IMR Press / JIN / Volume 22 / Issue 3 / DOI: 10.31083/j.jin2203079
Open Access Review
Craniofacial Encephalocele: Updates on Management
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1 Department of Neurosurgery, University of Florida, Gainesville, FL 32608, USA
*Correspondence: (Brandon Lucke-Wold)
J. Integr. Neurosci. 2023, 22(3), 79;
Submitted: 20 December 2022 | Revised: 20 February 2023 | Accepted: 24 February 2023 | Published: 19 May 2023
(This article belongs to the Special Issue Advances in Traumatic Brain Injury)
Copyright: © 2023 The Author(s). Published by IMR Press.
This is an open access article under the CC BY 4.0 license.

Craniofacial encephaloceles are rare, yet highly debilitating neuroanatomical abnormalities that result from herniation of neural tissue through a bony defect and can lead to death, cognitive delay, seizures, and issues integrating socially. The etiology of encephaloceles is still being investigated, with evidence pointing towards the Sonic Hedgehog pathway, Wnt signaling, glioma-associated oncogene (GLI) transcription factors, and G protein-coupled receptors within primary cilia as some of the major genetic regulators that can contribute to improper mesenchymal migration and neural tube closure. Consensus on the proper approach to treating craniofacial encephaloceles is confounded by the abundance of surgical techniques and parameters to consider when determining the optimal timing and course of intervention. Minimally invasive approaches to encephalocele and temporal seizure treatment have increasingly shown evidence of successful intervention. Recent evidence suggests that a single, two-stage operation utilizing neurosurgeons to remove the encephalocele and plastic surgeons to reconstruct the surrounding tissue can be successful in many patients. The HULA procedure (H = hard-tissue sealant, U = undermine and excise encephalocele, L = lower supraorbital bar, A = augment nasal dorsum) and endoscopic endonasal surgery using vascularized nasoseptal flaps have surfaced as less invasive and equally successful approaches to surgical correction, compared to traditional craniotomies. Temporal encephaloceles can be a causative factor in drug-resistant temporal seizures and there has been success in curing patients of these seizures by temporal lobectomy and amygdalohippocampectomy, but magnetic resonance-guided laser interstitial thermal therapy has been introduced as a minimally invasive method that has shown success as well. Some of the major concerns postoperatively include infection, cerebrospinal fluid (CSF) leakage, infringement of craniofacial development, elevated intracranial pressure, wound dehiscence, and developmental delay. Depending on the severity of encephalocele prior to surgery, the surgical approach taken, any postoperative complications, and the age of the patient, rehabilitation approaches may vary.

neural tube defects
Tessier's box osteotomy
endoscopic endonasal surgery
vascularized pedicled nasoseptal flap
HULA procedure
cerebrospinal fluid leakage
temporal seizures
neural crest cells
sonic hedgehog
Chiari malformation
Fig. 1.
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