Temporal Encephalocele: A Treatable Etiology of Drug-Resistant Pediatric Temporal Lobe Epilepsy.

BACKGROUND: Temporal lobe encephaloceles (TEs) are a rare cause of drug-resistant temporal lobe epilepsy (DR-TLE), with head trauma and obesity identified as risk factors in adults. This study evaluated the clinical characteristics of childhood-onset DR-TLE due to TE. METHODS: This is a single-institution retrospective review of childhood-onset DR-TLE with radiographic TE identified between 2008 and 2020. The epilepsy history, brain imaging features, and surgical outcomes were collected. RESULTS: Eleven children with DR-TLE due to TE were included (median age at epilepsy onset was 11 years, interquartile range 8.5 to 13.5 years). Median latency between epilepsy diagnosis and TE detection was 3 years (range of 0 to 13 years). None had history of head trauma. Body mass index greater than 85 percentile for age and sex was seen in 36% of the children. No patient had bilateral TE identified. TEs were diagnosed based on epilepsy surgery conference re-review of imaging in 36% of cases. All herniations were contained defects without osseous dehiscence. Regional fluorodeoxyglucose (FDG) hypometabolism ipsilateral to the encephalocele was seen in all children who had FDG-positron emission tomography (PET) of the brain. Of the children who had surgery, 70% were seizure free or had nondisabling seizures at last follow-up (mean follow-up 52 months). CONCLUSIONS: TE is a surgically remediable etiology of DR-TLE in childhood. TEs are often overlooked at pediatric epilepsy diagnosis, calling for the need to increase awareness of this entity. FDG-PET temporal hypometabolism in children with presumed nonlesional DR-TLE should be carefully examined for occult TEs.

Tonsillar herniation as a complication of lumboperitoneal shunt: case report and literature review.

Tonsillar herniation is a rare and seldom reported complication after lumboperitoneal (LP) shunting. There have been only few reports that have presented possible options for treatment with varying degrees of success. In this report, we describe a rare case of tonsillar herniation after LP shunting and review related literature.A 17-year-old girl with hydrocephalus related to a traumatic brain injury underwent implantation of an adjustable pressure shunt (valve setting2.5) and a small lumen peritoneal catheter via the L4-L5 interspinal space. One month later, she was admitted to the emergency room with a Glasgow Coma Scale score ofE1M1Vt and dilated pupil. Image studies demonstrated new-onset tonsillar herniation and a mild cervical syrinx. Emergent suboccipital decompressive craniectomy, C1 laminectomy, and duraplasty were performed. This was followed with ligation of the LP shunt and implantation of a ventriculoperitoneal (VP) shunt a few days later. The patient's Glasgow Coma Scale score gradually recovered to 6, which was her previous neurologic status.Tonsillar herniation as a complication after LP shunting is best treated with decompression, ligation or removal of the LP shunt, and a shift to a VP shunt. The tonsillar herniation should be rapidly addressed to avoid persistent symptoms or progression of the neurologic deficits.

Natural History of Traumatic Encephaloceles: A Systematic Literature Review.

Encephaloceles rarely develop following traumatic skull fractures. Given their low incidence, the clinical presentations and management strategies of these lesions are confined to case reports and limited case series. A systematic literature review was performed using PubMed, Ovid, and Web of Science databases to identify relevant articles using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A total of 37 articles met inclusion criteria, including the case presented herein. These articles reported 52 traumatic encephaloceles. Mean patient age was 25.3 years (range 6 mo-66 y) with a male predominance (63%, 33/52). The most common bony defects resulting in encephalocele formation were the orbital roof (52%, 27/52), ethmoid (35%, 18/52), and sphenoid (10%, 5/52). Mean time from traumatic injury to initial presentation was 21.3 months (range 0 d-36 y) with a bimodal distribution split between immediately following the traumatic injury (57%, 26/46) or in a delayed manner (43%, 20/46). Common presentations of orbital roof, frontonasal, and temporal bone encephaloceles were exophthalmos (85%, 23/27), cerebrospinal fluid rhinorrhea (71%, 17/24), and hearing loss (100%, 4/4), respectively. Operative approach, repair technique, and materials used for encephalocele reduction were highly variable. Surgical intervention afforded definitive symptomatic improvement or resolution in the majority of cases (89%, 42/47). Clinical outcomes did not differ between orbital, frontonasal, or temporal bone encephaloceles ( P =0.438). Traumatic encephaloceles are a rare entity with diverse presenting symptomatology dependent upon the location of fracture dehiscence. Surgical intervention affords symptomatic improvement in the majority of cases irrespective of encephalocele location, time to presentation, or operative approach.

The neurodevelopmental outcomes of children with encephalocele: a series of 102 patients.

OBJECTIVE: The overall prognosis of encephalocele (EC) is not well described. However, the presence of some risk factors may result in neurodevelopmental delay (NDD) and negatively affect the prognosis of affected patients. The goal of this study was to evaluate neurodevelopmental outcome, as well as the impact of a number of factors on the outcome in patients with ECs. METHODS: This was an observational, retrospective study including 102 children with EC who were followed at the pediatric neurosurgery department of a tertiary medical center between the years 2010 and 2021. The authors evaluated NDD status according to the Centers for Disease Control and Prevention classification via clinical evaluation and parent interviews in the outpatient setting. RESULTS: There were 52 boys and 50 girls. The median age at the time of surgery was 4 months (range 1 day-7.5 years). Seventy-one patients (69.6%) had posterior ECs, whereas 31 (30.4%) had anterior ECs. Forty-three (42.2%) of the ECs contained neural tissue. Of the 102 patients, 33 (32.4%) had ventriculomegaly. In terms of NDD, 14 (14.9%) had mild/moderate delay, whereas 17 patients (18.1%) had severe NDD. On univariate analysis, posterior location, size of sac, presence of neural tissue, ventriculomegaly, symptomatic hydrocephalus, and postoperative infection were correlated with NDD. On a multivariate logistic regression model, only neural tissue presence had a statistically significant association with NDD (OR 7.04, 95% CI 1.33-37.2, p = 0.022). Although not statistically significant, children with ventriculomegaly were 2.6 times as likely to have NDD (95% CI 0.59-11.19, p = 0.362). CONCLUSIONS: This is a single-center study with a large sample size in which the neurodevelopmental status of patients with EC was assessed, and the authors tried to find the risk factors of NDD in these patients. The results showed that the presence of neural tissue within the EC sac was the only risk factor that had independent statistically significant association with NDD.

A Clinical Case of Delayed Posttraumatic Frontal Sinus Cerebrospinal Fluid Leakage Management Via External Surgical Approach.

ABSTRACT: Cerebrospinal fluid rhinorrhea is frequently encountered after a fracture of the skull base. Individual fractures of the posterior wall of the frontal sinus after brain injury are uncommon. The authors present a case of a 33-year-old man with a distant history of skull base injury after a traffic accident, 12 years ago. He presented with intermittent rhinorrhea and reported 2 episodes of bacterial meningitis the last 7 years since the injury. Cerebrospinal fluid leakage was confirmed with ß2-transferrin testing. Computed tomography and magnetic resonance imaging revealed a small defect of the posterior and superior wall of the left frontal sinus and an ipsilateral meningoencephalocele.Finally, there was a communication between intracranial space and frontal sinus that caused meningitis.A coronal incision and frontal craniotomy with preservation of anterior pericranium was performed (frontal sinus cranialization using osteoplastic flap). In order to determine the precise margins of the frontal sinus and allow an accurate anterior table bone osteotomy and complete exposure of the sinus, the authors used a coronal view of skull X-ray. Scissors are then used to cut along the margins of the sinus. An "R" is scratched into the right side of template to record orientation. The template was sterilized and brought onto the surgical field and then placed over the left sinusAn external surgical approach (anterior table bone osteotomy) with the modern technique of osteoplastic flap access was performed. An elevator was used to separate the dura from the posterior table along the entire margin of the defect. Meningoplasty (cauterization of the meningoencephalocele) and closure of the osseous defect was followed. The authors repaired the defect of the posterior wall of the left frontal sinus using a combination of underlay and overlay technique. Femoral fascia from right thigh and fibrin glue were placed in order to cover the leak of the posterior wall of the left frontal sinus, respectively.Patients who present with a short or/ and distant history of traumatic brain injury should be evaluated for complication of a cerebrospinal fluid leak.

[On the tactics of management and treatment of patients with congenital cerebral hernias in their localization in the maxillofacial region]. / O taktike vedeniya i lecheniya bol'nykh s vrozhdennymi mozgovymi gryzhami pri ikh lokalizatsii v chelyustno-litsevoi oblasti.

The analysis of the state of two patients with congenital cerebral hernias was carried out, which made it possible to establish differences in the effect of hernias on the state of the body. In the first case, the hernia is localized in the nasal cavity, and after its removal, the postoperative cerebrospinal fluid (CSF) leakage was stopped by a flap of the mucous membrane from the opposite side of the nasal septum. In the second case clinical analysis and computed tomography made it possible to state that the hernia was in the retromaxillary space and did not affect the patient's condition. Computed tomography shows signs of moderate blood pressure on the adjacent formations, and removal of the hernia and stopping the subsequent CSF leakage were impossible. The presented observations demonstrate an ambiguous approach to resolving the issue of surgical intervention in such cases.

Comparison of Outcomes of Surgical Repair of Spontaneous Temporal Bone CSF Leaks and Encephaloceles Using Bone Cement and Autologous Material.

OBJECTIVE: To compare outcomes transmastoid repair of spontaneous middle fossa cerebrospinal fluid (CSF) leak using only bone cement (BC) versus only autologous material (AM) or combined materials (CM) with both bone cement and autologous material. STUDY DESIGN: Retrospective Chart Review. SETTING: Tertiary Care Hospital. PATIENTS: Forty-three adult patients undergoing transmastoid repair of spontaneous middle fossa CSF leak between 2014 and 2020 (BC:12, AM:15, CR:16). INTERVENTIONS: Cortical mastoidectomy, identification of defect, and repair with BC (Cranios® hydroxyapatite), AM (local bone, fascia, fat, and/or cartilage), or CM (Cranios® combined with autologous materials). MAIN OUTCOME MEASURES: Successful repair without recurrent CSF leak or encephalocele throughout follow up. RESULTS: Fifty-one percent of subjects were female. Mean age at repair was 58.6 years (SD 10.9). Mean BMI was 35.4 (SD 7.6; BC:36.3, AM:36.5, CM:33.6). Forty (93%) patients had successful repair without known recurrent CSF leak or encephalocele since surgery (BC:11, 91.6%; AM:14, 93.3%; CM:15, 93.8%; p = 0.49) over a mean length of follow up of 49.6 months (BC: 37.9, AM: 59.2, CR: 49.5). The difference in mean operative time amongst the groups was faster for patients using bone cement (BC: 100.2 min, AM: 182.8, CM: 133.2; p < 0.00001). CONCLUSIONS: BC, AM, and CM techniques each demonstrate effective and sustained means of repair for middle fossa CSF leak and encephalocele, even in the presence of multiple defects. Use of isolated BC offers a significant decrease in operative time with a noninferior outcome. Active CSF leak at the time of surgery is associated with increased risk of recurrence.

An anatomical model for studying cerebellar tonsillar herniation related to raised intracranial pressure.

Brain herniation is one of the most feared complications of many neurological pathologies. However, current understanding of the mechanisms behind brain herniation syndromes is poor. By investigating the correlations between raised intracranial pressure (ICP) and herniation of the cerebellar tonsils, we hope to develop a model that can be used to study intracranial fluid dynamics and its effects on brain tissue. This will facilitate evaluation of patients with elevated ICP and development of novel treatments including surgical approaches for decompressing the posterior cranial fossa and upper cervical spine. A standard suboccipital surgical approach was used to expose the foramen magnum and observe movements of the cerebellar tonsils in fresh cadavers. A urinary Foley catheter balloon in the parietal extradural space was used to simulate a mass effect while ICP was monitored. The baseline anatomy differed widely among the cadaver specimens. However, and overall, we found that as ICP rises, the cerebellar tonsils descend through the foramen magnum at a rate of 0.3 mm per 1 mmHg increase in ICP. A mean descent of 6.2 mm was observed for a mean ICP increase of 17 mmHg. In this cadaveric study, we present a method and model for exploring brain herniation syndromes in the context of ICP changes. This could allow for further models to study the effects of other neuropathologies on the cerebellar tonsils, including posterior cranial fossa mass lesions and cerebellar hemorrhage.

Transnasal endoscopic surgery of sphenoid meningocele associated with pituitary adenoma. A case report.

BACKGROUND: Transsphenoid meningoencephalocele is a congenital anomaly formed by herniation of an ependyma delimited sac through a bony defect into the sphenoid sinus. The sac contains cerebrospinal fluid and neurovascular structures. The prevalence of transsphenoid meningoencephalocele in the adult population is rare. It usually manifests as nasal liquorrhoea. METHODS AND RESULTS: This case report presents an adult male who underwent surgery due to suspected pituitary macroadenoma. The surgery was performed endoscopically via the transnasal approach with a surprising finding of true transsphenoid meningoencephalocele. Ectopic solid tissue was found in the sphenoid sinus in which pituitary adenoma was histologically confirmed. CONCLUSION: This paper presents a previously unpublished combination of true transsphenoid meningoencephalocele and pituitary adenoma in an adult individual.

Impact of the volume of the myelomeningocele sac on imaging, prenatal neurosurgery and motor outcomes: a retrospective cohort study.

To investigate the association of the myelomeningocele (MMC) volume with prenatal and postnatal motor function (MF) in cases who underwent a prenatal repair. Retrospective cohort study (11/2011 to 03/2019) of 63 patients who underwent a prenatal MMC repair (37 fetoscopic, 26 open-hysterotomy). At referral, measurements of the volume of MMC was performed based on ultrasound scans. A large MMC was defined as greater than the optimal volume threshold (ROC analysis) for the prediction of intact MF at referral (2.7 cc). Prenatal or postnatal intact motor function (S1) was defined as the observation of plantar flexion of the ankle based on ultrasound scan or postnatal examination. 23/63 participants presented a large MMC. Large MMC lesions was associated with an increased risk of having clubfeet by 9.5 times (CI%95[2.1-41.8], p < 0.01), and reduces the chances of having an intact MF at referral by 0.19 times (CI%95[0.1-0.6], p < 0.01). At birth, a large MMC reduces the chance of having an intact MF by 0.09 times (CI%95[0.01-0.49], p < 0.01), and increases the risk of having clubfeet by 3.7 times (CI%95[0.8-18.3], p = 0.11). A lower proportion of intact MF and a higher proportion of clubfeet pre- or postnatally were observed in cases with a large MMC sac who underwent a prenatal repair.Trial registration: Clinicaltrials.gov NCT02230072 and NCT03794011 registered on September 3rd, 2014 and January 4th, 2019.

Temporal encephaloceles in epilepsy patients and asymptomatic cases: Size may indicate epileptogenicity.

OBJECTIVE: This study was undertaken to identify temporal encephaloceles (TEs) and examine their characteristics in patients with temporal lobe epilepsy (TLE) and extratemporal lobe epilepsy (ETLE), as well as in asymptomatic cases. METHODS: Four hundred fifty-eight magnetic resonance imaging scans were examined retrospectively to identify TE in 157 patients with TLE, 150 patients with ETLE, and 151 healthy controls (HCs). RESULTS: At least one TE was identified in 9.6% of the TLE patients (n = 15, 95% confidence interval [CI] = 5.3%-15.3%), in 3.3% of patients with ETLE (n = 5, 95% CI = 1.1%-7.6%), and in 2.0% of the HCs (n = 3, 95% CI = .4%-5.7%), indicating a significantly higher frequency in patients with TLE compared to ETLE and HC subjects (p = .027, p = .005). Examining the characteristics of TEs in both asymptomatic and epilepsy patients, we found that TEs with a diameter of less than 6.25 mm were more likely to be asymptomatic, with a sensitivity of 91.7% and a specificity of 73.3% (area under the curve = .867, 95% CI = .723-1.00, p = .001). SIGNIFICANCE: Temporal encephaloceles may occur without presenting any clinical symptoms. Patients with TLE show a higher frequency of TEs compared to the ETLE and HC groups. According to our study, TE size could be used to suggest potential epileptogenicity.

Posterior occipital gunshot wound causing orbital roof blow-in fracture with encephalocele.

Isolated orbital roof fractures are rare fractures that usually occur in conjunction with other facial bone fractures during high velocity trauma. This report concerns a patient with an isolated orbital roof fracture with encephalocele, including its diagnosis, surgical management, and clinical follow-up. This case required a multidisciplinary approach to safely repair the fracture, reduce the encephalocele, restore the orbital volume, and restore the patient's form and function.

Brain Herniation and Intracranial Hypertension.

This article introduces the basic concepts of intracranial physiology and pressure dynamics. It also includes discussion of signs and symptoms and examination and radiographic findings of patients with acute cerebral herniation as a result of increased as well as decreased intracranial pressure. Current best practices regarding medical and surgical treatments and approaches to management of intracranial hypertension as well as future directions are reviewed. Lastly, there is discussion of some of the implications of critical medical illness (sepsis, liver failure, and renal failure) and treatments thereof on causation or worsening of cerebral edema, intracranial hypertension, and cerebral herniation.

CSF rhinorrhoea post COVID-19 swab: A case report and review of literature.

We report the case of a 59 year old male who presented with 2 months of persistent rhinorrhoea from left nostril post a nasal swab done for coryzal symptoms at the peak of the COVID-19 pandemic. Beta-2-transferrin confirmed it to be a CSF leak and imaging showed a left middle cranial fossa encephalocele herniating into the sphenoid sinus as the site of the leak post swab. The leak was treated endoscopically. We describe the case history and management of this exceedingly rare complication of nasal swab for respiratory testing.

Intradiploic encephalocele following linear skull fracture: a rare evolution of growing skull fracture.

Growing skull fracture (GSF) is a rare complication of pediatric head trauma. It usually arises from a linear fracture with an underlying dural tear, which results in herniation of the intracranial contents. In rare cases, the herniated intracranial contents can be restricted in the diploe, resulting in an expanded diploic cavity. If there is brain parenchyma herniating into the expanded diploic cavity, the condition is termed as intradiploic encephalocele (IE). Here we present a peculiar pediatric occipital GSF case that resulted from the silent progression of a linear fracture with a small brain herniation into the widened fracture finally leading to an IE after approximately 7 years. Detailed imaging findings from the early injury to the late phase of the disease were provided to record this natural process. To the best of our knowledge, this is the first case providing the imaging data of early injury before developing into IE.

CSF Leak After COVID-19 Nasopharyngeal Swab: A Case Report.

The nasopharyngeal swab has been used with increased frequency since the beginning of the COVID-19 pandemic. Little has been written in the literature regarding the complications arising from this procedure, as it is generally accepted as safe. In this report, we describe a case in which a young woman sustained a traumatic skull base injury during a nasopharyngeal swab for COVID-19. We then discuss the subsequent treatment and outcome. This case demonstrates the potential for significant complications arising from this widespread procedure and the necessity for awareness of these potential complications. Laryngoscope, 131:1927-1929, 2021.

Effects of Primary Decompressive Craniectomy on the Outcomes of Serious Traumatic Brain Injury with Mass Lesions, and Independent Predictors of Operation Decision.

BACKGROUND: Although operative indications for traumatic brain injury (TBI) are known, neurosurgeons are unsure whether to remove the bone flap after mass lesion extraction, and an efficient scoring system for predicting which patients should undergo decompressive craniectomy (DC) does not exist. METHODS: Nine parameters were assessed. In total, 245 patients with severe TBI were retrospectively assessed from June 2015 to May 2019, who underwent DC or craniotomy to remove mass lesions. The 6-month mortality and Extended Glasgow Outcome Scale scores were compared between the DC and craniotomy groups. Using univariable and multivariable logistic regression equations, receiver operating characteristic curves were obtained for predicting the decision for DC. RESULTS: The overall 6-month mortality in the entire cohort was 11.43% (28/245). Patients undergoing DC had lower mean preoperative Glasgow Coma Scale scores (P = 0.01), and higher amounts of individuals with a Glasgow Coma Scale score of 6 (P = 0.007), unresponsive pupillary light reflex (P < 0.001), closed basal cisterns (P < 0.001), and diffuse injury (P = 0.025), compared with the craniotomy group. Because of high disease severity, individuals administered primary DC showed increased 6-month mortality compared with the craniotomy group. However, in surviving patients, favorable Extended Glasgow Outcome Scale rates were similar in both groups. Pupillary light reflex and basal cisterns were independent predictors of the DC decision. Based on receiver operating characteristic curves, the model had sensitivity and specificity of 81.6% and 84.9%, respectively, in predicting the probability of DC. CONCLUSIONS: These preliminary data showed that primary DC may benefit some patients with severe TBI with mass lesions. In addition, unresponsive preoperative pupil reaction and closed basal cistern could predict the DC decision.