Summary
Cephaloceles are congenital malformations with herniation of intracranial structures through a defect in the cranium. On the basis of a review of the literature and 31 personal observations the authors discuss the clinical and neuroradiological presentation of their various anatomical locations: sphenoidal, ethmoidal, frontal, occipital and parietal.
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References
Anderson FM (1947) Intranasal (sphenopharyngeal) encephalocele. A report of a case with intracranial repair and a review of the subject. Arch Otolaryngol 46: 644–654
Avanzini G, Crivelli G (1970) A case of sphenopharyngeal encephalocele. Acta Neurochir 22: 205–212
Baraton J, Ernest C, Porée C, et al (1976) The neuroradiological examination of endocrine disorders of central origin in children. Pediatr Radiol 4: 69–78
Barrow N, Simpson DA (1966) Cranium bifidum. Investigation, prognosis, management. Aust Paediatr J 2: 20–26
Binet EF, Kieffer SA, Martin SH, et al (1969) Orbital dysplasia in neurofibromatosis. Radiology 93: 829–833
Blumenfeld R, Skolnik EM (1965) Intranasal encephaloceles. Arch Otolaryngol 82: 527–531
Burrows EH (1963) Bone changes in orbital neurofibromatosis. Br J Radiol 36: 549–561
Cassidy WA, Wahil JW (1954) Congenital displacement of central nervous system tissue in the nasal fossae. Report of 2 cases. Arch Otolaryngol 59: 93–99
Cimino A, Ferrara P (1967) Meningo-encefalocele sfeno-etmoidale associato a palatoschisi, cheiloschisi e naso bifido. Minerva Otorinolaryngol 17: 160–163
Consul BN, Kulshresta OP (1965) Orbital menigocele. Br J Ophthalmol 49: 374–376
Davis CH Jr, Alexander E Jr (1959) Congenital nasofrontal encephalomeningoceles and teratomas. J Neurosurg 16: 365–377
Ellyin F, Khatir AH, Singh SP (1980) Hypothalamic-pituitary functions in patients with transsphenoidal encephalocele and midfacial anomalies. J Clin Endocrinol Metab 51: 854–856
Evrard P, Caviness VS (1974) Extensive developmental defect of the cerebellum associated with posterior fossa ventriculocele. J Neuropathol Exp Neurol 33: 385–390
Evrard P, Belpaire MC, Boog G, et al (1982) Diagnostic prénatal des affections du système nerveux de l'enfant. Société Internationale de Neurologie Infantile, Lausanne
Fawcett P (1910) Notes on the development of the human sphenoid. J Anat Physiol 44: 207–222
Fitz-Hugh GS (1953) Intranasal encephalomeningocele. Arch Otolaryngol 58: 188–189
Gerber SS, Plotkin R (1982) Lipoma of the corpus callosum. J Neurosurg 57: 281–285
Gilmor RL, Kalsbeck JE, Goodman JM, et al (1972) Angiographic assessment of occipital encephaloceles. Radiology 103: 127–130
Giroud A, Martinet M, Deluchat C (1966) Au sujet des meningoceles intranasaux. Bull Assoc Anat: 449–452
Gisselsson L (1947) Intranasal forms of encephalomeningocele. Acta Otolaryngol 35: 517–531
Goldhammer Y, Lawton-Smith J (1975) Optic nerve anomalies in basal encephalocele. Arch Ophthalmol 93: 115–118
Guthkelch AN (1970) Occipital cranium bifidum. Arch Dis Child 45: 104–109
Harwood-Nash DC, Fitz CR (1976) Neuroradiology in infants and children. Vol II. Mosby, St Louis, pp 1025–1026
Ingraham FD, Swan H (1943) Spina bifida and cranium bifidum: survey of 546 cases. N Engl J Med 228: 559–563
Ingraham FD, Matson DD (1943) Spina bifida and cranium bifidum. Unusual nasopharygeal encephalocele. N Engl J Med 228: 815–820
Inman VT, Saunders JB de CM (1937) The ossification of the human frontal bone. J Anat Physiol 71: 383–394
Jackson FE, Moore BS (1969) Ectopic glial tissue in the occipital scalp. Arch Dis Child 44: 428–430
Jones RE, Bennington JL, Warner NE (1962) Encephalocele masquerading as nasal polyp. JAMA 181: 640–642
Joseph R, Ribierre M (1961) L'encephalocèle endonasale. Arch Fr Pediatr 18: 1128–1134
Karch SB, Urich H (1972) Occipital encephalocele. A morphological study. J Neurol Sci 15: 89–112
Kushnet MW, Goldman RL (1978) Lipoma of the corpus callosum associated with a frontal bone defect. AJR 131: 517–518
Kazner E, Stochdorph O, Wende S, et al (1980) Intracranial lipoma. J Neurosurg 52: 234–245
Larsen JL, Bassoe HH (1979) Transsphenoidal meningocele with hypothalamic insufficiency. Neuroradiology 18: 205–209
Lau BP, Newton TH (1965) Sphenopharyngeal encephalomeningocele. Radiol Clin Biol 34: 386–398
Leck I, Record RG, McKeown T, et al (1968) The incidence of malformations in Birmingham, England. 1950–1959. Teratology 1: 263–280
Lee CM Jr, McLaurin RL (1955) Heterotopic brain tissue an isolated embryonic rest. J Neurosurg 12: 190–192
Lemire RJ, Loeser JD, Leech RW (1975) Normal and abnormal development of the human nervous system. Harper and Row, Hagerstown, pp 289–309
Lieblich JM, Rosen SW, Guyden H, et al (1978) The syndrome of basal encephalocele and hypothalamic-pituitary dysfunction. Ann Intern Med 89: 910–916
List CR, Holt JF, Everett M (1946) Lipoma of the corpus callosum. A clinico-pathologic study. AJR 55: 125–134
Lorber J (1967) The prognosis of occipital encephalocele. Dev Med Child Neurol [Suppl] 13: 75–86
Low NL, Scheinberg L, Andersen DH (1956) Brain tissue in the nose and the throat. Pediatrics 18: 254–259
McLaurin RL (1964) Parietal cephaloceles. Neurology 14: 764–772
McLaurin RL (1977) Cranium bifidum and cranial cephaloceles. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, Vol 30. North Holland, Amsterdam, pp 209–217
Manelfe C, Starling-Jardin D, Toulbi S, et al (1978) Transsphenoidal encephalocele with agenesis of corpus callosum: value of metrizamide computed cisternography. J Comput Assist Tomogr 2: 356–361
Marin-Pedilla M (1980) Morphogenesis of experimental encephalocele (cranioschisis occulta). J Neurol Sci 46: 83–99
Matson DD (1969) Neurosurgery of infancy and childhood. Thomas, Springfield, IL, pp 61–75
Modesti LM, Glasauer FE, Terplan KL (1977) Sphenoethmoidal encephalocele. Child Brain 3: 140–153
Mortada A, El-Toraei I (1960) Orbital meningo-encephalocele and exophthalmos. Br J Ophthalmol 44: 309–314
Myrianthopoulos NC (1979) Oue load of centralnervous system malformations. Birth Defects 15: 1–19
Nabawi P, Dobben GD, Mafee M et al (1981) Diagnosis of lipoma of the corpus callosum by CT in five cases. Neuroradiology 21: 159–162
Nordin WA, Tesluk H, Jones RK (1955) Lipoma of the corpus callosum. J Neurol Neurosurg Psychiatry 39: 1179–1185
Pagon RA, Chandler JW, Collie WR, et al (1978) Hydrocephalus, agyria, retinal dysplasia, encephalocele (HARD-E) syndrome: an autosomal recessive condition. Brith Defects 14: 233–241
Pilpel R (1922) Persistenz des Canalis cranio-pharyngeus mit Rachendachhypophyse und Kephalokele. Monatschr Ohrenheikd 56: 793–802
Pollock JA, Newton TH, Hoyt WF (1968) Transsphenoidal and transethmoidal encephaloceles. Radiology 90: 442–453
Record RG, McKeown T (1949) Congenital malformations of the central nervous system. I. A survey of 930 cases. Br J Social Med 3: 183–219
Robinson EG (1957) Anterior encephalocele. Br J Surg 45: 36–40
Sadeh M, Goldhammer Y, Shacked, I et al (1982) Basal encephalocele associated with suprasellar epidermoid cyst. Arch Neurol 39: 250–252
San Galli F (1982) Les ectopies congénitales transsphénoidales et intrasellaire de la partie antérieure du Illème ventricule. Thèse, Bordeaux, France
Schowing J (1968) Influence inductrice de l'encéphale embryonnaire sur le developpement du crâne chez le poulet. J Embryol Exp Morphol 19: 1–32
Shirataki K, Sakamoto K, Ohama Y (1976) Diagnosis and treatment of transsphenoidal encephalocele. Brain Nerve (Tokyo) 28: 281–291
Strandberg B (1949) Cephalocele of posterior part of orbit. Arch Ophthalmol 42: 254–265
Suwanwela C, Hongsaprabhas C (1966) Fronto-ethmoidal encephalomeningocele. J Neurosurg 25: 172–182
Suwanwela C, Suwanwela N (1972) A morphological classification of sincipital encephalomeningoceles. J Neurosurg 36: 201–211
Tandon PN (1970) Meningoencephaloceles. Acta Neurol Scand 46: 369–383
Terrafranca RJ, Zellis A (1953) Congenital hereditary cranium bifidum occultum frontalis. Radiology 61: 60–66
Toublanc JE, Chaussain JL, Lejeune C, et al (1976) Hypopituitarisme avec hypoplasie des nerfs optiques. Arch Fr Pediatr 33: 67–75
Van Nouhuys JM, Bruyn GW (1964) Nasopharyngeal transsphenoidal encephalocele, craterlike hole in the optic disc and agenesis of the corpus callosum. Pneumoencephalographic visualisation in a case. Psychiatr Neurol Neurochir 67: 243–258
Walker E, Moore WW, Simpson JR (1952) Instranasal encephalocele. Survey of problem, with recommendations for reducing mortality. Arch Otolaryngol 55: 182–187
Wallace D (1976) Lipoma of the corpus callosum. J Neurol Neurosurg Psychiatry 39: 1179–1185
Walsh FB, Hoyt WF (1969) Clinical neuro-ophthalmology. Vol 1, 3rd ed, William and Wilkins, Baltimore, pp 713–719
Warkany J (1971) Congenital malformations. Year Book Medical Publishers, Chicago, pp 211–216
Wiese GM, Kempe LG, Hammon WM (1972) Transsphenoidal meningohydroencephalocele. J Neurosurg 37: 475–478
Winter RM, Garner A (1981) Hydrocephalus, agyria, pseudoencephalocele, retinal dysplasia, and anterior chamber anomalies. J Med Genet 18: 314–317
Wolpert SM (1974) Vascular studies in congenital anomalies. In: Newton TH, Potts DG (eds) Radiology of the skull and brain, Vol 2, Book 4. Mosby, St Louis, pp 2706–2715
Zimmerman RA, Bilaniuk LT, Metzger RA, et al (1983) Computed tomography of orbital-facial neurofibromatosis. Radiology 146: 113–116
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Diebler, C., Dulac, O. Cephaloceles: clinical and neuroradiological appearance. Neuroradiology 25, 199–216 (1983). https://doi.org/10.1007/BF00540233
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DOI: https://doi.org/10.1007/BF00540233