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Image Based Question-66199

Question

Diagnose the Underlying Condition as shown in Photograph

A. Dolichocephaly

B. Macrocephaly

C. Plagiocephaly

D. Scaphocephaly

 

Show Answer

Correct Answer » B

Explanation

Ans:B. Macrocephaly

Macrocephaly

  • It is defined as head circumference more than two standard deviations (SD) above the mean value for a given age and gender.
  • It has to be differentiated from megalencephaly, which is defined as increase in the size of the brain parenchyma.

Etiology

Enlargement of skull bones

  • Hyperostosis cranii – associated with disorders such as osteogenesis imperfecta, achondroplasia, and osteopetrosis
  • Secondary enlargement due to bone marrow expansion – as seen in thalassemia major
    Increase in volume of cerebrospinal fluid

Hydrocephalus

  • Choroid plexus papilloma
  • Benign familial macrocephaly(most common cause)

Megalencephaly

  • Leukodystrophies – Canavan disease, Alexander disease, megalencephalic
  • leukoencephalopathy with subcortical cysts
  • Lysosomal storage disorders
  • Neurocutaneous disroders – Tuberous sclerosis, Sturge-weber syndrome, neurofibromatosis, Gorlin syndrome
  • Autism spectrum disorder
  • Other syndromes – Fragile X syndrome, Cowden syndrome, Sotos syndrome

Increased intracranial pressure (ICP)

  • CNS infections
  • Pseudotumor cerebri

Mass lesions and increase in volume of blood

  • Tumor
  • Intraventricular hemorrhage, subdural hematoma, arteriovenous malformation.

Dolichocephaly is a condition where the head is longer than would be expected,relative to its width.

Brachycephaly is the shape of a skull shorter than typical for its species.

Plagiocephaly, also known as flat head syndrome,is a condition characterized by an asymmetrical distortion (flattening of one side) of the skull.

Scaphocephaly describes a specific shape of a long narrow head that resembles an inverted boat

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