Macrocranium and macrencephaly in neurofibromatosis

Data were collected on 52 patients (30 males and 22 females) with neurofibromatosis. Using the volumetric method of Gordon, it was found that 44% of the patients had cranial capacities above the 95th percentile (70% were above the 50th percentile). The presence of intracranial tumors and/or hydrocephalus did not influence skull size. Four patients above the 95th percentile had normal pneumoencephalograms and cerebral angiograms, indicating that the cause of the macrocranium, in some instances at least, is macrencephaly. Volumetric measurement of the sella turcica in 27 of the 52 patients gave results which strongly suggest that idiopathic enlargement of the sella in neurofibromatosis is uncommon, if not rare. Skull films of 26 patients with tuberous sclerosis did not show an increase in cranial capacity similar to that found in neurofibromatosis.