Cephalocele: Report of 55 Cases over 8 Years

Abstract

Objective: Cephalocele is a central nervous system (CNS) birth defect. Various CNS and extra-CNS anomalies, as well as prognostic factors have been reported with cephalocele. The aim of this study was to discuss prognostic factors and current possible theories explaining associated anomalies seen in a series of 55 patients with cephalocele. Methods: A retrospective study was performed using the records of 55 children with cephalocele at the Children’s Hospital Medical Center in Tehran, Iran, from October 2000 through October 2008. Patients’ sex, age at the operation time, characteristics of the lesion including location, size, cerebrospinal fluid leakage, radiological assessments and intraoperative findings (sac contents), intracranial and extracranial associated anomalies, and the last situation of the children were reviewed. Conclusion: A genetic role can be proposed while a female predominance is seen in our results as well as in other reports. CNS and extra-CNS anomalies, as well as several genetic syndromes are not explicable as a causal consequence of cephalocele and the probability of a mere coincidence cannot be ruled out; therefore, we propose that the combination of these anomalies is rather a low-frequency association. Ventriculomegaly was the only poor prognostic determinant in our study, which can compel more severe neuroradiologic studies in such patients as a reasonable prognostic evaluation.

Encephalocele in Uganda: ethnic distinctions in lesion location, endoscopic management of hydrocephalus, and survival in 110 consecutive children

“…Our overall 32% incidence of hydrocephalus requiring treatment is very similar to that reported by others, 7,13 as is our 32% incidence among patients with occipital EC. However, whereas others have reported hydrocephalus to be much less common among children with frontal lesions, we found it to be at least as common in this group.…”

“…23,27 Occipital lesions account for 66%-95% of ECs in North America, Western Europe, and the Middle East. 5,7,11,19,23,25,27 Sincipital lesions predominate in Thailand, Malaysia, Burma, Cambodia, the Philippines, and the Pacific Islands, 3,4,8,16,20,22,27,28 comprising 20 of 21 EC cases in one report. 3 Monteith and coauthors 16 reported ethnic distinctions in New Zealand, with patients of Pacific Island descent having a much higher incidence of sincipital lesions (44%) than those of European origin.…”

Endoscopic endonasal resection of a nasal meningoencephalocele - Case report

“…More modern endoscopic endonasal surgical techniques are used in cases were the tumor size and the skull base defects are manageable. These techniques have been very successful in many cases 8,12,13 . In the case presented by us, the tumor was resected en-block using an endoscopic endonasal approach, in a mixed ENT-neurosurgical operating team.…”

“…Surgical treatment is the only choice for MEC and, depending on the size and location, it can be done through different approaches, such as lateral rhinotomy, bicoronal flap, or intranasal techniques 8,11 . Recent advancement of endoscopic endonasal techniques has made this type of surgery more frequently used, and it has been successful in many cases of nasal MEC 12,13 . ABSTRACT Nasal meningoencephaloceles are rare findings, represented by protrusions of intracranial contents into the nasal cavity.…”

Encephalocele presenting as an oropharyngeal mass

“…Neurological deficits are common and depend on the size and types of neural tissues enclosed in the sac. 2 Treatment requires a multidisciplinary approach. …”