The child with a cephalocele: etiology, neuroimaging, and outcome

Abstract

We report a series of 46 children who were treated for one of the diverse forms of cranium bifidum during a period of 22 years. The purpose of the survey was to investigate pathogenetic factors involved in the development of cranial dysraphism and to analyze clinical and pathological factors that influence the patients' outcome. We also investigated the existence of associated intracranial anomalies, in a systematic way, using modern methods of neuroimaging, and related the findings to the patients' final results. The lesions were classified as encephalocele (n = 15), cranial meningocele (n = 3), atretic cephalocele (n = 26), cranium bifidum occultum (n = 1), and exencephaly (n = 1). There was an excess of the atretic form of cephaloceles in our series, a fact that probably reflects geographical variations described for cephaloceles in general. The location of the lesions was occipital in 29 children, parietal in 16, and temporal and frontobasal in one case each. In seven cases there was parental consanguinity. A familial history of malformations of the central nervous system was encountered in eight instances. Associated systemic abnormalities were present in 23 patients, while central nervous system anomalies were found in 36 children. Cephalocele repair was undertaken on 35 occasions. There were no surgical fatalities in the series. The mean follow-up time was of 7 years. Overall mortality for the whole group was of 17/46 or 36%. Twenty of the 29 survivors had no neurological sequelae, but only 18 children exhibited a competitive intelligence level. A good outcome was found to correlate well with: an average head size at birth, a normal initial neurological condition, operability of the lesions, and an absence of disorders of the neuronal migration. Neurological outcome depended also on the occurrence or not of hydrocephalus, while the intelligence level was mainly related to the absence of cerebral tissue within the sac of the malformation.

Lumps and Bumps on the Head in Children: Use of CT and MR Imaging in Solving the Clinical Diagnostic Dilemma

“…These encephaloceles are more common in South and Southeast Asian populations (12). They are found projecting along the nasal bridge between the nasofrontal sutures into the glabella (nasofrontal region), under the nasal bones and above the nasal septum (nasoethmoidal region) (Fig 3), or along the medial orbit at the level of the frontal process of the maxilla and the ethmoid-lacrimal bone junction (naso-orbital region) (Fig 4) (7,8,12). Frontoethmoidal encephaloceles manifest as a clinically visible mass along the nose.…”

“…Frontoethmoidal encephaloceles manifest as a clinically visible mass along the nose. The intracranial root of most frontoethmoidal encephaloceles lies at the foramen cecum, a small ostium located at the bottom of a small depression anterior to the crista galli and formed by the closure of the frontal and ethmoid bones (7,8).…”

“…Basal encephaloceles include transethmoidal, sphenoethmoidal, transsphenoidal, and frontosphenoidal varieties. Transsphenoidal and transethmoidal encephaloceles are the most common varieties, although they themselves are very rare (Fig 5) (8,13,14). The former project through a defect in the floor of the sella and into the nasal cavity, whereas the latter project through a midline or cribriform plate defect into the nasal cavity.…”

“…Transsphenoidal encephaloceles may be associated with a cleft palate and may also project into the oral cavity. A transsphenoidal encephalocele splays the sphenoid bone, displacing the cavernous sinus laterally, and is positioned anterior to the dorsum sella (8,13,14). Important structures such as the pituitary gland, hypothalamus, optic nerves and chiasm, and anterior third ventricle are typically involved in transsphenoidal encephaloceles (8,13,14).…”

Radiological Approach to Osteolytic Benign Calvarial Lesions

“…Radiography and CT are utilized to show the bone defect, MRI is used to show the sac, its content and the concomitant cerebral abnormalities. It might be observed together with some cerebral pathology such as cortical dysplasia, venous sinus abnormalities, corpus callosum abnormalities, Dandy-Walker and Chiarimal formation 4,12 . Four of our 2 cases were at the OB, 2 were at the PB.…”