Congenital Hereditary Cranium Bifidum Occultum Frontalis

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Abstract
Many cases of cranium bifidum frontalis associated with meningocele and meningo-encephalocele have been described, especially by neurosurgeons. In a review of the literature, we have found few reported cases of cranium bifidum occultum as such, and a survey of these cases revealed that the cranium bifidum was usually associated with craniofacial dysostosis, cleidocranial dysostosis, or abnormalities of the central nervous system. In the cases to be reported here, the cranium bifidum occultum was associated with large parietal foramina and spina bifida occulta. The patients were otherwise normal. Eckhardt (4), in 1919, reported a case showing a defect of the skull between the bregma and the frontal hairline without herniation of the brain. Associated with this defect were an atypical course of the frontal suture, abnormal configuration of the skull, hypertelorism along with divergent strabismus and horizontal nystagmus, and a gaping mouth producing a stupid and grotesque facies. A defect was also present in the right parietal bone. These findings, apparently, are consistent with craniofacial dysostosis. Laursen (9), in 1942, reported 2 cases of cranium bifidum occultum. The first was in a nine-year-old girl who had rickets during childhood and had sought treatment for a fracture of the tibia. The neurological examination proved negative. X-ray examination showed a caput quadratum type of skull with a mid-line defect along the whole sagittal region from the root of the nose to an area in the occiput. A brother of the patient was a midget and an imbecile. A sister was a dwarf. The second of Laursen's patients was a girl not quite two years of age, who was being placed for adoption. She was hospitalized for hydrocephalus. The mother had been treated for syphilis. The child had not grown normally during the first year but grew well thereafter. She was of low intelligence. The skull was wide and asymmetric, the right side being larger than the left. The forehead bulged forward. The frontal and parietal tuberosities were markedly prominent. The form of the skull represented a combination of hydrocephalic and rachitic cranium. A deep depression could be felt in the frontal suture region. The sagittal suture was wide open throughout its entire length. Caffey (3) in his book describes and illustrates a cranium bifidum occultum in the posterior sagittal region. Pancoast, Pendergrass, and Schaeffer (11) in their text demonstrate a frontal skull defect in a case of cleidocranial dysostosis. Many other cases of cleidocranial dysostosis with cranium bifidum occultum have been reported. Case Reports We are reporting 4 cases of cranium occultum frontalis occurring in three generations of a colored family. A 6-year-old colored girl (Fig. 1) was referred for roentgen examination of the skull for a possible depressed fracture of the frontal bone. She had fallen and suffered a superficial laceration in the mid-line of the forehead.

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