This post is an answer to the Case – A Baby With a Large Head
What is this condition called?
Congenital hydrocephalus.
What do you notice that is typical about the eyes in this child?
The eyes are deviated downwards due to compression of the upper brainstem. There may also be a squint and nystagmus in this condition.
Is papilloedema present on examining the fundi of these infants?
Characteristically, no.
What other physical signs might be commonly found on examining the enlarged head?
- The fontanelles bulge, the cranial sutures are widened (noticeably the sagittal suture) and dilated subcutaneous veins can be seen coursing over the cranial vault
- X-rays of the skull in older children may show ‘copper beating’ of the bones of the vault and erosion of the pituitary fossa.
What other congenital anomaly is typically associated with this condition?
Spina bifida.
What is the surgical treatment of this condition?
A shunt using a Spitz–Holter valve* between the lateral cerebral ventricle and the right atrium or peritoneal cavity.
