Chiari I and severe copper beaten skull

Case contributed by Frank Gaillard
Diagnosis almost certain

Patient Data

Age: Child

Severe plagiocephaly and copper beaten skull

Downward herniation of the cerebellar tonsils. There is also notable gyral impressions on the inner table and abnormal head shape. 

MRI - 6 months post surgery

mri

Interval marked improvement in skull shape and size of the posterior fossa. 

Case Discussion

This 6-month-old child has multiple sutural synostoses (sagittal suture and part of the lambdoid suture), and a Chiari I malformation

Severe copper beaten skull appearance is present which is more reminiscent of a lacunar skull, however, no myelomeningocele is present (i.e not a Chiari II malformation) and axial imaging demonstrates the correlation of gyri with the overlying skull defects. 

The patient was treated with surgery to open the sutures, and marked improvement to the skull shape and posterior fossa ensued. 

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