Intranasal encephalocele.

J Neurosurg

Published: March 1951

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http://dx.doi.org/10.3171/jns.1951.8.2.0231DOI Listing

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Article Synopsis
  • Intranasal meningoencephaloceles are rare congenital defects that can show symptoms similar to more common conditions like nasal polyps, making diagnosis challenging in pediatric neurosurgery.
  • The case of a 20-day-old boy with a basal meningoencephalocele was reported, involving symptoms such as cerebrospinal fluid leakage and respiratory issues, leading to two staged surgeries for repair.
  • Emphasis is placed on the necessity for prompt surgical intervention in infants and thorough intraoperative examination to ensure all defects are addressed, reducing the risk of complications.
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The uncommon, non-hereditary congenital abnormalities known as nasal glial heterotopias (NGH) are composed of heterotopic neuroglial tissue. Typically, NGH manifests in infancy, but occasionally it can also be seen in older children and adults. To rule out intracranial extension, magnetic resonance imaging (MRI) and computed tomography (CT) scans should be performed.

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Background And Importance: Basal encephaloceles are the result of a concomitant cranial and dural defect that allows for inferior displacement of cerebral tissue, meninges, and cerebrospinal fluid into the paranasal sinuses and outside the cranial vault. This work illustrates a step-by-step surgical approach of a successful single-stage, endoscopic repair of a congenital basal encephalocele in a 10-year-old child, using a free mucosal middle turbinate graft that provided effective results without utilization of traditional open reconstructive techniques or vascularized flaps.

Clinical Presentation: A previously healthy 10-year-old male with a history of unilateral clear rhinorrhea was admitted as an inpatient because of an acute episode of nausea, vomiting, and confusion, accompanied by fever, diplopia, and bilateral abducens nerve palsies.

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Primary pseudotumor cerebri syndrome (PPTS) is a rare disorder of elevated intracranial pressure (ICP) in the absence of an identifiable underlying etiology. Afflicted patients are usually obese women in their reproductive age presenting with symptoms of elevated ICP. Seldom, patients can present with an encephalocele.

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Histopathologic features of nasal glial heterotopia (nasal glioma).

Childs Nerv Syst

January 2022

Departments of Pathology, Neurology, Neurosurgery, University of Colorado, Aurora, CO, USA.

Purpose: Nasal glioneuronal heterotopia (NGH) is an uncommon developmental abnormality of the nasal cavity or paranasal soft tissue. Few detailed histologic studies of NGH exist, and molecular analyses have not been performed to date.

Methods: We describe six cases of pediatric NGH and two representative encephaloceles encountered in our practice over the past 20 years.

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