(1) The patient has a skull fracture at or before 18 months of age.
(2) The overlying skin is never broken.
(3) A dural tear occurs beneath the fracture, typically associated with some brain injury.
(4) The fracture defect progressively enlarges over time into a palpable cranial defect.
(5) The defect may feel soft and pulsating. There may be a visible swelling over the defect.
Neurologic findings:
(1) Some patients may present with seizures.
(2) Neurologic deficits may appear, typically after an initial silent period.
Imaging studies of the skull show the defect which can be monitored over time. Many patients will develop ventricular dilatation on the affected side and some patients will develop porencephaly.
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