Neurosurgical considerations in macrocephaly

Imithri D. Bodhinayake, Heather J. McCrea

Research output: Chapter in Book/Report/Conference proceedingChapter


Head circumference (HC) or occipitofrontal circumference (OFC) serves as an important indicator of head growth. For pediatric neurosurgeons, there may be no single more important objective measure and fortunately, this is a routine part of almost every well patient visit through 2 years. The head can be considered as several interconnected compartments: the brain parenchyma, blood, cerebrospinal fluid (CSF), and the bones of the skull. An increase in the volume of any one of these compartments prior to the fusion of the sutures and fontanels results in macrocephaly, which is defined as a HC greater than two standard deviations above normal. The most common pathologic cause of macrocephaly is hydrocephalus, the abnormal accumulation of CSF leading to dilation of the cerebral ventricles and increased intracranial pressure (ICP). The pathophysiology of hydrocephalus has been discussed in Chap. 13. The next most common cause of macrocephaly is megalencephaly. Megalencephaly refers to a true overgrowth of brain parenchyma greater than the 98th percentile of brain weight/volume ratio, which in turn increases HC to a degree that causes macrocephaly. Less frequently, syndromes associated with overgrowth of the actual cranial bones can lead to increased HC and macrocephaly. Macrocephaly may also be an early clinical sign of a complex neurocutaneous, neurometabolic, or genetic syndrome. Early recognition, treatment, and management of this condition is essential for the preservation of neurological development and function. In addition to providing a general overview, this chapter highlights important neurosurgical considerations in children with macrocephaly, particularly as they pertain to initial evaluation, treatment, and chronic management as a collaborative effort between pediatricians, pediatric neurologists, and pediatric neurosurgeons.

Original languageEnglish (US)
Title of host publicationCommon Neurosurgical Conditions in the Pediatric Practice
Subtitle of host publicationRecognition and Management
PublisherSpringer New York
Number of pages11
ISBN (Electronic)9781493938070
ISBN (Print)9781493938056
StatePublished - Jan 1 2016
Externally publishedYes


  • Endoscopic third ventriculostomy
  • Head circumference
  • Hydrocephaly
  • Macrocephaly
  • Ventriculoperitoneal shunt

ASJC Scopus subject areas

  • Medicine(all)


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