Traumatic encephalocele in the nasal cavity after 6 years of trauma: a case report.

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. CASE PRESENTATION: 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. CONCLUSION: 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.

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.

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.

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.