BACKGROUND: Encephalocele refers to protrusion of the meninges and brain tissue through a skull bone defect. It results from congenital, traumatic, neoplastic, or spontaneous reasons. Traumatic encephalocele occurs because of the posttraumatic fracture of the skull bone or iatrogenic causes. The manifestations vary a lot, such as rhinorrhea, seizures, headaches, and focal neurological deficits.
METHODS: A 20-year-old Syrian male presented to our department with the complaint of clear cerebrospinal fluid drainage from his right nostril, which started 6 years ago after a head trauma, moderate headache, and episodes of tonic-clonic seizures without any response to medical treatment. Then, 2 months ago, the patient had meningoencephalitis, so he was admitted to the intensive care unit and treated for a month until he was cured. The patient underwent radiological investigations, which showed that he had a base fracture with an encephalocele in the nasal cavity. The brain tissues with the meninges herniated through the skull base fracture with a significant expansion of the subarachnoid spaces in the right hemisphere. He was advised to undergo surgical repair at that time, but he refused the surgery. During this visit, surgery was indicated. The surgery was done by a specialist who returned the herniated brain tissues to their normal location, repaired the meninges, and reconstructed the skull base with bone cement and bio-glue. The patient\'s recovery after the surgery was uneventful.
CONCLUSIONS: Traumatic encephalocele is a rare and unexpected complication of trauma, but we should keep it in mind when the patient comes with head trauma because of its life-threatening consequences. This complication can happen after years of trauma if the patient refuses treatment, therefore, we must educate patients about the dangerous results of neglecting cerebrospinal fluid leakage and skull fractures.

OBJECTIVE: Skull base features, including increased foramen ovale (FO) cross-sectional area, are associated with lateral skull base spontaneous cerebrospinal fluid (sCSF) leak and encephalocele. Manual measurement requires skill in interpreting imaging studies and is time consuming. The goal of this study was to develop a fully automated deep learning method for FO segmentation and to determine the predictive value in identifying patients with sCSF leak or encephalocele.
METHODS: A retrospective cohort study at a tertiary care academic hospital of 34 adults with lateral skull base sCSF leak or encephalocele were compared with 815 control patients from 2013-2021. A convolutional neural network (CNN) was constructed for image segmentation of axial computed tomography (CT) studies. Predicted FO segmentations were compared to manual segmentations, and receiver operating characteristic (ROC) curves were constructed.
RESULTS: 295 CTs were used for training and validation of the CNN. A separate dataset of 554 control CTs was matched 5:1 on age and sex with the sCSF leak/encephalocele group. The mean Dice score was 0.81. The sCSF leak/encephalocele group had greater mean (SD) FO cross-sectional area compared to the control group, 29.0 (7.7) mm2 versus 24.3 (7.6) mm2 (P = .002, 95% confidence interval 0.02-0.08). The area under the ROC curve was 0.69.
CONCLUSIONS: CNNs can be used to segment the cross-sectional area of the FO accurately and efficiently. Used together with other predictors, this method could be used as part of a clinical tool to predict the risk of sCSF leak or encephalocele.

BACKGROUND: Encephalocele refers to protrusion of cranial contents through a bony skull defect. Prevalence of encephaloceles in East Africa is approximately 2 per 10,000 livebirths, with occipital encephaloceles making the least proportion of these in this region. We present a case which was diagnosed postnatally and managed surgically with good outcome and few anticipated complications.
METHODS: Newborn baby delivered to a 26-year-old mother at 38 weeks of gestation by spontaneous vaginal delivery, with swelling on the occipital region since birth. Physical examination revealed a mass measuring 8 cm by 6 cm over the occiput. Initial cranial ultrasound and MRI of the brain revealed an occipital myelomeningocele with part of the right cerebellar lobe, meninges, and CSF herniating through the defect in the occipital skull bone. Surgical correction was successfully done. The patient developed CSF leakage due to hydrocephalus 1-week post-surgery and VP shunt placed to relieve the increased intracranial pressure.
CONCLUSIONS: This case highlights a very rare neurosurgical congenital defect in East Africa that was managed as early as possible in a low resource setting with minimal post-surgical complications.
CONCLUSIONS: There is a need for high index of suspicion for encephalocele during antenatal ultrasound screening for prenatal diagnosis. Early surgical repair and prompt post operative follow up help to minimize complications especially in low resource settings where morbidity can be high due to high costs of managing complications.

BACKGROUND: Dermal sinuses and encephaloceles are uncommon central nervous system malformations. The occurrence of these malformations in different members of a family is very rare.
METHODS: This report documents the unique case of a 10-month-old boy with a frontonasal encephalocele, born to a mother with a nasal dermal sinus. These two specific neural tube defects have not been previously reported as occurring between parent and child.
CONCLUSIONS: This case demonstrates potential heritable links in central nervous system malformations through the occurrence of a frontonasal encephalocele in a child born to a mother with a dermal sinus. The connection between these two malformations in a heritable manner suggests a potential need for further research into the genetic pathogenesis of such defects to predict them more accurately within families. https://thejns.org/doi/10.3171/CASE23727.

Primary encephaloceles are congenital mesodermal defects that result in brain tissue protruding through the skull. These defects most commonly occur occipitally but can be present anywhere in the calvarium. Meningoencephaloceles are a subclassification that includes herniation of the meninges. Basal meningoencephaloceles with cleft palate defects are the rarest form, with very few reports discussing anesthetic implications. We report a case of a giant basal meningoencephalocele that involves the nasal and oral cavities with a risk of thecal sac rupture.

Duraplasty is one of the most common neurosurgical procedures which complications include iatrogenic pseudomeningocele, which is common, but ossification of pseudomeningocele following cranial surgery is a rare event. We present a case of a 2-year-old male patient who came to our hospital with a huge bulge in his head and weakness in the right arm and leg. He had a history of sagittal craniosynostosis with a postoperative cranioplasty complication of left parital pseudomeningocele. He underwent a duraplasty, but the bulge recurred with failed cerebrospinal fluid aspiration and external ventricular drain, changing in size periodically. Computed tomography showed that the bulge was a median and left paramedian parital encephalocele, so encephalocele with ossification was diagnosed and a cranioplasty was done. This case highlights that iatrogenic encephaloceles with ossification can develop after duraplasty repair in the parital region. Also, if a postoperative pseudomeningocele changes in size or consistency, clinicians should look for ossification.

OBJECTIVE: Recurrent cerebrospinal fluid (CSF) rhinorrhea caused by sequential, anatomically separated skull base defects is rarely reported in the literature. Neither management nor etiology has been sufficiently investigated. We present an illustrative case and a systematic review of the literature regarding etiology, diagnostics, and management of this rare phenomenon.
METHODS: A systematic literature search looking for articles reporting sequential CSF leaks with multiple skull base defects was performed. Data from included articles were descriptively reported, and the quality of the included studies was assessed with Grading of Recommendations Assessment, Development and Evaluation.
RESULTS: A 71-year-old woman with posttraumatic CSF rhinorrhea and left-sided CSF otorrhea due to a left-sided horizontal fracture of the petrous bone presented at our institution. After initial surgical repair and a 10-week symptom-free interval, CSF rhinorrhea recurred. Imaging revealed a preexisting contralateral meningoencephalocele of the lateral sphenoid recess causing recurrent CSF rhinorrhea most likely after initial traumatic laceration. The defect was successfully treated. A literature search identified 366 reports, 6 of which were included in the systematic review with a total of 10 cases. Quality was deemed good in 8 of 10 cases. The most common location for primary and sequential CSF leaks was along the sphenoid bone (4/10 and 5/10 patients, respectively). All publications except one reported the presence of a meningo (encephalo)cele as cause of the sequential CSF leak.
CONCLUSIONS: Occurrence of recurrent CSF rhinorrhea due to an anatomically separated sequential skull base lesion remains a rare phenomenon. Reassessment of imaging studies and a structured diagnostic workup to detect sequential CSF leaks independent of the primary lesion should is recommended.

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Temporal encephaloceles (TE) are an under-identified, potentially intervenable cause of epilepsy. This systematic review consolidates the current data to identify the major clinical, neuroimaging, and EEG features and surgical outcomes of epilepsy associated with TE. Literature searches were carried out using MEDLINE, Embase, PsycINFO, Scopus, and Cochrane Library databases from inception to December 7, 2023. Studies were included if they described clinical, neuroimaging, EEG, or surgical data in ≥5 patients with TE and epilepsy. Of 562 studies identified in the search, 24 met the eligibility criteria, reporting 423 unique patients with both epilepsy and TE. Compared to epilepsy patients without TE, those with TE had a higher mean age of seizure onset and were less likely to have a history of febrile seizures. Seizure semiologies were variable, but primarily mirrored temporal lobe onset patterns. Epilepsy patients with TE had a higher likelihood of having clinical or radiographic features of idiopathic intracranial hypertension (IIH) than those without. Brain MRI may show ipsilateral mesial temporal sclerosis (16 %). CT scans of the skull base usually revealed bony defects near the TE (90 %). Brain PET scans primarily showed ipsilateral temporal lobe hypometabolism (80 %), mostly in the anterior temporal lobe (67 %). Scalp EEG mostly lateralized ipsilateral to the implicated TE (92 % seizure onset) and localized to the temporal lobe (96 %). Intracranial EEG revealed seizure onset near the TE (11 of 12 cases including TE-adjacent electrodes) with variable timing of spread to the ipsilateral hippocampus. After surgical treatment of the TE, the rate of Engel I or ILAE 1 outcomes at one year was 75 % for lesionectomy, 85 % for anterior temporal lobectomy (ATL), and 80 % for ATL with amygdalohippocampectomy. Further studies are needed to better elucidate the relationship between IIH, TE, and epilepsy, improve the identification of TE, and optimize surgical interventions.

Anterior encephaloceles are rare neural tube defects posing anesthetic challenges. While anterior encephaloceles can cause airway obstruction at birth, this presentation is very rare and to our knowledge not reported in the literature. This case report describes a 34 weeks +0 days gestation, 2.6 kg, newborn with a massive nasoethmoidal anterior encephalocele creating significant external airway obstruction, necessitating emergent and thoughtful airway management and anesthetic care. Our most important perioperative considerations for this newborn included spontaneous ventilation using awake fiberoptic bronchoscopic intubation with lidocaine airway topicalization, secure endotracheal tube attachment, and avoiding noninvasive positive airway pressure postoperatively to avoid pneumocephalus.