Like in all posterior fossa surgeries the avoidance of cerebrospinal fluid (CSF) leaks is of paramount importance for foramen magnum decompression in Chiari I malformation in children. The present technical note decribes the experience with the creation of a T-shaped myofascial cuff to reduce the risk of postoperative CSF leaks.

The medical records of 98 children were evaluated. They underwent foramen magnum decompression without dural closure using a T-shaped myofascial cuff whose incision lines run completely within the fascia and the muscles not extending beyond their attachment line to the bone.

There were 2 CSF leaks and no infections in 98 operated patients. The follow-up was between 4 and 48 months.

The placement of a T-shaped myofascial cuff allows for watertight closure of the muscles and the fascia which is likely to reduce the rate of CSF leaks in pediatric Chiari I patients operated on without closure of the dura.

We designed this study to introduce the surgical strategy cerebrospinal fluid (CSF) decompression in treatment of Chiari malformation type I and compared CSF decompression with other surgical strategies to provide a solid basis for patient counseling.

The study enrolled 528 consecutive patients with CMI who underwent surgical interventions from 2012 to 2022. The surgical strategy for these patients was bony and dural decompression, anatomical reduction of herniated tonsils, or CSF decompression. Short-term results were determined after 3 months; long-term outcomes were evaluated at last follow-up (at least 18 months).

CSF decompression was independently associated with better long- or short-term primary outcomes than anatomical reduction of herniated tonsils or bony and dural decompression (P < 0.001). Compared with short-term, the long-term outcomes were better in patients who underwent CSF decompression (P = 0.035), but were worse in patients with bony and dural decompression (P = 0.03). Specific surgical techniques cannot affect the long- and short-term outcomes of patients with Chiari malformation type I. CSF decompression provided better long-term syringomyelia improvement than short-term (181/218, 83% vs. 169/218, 77.5%; P < 0.001).

CSF decompression, but not a specific surgical technique or operative method, was associated with favorable neurological outcomes in ADULT patients with Chiari malformation type I. The surgical technique and operative method should be selected according to the characteristics of each patient and the intraoperative condition to normalize CSF circulation at the craniovertebral junction area. The intraoperative target, smooth CSF flow out from the fourth ventricle and in to the bilateral Luschka foramina, could be observed.

Our study aims to prospectively compare an autologous duraplasty in situ technique (IS group) with the synthetic dural graft duraplasty (SDG group) to clarify the effectiveness and superiority of the former in the treatment of patients with Chiari malformation type 1 (CM-I).

29 patients with CM-I were randomly assigned to either IS or SDG group. In both groups, a dissection from the occipital bone was performed. All procedures were performed by the same surgeon. The two duraplasty methods were compared in terms of surgical factors and complications. Data analysis was done for the baseline material, the neurological outcome and MRI-documented syrinx size at the 6 month follow-up.

29 patients were enrolled in this study, 14 in the IS group and 15 in the SDG group. The results showed no significant difference in operation time (P = 0.916), amount of bleeding (P = 0.120), operation complications, hospitalization time (P = 0.854) and prognosis between the two groups. The hospitalization cost of IS group was 15,125 yuan less than that of SDG group (P < 0.05).

The autogenous duraplasty in situ technique is a novel, simple, effective and economical surgical management for patients with CM-I.

Treatment for a large symptomatic syrinx associated with a Chiari 1 is predominately surgical, via a foramen magnum decompression (FMD), with the aim to normalise cerebrospinal fluid (CSF) movement. Whilst theories of underlying hyperdynamic states in Chiari 1 and Syringomyelia exist, to date there is no effective medical treatment to reverse Syringomyelia. A 17-year-old female was referred with a seven-month history of gradually progressive impaired temperature sensation in her left upper limb. She had also been concomitantly diagnosed with thyrotoxicosis. Magnetic resonance imaging (MRI) confirmed a Chiari 1 with a large syrinx. The patient preferred to avoid surgery in the first instance. She underwent treatment for her thyrotoxicosis. The eight-month, 20- and 36-month follow-up MRI scans demonstrated a gradual resolution of the Chiari 1 malformation and the syrinx. Whilst there have been reports of Chiari 1 malformation association with hyperthyroidism, this is the first report describing syrinx resolution following treatment of thyrotoxicosis. Hyperdynamic circulation can result in syrinx formation through various mechanisms. We hypothesise that the treatment of thyrotoxicosis resulted in normalisation of CSF pulse amplitude and subsequent syrinx resolution. Hyperthyroidism evaluation may be explored in studies of CM1 and Syrinx or other CSF disorders.

Chiari type 1 malformation (CIM) is defined as tonsillar ectopia of >5 mm, while syringomyelia (SM) is defined as a cerebrospinal fluid (CSF)-filled cavity larger than 3 mm dissecting the spinal cord. Over the last decades, our understanding of these pathologies has grown; however, many controversies still exist almost in every aspect of CIM and SM, including etiology, indication for treatment, timing of treatment, surgical technique, follow-up regime, and outcome. This chapter provides a comprehensive overview on different aspects of CIM and SM and on the still existing controversies, based on the evidence presently available. Future directions for clinical research concerning CIM and SM treatment and outcome are elaborated and discussed as well.

Patients showing typical Chiari malformation type 1 (CM1) signs and symptoms frequently undergo cranial and cervical MRI. In some patients, MRI documents >5 mm of cerebellar tonsillar herniation (TH) and the diagnosis of CM1. Patients with 3−5 mm TH have “borderline” CM1. Patients with less than 3 mm of TH and an associated cervical syrinx are diagnosed with Chiari “zero” malformation (CM0). However, patients reporting CM1 symptoms are usually not diagnosed with CM if MRI shows less than 3−5 mm of TH and no syrinx. Recent MRI morphometric analysis of the posterior fossa and upper cervical spine detected anatomical abnormalities in and around the foramen magnum (FM) that explain these patients’ symptoms. The abnormalities include a reduced size of the posterior fossa, FM, and upper cervical spinal canal and extension of the cerebellar tonsils around the medulla rather than inferior to the foramen magnum, as in CM1. These morphometric findings lead some neurologists and neurosurgeons to diagnose CM0 in patients with typical CM1 signs and symptoms, with or without cervical syringes. This article reviews recent findings and controversies about CM0 diagnosis and updates current thinking about the clinical and radiological relationship between CM0, borderline CM1, and CM1.

Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children.

A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three).

Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery."

The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.

The widespread use of imaging has increased Chiari malformation (CM) diagnosis. CM shows clinical heterogeneity that makes management controversial. We aimed to evaluate the occurrence and clinical and radiographic presentation of children with CM-1 and CM-1.5, reporting possible differences according to age and management.

We retrospectively reviewed 46 children diagnosed with CM-1 or CM-1.5, between 2006 and 2019 at our institute. We evaluated for each subject: reason for hospital admission, clinical presentation, age at diagnosis, extent of cerebellar tonsillar herniation (CTH) and type of treatment when carried out. Affected children were assigned to three age groups. In some patients, a clinical follow-up was carried out.

Mean age at diagnosis was 7.61 years. Mean CTH was 8.72 mm. Syringomyelia was found in 10.9%. Twenty-six individuals (56.5%) were symptomatic. The most frequent symptom was headache (34.8%). There were no statistically significant differences between the age groups with regard to the amount of CTH (p = 0.81). Thirteen children (28.3%) underwent surgical treatment. CTH was significantly higher in the surgical group (p < 0.01). Twenty-three patients (50%) performed a 3-year mean follow-up, 17 of whom had no surgery treatment. CTH was stable in 58.8%, reduced in three and increased in three, without any change in symptoms. Only one child showed a worsening in herniation and symptoms, then requiring surgery.

Frequency and type of symptoms were consistent with those reported in the literature. Conservative approach is a viable option for minimally symptomatic patients, most of whom did not show clinical worsening at follow-up.

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.

Incidental cerebellar tonsillar ectopia (ICTE) that meets the radiographic criterion for Chiari malformation type I (CMI) is an increasingly common finding in the clinical setting, but its significance is unclear. The present study examined posterior cranial fossa (PCF) morphometrics and a broad range of health instruments of pediatric ICTE cases and matched controls extracted from the Adolescent Brain Cognitive Development (ABCD) dataset.

One-hundred-six subjects with ICTE and 106 matched controls without ICTE were identified from 11,411 anatomical MRI of healthy screened pediatric subjects from the ABCD project. Subjects were matched by sex, age, body mass index, race, and ethnicity. Twenty-two brain morphometrics and 22 health instruments were compared between the two groups to identify unrecognized CMI symptoms and assess the general health impact of ICTE.

Twelve and 15 measures were significantly different between the ICTE and control groups for females and males, respectively. Notably, for females, the anterior CSF space was significantly smaller (p = 0.00005) for the ICTE group than controls. For males, the clivus bone length was significantly shorter (p = 0.0002) for the ICTE group compared to controls. No significant differences were found among the 22 health instruments between the two groups.

This study demonstrated that pediatric ICTE subjects have similar PCF morphometrics to adult CMI. ICTE alone did not appear to cause any unrecognized CMI symptoms and had no impact on the subjects' current mental, physical, or behavioral health. Still, given their cranial and brain morphology, these cases may be at risk for adult-onset symptomatic CMI.

Chiari malformations encompass various radiological and clinical entities, sharing the herniation of the rhombencephalic structures through the foramen magnum as a common characteristic. They can be symptomatic or asymptomatic. The therapeutic strategies for these malformations differ on the basis of the diverse pathophysiologic processes that cause them. As Chiari malformations are caused by various pathophysiologic processes, they must be recognized promptly to select the best treatment for each single case.

The goals of this study were to compare clinical outcomes in patients with Chiari Malformation Type I (CMI) receiving posterior fossa decompression with (PFDD) or without duraplasty (PFD).

We conducted a retrospective analysis of 178 consecutive cases of 157 patients undergoing PFDD or PFD for CMI at Cohen Children's Medical Center between 2007 and 2017. Clinical improvement was defined as a clear reduction of preoperative symptoms after surgery as reported by patients and parents. An improvement of syrinx was derived from radiologic comparison of pre- and postoperative MR imaging. Chi-square analysis was performed to analyze the association between duraplasty and clinical parameters (alpha = 0.05).

The primary presenting complaint was headache (142/178; 80%), followed by neck, back, and upper extremity pain, and numbness or tingling (49/178; 28%). Seventy patients (78/178; 44%) underwent PFD, and 100 patients (100/178; 56%) underwent PFDD. Overall, 143 patients (143/178; 80%) experienced subjective improvement with no statistically significant difference between the two surgical techniques (p = 0.705). The number of patients receiving PFDD with syrinx improvement or stabilization (55/59; 93%) was statistically larger than those that received PFD (8/13; 62%) (p = 0.008). PFDD was associated with greater complications than PFD alone. There were 35 cases of reoperation overall (35/178; 19%), and there was no statistically significant difference in reoperation rate between PFD and PFDD (p = 0.255).

There appears to be a role for PFDD in patients with severe syringomyelia, but overall, PFD alone may be safely offered as the initial surgical intervention for symptomatic CMI patients.

It remains unclear which subgroups of scoliotic patients with CMI and syringomyelia are more likely to undergo unplanned neurosurgery after spinal deformity correction. The purpose of this study is to explore risk factors of unplanned neurosurgery for scoliotic patients with CMI and syringomyelia after spinal deformity correction.

This cohort consisted of 62 scoliotic patients with CMI and syringomyelia who underwent spinal deformity surgery with a mean follow-up of 4.3 year. 14 of them underwent unplanned neurosurgery (the NN group), and the other 48 patient underwent single spinal correction surgery (the SS group). The radiological parameters were compared between the two groups, and multivariate logistic regression analysis and Kaplan-Meyer survival curves were used to identify potential risk factors of unplanned neurosurgery.

The incidence of unplanned neurosurgery after spinal deformity surgery was 22.28 % (14/62), and delayed headache was the most common reason for unplanned neurosurgery with five patients (36 %) and follow by neck pain with three patients (21 %). Significantly increased tonsil ectopia (9.7 ± 3.8 vs. 6.9 ± 2.9; P = 0.021), syrinx/cord width ratio (0.62±0.11 vs. 0.45±0.13; P＜0.001), and syrinx/cord area ratio (0.45 ± 0.11 vs. 0.26 ± 0.15; P＜0.001) were found in the NN group. While, there were no significant differents in pBC2 line, clivus canal angle, and syrinx length between the two groups. The logistic regression analysis indicated that tonsil ectopia≥10 mm (P = 0.019; OR=6.440; 95 %CI = 1.361 to 30.467) and syrinx/cord area ratio ≥ 0.4 (P = 0.006; OR=7.664; 95 %CI = 1.819 to 32.291) were independent risk factors of unplanned neurosurgery. Kaplan-Meyer survival curves showed cumulative unplanned neurosurgery for patients with tonsil ectopia ≥ 10 mm (P < 0.001) and syrinx/cord area ratio ≥ 0.4 (P = 0.001) after spinal deformity correction.

After spinal deformity correction, 78 % of the patients did not require later neurosurgery and those that needed it had a delay of more than nine months. Tonsil ectopia ≥ 10 mm and syrinx/cord area ratio ≥ 0.4 were independent risk factor of unplanned neurosurgery after spinal deformity correction. It is reasonable to perform spinal corrective surgery in patients with minimal symptoms and signs without the need for prior neurosurgical intervention.

Surgical decision-making in Chiari malformation type I (CM-I) patients tends to depend on the presence of neurological signs and symptoms, syringomyelia, and/or scoliosis, but significant variability exists from center to center. Here, we review the symptoms of CM-I in children and provide an overview of the differences in opinion regarding surgical indications, preferred surgical techniques, and measures of outcome.

A review of the literature was performed to identify publications relevant to the surgical management of pediatric CM-I patients.

Most surgeons agree that asymptomatic patients without syringomyelia should not undergo prophylactic surgery, while symptoms of brainstem compression and/or lower cranial nerve dysfunction warrant surgery. Patients between these extremes, however, remain controversial, as does selection of the most appropriate surgical technique.

The optimal surgical procedure for children with CM-I remains a point of contention, and widespread variability exists between and within centers.

The aim of this study was to evaluate the outcomes and complications for individual surgeons at British Columbia Children's Hospital for the treatment of Chiari I Malformation (CMI) in children.

This was a retrospective review of patients with CMI who had surgery from 1986 to 2015. We assessed the Chicago Chiari Outcome Scores (CCOS) and complication rates by surgeon.

Seventy patients, 38 males and 32 females, underwent posterior fossa decompression including 14 extradural and 56 intradural approaches. Syringomyelia was present in 74.3%. Most syringomyelia improved with no difference between intradural and extradural surgeries. After initial surgery, 13 patients (18.6%) had complications including 2/14 (14.3%) of extradural and 11/56 (19.6%) of intradural surgeries. Two patients required surgical intervention for complications whereas 11 had transient complications. The complication rate by surgeon ranged from 11 to 20% for extradural (2 surgeons only) and 10.5 to 40% for intradural surgeries (4 surgeons). The CCOS ranged from 12 to 15 for extradural and 6 to 16 for intradural. The CCOS ranges for surgeons 1 and 2 were 12-15 and 13-15 respectively for extradural. The CCOS ranges for surgeons 1, 2, 3, and 4 were 12-16, 6-15, 12-16, and 12-16 respectively for intradural. Thirteen patients had a second surgery for CMI. The final CCOS was good in 86% and moderate in 14%.

There was variability in surgeries performed at BCCH by different surgeons, with variations in CCOS and complication rates. This information is important during decision making, consent process, and for quality improvement.

There are numerous publications about the technical aspects of decompressive surgery for Chiari I malformation highlighting many variations of this procedure. Each approach has its followers. Bony decompression of the foramen magnum alone or with the removal of a portion of the posterior arch of C1, dural splitting with keeping arachnoid intact, and durotomy are described. Dural closure is done with various materials. We retrospectively reviewed foramen magnum decompression without dural repair (FMDWDR) following the technique used by Gardener and Williams as an option in pediatric patients with Chiari I malformation in terms of complication rate and clinical outcome.

The surgical database of our unit identified 65 consecutive children who underwent FMDWDR surgery for Chiari I malformation between 2009 and 2016. The retrospective assessment included patient demographics, clinical data, surgical technique, revision rate, complications, and clinical outcome.

Durotomy without repair was performed in 65 patients. Complications included aseptic meningitis and subdural hematoma respectively in three cases, intradiploic CSF collections in three patients, and CSF leaks in six children. The CSF leak rate has reduced dramatically after introducing a technical modification. Revision surgery was performed in seven cases. None of the patients was identified with postoperative hydrocephalus or infection. There was no mortality and no long-term surgical morbidity. In terms of clinical outcome, 52 patients reported postoperative improvement, 10 were clinically unchanged, and three noticed worsening of symptoms.

Applying a "T"-shaped fascial incision which allows a watertight closure of the fascia FMDWDR is still a safe and effective treatment option for Chiari I malformation in children.

Despite decades of experience and research, the etiology and management of Chiari I malformations (CM-I) continue to raise more questions than answers. Controversy abounds in every aspect of management, including the indications, timing, and type of surgery, as well as clinical and radiographic outcomes. This review aims to outline past experiences, consolidate current evidence, and recommend directions for the future management of the Chiari I malformation.

A review of recent literature on the management of CM-I in pediatric patients is presented, along with our experience in managing 1073 patients who were diagnosed with CM-I over the past two decades (1998-2018) at Children's National Medical Center (CNMC) in Washington DC.

The general trend reveals an increase in the diagnosis of CM-I at younger ages with a significant proportion of these being incidental findings (0.5-3.6%) in asymptomatic patients as well as a rise in the number of patients undergoing Chiari posterior fossa decompression surgery (PFD). The type of surgical intervention varies widely. At our institution, 104 (37%) Chiari surgeries were bone-only PFD with/without outer leaf durectomy, whereas 177 (63%) were PFD with duraplasty. We did not find a significant difference in outcomes between the PFD and PFDD groups (p = 0.59). An analysis of failures revealed a significant difference between patients who underwent tonsillar coagulation versus those whose tonsils were not manipulated (p = 0.02).

While the optimal surgical intervention continues to remain elusive, there is a shift away from intradural techniques in favor of a simple, extradural approach (including dural delamination) in pediatric patients due to high rates of clinical and radiographic success, along with a lower complication rate. The efficacy, safety, and necessity of tonsillar manipulation continue to be heavily contested, as evidence increasingly supports the efficacy and safety of less tonsillar manipulation, including our own experience.

This manuscript describes our management philosophy of Chiari I malformation in children based on a single neurosurgeon's personal experience.

Based on 61 infants and children with Chiari I malformation treated from 2007 to 2017, typical symptoms, surgical indications, types of surgery, and evaluation of surgical decompression are reviewed.

Sixty-one patients had 69 decompressions, with 90% having symptom improvement. Seven (11.5%) needed reoperation, 1 of which needed 2 reoperations for recurrence. The recurrence rates were 20% (5 of 25) after dural scoring and 5.6% (2 of 36) after duraplasty (p = 0.1116, Fisher's exact test). Six (16%) of 36 patients developed pseudomeningocele or CSF leak.

We recommend surgical intervention for Chiari I malformation for clearly symptomatic patients and those with significant hydromyelia regardless of symptoms. A bony decompression with dural scoring is recommended for patients with typical occipital headaches with a lesser degree of tonsillar descent, while an expansile duraplasty is standard for those with high-grade tonsillar descent, medullary kink, or hydromyelia. Intraoperative ultrasound is often helpful to ensure the adequacy of the decompression. Most patients will have improvements in symptom and imaging after either type of decompressive surgery.