Abstract
Craniocerebral disproportion is rarely considered as a cause for chronic, debilitating headache in adults. Children reported with this disorder typically suffer from headaches and lethargy for many years and have multisutural synostosis. The terms craniocerebral disproportion, craniostenosis, and slit-ventricle syndrome are used inconsistently as diagnostic designations. Three adults with craniocerebral disproportion who had been treated in infancy for two different pathologies are reported. All benefited greatly from cranial vault expansion.
Highlights
A cranial vault that is too small to safely and satisfactorily accommodate a patient’s normal intracranial contents, regardless of circumference and cause, is designated craniocerebral disproportion
One was a 20-year old who had undergone surgery for scaphocephaly in infancy and began in adolescence to have severe and progressively worsening headaches and lethargy but no cause had been identified. His evaluation at University of Colorado Hospital revealed multisutural synostosis, small ventricles, and very high intracranial pressure despite a head circumference at the 90th percentile. These findings were interpreted as consistent with the diagnosis of craniocerebral disproportion caused by multisutural synostosis, which occurred as a consequence of surgery in infancy for craniosynostosis [2,3,6,8]
Two patients ages 26 and 30, both with cerebral spinal fluid (CSF) shunts implanted in infancy, had experienced many years of repeated episodes of high intracranial hypertension, which had always been attributed to cerebrospinal fluid (CSF) shunt failures
Summary
A cranial vault that is too small to safely and satisfactorily accommodate a patient’s normal intracranial contents, regardless of circumference and cause, is designated craniocerebral disproportion. It may be caused by multisutural synostosis, either syndromic or occurring following surgery for craniosynostosis or secondary to long-term exposure to low intracranial pressure in infancy [1,2,3,4,5,6,7,8]. Craniocerebral disproportion either rarely occurs or is perhaps only rarely recognized in adults [6]. This report presents adults with craniocerebral disproportion who benefited from cranial vault expansion
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