A systematic literature review and consensus using Delphi method.

This review aims to provide an overview on Chiari malformation in pediatric patients, highlighting the specific clinical manifestations and surgical treatment options.

Chiari malformation in children presents a real difficulty to the general neurosurgeon because children are not smaller adults. In the absence of pediatric neurosurgeons, as in many countries of the world, a need for education of general neurosurgeons on the management of Chiari malformation in children was identified.

The authors carried out an extensive review of the literature in PubMed database of the last 10 years addressing the topic of Chiari malformation in children. A total of 64 studies were selected for analysis and five statements were drawn to be voted by a panel of expert spine surgeons in two consensus meetings organized by the World Federation of Neurosurgical Societies (WFNS) Spine Committee. A consensus was reached using the Delphi method.

In children with CM1, a decompressive surgery with duraplasty before puberty may avoid scoliosis progression. In Chiari type 2, the recommendation is to perform urgently extensive decompression of the craniovertebral junction and cervical canal if there is no decompensation of hydrocephalus. Cranial vault expansion may be recommended in pediatric Chiari malformation associated with craniosynostosis. Children with Chiari type I can play sports because of the low risk of worsening.

Pediatric age is a modifier for the management of Chiari malformation (CM). Prompt diagnosis and appropriate decompressive surgery with duraplasty before puberty are essential to mitigate the impact of the condition on the child's well-being. Increased awareness among health care professionals, timely access to specialized expertise in neurosurgical interventions are crucial, especially for type 2 CM patients that require urgent decompression of CVJ and cervical spine.

Chiari malformation type I (CIM) diagnoses have increased in recent years. Controversy regarding the best operative management prompted a review of the literature to offer guidance on surgical interventions.

To assess the literature to determine (1) whether posterior fossa decompression or posterior fossa decompression with duraplasty is more effective in preoperative symptom resolution; (2) whether there is benefit from cerebellar tonsillar resection/reduction; (3) the role of intraoperative neuromonitoring; (4) in patients with a syrinx, how long should a syrinx be observed for improvement before additional surgery is performed; and 5) what is the optimal duration of follow-up care after preoperative symptom resolution.

A systematic review was performed using the National Library of Medicine/PubMed and Embase databases for studies on CIM in children and adults. The most appropriate surgical interventions, the use of neuromonitoring, and clinical improvement during follow-up were reviewed for studies published between 1946 and January 23, 2021.

A total of 80 studies met inclusion criteria. Posterior fossa decompression with or without duraplasty or cerebellar tonsil reduction all appeared to show some benefit for symptom relief and syrinx reduction. There was insufficient evidence to determine whether duraplasty or cerebellar tonsil reduction was needed for specific patient groups. There was no strong correlation between symptom relief and syringomyelia resolution. Many surgeons follow patients for 6-12 months before considering reoperation for persistent syringomyelia. No benefit or harm was seen with the use of neuromonitoring.

This evidence-based clinical guidelines for the treatment of CIM provide 1 Class II and 4 Class III recommendations. In patients with CIM with or without syringomyelia, treatment options include bone decompression with or without duraplasty or cerebellar tonsil reduction. Improved syrinx resolution may potentially be seen with dural patch grafting. Symptom resolution and syrinx resolution did not correlate directly. Reoperation for a persistent syrinx was potentially beneficial if the syrinx had not improved 6 to 12 months after the initial operation. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/3-surgical-interventions .

In anticipation of the "Chiari and Syringomyelia Consensus Conference" held in Milan in 2019, we performed a systematic literature review on the management of Chiari malformation type 1 (CM1) and syringomyelia (Syr) in children.We aimed to summarize the available evidence and identify areas where consensus has not been reached and further research is needed.In accordance with PRISMA guidelines, we formulated seven questions in Patients-Interventions-Comparators-Outcomes (PICO) format. Six PICOs concerned CM1 children with/without additional structural anomalies (Syr, craniosynostosis, hydrocephalus, tethered cord, and cranio-vertebral junction anomalies), and one PICO Syr without CM1. We searched Medline, Embase, Cochrane, and NICE databases from January 1, 1999, to May 29, 2019. Cohort studies, controlled and randomized clinical trials (CCTs, RCTs), and systematic reviews were included, all pertinent only to patients ≤ 18 years of age.For CM1, 3787 records were found, 460 full texts were assessed and 49 studies (46 cohort studies, one RCT, and two systematic reviews) were finally included. For Syr, 376 records were found, 59 full texts were assessed, and five studies (one RCT and four cohort studies) were included. Data on each PICO were synthetized narratively due to heterogeneity in the inclusion criteria, outcome measures, and length of follow-up of the included studies.Despite decades of experience on CM1 and Syr management in children, the available evidence remains limited. Specifically, there is an urgent need for collaborative initiatives focusing on the adoption of shared inclusion criteria and outcome measures, as well as rigorous prospective designs, particularly RCTs.

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.

To present a unique case in which resection of a ventral tumor allowed for spontaneous resolution of a simultaneously noted dorsal Chiari I malformation.

Pertinent data, including presenting symptoms, hospital course, surgical notes, preoperative images, and postoperative images, were collected using the electronic medical record.

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.

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.

While a select population of pediatric patients with Chiari malformation type I (CM-I) remain asymptomatic, some patients present with tussive headaches, neurological deficits, progressive scoliosis, and other debilitating symptoms that necessitate surgical intervention. Surgery entails a variety of strategies to restore normal CSF flow, including increasing the posterior fossa volume via bone decompression only, or bone decompression with duraplasty, with or without obex exploration. The indications for duraplasty and obex exploration following bone decompression remain controversial. The objective of this study was to describe an institutional series of pediatric patients undergoing surgery for CM-I, performed by a single neurosurgeon. For patients presenting with a syrinx, the authors compared outcomes following bone-only decompression with duraplasty only and with duraplasty including obex exploration. Clinical outcomes evaluated included resolution of syrinx, scoliosis, presenting symptoms, and surgical complications.

A retrospective review was conducted of the medical records of 276 consecutive pediatric patients with CM-I operated on at a single institution between 2001 and 2015 by the senior author. Imaging findings of tonsillar descent, associated syrinx (syringomyelia or syringobulbia), basilar invagination, and clinical assessment of CM-I-attributable symptoms and scoliosis were recorded. In patients presenting with a syrinx, clinical outcomes, including syrinx resolution, symptom resolution, and impact on scoliosis progression, were compared for three surgical groups: bone-only/posterior fossa decompression (PFD), PFD with duraplasty (PFDwD), and PFD with duraplasty and obex exploration (PFDwDO).

PFD was performed in 25% of patients (69/276), PFDwD in 18% of patients (50/276), and PFDwDO in 57% of patients (157/276). The mean follow-up was 35 ± 35 months. Nearly half of the patients (132/276, 48%) had a syrinx. In patients presenting with a syrinx, PFDwDO was associated with a significantly higher likelihood of syrinx resolution relative to PFD only (HR 2.65, p = 0.028) and a significant difference in time to symptom resolution (HR 2.68, p = 0.033). Scoliosis outcomes did not differ among treatment groups (p = 0.275). Complications were not significantly higher when any duraplasty (PFDwD or PFDwDO) was performed following bone decompression (p > 0.99).

In this series of pediatric patients with CM-I, patients presenting with a syrinx who underwent expansile duraplasty with obex exploration had a significantly greater likelihood of syrinx and symptom resolution, without increased risk of CSF-related complications, compared to those who underwent bone-only decompression.

The pathophysiological connection between Chiari malformation and syringomyelia is accepted. Debate remains, however, how can we best define changes in syringomyelia following surgery.

To introduce a grading system focusing on syrinx reduction based on routinely and reproducible radiological information, and provide a suggestion of the application of this scale for prediction of patient's prognoses.

Data from 48 patients with Chiari malformation and syringomyelia were compiled. We calculated syrinx cross-sectional area by approximating an ellipse in the largest axial plane. We compared the percentage of reduction or enlargement following surgery. The percentage change was grouped into four grades: Grade 0 = Increasing size, grade I ≤ 50% reduction, grade II = 50% to 90% reduction, grade III ≥ 90% reduction.

A total of 89.6% of patients had syrinx improvement after surgery. A total of 5 patients were grade 0, 14 were grade I, 20 patients were grade II, and 9 patients met criteria for grade III. The mean postoperative syrinx area was 24.1 mm2 (0-169 mm2) with a mean syrinx reduction of 62.7%.

Radiological improvement of syringomyelia can be mathematically defined and standardized to assist in communication in outcome-based trials. Radiological resolution is expected most patients.

The aim of this study was to determine the timeline of syrinx regression and to identify factors mitigating syrinx resolution in pediatric patients with Chiari malformation type I (CM-I) undergoing posterior fossa decompression (PFD).

The authors conducted a retrospective review of records from pediatric patients (< 18 years old) undergoing PFD for the treatment of CM-I/syringomyelia (SM) between 1998 and 2015. Patient demographic, clinical, radiological, and surgical variables were collected and analyzed. Radiological information was reviewed at 4 time points: 1) pre-PFD, 2) within 6 months post-PFD, 3) within 12 months post-PFD, and 4) at maximum available follow-up. Syrinx regression was defined as ≥ 50% decrease in the maximal anteroposterior syrinx diameter (MSD). The time to syrinx regression was determined using Kaplan-Meier analysis. Multivariate analysis was conducted using a Cox proportional hazards model to determine the association between preoperative, clinical, and surgery-related factors and syrinx regression.

The authors identified 85 patients with CM-I/SM who underwent PFD. Within 3 months post-PFD, the mean MSD regressed from 8.1 ± 3.4 mm (preoperatively) to 5.6 ± 2.9 mm within 3 months post-PFD. Seventy patients (82.4%) achieved ≥ 50% regression in MSD. The median time to ≥ 50% regression in MSD was 8 months (95% CI 4.2-11.8 months). Using a risk-adjusted multivariable Cox proportional hazards model, the patients who underwent tonsil coagulation (n = 20) had a higher likelihood of achieving ≥ 50% syrinx regression in a shorter time (HR 2.86, 95% CI 1.2-6.9; p = 0.02). Thirty-six (75%) of 45 patients had improvement in headache at 2.9 months (IQR 1.5-4.4 months).

The maximum reduction in syrinx size can be expected within 3 months after PFD for patients with CM-I and a syrinx; however, the syringes continue to regress over time. Tonsil coagulation was associated with early syrinx regression in this cohort. However, the role of surgical maneuvers such as tonsil coagulation and arachnoid veil identification and sectioning in the overall role of CM-I surgery remains unclear.

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.

Chiari I Malformation (CMI) and the topics concerning it have been the subject of numerous discussions and polarizing controversies over the course of the past 20 years.

The opinions of 63 recognized international Neurosurgical CMI experts from 4 continents, with a collective surgical experience of more than 15,000 CMI cases, were gathered through a detailed questionnaire, divided in two parts: diagnostic and therapeutic. The therapeutic part was organized into four sections: Professional Profile, Technical Preferences, Surgical Opinions, and Clinical Scenarios.

The data reflected a wide spectrum of opinions, approaches, and expertise. The second part of the questionnaire dealt with the surgical aspects of CMI care and painted a more complex picture: • 81% of the surgeons preferred the Intradural technique. • 88% of the experts agreed that CMI surgery is not indicated for minimal non-debilitating symptoms alone, or as prophylaxis. • In the face of given clinical scenarios, a wide spectrum of therapeutic approaches was chosen by the whole group, but the 4 Surgeons with the largest case series expressed the same opinion. • Eight out of 63 Surgeons had a surgical experience above 600 cases, were responsible for more than half of the total 15,000 declared CMI cases, and shared a similar profile in terms of technical surgical choices, therapeutic opinions, and low complication rate, with a marked preference for Intradural techniques and tonsillar manipulation. • Once large individual case series were accumulated, we did not see any differences in the opinions and preferences between Adult and Pediatric Neurosurgeons.

Surgeons who have focused on CMI have been able to accumulate large surgical series, have chosen in their practices the more aggressive (and intrinsically more effective) CMI surgical techniques, and have achieved a low complication rate which compares favorably with that one of the extradural techniques.

Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction.

Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx.

Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications.

This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

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.

OBJECT Techniques for combined extradural and intradural decompression with expansile duraplasty for Chiari malformation type I (CM-I) have been well described, with various allogenic and autologous materials used for duraplasty. However, the approach and surgical technique used for duraplasty in our treatment of CM-I and developed by the senior author in the 1990s has not been described. METHODS A prospective database was initiated in March 2003 to denote the use of cervical fascia for duraplasty and incorporate an ongoing detailed record of complications during the surgical treatment of children and adults with CM-I with and without syringomyelia. A total of 389 surgeries for CM-I were performed on 379 patients from March 2003 to June 2016. A total of 123 posterior procedures were performed on 123 patients in which both a posterior fossa extradural and intradural decompression with duraplasty (extra-intradural) was performed. In this paper the authors describe the surgical technique for harvesting and using cervical fascia for duraplasty in the surgical treatment of CM-I and analyze and discuss complications from a prospective database spanning 2003-2016. RESULTS The authors found that cervical fascia can be harvested in patients of all ages (2-61 years old) without difficulty, and it provides a good substitute for dura in creating an expansile duraplasty in patients with CM-I. Cervical fascia is an elastic-like material with a consistency that allows for a strong watertight closure. Harvesting the cervical fascia graft does not require any further extension of the incision superiorly or inferiorly to obtain the graft. Complications were uncommon in this study of 123 children and adults. The risk of any type of complication (aseptic meningitis, CSF leak, pseudomeningocele, infection, development of hydrocephalus, and need for ventriculoperitoneal shunt) for the 78 patients in the pediatric age group was 0%. The risk of complication in the adult group was 6.7% (1 patient with aseptic meningitis and 2 patients with CSF leak). CONCLUSIONS Autologous cervical fascia is easy to obtain in patients of all ages and provides an effective material for duraplasty in the treatment of CM-I. Complications from the combination of both an extradural and intradural decompression with autologous cervical fascia duraplasty are uncommon.

Chiari I malformation, defined as herniation of the cerebellar tonsils at least 5 mm below the foramen magnum, can result from congenital or acquired pathology. While the mechanism is not well understood, an association between Chiari I and cystic fibrosis has been described in the literature. The lifelong respiratory status management necessitated by cystic fibrosis creates a greater risk of Chiari symptomatology as well as post-operative CSF-related complications in the setting of duraplasty secondary to recurrent transient increases in intracranial pressure. We will review the literature, describe our experience with these patients, and propose bony decompression as an approach to treatment.

A retrospective review of pediatric patients treated at our institution with both cystic fibrosis and Chiari I was performed. Since our first case in 2016, our department has evaluated four patients carrying that dual diagnosis. All four underwent posterior fossa decompression surgery. Two patients had incidental pathology. Two symptomatic patients exhibited headaches and/or coordination difficulty. Half of the patients had associated syringomyelia. All patients were offered posterior fossa decompression utilizing intraoperative ultrasound.

All four patients underwent posterior fossa decompression without duraplasty. Average operative time was 128 min. There were no complications post-operatively. Average hospital stay was 3.8 days. Average surgical length of stay was 2.3 days. Morbidity and mortality were 0%. The longest follow-up to date is 20 months. The two asymptomatic patients remained so post-operatively. The child with headaches and imbalance had complete resolution of his symptoms after surgery, as did the toddler with headaches. Both patients with syringomyelia demonstrated significant decrease in the size of their syrinxes on imaging performed at least 3 months post-operatively.

Based on the literature and our experience, we recommend considering posterior fossa decompression without duraplasty as treatment for pediatric cystic fibrosis patients with Chiari I malformation. This approach can be effective for symptomatic and prophylactic cases in this particular patient demographic because their comorbidities predispose them to Chiari pathology and symptomatology as well as certain post-operative complications.

Duraplasty is a technique successfully used to treat Chiari malformation type I (CM-I). This study describes the timely manner of clinical outcomes and the postoperative course after craniectomy and duraplasty for the treatment of symptomatic CM-I in children.

A retrospective chart review was performed in 105 consecutive children who underwent surgical decompression of symptomatic CM-I with dural opening by a single surgeon between 1999 and 2015.

In 16 of 28 children (57%) with typical Valsalva-related/tussive and mixed headaches, the symptoms resolved before discharge; by 6 months, all children were headache-free. Two of 28 children (7%) had recurrent headaches 9 months after surgery. Among the 78 children with syrinx, syrinx resolved or decreased in 68 (87%), recurred in 8 (10%), and was stable in 2 children (3%). Syrinx was resolved or decreased by 3 months in 51 children (65%) and by 6 months in 62 children (79%). Complications included aseptic meningitis requiring reoperation in 3 children (3%) and infection in one child (1%). Twelve children underwent reoperation, none within the first 30 days. No child had a major morbidity or mortality.

In carefully selected children with CM-I, a high success rate can be achieved with suboccipital decompression and duraplasty. Valsalva-related/tussive headaches resolved by the time of discharge from the hospital in the majority of children, and syrinx resolved or decreased in two-thirds of the children by 3 months. By 6 months, headaches were resolved in all cases, and syrinx was resolved or decreased in 79% of cases.

to develop the algorithm for defining the amount of posterior decompression of the craniovertebral junction in children with syringomyelia combined with Chiari-1 malformation.

Sixty eight children with syringomyelia and Chiari-1 malformation, under age of 18 years, underwent posterior decompression of the craniovertebral junction (PDCVJ) in the period from January 2001 to June 2016. Seven (10%) patients underwent extradural decompression (EDD), 16 (24%) patients underwent extra-arachnoid duraplasty (EAD), 25 (37%) patients underwent intra-arachnoid dissection (IAD) and duraplasty, and 20 (29%) patients underwent PDCVJ and placement of a fourth ventricle-subarachnoid shunt.

Clinical improvement occurred in 85% of patients, and stabilization was observed in 11% of patients. Syringomyelia regressed in 78% of cases. There were no complications associated with EDD; however, re-operation was required in 3 (43%) cases. In the case of EAD, treatment results were satisfactory in 11 (79%) patients; re-operation was required in 2 (12.5%) cases; there were no complications in the early postoperative period. The highest complication rate of 6 (30%) cases was associated with shunt placement and duraplasty. However, long-term results in this group of patients were satisfactory in 16 (94%) cases, and MRI-based positive changes were observed in 100% of cases.

According to our analysis, EAD is the method of choice for PDCVJ in children with syringomyelia and Chiari-1 malformation without myelopathy symptoms. In the presence of myelopathy symptoms, intra-arachnoid dissection (with or without shunting) is an acceptable alternative. To our opinion, the use of EDD in syringomyelia is unadvisable.