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OBJECTIVE The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

OBJECTIVE There is little research on the effect of social determinants of health on Chiari malformation type I (CM-I). The authors analyzed data on all children evaluated for CM-I at a single institution to assess how socioeconomic factors and race affect the surgical treatment of this population. METHODS Medical records of patients treated for CM-I at the authors’ institution between 1992 and 2017 were reviewed. Area Deprivation Index (ADI) and Rural-Urban Commuting Area (RUCA) codes for each patient were used to measure neighborhood disadvantage. Non-Hispanic White patients were compared to non-White patients and Hispanic patients of any race (grouped together as non-White in this study) in terms of insurance status, ADI, and RUCA. Patients with initially benign CM-I, defined as not having undergone surgery within 9 months of their initial visit, were then stratified by having delayed symptom presentation or not, and compared on these same measures. RESULTS The sample included 665 patients with CM-I: 82% non-Hispanic White and 18% non-White. The non-White patients were more likely to reside in disadvantaged (OR 3.4, p < 0.001) and urban (OR 4.66, p < 0.001) neighborhoods and to have public health insurance (OR 3.11, p < 0.001). More than one-quarter (29%) of patients underwent surgery. The non-White and non-Hispanic White patients had similar surgery rates (29.5% vs 28.9%, p = 0.895) at similar ages (8.8 vs 9.7 years, p = 0.406). There were no differences by race/ethnicity for symptoms at presentation. Surgical and nonsurgical patients had similar ADI scores (3.9 vs 4.2, p = 0.194), RUCA scores (2.1 vs 2.3, p = 0.252), and private health insurance rates (73.6% vs 74.2%, p = 0.878). A total of 153 patients underwent surgery within 9 months of their initial visit. The remaining 512 were deemed to have benign CM-I. Of these, 40 (7.8%) underwent decompression surgery for delayed symptom presentation. Patients with delayed symptom presentation were from less disadvantaged (ADI 3.2 vs 4.2; p = 0.025) and less rural (RUCA 1.8 vs 2.3; p = 0.023) areas than those who never underwent surgery. CONCLUSIONS Although non-White patients were more likely to be socioeconomically disadvantaged, race and socioeconomic disadvantage were not associated with undergoing surgical treatment. However, among patients with benign CM-I, those undergoing decompression for delayed symptom presentation resided in more affluent and urban areas.

Study Design: Retrospective study.Purpose: This study was undertaken to compare the patterns of syringomyelia in patients with presumed idiopathic and congenital scoliosis.Overview of Literature: The incidence of neuraxial anomalies presenting as idiopathic scoliosis ranges from 2% to 14%; the common ones are idiopathic syringomyelia (IS) and Chiari malformation type 1 (CM1) with syringomyelia. Some authors have speculated that scoliosis is caused by the asymmetrical compression of anterior horn cells by a syrinx, which causes an imbalance of the trunk musculature. In congenital scoliosis, syringomyelia is the second commonest cord anomaly, and the deformity progression depends upon the underlying vertebral abnormality, the location of the abnormality, and the age of patient, and is independent of the intraspinal anomaly.Methods: We analyzed the radiological records of 44 consecutive patients with scoliosis and syringomyelia. Of these 44 patients, 13 had IS, 12 had CM1, and 19 had congenital scoliosis. The radiographs were evaluated to determine the curve magnitude, sagittal alignment, side of convexity, and type of vertebral anomaly, if any. T1- and T2-weighted magnetic resonance imaging of the whole spine was analyzed to determine the presence of craniovertebral anomalies, syrinx length, syrinx diameter, and syrinx-cord ratio (SCR).Results: The frequency of left convex curves was 26.1%, with no significant differences across the three groups. The mean length of the syrinx was 7.2±4.9 vertebral levels, and the mean SCR was 0.39±0.2. The mean syrinx length was significantly higher in patients with CM1 and IS, compared to patients with congenital scoliosis. The mean SCR was highest in patients with CM1. In congenital scoliosis, syringomyelia was seen most frequently in patients with a failure of formation, and 63.2% had concomitant cord anomalies.Conclusions: Syrinxes were smaller in size and length in patients with congenital scoliosis, and are distinct from those seen in IS and CM1.

The most common congenital intraspinal abnormalities presenting to medical attention is either a Chiari malformation with or without associated syringomyelia or caudal anomalies, such as tethered cord and fatty filum terminale. These intraspinal defects are usually diagnosed at birth or in early childhood, but they may also be identified in adult life due to newly presented neurological symptoms. Availability and use of appropriate imaging modalities, such as MRI and regular fetal screening has reduced the incidence of delayed diagnosis of spinal dysraphism and other accompanying congenital systemic anomalies. In view of the risk for neurological complications, prophylactic surgery is often recommended in spinal dysraphism; hence knowledge of various intraspinal abnormalities, their clinical presentation and natural history will allow early diagnosis and treatment in order to prevent neurological deterioration and irreversible morbidity. This review summarizes the embryological aspects and clinical manifestations of intraspinal abnormalities and provides a rational approach for evaluation and management in this group of patients.

This CASE report presents a 16-year-old female volleyball, basketball and track & field athlete who was diagnosed with a Chiari I Malformation following a concussion. Surgical decompression was recommended and performed 3 months following her initial diagnosis. This patient presented unique challenges due to her age, desire to return-to-sport, and the lack of access to medical care due to living in a rural area. There are few evidence-based best-practice recommendations for the management and return-to-sport of Chiari I Malformation patients, particularly for post-surgical Chiari I Malformation cases. This case study discusses the treatment and return-to-sport process for the patient, and also provides a comprehensive review of the published literature on patients attempting to return-to-sport following Chiari I Malformation diagnosis. Additionally, this case report suggests and explores the utilization of an athletic trainer to reconcile various barriers in management and return-to-sport evident in this case and the reviewed literature.

This case report presents a female infant whose chief complaint of choking episodes resulted in a diagnosis of Chiari malformation type I and subsequent neurosurgical decompression. Although feeding difficulties and irritability are common complaints in pediatric primary care, dysphagia requires a more significant workup to identify a cause. This case report highlights red flags in the history and diagnostic studies and provides details for managing an unusual presentation of Chiari malformation type I.

BACKGROUND Foramen magnum decompression (FMD) is an established technique for patients with Chiari-1 malformation. However, the extent of decompressive surgery is controversial, in particular whether dural opening is undertaken. OBJECTIVE In this study, we report outcomes with our technique utilising intraoperative ultrasound (iOU) findings. Decision to proceed for further decompression was based on CSF flow around foramen magnum and visualisation of tip of tonsils. METHODS Review of adults with CM-1 undergoing FMD 2013-2018. Patients underwent a suboccipital craniectomy ± partial C1 superior laminectomy ± duraplasty in a stepwise manner. Favourable iOU findings of CSF flow around the foramen magnum and pulsation of the tonsillar tips were sought at each step. Post-operative outcomes were assessed using the Chicago Chiari Outcome Score (CCOS). RESULTS Fifty-seven patients were included. The mean age was 47 years (range 18-77 years). There was a strong female preponderance (48 females to nine males). The mean follow-up was 3.2 years (range 1-5 years). Surgery included: suboccipital craniectomy (52/57, 91%), plus partial C1 superior laminectomy (2/57, 4%), and plus duraplasty (3/57, 6%). Redo surgery was undertaken in 7/57 patients (12%). At final follow-up, 56/57 (98%) patients achieved a good (CCOS 13-16) or satisfactory (CCOS 9-12) outcome based on COSS. CONCLUSIONS A patient centred approach to surgery utilising findings from iOU can achieve favourable outcomes. Dural opening is not routinely required.

To evaluate the effectiveness of brace treatment in patients with Chiari malformation type 1 (CM-1) or syringomyelia associated scoliosis without neurosurgical intervention. This was a retrospective case–control study. 34 CM-1 or syringomyelia (CMS) patients who received brace treatment without neurosurgical intervention were recruited. Another 68 matched patients with idiopathic scoliosis who received bracing served as the control group. The matching criteria included gender, age (± 1 years), Risser sign (± 1 grade), initial curve magnitude (± 5°), curve patterns and follow-up time (± 6 months). Patients who encountered curve progression and scoliosis surgery were compared between different groups. Until the last visit, 16 (47%) patients in CMS group and 18 (26%) patients in IS group occurred curve progression; 9 (26%) patients and 15 (22%) patients underwent scoliosis surgery, respectively. Compared to idiopathic scoliosis, patients with CMS-associated scoliosis had a significantly higher rate of curve progression (P = 0.038). However, no significant difference was observed between two groups regarding to the rate of surgery (P = 0.867). Patients with combined CM-1 and syringomyelia had a higher rate of surgery than patients with isolated CM-1 or syringomyelia (P = 0.049). The double major curve pattern was identified as the risk factor for curve progression. Brace treatment is effective for CMS-associated scoliosis without neurosurgical intervention. Compared to idiopathic scoliosis, brace can provide similar prevention for scoliosis surgery in CMS patients, but slight or moderate curve progression may occur. Specifically, patients with combined CM-1 and syringomyelia should be followed closely with a higher expectation of curve progression.

BACKGROUND Chiari I malformations secondary to other causes represent a small subset of presenting symptomatic cases. Typically, the primary cause of the malformation is addressed first and results in resolution of the malformation and symptoms. However, in some cases, a patient may present with both a primary Chiari I malformation and another unrelated neurosurgical lesions. OBJECTIVE To present a unique case in which resection of a ventral tumor allowed for spontaneous resolution of a simultaneously noted dorsal Chiari I malformation. METHODS Pertinent data, including presenting symptoms, hospital course, surgical notes, preoperative images, and postoperative images, were collected using the electronic medical record. RESULTS We present a case of a 46-yr-old man with a Chiari I malformation in conjunction with a ventral cranio-cervical junction chordoma. Endoscopic endonasal resection of the chordoma and ventral foramen magnum decompression resulted in radiographic resolution of the Chiari malformation and resolution of his symptoms. Our report represents a rare case of ventral foramen magnum decompression as a treatment for Chiari I malformation. CONCLUSION It is felt that the chordoma mass effect was not the source of the Chiari I malformation. Thus, both ventral and dorsal decompressions of the posterior fossa may be considered for Chiari I decompression in select circumstances.

Background Idiopathic intracranial hypertension is a disorder of increased intracranial pressure in the absence of cerebrospinal outflow obstruction, mass lesion, or other underlying cause. It is a rare phenomenon in prepubertal children and is most typically found in women of childbearing age. The classic presentation consists of headaches, nausea, vomiting, and visual changes; however, children present more atypically. We report a case of idiopathic intracranial hypertension in an otherwise healthy, 4-year-old child with atypical symptoms resembling those of cyclic vomiting syndrome. Case presentation A 4-year-old Caucasian, otherwise healthy, male child presented to our emergency department with episodic intermittent early-morning vomiting occurring once every 1–3 weeks without interepisodic symptoms, starting 10 months prior. With outpatient metabolic, autoimmune, endocrine, allergy, and gastroenterology work-up all unremarkable, he was initially diagnosed with cyclic vomiting syndrome. Discovery of mild optic nerve sheath distension on magnetic resonance imaging of the brain 10 months after symptom onset led to inpatient admission and a lumbar puncture notable for an opening pressure of 47 mmHg, with normal cell count and protein levels. He had no changes in visual acuity or optic disc edema on dilated fundoscopic examination. The patient was started on acetazolamide, with resolution of episodic emesis at his last follow-up visit 12 weeks after discharge. Conclusions Idiopathic intracranial hypertension presents atypically in prepubescent children, with about one-fourth presenting asymptomatically, and only 13–52% presenting with “classic” symptoms. With a prevalence of only 0.6–0.7 per 100,000, much remains unknown regarding the underlying pathophysiology in this demographic. Cyclic vomiting syndrome, however, has a much higher prevalence in this age group, with a prevalence of 0.4–1.9 per 100. It is thought to be an idiopathic, periodic disorder of childhood, often linked to neurological conditions such as abdominal migraines, epilepsy, mitochondrial disorders, and structural lesions such as chiari malformation and posterior fossa tumors. While cyclic vomiting syndrome is thought to have a benign course, untreated idiopathic intracranial hypertension can have long-term detrimental effects, such as visual loss or even blindness. We present a case of idiopathic intracranial hypertension presenting with symptoms resembling cyclic vomiting syndrome in a 4-year-old child, diagnosed 10 months after initial onset of symptoms. We aim to demonstrate the need for a high level of clinical suspicion and the need for further investigation into underlying pathophysiology in this vulnerable population.

Sleep-disordered breathing (SDB) is common in Chiari Malformation (CM) and Spina Bifida (SB) and can lead to adverse consequences if untreated. Therefore, screening is very important but the Pediatric Sleep Questionnaire (PSQ) has not been validated in this population. Further, there is limited data on the validity of this tool in children with central nervous system malformations. Retrospective chart review of CM and SB patients evaluated in our sleep center between 2008 and 2018. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the PSQ and several of its components were calculated to predict obstructive sleep apnea (OSA). A total of 149 patients met criteria for analysis. The majority were referred to a sleep specialist due to concern for SDB. OSA was found in 36% (53/149) of all patients. The sensitivity and specificity of the PSQ to predict OSA was 73.58% and 20.83%, respectively. The PPV was 33.91%, and the NPV was 58.82%. Specificity values were higher for PSQ as negative predictors of moderate or severe OSA. In this population, the sensitivity of PSQ for OSA is reasonable but lower than values described in other populations. The specificity and NPV are low. Even with a high prevalence of OSA, symptoms of SDB may overlap with those of other comorbidities leading to a low specificity. A PSQ could be used to prioritize which patients need a PSG more urgently than others. Further studies are needed to define an optimal cut-off value of the PSQ in this population.

Chiari malformation type 1 (CM1) is often found incidentally. However, patients with symptoms or signs referable to CM1 or an associated syrinx will likely benefit from surgical intervention. Patients who are not symptomatic from CM1 at presentation are unlikely to become symptomatic at follow-up.

Spontaneous third ventriculostomy (STV) is characterized by the spontaneous rupture of one of the ventricle walls due to increased pressure in the third ventricle caused by obstructive hydrocephalus. Clinically, STV results in resolution of signs and symptoms of intracranial hypertension and head circumference stabilization. No spontaneous STV cases in patients with myelomeningocele have been reported in the literature. The objective of this study was to report three cases of STV in patients with type 2 Chiari malformation who underwent intrauterine treatment. All patients presented clinically with increased head circumference during outpatient follow-up. Only one patient required a ventriculoperitoneal shunt implantation. The other patients did not require further intervention. STV is a rare entity that is difficult to diagnose and should always be suspected in spontaneous hydrocephalus resolution, especially in early childhood. STV is not synonymous with hydrocephalus resolution.

Sleep apnoea is common in patients with Basilar Invagination with Arnorld Chiari Malformation (ACM). Various studies have shown its incidence in the range of 60-70% among such patients. Most of the studies have shown improvement in sleep disturbances after decompressive surgeries for Chiari Malformations. There is no report of postoperative deterioration due to sleep apnoea in these patients. Authors report two cases of basilar invagination associated with ACM and Platybasia, who deteriorated probably due to worsening of pre-existing sleep disorders on 3rd and 7th postoperative days after their surgeries, despite clinico-radiological improvements during their early post-operative courses. Authors discuss literature related to sleep apnoea in basilar invagination associated with Chiari Malformations and share precautions, which are relevant and should be undertaken in such patients especially during early post-operative periods to avoid alarming complication which may occur even in experienced hands.

The role of an osseous-only posterior fossa decompression (PFD) for Chiari malformation type 1 (CM1) remains controversial. We reviewed long-term outcomes for patients with CM1 undergoing a PFD to evaluate if there was any difference for failure when compared to patients undergoing a PFD with duraplasty (PFDD). Consecutive patients surgically treated at a single tertiary pediatric neurosurgery clinic over a 25-year period with at least 5 years of follow-up were evaluated. PFD patients were compared to those that initially received a PFDD. Demographics, surgical indications, surgical approach, outcomes, and complications were reviewed. A total of 60 patients were included in this study of which 25 (41.67%) underwent PFD and 35 (58.33%) underwent PFDD. Mean age at surgery was 7.41 years (range 0.4 to 18 years) with a mean follow-up of 8.23 years (range 5 to 21 years). Those that received a PFD had a lower rate of radiographic syrinx improvement (p = 0.03), especially in the setting of holocord syringes. Failure rate was significantly higher in the PFD group (20% vs 2.90%, p = 0.03). However, complications were significantly higher in the PFDD group (17.14% vs 4.0%, p = 0.04). PFD provides a safe treatment option with similar clinical improvements and lower post-operative complication rate compared to PFDD, albeit at the cost of greater chance of reoperation, especially in the setting of a holocord syrinx. Patients with a holocord syrinx should be considered for a PFDD as their initial procedure.

Chiari malformation (CM) type 1 is characterized by descent of the cerebellar tonsils resulting from crowding of the posterior fossa. In 30% of cases, it is associated with syringomyelia. When symptomatic, it may result in a classic constellation of symptoms. Here we describe a case of a 16-year-old male who presented with isolated, unilateral foot drop due to CM type 1 and holosyrinx. This unique presentation is extremely rare, and we additionally present a review of all other reported cases in the literature. After undergoing posterior fossa decompression with C1 laminectomy and duraplasty, our patient made a complete neurological recovery within 2 weeks postoperatively and his MRI entire spine at 3 months postoperatively demonstrated a nearly complete resolution of the holosyrinx with significant decompression of the foramen magnum. This rare presentation highlights the importance of maintaining a broad differential, particularly in pediatric patients, and expediting the workup in order to offer a surgical decompression within 1–2 months of foot weakness to maximize the probability of a full neurological recovery.

Atlas assimilation (AA) may be associated with atlantoaxial dislocation, Chiari malformation (CM), and basilar invagination. The importance of AA in the context of craniocervical junction (CVJ) anomalies is unclear. Considering this context, this study's objective is to discuss the role of AA in the management of CVJ anomalies, especially in CM. A comprehensive literature review was performed. In addition, some illustrative cases were discussed on the basis of our review. Finally, we propose a theoretic algorithm to evaluate patients with AA and CM. AA is a proatlas segmentation anomaly that may be complete or incomplete. It may be totally asymptomatic or symptomatic as the result of transferred shifted forces onto the C1-2 joints, leading to clear instability (atlantoaxial dislocation) or mild C1-2 instability. Cautious surgical planning may be required due to associated vertebral artery anomalies. AA with concomitant C2-C3 segmentation failure is highly associated with late C1-C2 instability. CVJ decompression failure was reported in patients with CM and a low clivus canal angle (130-135 degrees). Patients with assimilated anterior C1 arches usually have evident AAD. CM patients with AA generally have type 1 BI or type 2 BI and are reported with higher rates of CVJ instabilities when compared with those "pure" CM. Dynamic examinations may provide additional evidence of atlantoaxial instability. Although AA per se is not considered an unstable configuration, further and detailed evaluations of patients with CM associated with AA are necessary. Some associated unstable configurations required concomitant CVJ fixation.

Background The best surgical treatment for adult Chiari malformation type 1 remains widely debated. Objective This study aimed to assess the efficacy of posterior fossa decompression and duraplasty with arachnoid preservation compared with arachnoid dissection for the treatment of adult Chiari malformation type 1. Methods Two reviewers (M.O.-G. and M.A.) performed a PubMed, MEDLINE, and Embase literature search using the following terms: (“Chiari” OR “Chiari 1”) AND (“duraplasty” OR “arachnoid preservation” OR “arachnoid spar∗” OR “posterior fossa surgery” OR “posterior fossa decompression” OR “foramen magnum decompression”). Studies assessing the efficacy of posterior fossa decompression with duraplasty for the treatment of patients aged >18 years with Chiari malformation type 1 were included. Case reports with Results Of 195 studies identified, 24 were included for meta-analysis. In the 1006 participants, there was no difference in postoperative clinical or radiologic improvement between the 2 techniques. Patients who underwent posterior fossa decompression with duraplasty and arachnoid dissection had a greater prevalence of total complications (0.20, 95% confidence interval [CI], 0.13–0.29 vs. 0.09, 95% CI, 0.05–0.14; Q = 6.47; P = 0.01) and cerebrospinal fluid–related complications (0.15, 95% CI, 0.10–0.22 vs. 0.05, 95% CI, 0.02–0.12; Q = 4.88; P = 0.03) compared with arachnoid preservation. Furthermore, the prevalence of reoperation in the arachnoid dissection group was 25 times greater than in the arachnoid preservation group (0.08, 95% CI, 0.06–0.10 vs. 0.003, 95% CI, 0.00–0.02; Q = 10.73; P > 0.001). Conclusions Posterior fossa decompression and duraplasty with arachnoid preservation is a beneficial technique to treat Chiari malformation type 1 and reduces the risk of complications, particularly cerebrospinal fluid–related complications and the rate of reoperation.

Syringomyelia (SM) with Chiari malformation is a rare disease with an unpredictable course. Surgery and other interventions help reduce the severity of symptoms, but over 50% patients require re-operation. Auto-resolution is rare in this type of SM, and most cases progress to complications, which may amount to a great burden. The patient of SM with Arnold-Chiari malformation type 1 in a 54-year-old Russian woman who was treated with individualized classical homeopathy for over eight years with remarkable improvement in the clinical signs and symptoms of the condition and comorbidities. On MRI, the syrinx completely resolved, which further confirmed the benefit of this therapy. This case of SM with Arnold-Chiari malformation type 1 seemed to benefit from individualized classical homeopathy. Scientific investigation into an individualized classical homeopathic approach towards SM is necessary to establish its relevance in this condition.

Introduction Arachnoid cysts are benign cerebrospinal fluid collection within a duplication of arachnoid membrane and, when found in the retrocerebellar site, they may be associated with tonsils herniation. This rare situation of coexisting retrocerebellar arachnoid cyst (AC) and Chiari malformation type 1 (CM-1) have been previously reported in few cases (10 patients) with syringomyelia and hydrocephalus described to be the most relevant issues. The aim of this paper is to describe 3 pediatric cases of this condition with a systematic review of the literature, underlining the importance of surgical management tailored to the pathogenic mechanism. Methods A restrospective analysis of patients treated for coexisting CM-1 and ACs at the authors' institution has been carried out. Results A case of a 10-month-old baby with coexisting AC and CM-1 with tri-ventricular hydrocephalus treated with endoscopic third ventriculostomy, a case of a 1-year-old child with a huge retrocerebellar AC and CM-1 treated with a cysto-peritoneal shunt, and a case of a 15-year-old child with retrocerebellar AC causing symptomatic CM-1 treated with C0-C2 decompression, AC fenestration and duraplasty are described. A long-term follow-up is reported. Conclusions Surgical management of coexisting ACs and CM-1 should not aim at the complete resolution of the cyst or of tonsil herniation, especially when pediatric patients are treated. Rather, the purpose of the neurosurgeon should be to understand the underlying pathogenic mechanism, and then restoring both the cerebrospinal fluid flow in the posterior fossa and the dynamic equilibrium between ventricles, cyst, and subarachnoid space.

Chiari malformation type I (CMI) provides an opportunity for examining possible moderators of allostatic load. CMI patients who had (n = 43) and had not (n = 19) undergone decompression surgery completed questionnaires regarding pain, disability, and loneliness, and provided serum samples for IL-6, CRP, estrogen, and free estradiol assays, and saliva samples to assess diurnal cortisol curves. ANOVAs examining surgical status (decompressed versus non-decompressed), loneliness (high vs. low), and disability (high vs. low) as independent variables and biomarker variables as dependent factors found that loneliness was associated with higher levels of cortisol, F(1, 37) = 4.91, p = .04, η2P = .11, and lower levels of estrogen, F(1, 36) = 7.29, p = .01, η2P = .17, but only in decompressed patients. Results highlight the possible impact of loneliness on biological stress responses and the need to intervene to reduce loneliness in patients with symptomatic CMI.

Surgery is the definitive treatment option for symptomatic Chiari malformation I (CMI), but there is no clear consensus as to the preferred surgical method. This study aimed to quantitatively assess and compare the effect and safety of dura splitting decompression (DSD) and posterior fossa decompression with duraplasty (PFDD) in treating patients with CMI.A literature search of EMBASE, MEDLINE, PubMed, Cochrane Library, and Web of Science databases was conducted. References from January 1990 to September 2020 were retrieved. We only included papers containing original data, comparing the use of DSD and PFDD in CMI patients.Overall, 11 relevant studies were identified, wherein 443 patients treated for CMI by DSD were compared with 261 patients treated by PFDD. No difference was observed between PFDD and PFD in terms of clinical improvement (P = 0.69), syringomyelia improvement (P = 0.90), or reoperation (P = 0.22). DSD was associated with shorter operation durations (P = 0.0007), shorter length of stay (P = 0.0007), and shorter overall postoperative complications (P0.0001) (especially cerebrospinal fluid [CSF] leak [P = 0.005], meningitis [P = 0.002], and pseudomeningocele [P = 0.002]), as compared with PFDD.This study confirmed that dura splitting decompression has clinical and syringomyelia improvement outcomes comparable to posterior fossa decompression with duraplasty. Compared with PFDD, DSD not only significantly shortened the operation time and length of stay, but also significantly reduced the overall complication rate, especially those related to incidence of CSF-related complications. More evidence from advanced multicenter studies are needed to require to validate the findings.

Objective Posterior fossa decompression is the treatment of choice in type 1 Chiari malformation (CM-1) without bony instability. Although surgical fixation has been recommended by a few authors recently, comparative studies to evaluate these treatment strategies using objective outcome tools are lacking. Methods Seventy-three patients with pure CM-1 (posterior fossa bony decompression [PFBD], n = 21; posterior fossa bony and dural decompression [PFBDD], n = 40; and posterior fixation [PF], n = 12) underwent a postoperative outcome assessment using Chicago Chiari Outcome Score (CCOS). Logistic regression analysis detected predictors of an unfavorable outcome. Results Minimally symptomatic patients generally underwent a PFBD while most of the clinically severe patients underwent a PFBDD (p = 0.049). The mean CCOS score at discharge was highest in the PF (12.0 ± 1.41) and lowest in PFBDD group (10.98 ± 1.73, p = 0.087). Patients with minimal preoperative clinical disease severity (adjusted odds ratio [AOR], 4.58; 95% confidence interval [CI], 1.29–16.31) and PFBDD (AOR, 7.56; 95% CI, 1.70–33.68) represented risks for an unfavorable short-term postoperative outcome. Though long-term outcomes (CCOS) did not differ among the 3 groups (p = 0.615), PFBD group showed the best long-term improvements (mean follow-up CCOS, 13.71 ± 0.95), PFBDD group improved to a comparable degree despite a poorer short-term outcome while PF had the lowest scores. Late deteriorations (n = 3, 4.1%) occurred in the PFBDD group. Conclusion Minimally symptomatic patients and PFBDD predict a poor short-term postoperative outcome. PFBD appears to be a durable procedure while PFBDD group is marred by complications and late deteriorations. PF does not provide any better results than posterior fossa decompression alone in the long run.

Hydrocephalus by the fourth ventricle outlet obstruction (FVOO) associated with a Chiari malformation type I and syringomyelia is a well-known entity but a rare situation in clinical practice. Although suboccipital craniectomy with the opening of the obstruction membrane appears to be the most physiological approach, by restoring the original pathway of cerebrospinal fluid flow, the endoscopic third ventriculostomy (ETV) represents an important minimally invasive alternative. We report the case of an adult patient with tetra ventricular hydrocephalus by FVOO associated with Chiari malformation and syringomyelia. The ETV alone completely resolved all symptoms, as well as neuroimaging abnormalities on the control magnetic resonance imaging. The ETV is a minimally invasive option for the treatment of hydrocephalus in patients with obstruction at the exit of the fourth ventricle, even in cases associated with Chiari malformation and syringomyelia.

STUDY DESIGN This was a retrospective cohort study. OBJECTIVE The objective of this study was to evaluate posterior fossa structural differences between Chiari type 1 malformation with and without syringomyelia, and assess data of the improvement of syringomyelia after different surgical options. SUMMARY OF BACKGROUND DATA Syringomyelia is among the most common concomitant complications of Chiari malformation (CM). However, posterior fossa decompression cannot definitely make the syringomyelia disappear even in the long term. Also, there are no universal criteria defining improvement in syrinx. MATERIALS AND METHODS All admitted CM patients at our institution from 2013 to 2018 with a 1-year follow-up were analyzed. Patients without syringomyelia were compared with those who had syringomyelia. Patients were divided into 3 groups according to the procedures performed: posterior fossa decompression versus posterior fossa decompression with duralplasty (PFDD) versus PFDD plus obex unblocking. Divergent prognosis of syringomyelia was defined as a 3-category ordinal variable. A multivariable ordinal regression model was used to estimate the relationship between patient variables and increased odds for better resolution of syringomyelia. RESULTS No significant linear difference in bony structure was found between syringomyelia and nonsyringomyelia patients. Among syringomyelia patients, the regression analysis demonstrated that patients with shorter posterior fossa height (P=0.032), lower Pavlov ratio (P=0.029), and obex unblocking (vs. PFDD, P

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

Background Foramen magnum decompression (FMD) is the first-choice treatment for Chiari malformation (CM). However, it has been suggested that cerebellar herniation and syringomyelia occur as a natural protective event to prevent neural damage caused by atlantoaxial instability. It is argued that treating instability is the main treatment. Positive results of atlantoaxial fusion have been reported in the literature, but there are no studies including the results of atlantoaxial fusion as the second treatment in patients in whom classical decompression failed. In our study, we report the results of these patients to help in the selection of treatment and we present our treatment algorithm for CM with syringomyelia. Methods Thirteen patients who had undergone FMD and duraplasty due to CM and syringomyelia in our clinics and who had recovered clinically and radiologically but had recurrent complaints during long-term follow-up were evaluated. C1–C2 distraction and fusion were performed. We evaluated these patients radiologically and clinically. Results The mean age of the 13 patients was found to be 32.4 years. Male to female ratio was 6:7. The complaints recurred after an average of 2.1 years. Also, 3 cases were presented with their clinical characteristics and radiologic findings. Conclusions FMD may fail even with duraplasty, and treatment of CM in recurrent cases is still controversial. Recently, atlantoaxial instability has been reported to be the main pathology of CM, and the cure for pathology is to treat instability. Recurrent CMs with syringomyelia in which FMD has failed should be treated by atlantoaxial fixation.

Megalencephaly-CApillary malformation-Polymicrogyria (MCAP) syndrome results from somatic mosaic gain-of-function variants in PIK3CA. Main features are macrocephaly, somatic overgrowth, cutaneous vascular malformations, connective tissue dysplasia, neurodevelopmental delay, and brain anomalies. The objectives of this study were to describe the clinical and radiological features of MCAP, to suggest relevant clinical endpoints applicable in future trials of targeted drug therapy. Based on a French collaboration, we collected clinical features of 33 patients (21 females, 12 males, median age of 9.9 years) with MCAP carrying mosaic PIK3CA pathogenic variants. MRI images were reviewed for 21 patients. The main clinical features reported were macrocephaly at birth (20/31), postnatal macrocephaly (31/32), body/facial asymmetry (21/33), cutaneous capillary malformations (naevus flammeus 28/33, cutis marmorata 17/33). Intellectual disability was present in 15 patients. Among the MRI images reviewed, the neuroimaging findings were megalencephaly (20/21), thickening of corpus callosum (16/21), Chiari malformation (12/21), ventriculomegaly/hydrocephaly (10/21), cerebral asymmetry (6/21) and polymicrogyria (2/21). This study confirms the main known clinical features that defines MCAP syndrome. Taking into account the phenotypic heterogeneity in MCAP patients, in the context of emerging clinical trials, we suggest that patients should be evaluated based on the main neurocognitive expression on each patient.

The presenting report describes the use of a posterior cranial vault distraction procedure in resolving a Chiari malformation with associated syringomyelia following a surgically-treated nonsyndromic craniosynostosis. Chiari malformations are typically treated with posterior fossa decompression with or without expansion duraplasty. The objective of this report is to describe the effectiveness in resolving both Chiari malformation and secondary syringomyelia with posterior cranial vault distraction osteogenesis. A 5-year-old male, with a history of surgery for sagittal synostosis during infancy, presented with daily severe headaches and dysesthesias in the hands and feet. Imaging demonstrated a copper-beaten calvarium and a Chiari 1 malformation with a 7 mm diameter C4-T1 syrinx. He underwent posterior cranial vault distraction osteogenesis over 3 months to increase his intracranial volume. The patients' headaches improved significantly, and his dysesthesias resolved postoperatively. A magnetic resonance imaging performed 7 months after completion of distraction demonstrated resolution of the Chiari malformation and decompression of his syrinx. A computed tomography scan at 9 months postdistraction showed resolution of the copper-beaten calvarium. Patients with a history of craniosynostosis can develop inadequate cranial volume over time due to abnormal skull growth, leading to secondary Chiari malformation with or without syringomyelia. Posterior vault distraction is an effective strategy to address these conditions and can be employed later in childhood to treat the underlying pathology. The mechanism potentially expands both calvarium and dura, which in turn addresses both the Chiari malformation and secondary syringomyelia.

Introduction: Children aged 0–6 years with Chiari malformation (CM) often present with atypical symptoms and require revision surgery more often than older children. We studied characteristics and outcomes of CM patients in this age-group who underwent one or more revision surgeries to assess how often revision surgery is necessary and successful in this age-group. Methods: We retrospectively reviewed patients who were diagnosed with CM 1 or CM 1.5 and surgically treated with posterior fossa decompression (PFD) with or without duraplasty before their 7th birthday. Basic demographics, preoperative presentation, operative details, and postoperative outcomes were analyzed. Results: Forty patients (mean age 3.2 ± 1.7 years, 35% female) were reviewed. The most common presenting symptoms were headache, dysphagia, and respiratory problems. Eight patients required one or more revision surgeries 11.6 ± 7.6 months on average after their initial surgery. Comparing the revision and no revision groups, dysautonomia was significantly more common prior to initial surgery in the children requiring revision (37.5 vs. 3.1%, p = 0.02). The revision group also trended toward more dysphagia (75.0 vs. 46.9%, p = 0.24) and respiratory problems (75.0 vs. 40.6%, p = 0.12). The most frequent reasons for reoperation were symptom recurrence (6/8), residual posterior fossa compression (3/8), significant scar tissue formation (2/8), ventral brainstem compression (1/8), and suspected craniocervical instability (1/8). Of the 8 children undergoing reoperation, surgery achieved symptom resolution or improvement in 5, while 3 had unchanged symptoms. Two patients underwent a third surgery, after which 1 showed improvement and the other did not. This last patient showed short-term improvement after a fourth surgery but had symptom recurrence 12 months later. Discussion/Conclusion: Oropharyngeal and respiratory problems are particularly common in children aged 0–6 years with CM. Presentation with dysautonomia or other signs of brainstem compression will often predict an additional surgery will be needed after an initial PFD. Symptom recurrence is the most frequent reason for reoperation, and revision surgeries lead to improved clinical outcomes in the majority but not all of these young patients. Surgery in very young children is successful, but reoperation should be integrated into an up-front discussion algorithm particularly in children with severe symptoms.

Background The coexistence of falcine and occipital sinuses is rare and its natural course has not been reported. Case reports Two patients with persistent falcine and occipital sinuses are described. Both patients had straight sinuses. In one, both the transverse and sigmoid sinuses were hypoplastic and the patient had an acquired Chiari I malformation. The other patient had no other venous anomalies and had a normal posterior cranial fossa. Conclusion The coexistence of falcine and occipital sinuses can lead to an acquired Chiari I malformation. These cases suggest the importance of checking other venous and brain anomalies in this situation.

Aim:The causes of the syringomyelia in Chiari are not certainly defined. We have done morphometric analyses which can be useful to understand natural course and causes which increase the surgery requirement of patients.Methods:Eighty nine patients (operated) and 41 patients (non-operated) were included the study. Investigations were done as randomized. Accompanying syringomyelia, area of foramen magnum, herniated tonsil volume and clivo-dental angle were compared between operated and control groups.Results:Syringomyelia was seen in 50%. The mean values of FMA were 765.2±119.5 mm2. The mean value of critical discourse analysis (CDA) was 139.5±10.6°. Mean of the hydrophobicity (HTV) were found to be 54.5±24.1 mm3. The mean of age was 27±11.1, FMA was 876.5±131.9 mm2, CDA was 141.1±10.2° and HTV was 57.6±20.7 mm3 in the patients with syringomyelia. The mean of age was 33.9±12, FMA was 704.3±99.6 mm2, CDA was 137,8±10,8° and HTV was 51,4±26,8 mm3 in the patients who had not syringomyelia.Conclusion:Younger age, larger FMA and CDA increase the HTV and the rate of accompanying syringomyelia. As a result, the need for surgery increases. We think that it may be beneficial to consider these parameters in patients who are evaluated for surgery.

As headache is known as one of the most common symptoms in the patients with Chiari malformation type 1 (CM1), it is difficult to find out CM1-related headache among the symptoms because headache itself is commonly seen. Herein, we retrospectively review the cases of six CM1 patients complaining only of headache by which they complained of deterioration in daily life activities. The symptom of headache worsened during anteflexion (n = 2; 33%), retroflexion (n = 1; 17%), jumping (n = 3; 50%), going up the stairs (n = 1; 17%), and running (n = 1; 17%). Mean age at the onset was 15.7 years old (ranging 11–18) and four out of six were female. These inductive factors were clearly different from “Valsalva-like maneuvers,” although the mechanism might originate from dynamic tonsil changes. We named these headaches as “motion-specific.” These headaches radiated to the posterior side. MRI revealed that the extent of tonsillar ectopia was 11.3 mm, while syringomyelia was observed in three out of six patients (50%). All patients underwent surgical treatment, with the “motion-specific headache” completely disappearing 12.5 days thereafter. Although headaches are common, “motion-specific headache” may be a good candidate symptom to distinguish CM1 patients, especially among teenagers with headaches, and a good predictor for favorable outcomes after surgical treatment.

Introduction: Arachnoid membranes are well recognized as a cause of cerebrospinal fluid (CSF) flow impairment in disorders such as obstructive hydrocephalus and syringohydromyelia, but can be difficult to detect with standard noninvasive imaging techniques. True fast imaging with steady-state precession (TrueFISP) can exhibit brain pulsations and CSF dynamics with high spatiotemporal resolution. Here, we demonstrate the utility of this technique in the diagnosis and management of arachnoid membranes in the posterior fossa. Case Presentations: Three symptomatic children underwent cine TrueFISP imaging for suspicion of CSF membranous obstruction. Whereas standard imaging failed to or did not clearly visualize the site of an obstructive lesion, preoperative TrueFISP identified a membrane in all 3 cases. The membranes were confirmed intraoperatively, and postoperative TrueFISP helped verify adequate marsupialization and recommunication of CSF flow. Two out of the 3 cases showed a decrease in cerebellar tonsillar pulsatility following surgery. All children showed symptomatic improvement. Conclusion: TrueFISP is able to detect pulsatile arachnoid membranes responsible for CSF outflow obstruction that are otherwise difficult to visualize using standard imaging techniques. We advocate use of this technology in pre- and postsurgical decision-making as it provides a more representative image of posterior fossa pathology and contributes to our understanding of CSF flow dynamics. There is potential to use this technology to establish prognostic biomarkers for disorders of CSF hydrodynamics.

Background: Myoclonus is an involuntary movement disorder characterized by semirhythmic jerking movements of muscle groups but is rarely seen in association with Chiari malformation type I (CM-1). CM-1 is a frequently encountered clinical entity in pediatric neurosurgery characterized by caudal displacement of the cerebellar tonsils with or without syringomyelia. We report a pediatric patient who presented with upper extremity myoclonus and was found to have CM-1 and a complex septated cervicothoracic syrinx eccentric to the left. Case Presentation: A 12-year-old female presented with 6 months of headaches and upper extremity paresthesias who subsequently developed a left upper extremity segmental myoclonus after a fall. MRI demonstrated a CM-1 and a large complex cervicothoracic syrinx with a midline and left paracentral cavities. Her myoclonus was nonepileptic and refractory to clonazepam, cyclobenzaprine, and gabapentin. She underwent an intradural Chiari decompression and duraplasty. Postoperatively, she had complete resolution of her segmental myoclonus. Discussion: This case demonstrates a durable resolution of posttraumatic upper extremity segmental myoclonus after surgical decompression of a CM-1 with syringomyelia. Thus, Chiari decompression should be considered in cases of myoclonus with CM-1 and syringomyelia.

Objective: We aimed to evaluate the effectiveness and reliability of posterior fossa decompression (PFD) and superficial durotomy in patients who underwent surgery for Chiari malformation type 1 (CM1).Materials and Methods: Our study included 54 patients with the diagnosis of CM1 who had surgery between January 2012 and June 2019. The patients were divided into two groups according to the surgical technique applied. Group 1 included 10 patients who underwent classic PFD and duraplasty, and Group 2 included 44 patients who underwent PFD and superficial durotomy surgery. Pre- and postoperative clinical signs and symptoms of each patient were recorded. Simultaneously, morphometric measurements were compared from computed tomography (CT) and magnetic resonance (MRI) images taken pre- and postoperatively. The data of the two groups were compared.Results: Of the 54 patients, 18 were male, and the mean age was 37.51 ± 15.14. A statistically significant difference was found between the pre- and postoperative morphometric measurements of the subarachnoid distance, craniocervical angle, syringomyelia, and hydrocephalus at the cerebellum level in Group 2 patients who underwent PFD and superficial durotomy surgery (p 0.05). There was a 92% improvement in clinical signs and symptoms postoperatively. The complications seen in Group 1 decreased to a minimum in Group 2.Conclusion: We believe that a minimally invasive surgical method is superior to avoid major complications. We also found PFD and superficial durotomy shorten the duration of the patient's hospital stayAbbreviations: C1: cervical vertebra 1C2: cervical vertebra 2CM: Chiari malformationCM1: Chiari malformation type 1CSF: cerebrospinal fluidCT: computed tomographyMRI: magnetic resonance imagingPFD: posterior fossa decompressionUSG: ultrasonography.

Meningo encephalocele is a congenital anomaly and is a neural tube defect with occipital meningo encephalocele being the most common and is a result of a failure of the surface ectoderm to separate from the neuroectoderm. This condition can be identified in 1st trimester in 80% of cases and almost all by 2nd trimester. A 20-year-old third gravida was referred for antenatal Ultrasonography at five months of amenorrhoea to rule out fetal anomalies. On targeted imaging, for fetal anomalies, a defect was seen in occipital bone with herniation of posterior fossa contents with overlying meningeal covering. No other fetal anomalies were noted. A diagnosis of isolated occipital meningoencephalocele was made with additional fetal MRI correlation. The mother underwent termination of her pregnancy by Department of Obstetrics and Gynecology because of the grim fetal prognosis. The mother was advised to plan the subsequent pregnancies and was advised pre-conceptional folic acid supplementation. We present a case of isolated occipital meningoencephalocele- a rare congenital anomaly which was diagnosed prenatally in our hospital. This case provides an opportunity for identifying such neurological defects early and prompt termination of pregnancy to prevent comorbidity to mother. This study also helps to establish occipital meninigioencephalocele as an isolated clinicoradiological diagnosis and to distinguish it from syndrome associated occipital meninigioencephalocele or those associated with other neural tube defects like Chiari III malformations. It also allows us to stress once again the role of periconceptional folic acid in preventing the occurrence of neural tube defects.

Objective. To analyze possible radiological and clinical risk factors for syringomyelia in patients with scoliotic deformity. Material and Methods. An analysis of data from 3,285 patients with idiopathic scoliosis treated from 1997 to 2020 was performed. Syringomyelia was detected in 38 (1.16 %) cases. In 26 cases, syringomyelia was combined with Chiari malformation. In order to search for information on the topic under discussion, the international databases (Scopus, Medline, and GoogleScholar) were used. Additional search for publications listed in the article references was carried out. According to the literature data, possible risk factors for the presence of syringomyelia have been identified. Results. Among patients with confirmed syringomyelia, a left-sided thoracic scoliotic curve was found in 23.7 % of cases, a double thoracic curve in 18.4 %, an upper thoracic scoliotic curve in 7.9 %, an increase in thoracic kyphosis in 60.5 %, and initial neurological deficit in 47.4 %, which is significantly higher than the same indicators in the entire group of patients. The ratio of male and female patients in the entire group was 1.0 : 6.7, in the group with syringomyelia – 1.00 : 1.71. Conclusion. According to the literature review and statistically significant factors identified in the analysis of the monocentric group, it can be said that the left-sided thoracic scoliotic curve, upper thoracic curve, double thoracic curve, increased thoracic kyphosis, initial pyramidal insufficiency in the lower extremities, male sex, and a decrease in the initial somatosensory evoked potentials are risk factors for syringomyelia. If the above factors are present in a patient, the MRI study may be recommended to determine further treatment tactics. Before surgical correction of scoliotic deformity, MRI is recommended for all patients.

Chiari malformation type 1 (CM1) is an increasingly common incidental finding on magnetic resonance imaging (MRI). The proportion of children with an incidentally discovered CM1 who upon further evaluation require operative intervention for previously unrecognized signs and symptoms of neurological compromise or significant radiographic findings (syringomyelia) is unclear. An extensive long-term single-institution patient series was evaluated to better clarify the likelihood of surgery in patients who present with an incidentally discovered CM1. This study was conducted using prospective data for patients up to 18 years old that were evaluated for a CM1 at a large tertiary pediatric neurosurgery clinic between February 2009 and June 2019. Patients were excluded if they did not have an incidentally discovered CM1 and at least 12 months of clinical follow-up. A total of 218 consecutive patients were included in this study. The mean age at the initial neurosurgical evaluation was 6.5 years (range 5 months to 18.4 years), and the mean duration of clinical follow-up was 40.6 months (range 12 to 114 months). Initial MR imaging was most commonly obtained for the evaluation of seizures (15.1%), nonspecific headaches (not occipital or tussive) (14.7%), trauma (9.6%), and developmental delay (7.8%). Of the patients studied that eventually required surgery, we identified two groups: those operated before 6 months since presentation and those operated after 6 months. A total of 36 patients (16.5%) underwent a decompression with 22 patients (61.1%) receiving surgery within 6 months and the remaining 14 patients (38.9%) beyond 6 months. Patients undergoing early surgery (10.1%) initially presented with a significant syrinx or were noted to have an occult neurological dysfunction, whereas a smaller subset of patients (6.4%) eventually required surgery over time due to the development of new symptoms or a de novo syrinx. Only the presence of syringomyelia was statistically significant for the need of a surgical intervention, while age, sex and degree of tonsillar herniation were not. Evaluation of a large group of patients with an incidentally discovered Chiari malformation demonstrated that most patients may be managed conservatively, especially in the absence of syringomyelia. However, there is a subset of patients who will go on to develop a de novo syrinx or neurological symptoms that are new or progressive during follow-up, which should be evaluated by imaging of the brain and spinal cord. The presence of syringomyelia was associated with need for early surgical intervention. However, for patients without syringomyelia, surgical intervention is uncommon but may be delayed up to several years after presentation; therefore, long-term clinical follow-up is recommended.

Background. While the endocrine manifestations of pseudohypoparathyroidism are well known, less is known about the associated brain and spine abnormalities. These abnormalities may present with nonspecific symptoms in the paediatric population, and lack of awareness to these uncommon manifestations of the disease may result in a delay in necessary intervention. Case Presentation. We herein present a case of known pseudohypoparathyroidism type 1a who presented initially with minor head injury. She later developed progressive worsening headache, increased irritability, and vomiting. Repeated imaging showed hydrocephalus and Chiari malformation type 1 necessitating emergency craniectomy. Conclusion. Growth hormone deficiency, a common manifestation of pseudohypoparathyroidism type 1a, results in underdevelopment of the posterior cranial fossa and may account for the higher incidence of Chiari malformation in this group of patients. Other associated neurological features reported in pseudohypoparathyroidism type 1a include spinal stenosis, syringomyelia, and craniosynostosis. While less commonly seen, awareness to these associations is important in order to optimize the multidisciplinary care to this group of patients.

Syringomyelic affliction of spinal cord is usually seen in association with variety of craniovertebral junction anomalies i.e. Chiari malformations, basilar invagination/impression, atlantoaxial instability etc. The natural history is not very clearly understood and a majority of patient present with slowly progressive neurological deficit followed by sudden rapid deterioration. At present, there is a general consensus to offer surgical decompression in all patients diagnosed with Chiari I malformation with Syrinx irrespective of their symptoms in order to prevent delayed neurological worsening. Few authors have reported spontaneous resolution of syrinx with persistent tonsillar herniation without operative treatment. We report one such patient and propose anterior spinal cord fissuring as a plausible cause of spontaneous drainage of syrinx. We also propose an idea to keep such patients with anterior spinal cord fissure seen in index scans to be followed up conservatively closely instead of upfront Chiari decompression.

We describe the case of a 22-year-old woman of southeast-Asian origin, presenting with unilateral sudden visual loss after a self-healing hearing loss a week before. Ophthalmological examination showed visual acuity of light perception in the left eye, mild RAPD, normal ocular motility and an elevated optic disc with indistinct margins. Neurological examination showed no acute pathology and brain CT-MRI imaging revealed a small-almost subclinical-herniation of the cerebellar tonsils. As investigation eliminated every other infectious or inflammatory cause of papillitis, neurosurgical intervention was proposed. The patient underwent an uncomplicated occipital craniotomy with posterior fossa decompression and had a favorable revolution with regression of papilledema and a fully recovering visual acuity that reached 20/20. Chiari malformation type I refers to an abnormality of the posterior fossa that has a smaller volume than normal, leading to the herniation of cerebellar tonsils, at least 5 mm below the foramen magnum. The occurrence of papilledema associated with Chiari malformation type 1 is rare. Chiari malformation has, until today, mainly been studied among children populations, usually with a poor visual acuity recovery. The originality of our case report consists in the description of an adult patient case showing unilateral, unusual ophthalmological findings and complete recovery after surgical treatment.

Background While commonly seen in syndromic craniosynostosis, the incidence of Chiari malformation (CM) in nonsyndromic craniosynostosis has been reported at 5% and there is a lack of understanding of the pathophysiology and management of CM in this patient population. Case Description We present a 5-year-old male patient who underwent a sagittal craniosynostosis repair at the age of 5 months and returned at the age of 5 years with daily headaches associated with behavioral changes. He was found to have pan-synostoses and radiographic evidence of increased intracranial pressure, including a Chiari malformation. Neurologic and genetic workup was unremarkable. A cranial vault reconstruction was performed, and subsequent imaging demonstrated resolution of previously noted Chiari malformation. Conclusions In our case, we provided a unique window into the underlying pathophysiology for CM in patients with concurrent nonsyndromic craniosynostosis that we hope will add to the current foundation of literature supporting the intricate relation between cranial vault compliance and Chiari malformation or hindbrain herniation. Furthermore, we provide insight into the management of acquired CM and support isolated cranial vault reconstruction in those who do not appear to have symptomatic suboccipital compression.

Craniovertebral decompression is performed for symptomatic Chiari malformation type 1, with or without syringomyelia. In a few patients the symptoms and/or syrinx persist or recur, in which case revision surgery may be offered. The aim of this study was to examine the cause of failure of primary surgery and to assess the outcomes for revision cranio-vertebral decompression.We retrospectively reviewed all revision cranio-vertebral decompressions performed in our centre over an eighteen-year period. A total of 35 patients were identified (23 females; 12 males). Twenty patients had isolated Chiari malformation type 1 and a further fifteen had associated syringomyelia. The mean follow up period was 4-years (range:1-12 years).The mean time to revision was 7 years after the primary surgery. The commonest operative finding was that of dense arachnoid scar tissue, obstructing movement of CSF across the craniovertebral junction. Overall, 32 of the 35 patients reported improvement after surgery, including all 15 patients with syringomyelia. Improvement was maintained in the longer term in more than two thirds of those patients who underwent revision craniovertebral decompression.Failure of primary craniovertebral decompression for Chiari malformation most often results from the formation dense scar tissue at the operation site, obstructing CSF movement. Revision cranio-vertebral decompression can provide early symptomatic relief in most patients and this benefit is sustained in at least two thirds of cases, in the medium term at least. Patients with concomitant syringomyelia appear to derive most benefit from revision surgery.

While cases of acquired Chiari I malformation following ventriculoperitoneal shunting for posthemorrhagic hydrocephalus have been reported, true disproportionate cerebellar growth is rare, with no previous cases requiring posterior fossa decompression reported. We present a premature neonate who underwent ventriculoperitoneal shunt placement for suspected posthemorrhagic hydrocephalus. He subsequently developed a symptomatic Chiari I malformation with volumetric measurements demonstrating disproportionate growth of the cerebellum. He did not demonstrate thickening of the supratentorial or posterior fossa cranium. The patient underwent an extradural posterior fossa decompression, with resolution of symptoms. We review the extant literature regarding the development of Chiari malformation type I as a manifestation of craniocerebral disproportion (CCD) following shunt placement for posthemorrhagic hydrocephalus of prematurity. Most previous reports reflect a mechanism that includes underdevelopment of the intracranial posterior fossa (or supratentorial) volume. The case presented in this report, as well as one additional case, indicates that there may exist a variant mechanism, characterized by rapid growth of the cerebellum itself, in the absence of one of the rare syndromes associated with primary macrocerebellum. While this case was effectively managed with extradural posterior fossa decompression, previous reports indicate that supratentorial cranial expansion procedures are preferable in some cases. As such, pediatric neurosurgeons should be able to distinguish the patterns of craniocerebral disproportion when considering treatment options for these patients. Further investigation regarding these uncommon patients may better describe the underlying mechanisms.

Introduction : CSF leaks are the most common surgical complication in the posterior fossawhich can be minimized with watertight dural closure which can be done either with native available dura and if not available with other materials. Materials and Methods : Aim of the study was to compare the occurrence of CSF leak in posterior fossa surgeries where different dural closure materials are used. This was a 5 year retrospective cohort study, conducted in the department of Neurosurgery, Government medical college, Thrissur, Kerala, India. Results and Discussion : A total of 49 cases were included in the study. The mean age of the study was 44.86 16.99(4 years- 70 years).Out of the 49 cases 18(36.7%) were male patients and 31(63.3%) were female patients. Total posterior fossa procedures are distributed as CP angle tumor excision 27(55.1%), Foramen magnum decompression for chiari malformation 12(24.5%), Other tumors of posterior fossa 7(14.3%) and vascular lesions of posterior fossa 3(6.1%). Out of the total 49 cases 20 (40.85%) are closed with pericranium, 14 cases (28.6%) are closed with primary dura, 9 cases (18.4%) with fascia lata and 6(12.2%)are closed with artificial dural patch. Out of the total 49 procedures 10(20.4%) has CSF leak and no CSF leak detected in 39(79.6%). It is found that cases closed with pericranium has got a significant capacity to decrease the CSF leak 13 (65%) out of 20 cases (a24.43, df=1 p=0.035).Odds ratio found to be 0.21 that gives 79% protection against CSF leak(95% CI=0.047-0.0968). Conclusion : Study concludes that, in cases of posterior fossa surgeries, closure with pericranium has a significant protection against CSF leak. Keywords: Cerebrospinal fluid leak, Posterior fossa surgeries, Dural closure methods.

The article analyzes the origin and competence of using several eponyms widely known in neurology. It is shown that some of them are not used correctly. So, the alternates “Jakob–Creutzfeldt disease” and “Arnold– Chiari malformation” are more correct. The eponym “Steele–Richardson–Olszewski syndrome” should not be used as a synonym for progressive supranuclear palsy syndrome. The historical aspects and correct variants of the use of a number of other neurological eponyms are highlighted in the article.

Since the active introduction of neuroimaging methods, the prevalence of registered children with craniovertebral anomalies, including the Chiari malformation (CM), has a statistically significant tendency to increase. Minimally invasive surgical interventions are the most acceptable in pediatric neurosurgery, however, currently experience has been accumulated enough that does not confirm the universality of this method, due to the high frequency of reoperations at an older age, as well as a decrease of children’s quality of life in the interoperative periods. Considering the imperfection of treatment strategy mentioned above, the aim of this article was to analyze modern surgical interventions in pediatric and adult neurosurgeons with confirmation of our own results as an example of a clinical case of Chiari malformation in our clinic.

Background: Chiari malformations (CM) are congenital defects due to hypoplasia of the posterior fossa with cerebellar herniation into the foramen magnum and upper spinal canal. Despite the vast research done on this neurological and structural syndrome, clinical features and management options have not yet conclusively evolved. Quantification of proper treatment planning, can lead to potential perioperative benefits based on diagnoses and days to procedure. This study aims to identify if early operation produces better perioperative outcomes or if there are benefits to delaying CM surgery. Aims and Objective: Assess outcomes for Chiari type I. Methods: The KID database was queried for diagnoses of Chiari Malformation from 2003-2012 by icd9 codes (348.4, 741.0, 742.0, 742.2). Included patients: had complete time to procedure (TTP) data. Patients were stratified into 7 groups by TTP: Same-day as admission (SD), 1-day delay (1D), 2-day delay (2D), 3-day delay (3D), 4-7 days delay (4-7D), 8-14 days delay (8-14D), >14 days delay (>14D). Differences in pre-operative demographics (age/BMI) and perioperative complication rates between patient cohorts were assessed using Pearson's chi-squared tests and T-tests. Surgical details, perioperative complications, length of stay (LOS), total charges, and discharge disposition was compared. Binary logistic regressions determined independent predictors of varying complications (reference: same-day). Results: 13,812 Chiari type I patients were isolated from KID (10.12 ± 6.3, 49.2F%, .063 ± 1.3CCI). CM-1 pts were older (10.12 yrs vs 3.62 yrs) and had a higher Charlson Comorbidity Score (0.62 vs 0.53; all P < 0.05). Procedure rates: 27.8% laminectomy, 28.3% decompression, and 2.2% spinal fusion. CM-1 experienced more complications (61.2% vs 37.9%) with the most common being related to the nervous system (2.8%), anemia (2.4%), acute respiratory distress disorder (2.1%), and dysphagia (1.2%). SD was associated with the low length of stay (5.3 days vs 9.5-25.2 days, P < 0.001), total hospital charges ($70,265.44 vs $90, 945.33-$269, 193.26, P < 0.001) when compared to other TTP groups. Relative to SD, all delay groups had significantly increased odds of developing postoperative complications (1D-OR: 1.29 [1.1- 1.6] → 8-14D-OR: 4.77[3.4-6.6]; all P < 0.05), more specifically, nervous system (1D-OR: 1.8 [1.2-2.5] → 8-14D-OR: 3.3 [1.8-6.2]; all P < 0.05).Sepsis complications were associated with a delay of at least 3D(2.5[1.4-4.6]) while respiratory complications (6.2 [3.1-12.3]) and anemia (2 [1.1-3.5]) were associated with a delay of at least 8-14D (all P < 0.05).