A morphological classification of sincipital encephalomeningoceles

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✓ Cranial defects and cerebral abnormalities as revealed by postmortem dissection in 12 patients with sincipital encephalomeningocele are reported. The various methods of classifying this lesion are discussed. A classification based on the location of the defect in the cranium is outlined. The clinical application of such a classification and its usefulness in the surgical management are emphasized.

Article Information

Address reprint requests to: Charas Suwanwela, M.D., Section of Neurological Surgery, Department of Surgery, Chulalongkorn Hospital and Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

© AANS, except where prohibited by US copyright law.

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Figures

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    Drawings to illustrate the findings in all 12 patients. The appearance and location of the mass are shown in the upper row, the defects of the bones of the face in the middle row. The intracranial openings in Cases 1 through 10 and the defects of the cranial vault in Cases 11 and 12 are shown in the lower row.

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    Drawings to illustrate the location of the bone defect in the three subgroups of the fronto-ethmoidal encephalomeningocele. The anterior aspect and midsagittal plane of the cranium together with the nasal cartilage are shown: A. Normal. B. Nasofrontal. C. Naso-ethmoidal. D. Naso-orbital. The medial wall of the orbit instead of the midsagittal plane is shown in D. E = ethmoidal bone, F = frontal bone, M = maxillary bone (shaded area), N = nasal bone (striped area), nc = nasal cartilage, and S = sphenoid bone.

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    Case 1. Nasofrontal subgroup of fronto-ethmoidal encephalomeningocele showing the defect between the frontal bone on one side and the ethmoidal and nasal bones on the other. The crista galli is seen protruding into the defect. Note the low position of the cribriform plates in relation to the roof of the orbits. This patient is Case 5 in our previous report.39

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    Case 4. Nasofrontal subgroup of the fronto-ethmoidal encephalomeningocele showing the typical location of the mass in this subgroup.

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    Case 1. Superior view (left) and coronal section (right) of the brain in an 8-day-old boy with a nasofrontal encephalomeningocele showing holotelencephaly.

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    Case 6. Brain showing constriction in the middle corresponding to the neck of a nasofrontal encephalomeningocele. About one-half of the brain was outside the cranial cavity. Note the rather well-formed cortical gyri.

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    Case 4. Undersurface of the brain showing the herniated front tip. Note the elongation of the anterior cerebral artery and the far-forward displacement of the anterior communicating artery. Smooth cortical surface is also seen.

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    Case 7. Naso-ethmoidal subgroup of fronto-ethmoidal encephalomeningoceles. Postmortem dissection showing the defect in the facial bones. The nasal bones and the frontal processes of the maxillary bone are attached to the frontal bone above the defect. The nasal cartilage adjoins the ethmoidal bone below the defect. The lateral wall of the defect, being membranous, was removed.

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    Case 9. Naso ethmoidal subgroup of fronto-ethmoidal encephalomeningocele showing the typical location of the mass or masses.

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    Case 9. Postmortem dissection showing herniation of the tips of both frontal lobes through the defect. The fibrous band in the middle is the herniated falx cerebri.

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    Case 10. Naso-orbital subgroup of front-ethmoidal encephalomeningocele. Postmortem dissection showing the thick-walled sacs. This patient is Case 11 in our previous report.39

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    Case 11. Interfrontal encephalomeningocele in an 8-day-old girl who also had a lumbosacral myelomeningocele, flexion deformity of the metacarpophalangeal joints of both hands, claw toes, and an interatrial septal defect.

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    Case 12. Anterior-cranial upper-facial cleft. This newborn boy had two large cystic masses overlying a large skull defect. He also had a complete cleft lip and palate on the left side.

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