magendia and Luschka). Excessive amounts of fluid accumulate around the brain and cause abnormally high pressure within the skull, swelling of the head (congenital hydrocephalus), and neurological impairment. Motor delays and learning problems may also occur. Dandy-Walker Malformation is a form of "Obstructive" or "Internal Noncommunicating Hydrocephalus," meaning that the normal flow of cerebrospinal fluid is blocked resulting in the widening of the ventricles

  #6

Arnold-Chiari type 2 malformation---->associated with myelomeningocele

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  #7

c

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  #8

yes type 2

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live and let live.

  #9

dandy walker----failure of foramina of luschka & magendie to open ----> dilatataion of 4 th ventricle---associated with occipital meningiocele,elevation of confluence of sinuses,agenis of cerebellar vermis &splenium of corpus callosum







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