What is the significance of foramen magnum stenosis in achondroplasia?
a) It is a cosmetic issue with no medical consequences.
b) It can lead to compression of the brainstem and spinal cord, resulting in severe health problems.
c) It only affects adults with achondroplasia.
d) It is completely unrelated to achondroplasia.
What contributes to the narrowing of the foramen magnum in infants with achondroplasia?
a) Growth spurt during adolescence
b) Restricted growth in the first 2 years of life and premature closure of skull plate synchondroses
c) Diet and nutrition
d) Lack of physical activity
How can foramen magnum stenosis be diagnosed?
a) By physical examination alone
b) Through a blood test
c) By acquiring effective neuroimaging
d) By measuring head circumference
Why is standardized imaging protocol essential for children with achondroplasia?
a) It helps in diagnosing achondroplasia itself.
b) It ensures that clinically useful neuroimaging is performed and reduces unnecessary radiation exposure.
c) It is required for insurance purposes.
d) It helps in determining the child's future height.
How did the European Achondroplasia Forum (EAF) contribute to the management of foramen magnum stenosis?
a) By recommending surgery for all cases
b) By providing guidelines for the detection and management of foramen magnum stenosis
c) By developing a new drug
d) By organizing awareness campaigns
What is the Achondroplasia Foramen Magnum Score (AFMS) used for?
a) To assess the severity of sleep apnea in achondroplasia patients
b) To diagnose achondroplasia in infants
c) To evaluate the severity of foramen magnum stenosis in infants with achondroplasia
d) To measure head circumference
What does a high Total Apnea and Hypopnea Index (TAHI) indicate in relation to foramen magnum stenosis?
a) It suggests that the patient has no stenosis.
b) It indicates severe foramen magnum stenosis.
c) It has no correlation with foramen magnum stenosis.
d) It indicates a need for dietary changes.
What is the sensitivity of clinical examination and CRS (cardiorespiratory sleep studies) for predicting the effects of foramen magnum stenosis on the spinal cord?
a) High sensitivity
b) Low sensitivity
c) No sensitivity
d) Moderate sensitivity
How can routine screening with MRI using AFMS benefit infants with achondroplasia?
a) It helps in cosmetic improvements.
b) It has no benefits.
c) It aids in detecting early spinal cord changes and can reduce infant morbidity and mortality.
d) It is only useful for diagnosing other medical conditions.
What percentage of infants required neurosurgery in the study mentioned in the text?
a) 0%
b) 10%
c) 25%
d) 50%
Answers:
b) It can lead to compression of the brainstem and spinal cord, resulting in severe health problems.
b) Restricted growth in the first 2 years and premature closure of skull plate synchondroses
c) By acquiring effective neuroimaging
b) It ensures that clinically useful neuroimaging is performed and reduces unnecessary radiation exposure.
b) By providing guidelines for the detection and management of foramen magnum stenosis
c) To evaluate the severity of foramen magnum stenosis in infants with achondroplasia
b) It indicates severe foramen magnum stenosis.
b) Low sensitivity
c) It aids in detecting early spinal cord changes and can reduce infant morbidity and mortality.
c) 25%
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