Macrocephaly in Children: What to Consider Beyond Ventriculomegaly?

Pediatric macrocephaly may result from many benign, transient, acute or chronic entities. Macrocephaly can be secondary to ventriculomegaly, widening of the cerebrospinal fluid filled spaces overlying the brain as well as multiple intrinsic brain pathologies. Early and correct identification of the underlying etiology is essential for patient management and prognosis. The principal goal of this manuscript is to review and discuss the various etiologies of macrocephaly including matching neuroimaging findings beyond the classical well recognized macrocephaly secondary to ventriculomegaly. In addition, the delicate interaction between the ventricular system, the intra- and extracranial arterio-venous system as well as the glymphatic system are briefly reviewed.