Preemptive strategies and lessons learned from complications encountered with microvascular decompression for hemifacial spasm.

OBJECTIVE: Microvascular decompression (MVD) is the only curative treatment modality for hemifacial spasm (HFS). Although generally considered to be safe, this surgical procedure is surrounded by many risks and possible complications. The authors present the spectrum of complications that they met in their case series, the possible causes, and the strategies recommended to minimize them. METHODS: The authors reviewed a prospectively maintained database for MVDs performed from 2005 until 2021 and extracted relevant data including patient demographics, offending vessel(s), operative technique, outcome, and different complications. Descriptive statistics with uni- and multivariable analyses for the factors that may influence the seventh, eighth, and lower cranial nerves were performed. RESULTS: Data from 420 patients were obtained. Three hundred seventeen of 344 patients (92.2%) with a minimum follow-up of 12 months had a favorable outcome. The mean follow-up (standard deviation) was 51.3 ± 38.7 months. Immediate complications reached 18.8% (79/420). Complications persisted in only 7.14% of patients (30/420) including persistent hearing deficits (5.95%) and residual facial palsy (0.95%). Temporary complications included CSF leakage (3.10%), lower cranial nerve deficits (3.57%), meningitis (0.71%), and brainstem ischemia (0.24%). One patient died because of herpes encephalitis. Statistical analyses showed that the immediate postoperative disappearance of spasms and male gender are correlated with postoperative facial palsy, whereas combined vessel compressions involving the vertebral artery (VA) and anterior inferior cerebellar artery can predict postoperative hearing deterioration. VA compressions could predict postoperative lower cranial nerve deficits. CONCLUSIONS: MVD is safe and effective for treating HFS with a low rate of permanent morbidity. Proper patient positioning, sharp arachnoid dissection, and endoscopic visualization under facial and auditory neurophysiological monitoring are the key points to minimize the rate of complications in MVD for HFS.

Late Frontal Bone Reconstruction Using Three-Dimensional Printed Models for Titanium Mesh Customization: A Case Series.

BACKGROUND: The convex frontal bone is covered by thin skin, rendering its reconstruction cosmetically challenging. Customized alloplastic implants provide better contouring than autologous bone, yet their high cost and availability limit their application. We assess customized titanium mesh implants precontoured using patient-specific three-dimensional (3D) printed models for late frontal cranioplasty. METHODS: We retrospectively analyzed the prospectively collected cases of unilateral frontal titanium mesh cranioplasty with 3D printing-assisted preplanning from 2017 to 2019. We used two 3D-printed patient-specific skull models for preoperative planning: a mirrored normal model for implant contouring and a defect model for edge trimming and fixation planning. The endoscope was used in 4 cases for percutaneous mesh fixation. We documented postoperative complications. We assessed the reconstruction symmetry clinically, and radiologically on postoperative computed tomography. RESULTS: Fifteen patients were included. The duration after previous surgery ranged from 8 to 24 months. Four patients developed complications, which were managed conservatively. Favorable cosmetic outcomes were achieved in all patients. CONCLUSIONS: Precontouring of titanium mesh implants using in-house 3D-printed models could optimize cosmetic and surgical outcomes in late frontal cranioplasty. Preoperative planning could permit minimal access surgery, which could be aided by the endoscope in select cases.

Risk factors of congenital hydrocephalus: a case-control study in a lower-middle-income country (Egypt).

OBJECTIVE: Hydrocephalus is the most common brain disorder in children and is more common in low- and middle-income countries. Research output on hydrocephalus remains sparse and of lower quality in low- and middle-income countries compared with high-income countries. Most studies addressing hydrocephalus epidemiology are retrospective registry studies entailing their inherent limitations and biases. This study aimed to investigate child-related, parental, and socioeconomic risk factors of congenital hydrocephalus (CH) in a lower-middle-income country. METHODS: An investigator-administered questionnaire was used to query parents of patients with CH and controls who visited the authors' institution from 2017 until 2021. Patients with secondary hydrocephalus and children older than 2 years of age at diagnosis were excluded. Uni- and multivariable logistic regression was performed to identify the factors affecting CH development. RESULTS: Seven hundred forty-one respondents (312 cases and 429 controls) were included in this study. The authors showed that maternal diseases during pregnancy (OR 3.12, 95% CI 1.96-5.03), a lack of periconceptional folic acid intake (OR 1.92, 95% CI 1.32-2.81), being a housewife (OR 2.66, 95% CI 1.51-4.87), paternal illiteracy (OR 1.65, 95% CI 1.02-2.69), parental consanguinity (OR 3.67, 95% CI 2.40-5.69), a history of other CNS conditions in the family (OR 2.93, 95% CI 1.24-7.34), conceiving a child via assisted fertilization techniques (OR 3.93, 95% CI 1.57-10.52), and the presence of other congenital anomalies (OR 2.57, 95% CI 1.38-4.87) were associated with an independent higher odds of a child having CH. Conversely, maternal hypertension (OR 0.22, 95% CI 0.09-0.48), older maternal age at delivery (OR 0.93, 95% CI 0.89-0.97), and having more abortions (OR 0.80, 95% CI 0.67-0.95) were negatively correlated with CH. CONCLUSIONS: Multiple parental, socioeconomic, and child-related factors were associated with higher odds for developing CH. These results can be utilized to guide parental counseling and management, and direct social education and prevention programs.

An eye on the future for defeating hydrocephalus, ciliary dyskinesia-related hydrocephalus: review article.

Congenital hydrocephalus affects approximately one in 1000 newborn children and is fatal in approximately 50% of untreated cases. The currently known management protocols usually necessitate multiple interventions and long-term use of healthcare resources due to a relatively high incidence of complications, and many of them mostly provide a treatment of the effect rather than the cause of cerebrospinal fluid flow reduction or outflow obstruction. Future studies discussing etiology specific hydrocephalus alternative treatments are needed. We systematically reviewed the available literature on the effect of ciliary abnormality on congenital hydrocephalus pathogenesis, to open a discussion on the feasibility of factoring ciliary abnormality in future research on hydrocephalus treatment modalities. Although there are different forms of ciliopathies, we focused in this review on primary ciliary dyskinesia. There is growing evidence of association of other ciliary syndromes and hydrocephalus, such as the reduced generation of multiple motile cilia, which is distinct from primary ciliary dyskinesia. Data for this review were identified by searching PubMed using the search terms 'hydrocephalus,' 'Kartagener syndrome,' 'primary ciliary dyskinesia,' and 'immotile cilia syndrome.' Only articles published in English and reporting human patients were included. Seven studies met our inclusion criteria, reporting 12 cases of hydrocephalus associated with primary ciliary dyskinesia. The patients had variable clinical presentations, genetic backgrounds, and ciliary defects. The ependymal water propelling cilia differ in structure and function from the mucus propelling cilia, and there is a possibility of isolated non-syndromic ependymal ciliopathy causing only hydrocephalus with growing evidence in the literature for the association ependymal ciliary abnormality and hydrocephalus. Abdominal and thoracic situs in children with hydrocephalus can be evaluated, and secondary damage of ependymal cilia causing hydrocephalus in cases with generalized ciliary abnormality can be considered.

A multivariable prediction model for recovery patterns and time course of symptoms improvement in hemifacial spasm following microvascular decompression.

BACKGROUND: Microvascular decompression (MVD) success rates exceed 90% in hemifacial spasm (HFS). However, postoperative recovery patterns and durations are variable. OBJECTIVE: We aim to study factors that might influence the postoperative patterns and duration needed until final recovery. METHOD: Only patients following de-novo MVD with a minimum follow-up of 6 months were included. Overall trend of recovery was modeled. Patients were grouped according to recognizable clinical recovery patterns. Uni- and multivariable analyses were used to identify the factors affecting allocation to the identified patterns and time needed to final recovery. RESULTS: A total of 323 (92.6%) patients had > 90% symptom improvement, and 269 (77.1%) patients had complete resolution at the last follow-up. The overall trend of recovery showed steep remission within the first 6 months, followed by relapse peaking around 8 months with a second remission ~ 16 months. Five main recovery patterns were identified. Pattern analysis showed that evident proximal indentation of the facial nerve at root exit zone (REZ), males and facial palsy are associated with earlier recovery at multivariable and univariable levels. anterior inferior cerebellar artery (AICA), AICA/vertebral artery compressions and shorter disease durations are related to immediate resolution of the symptoms only on the univariable level. Time analysis showed that proximal indentation (vs. distal indentation), males and facial palsy witnessed significantly earlier recoveries. CONCLUSION: Our main finding is that in contrast to peripheral indentation, proximal indentation of the facial nerve at REZ is associated with earlier recovery. Postoperative facial palsy and AICA compressions are associated with earlier recoveries. We recommend a minimum of 1 year before evaluating the final outcome of MVD for HFS.

Soft tissue density within the foramen magnum, a predictor for surgical intervention in pediatric patients with Chiari malformation type I.

OBJECTIVE: Chiari malformation type I (CMI) is diagnosed as herniation of the cerebellar tonsils by at least 5 mm below the foramen magnum. However, the degree of tonsillar herniation is a poor predictor of the need for decompression surgery. Exploration for an alternative morphological predictor for surgical intervention could provide greater insight into the development of an appropriate treatment plan for these patients. To investigate this issue, the authors calculated the soft tissue density within the foramen magnum as a measure of impaction of the cerebellar tonsils. Soft tissue density within the foramen magnum and degree of tonsillar herniation were then assessed for their correlation with the need for surgical intervention. METHODS: The authors conducted a retrospective, longitudinal chart review of pediatric patients with CMI. Those who had undergone surgical intervention were considered symptomatic and those who had been treated conservatively, as asymptomatic. Soft tissue density was found by dividing the soft tissue occupancy of the foramen magnum (brainstem and cerebellar tonsils) by the total area of the bony foramen magnum. The predictive value of these two measurements for the need of surgery was determined. RESULTS: Of the 465 patients seen for CMI at the authors' institution between July 1, 2011, and May 31, 2017, 80 underwent surgical intervention and 385 were asymptomatic. The average tissue density was significantly greater in the surgical group than in the asymptomatic group (83.3% and 78.6%, respectively, p < 0.0001). The average tonsillar descent for surgical patients was 10.8 mm compared to 9.8 mm for asymptomatic patients (p = 0.140). The point-biserial correlation coefficient was assessed, and soft tissue density was found to positively correlate with the need for surgical intervention (rpb = 0.199, p = 0.0001), whereas tonsillar herniation did not correlate with the need for surgery (rpb = 0.083, p = 0.115). Additionally, the degree of tonsillar herniation did not correlate with soft tissue density (r = 0.09), indicating that soft tissue density is an independent morphological parameter. CONCLUSIONS: The study findings suggest that the need for surgical intervention in CMI patients is positively correlated with increasing soft tissue density within the foramen magnum, whereas the degree of tonsillar herniation did not show a correlation with the need for surgical intervention. Additionally, soft tissue density is a factor independent of the degree of tonsillar herniation. Further investigation of tissue density within the foramen magnum is needed in the hope of discovering a clinically applicable parameter that would indicate a need for surgical intervention in patients with CMI.

Visual changes after space flight: is it really caused by increased intracranial tension? A systematic review.

INTRODUCTION: Spaceflight-Associated Neuro-ocular Syndrome (SANS) was linked to increased intracranial pressure (ICP) attributable to the combined effects of microgravity and environmental conditions encountered during spaceflight. Microgravity countermeasures as lower body negative pressure (LBNP) are potential interventions for SANS. Our aim is to provide a comprehensive qualitative analysis of literature contrasting simulation and spaceflight studies, focusing on the pathophysiology of SANS, and highlighting gaps in current knowledge. EVIDENCE ACQUISITION: We systematically searched PubMed electronic database for English primary research published until February 2019 discussing intracranial changes in spaceflight or simulated microgravity, excluding animal and experimental studies. Two authors screened all the abstracts with a third author resolving disagreements. The full-text manuscripts were analyzed in pilot-tested tables. EVIDENCE SYNTHESIS: Nineteen studies were reviewed; 13 simulation, and two out of six spaceflight studies were prospective. ICP changes were investigated in 11 simulation studies, where eight demonstrated a significant increase in ICP after variable periods of head-down tilt. three showed a significant increase in intraocular pressure (IOP) in conjunction with ICP elevation. With increasing ambient CO2: one showed an increase in IOP without further increase in ICP, while another showed a slight further decrease in ICP. LBNP demonstrated no significant effect on ICP in one and a decrease thereof in another study. After spaceflight, increased ICP on lumbar puncture was demonstrated in five studies. CONCLUSIONS: Exposure to microgravity increases ICP possibly precipitating ocular changes. Whether other factors come into play is the subject of investigation. Further randomized studies and methods of direct ICP measurement during spaceflight are needed.

Effects of growth hormone therapy in pediatric patients with growth hormone deficiency and Chiari I malformation: a retrospective study.

PURPOSE: The safety and efficacy of growth hormone replacement therapy (GHRT) on pediatric patients with growth hormone deficiency (GHD) and Chiari I malformation (CIM) are not well investigated within the current body of literature. With no clear indication of the effects of GHRT on CIM disease progression, we sought to determine the effect of GHRT on tonsillar herniation and progression of CIM symptomatology. METHODS: From a previously established database of 465 patients with radiologically confirmed CIM defined as > 5 mm of tonsillar descent on head magnetic resonance imaging (MRI), we identified 20 patients who also had GHD. Using the imaging analysis software package, ANALYZE, the degree of change in tonsillar herniation was documented between initial and final MRI measurements. The radiologic and clinical changes over time were examined via a proportional odds model, Student's t test, Mann-Whitney test, or a mixed model corresponding to the outcomes measured either on an ordinal scale or on a quantitative scale. RESULTS: Incidence of GHD in our CIM population was 4.3%. There was no significant effect of GHRT on the degree of tonsillar herniation in patients with GHD and CIM. No patient became symptomatic, developed syringomyelia, or required surgical intervention for CIM. CONCLUSION: Based on our findings with a larger sample size, along with recent reports, the incidence of patients with CIM and GHD we reported (0.86-5%) is likely more indicative of the actual incidence of GHD and CIM than the prior findings within the literature (9.1-20%). We also suggest that GHRT does not significantly affect CIM morphology or symptomatology. Therefore, neurosurgeons should have no hesitation clearing these patients for GHRT.

Incidence of and Causes for Ventriculoperitoneal Shunt Failure in Children Younger Than 2 Years: A Systematic Review.

OBJECTIVES: Ventriculoperitoneal (VP) shunting is commonly used to treat pediatric hydrocephalus, but failure rates are high. VP shunt failure in children is mostly caused by infection and/or proximal/distal shunt obstruction. However, to our knowledge, no previous reviews have discussed this topic using only clinical studies when age-related data could be obtained. This systematic review aimed at reevaluating what is already known as the most common causes of shunt failure and to determine the incidence and causes of VP shunt failure during the first 2 years of life as a step to establish solid evidence-based guidelines to avoid VP shunt failure in infants. METHODS: We performed a search using the search terms "Cerebrospinal Fluid Shunts" (Medical Subject Headings [MeSH]) AND failure [All Fields] AND ("humans" [MeSH] AND English [lang] AND "infant" [MeSH]). Only articles that specifically discussed VP shunt complications in children < 2 years were included. RESULTS: We found that the most common causes of VP shunt failure in children < 2 years were shunt obstruction and infection, both observed in a range. CONCLUSION: VP shunt failure is very common in infants, mostly resulting from obstruction and infection. Future studies should focus on methods designed to avoid these complications or on alternative treatments for hydrocephalus.

C5 palsy following posterior decompression and instrumentation in cervical stenosis: Single center experience and review.

OBJECTIVE: Causation and avoidance of C5 palsy after laminectomy have proven elusive, with multiple factors incriminated including width of the laminectomy, spinal cord migration, C5 neural foraminal stenosis, or intraoperative C5 root traction. In an attempt to identify risk factors for C5 palsy after decompression in cervical stenosis and myelopathy, the following review was conducted. This report is from a single center with consistent criteria for diagnosis and management of cervical stenosis and myelopathy. PATIENTS AND METHODS: We retrospectively reviewed 63 patients with cervical stenosis and myelopathy who had been treated with laminectomy with instrumentation at the C4-6 level. Imaging studies reviewed included plain X-ray films, magnetic resonance imaging (MRI), and computed tomography (CT) scans of the cervical spine. Health-related outcomes were assessed before and at follow-up and included Visual Analog Scale (VAS) for pain (1-10), Japanese Orthopedic Association (JOA) score for myelopathy (0-18), and SF-36 physical functioning, energy and fatigue, and general health categories (0-100). RESULTS: In 53 patients (control group), decompression and instrumentation was accomplished without incident, but 5 patients developed lasting postoperative C5 palsy. At follow-up, there were overall significant improvements in VAS, JOA, and SF-36 physical functioning and general health domains. Subsequent to surgery, a loss of lordosis of 5° and an increase in C2 sagittal vertical axis (SVA) of 17 mm was significant. There was, however, no significant difference between control and C5 palsy patients in lordosis and C2 SVA, before or after surgery. Postoperative MRI studies were obtained in 15 of the control patients and 6 of the C5 palsy patients. Postoperative width of the laminectomy as well as the caliber of the C5 neural foramina in the control and C5 palsy cohorts were not statistically different. Though the posterior displacement of the cord in the C5 palsy cohort was larger than in controls, this difference was also not significant. CONCLUSION: The above findings suggest that the cause of C5 palsy remains elusive. Though our incidence of lasting C5 palsy subsequent to laminectomy and instrumentation was 8%, it is probably under-reported. In our experience, laminectomy and instrumentation failed to increase lordosis and, in fact, were associated with an increase in positive cervical balance. Complications with cervical laminectomy and instrumentation are not by any means rare, and need to be emphasized in counselling patients, and selecting the approach.

Intraventricular extension of an aneurysmal subarachnoid hemorrhage is an independent predictor of a worse functional outcome.

OBJECTIVE: The objective of this study is to determine the impact of intraventricular hemorrhage (IVH) on the cognitive prognosis of subarachnoid hemorrhage (SAH) due to ruptured cerebral aneurysm, independent of the presence of intraparenchymal hemorrhage, hydrocephalus or vasospasm. PATIENT AND METHODS: A Retrospective review of a prospectively collected database of patients with aneurysmal SAH from July 2009 to November 2016 was performed. Patients were included if they had a saccular aneurysm with a Hunt-Hess grade (HHG) 1-3. Those who underwent craniectomy/clipping and those with vasospasm were excluded. Patients with IVH were grouped into 5 groups depending on the blood distribution in the ventricles. Functional outcomes studied were modified Rankin score (mRS) 0-2, cognitive impairment and memory impairment, and the presence of amnesia to the event. A univariate followed by a multivariate analysis ware performed. RESULTS: A total of 443 patients were identified and 124 patients met the criterion. There were no significant differences in the proportion of patients with mRS of 0-2 between patients with IVH and those without IVH but with EVD (external ventricular drain). There was a higher proportion of cognitive deficits in patients with IVH (71.95%), compared to those without (31.58%; p = 0.01). Patients with IVH had a higher rate of anterograde amnesia (100% vs. 4.3% p < 0.0001), lower rate of mRS 0-2 (78% vs 100% p < 0.001), and higher rate of cognitive impairment (71.9% vs. 13% p < 0.0001) compared with those who did not require an EVD. Grade 3 and grade 4 were shown to have lower rate of patients with mRS 0-2 and a higher rate of cognitive impairment. In multivariate analysis, independent predictors of cognitive and memory impairment were increasing HHG (OR = 155.33; P < 0.01), ACOM/A1/ACA/anterior choroidal aneurysms, (OR = 5.24; P = 0.04), increasing Fischer scale (OR = 6.93; P = 0.01), and increasing IVH grade (OR = 6.9; P = 0.01). Only worse HHG (OR = 2704.22; P = 0.01) and IVH grade 2-4 were associated (perfect predictor, OR cannot be extracted) with anterograde amnesia. CONCLUSION: IVH is an independent prognosticator of SAH cognitive outcomes. The effect of IVH drainage and other intraventricular therapies on SAH course is an attractive topic for further investigation.

Emergency Optic Canal Decompression for Vision Salvage in Fibrous Dysplasia.

BACKGROUND: The most common neurologic manifestations of fibrous dysplasia (FD) are vision and hearing loss. Optic decompression for progressive vision loss has been shown to yield positive results in terms of visual outcomes; however, emergency optic decompression surgery for sudden loss of vision in FD has not to date been reported in the pediatric population. We report the first case of FD presenting with sudden vision loss and successfully managed with emergency optic decompression. CASE DESCRIPTION: A 10-year-old male patient known to have FD with a 2-month history of progressive vision loss in his left eye presents with sudden blindness. Emergency decompression surgery restored vision in his left eye to 20/40, which remained stable on long-term follow-up. CONCLUSION: There is no consensus on the benefit and safety of prophylactic optic decompression, yet therapeutic decompression has been shown to prevent vision deterioration. Our findings suggest that therapeutic decompression even when done in the emergency setting yields positive results, while prophylactic decompression carries an inherent risk for loss of vision in a seeing eye.

Development of an Arabic version of the National Eye Institute Visual Function Questionnaire as a tool to study eye diseases patients in Egypt.

AIM: To develop and test an Arabic version of the National Eye Institute Visual Function Questionnaire-25 (NEI-VFQ-25). METHODS: NEI-VFQ-25 was translated into Arabic according to WHO translation guidelines. We enrolled adult consenting patients with bilateral chronic eye diseases who presented to 14 hospitals across Egypt from October to December 2012, and documented their clinical findings. Psychometric properties were then tested using STATA. RESULTS: We recruited 379 patients, whose mean age was (54.5±15)y. Of 46.2% were males, 227 had cataract, 31 had glaucoma, 23 had retinal detachment, 37 had diabetic retinopathy, and 61 had miscellaneous visual defects. Non-response rate and the floor and ceiling numbers of the Arabic version (ARB-VFQ-25) were calculated. Internal consistency was high in all subscales (except general health), with Cronbach-α ranging from 0.702-0.911. Test-retest reliability was high (intraclass correlation coefficient 0.79). CONCLUSION: ARB-VFQ-25 is a reliable and valid tool for assessing visual functions of Arabic speaking patients. However, some questions had high non-response rates and should be substituted by available alternatives. Our results support the importance of including self-reported visual functions as part of routine ophthalmologic examination.