Approach Considerations
The management is variable depending upon the etiology of macrocephaly.
Benign familial macrocephaly does not necessitate treatment and children mostly remain asymptomatic. Periodic monitoring of head size is sufficient in these cases along with regular monitoring for physical growth and neurologic development.
Medical Care
Macrocephalic children with epilepsy require therapy with appropriate antiepileptic medication.
Surgical Care
Neurosurgical intervention may be warranted in children with hydrocephalus and consequent symptoms of increased intracranial pressure. Various procedures may be considered for drainage of CSF in order to reduce the fluid volume including placement of a extraventricular drain (EVD) or a ventriculoperitoneal (VP) shunt (see image below). [10]
Another surgery reported in the literature is complete reconstruction of the skull in children with grossly deformed skull architecture. It has been tried in a case of macrocephaly due to hydrocephalus. [11]
Consultations
Consultation with any of the following may prove helpful:
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Pediatrician
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Geneticist
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Pediatric neurologist
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Pediatric neurosurgeon
Long-Term Monitoring
It is recommended to measure and plot the head size monthly for six months to ascertain the growth curve of the child and to compare it to the normal curve. Physical and neurologic examination should be carried out regularly. Neuroimaging is not routinely recommended unless there is abnormal or rapid increase in head size or there is motor, cognitive, or growth retardation or loss of milestones.
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A 1-year-old boy with macrocephaly. The image illustrates the increased head circumference (large head).
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A 1-year-old boy with macrocephaly. The image illustrates the increased head circumference (large head).
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A 1-year-old boy with macrocephaly. The image illustrates the increased head circumference (large head).
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Antero-posterior and lateral views on skull radiographs of a 1-year-old boy with macrocephaly.
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Antero-posterior view on skull radiograph of a 1-year-old boy with macrocephaly.
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Lateral view on skull radiograph of a 1-year-old boy with macrocephaly. The increased antero-posterior diameter can be appreciated.
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Noncontrast CT scan of brain of a 2-year-old boy showing gross hydrocephalus with paper thin brain tissue (coronal view).
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Noncontrast CT scan of brain of a 2-year-old boy showing gross hydrocephalus with paper thin brain tissue (coronal view).
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Noncontrast CT scan of brain of a 2-year-old boy showing gross hydrocephalus with paper thin brain tissue (coronal view).
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Noncontrast CT scan of brain of a 2-year-old boy showing gross hydrocephalus with paper thin brain tissue (coronal view).
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Ventriculoperitoneal shunt in situ.
