Chiari type I malformation in children

Massimi, Luca; Novegno, Federica; Rocco, Concezio Di

doi:10.1007/978-3-7091-0673-0_6

Cited by 30 publications

(9 citation statements)

References 235 publications

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“…However, in many dogs, SM also occurs in the thoracic and lumbar spinal cord and although thoracolumbar (TL) pain is frequently noted we also observed that many CKCS have lumbosacral (LS) pain . This is consistent with the findings in Chiari‐type 1 malformation (CM1), the equivalent human condition . Headaches, neck pain, and paresthesia of the upper extremities dominate the clinical presentation in these patients, but lower back pain also occurs …”

Section: Introductionsupporting

confidence: 85%

“…Alterations in this anchoring can increase tension on the caudal spinal cord with clinical consequences, so‐called tethered cord syndrome (TCS) in people . There is an association among a subset of CM1 patients, as well as those suffering from SM, with TCS . To date, the relationship between Chiari malformations, syringomyelia (CMSM) and TCS has not been explored in dogs; although, a recent study using a 3‐dimensional computer model suggested tethering as a potential mechanism for SM development within the canine spinal cord …”

Section: Introductionmentioning

confidence: 99%

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Morphometric analysis of spinal cord termination in Cavalier King Charles Spaniels

Sparks

Robertson

Olby

2019

Veterinary Internal Medicne

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Background There is an association between Chiari malformations, syringomyelia (CMSM) and tethered cord syndrome (TCS) in people, suggesting Cavalier King Charles Spaniels (CKCS) with CMSM could also have TCS. Currently there are no data on the position of the caudal spinal cord structures in CKCS. Objective To describe and compare location of spinal cord termination in CKCS with weight‐matched controls and to examine the relationship between SM and spinal cord termination. Animals Thirty‐nine CKCS and 33 controls with thoracolumbar MRIs; 34 of 39 CKCS also had cervical MRIs. Methods Blinded retrospective study. Spinal cord and dural sac termination were determined from T2‐weighted sagittal and transverse images and half‐Fourier acquisition single‐shot turbo spin echo sequences. Intra‐observer reliability was determined using kappa analysis. Presence of SM was compared with location of spinal cord and dural sac termination. Results Intra‐observer reliability was moderate for identifying spinal cord termination (Kappa = 0.6) and good for dural sac termination (Kappa = 0.8). The spinal cord terminated at lumbar vertebra 6 (L6) in 1, 7 (L7) in 22, and sacrum in 16 CKCS versus 9 at L6, 23 at L7, 1 at sacrum in controls. Spinal cord (P < .001) and dural sac (P = .002) termination were significantly more caudal in CKCS compared to controls. The presence of thoracolumbar SM was associated with more caudal dural sac termination in CKCS (P = .03). Conclusions and Clinical Importance The relationship between TL SM and possible spinal cord tethering because of a more caudal dural sac termination should be investigated.

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Section: Introductionsupporting

confidence: 85%

Section: Introductionmentioning

confidence: 99%

Morphometric analysis of spinal cord termination in Cavalier King Charles Spaniels

Sparks

Robertson

Olby

2019

Veterinary Internal Medicne

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“…Section 3-Isolated/non-CM1 pediatric syringomyelia Part A: Differential diagnosis (Table 7) As shown, syringomyelia is frequently associated with CM1 and it is a discriminating factor influencing the indication for surgery and the assessment of outcome [75][76][77]. Its management is strictly related to the management of CM1.…”

Section: Agreement: 905%mentioning

confidence: 99%

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

et al. 2021

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Background 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. Methods 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). Results 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.” Conclusions 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.

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“…Radiographic evidence if CM1 is common, with a magnetic resonance imaging (MRI) prevalence in children close to 4% [2]. While some affected individuals are asymptomatic, crowding in the posterior fossa often leads to compression of the cerebellum and cervicomedullary junction, causing a variety of signs and symptoms, ranging from headache to brainstem compromise [3–5]. …”

Section: Introductionmentioning

confidence: 99%

Comparison of posterior fossa decompression with or without duraplasty in children with Type I Chiari malformation

et al. 2014

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Purpose Chiari Malformation Type I (CM1) is a common and often debilitating neurosurgical disease. Whether to treat CM1 patients with a traditional posterior fossa decompression with duraplasty (PFDD) or a less invasive extradural decompression (PFDO) is controversial. The purpose of this study was to compare clinical outcome and syrinx resolution between the two procedures. Methods We retrospectively reviewed the records of 36 patients treated with PFDD and 29 patients with PFDO between 2003 and 2011. We compared baseline demographic, clinical, and radiographic characteristics. The primary clinical outcome was the Chicago Chiari Outcome Scale (CCOS). The primary radiographic outcome was qualitative syrinx improvement or resolution. Results At baseline, age and sex distributions, radiographic characteristics, and presenting symptoms were similar in patients undergoing PFDD and PFDO. Patients undergoing PFDO had shorter surgical time (1.5 vs. 2.8 hours; p < 0.001) and length of hospital stay (2.1 compared to 3.3 days; p < 0.001). Cerebrospinal fluid-related complications were more common in patients receiving PFDD (7/36) than PFDO (0/29) (p=0.014). Clinical improvement, defined by the mean CCOS score, was comparable in patients receiving PFDO (14.7) and PFDD (14.6) (p=0.70). Among patients with postoperative syrinx imaging, 10/13 in the PFDD group improved or resolved, compared to 8/8 in the PFDO group (p=0.26). Conclusions Extradural decompression for CM1 produces comparable rates of clinical and radiographic improvement as the more-invasive decompression with duraplasty. Given the increased morbidity and resource utilization associated with PFDD, PFDO should be considered an attractive first-line option for most CM1 patients.

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Chiari type I malformation in children

Cited by 30 publications

References 235 publications

Morphometric analysis of spinal cord termination in Cavalier King Charles Spaniels

Morphometric analysis of spinal cord termination in Cavalier King Charles Spaniels

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

Comparison of posterior fossa decompression with or without duraplasty in children with Type I Chiari malformation

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