Raised intracranial pressure and hydrocephalus following hindbrain decompression for Chiari I malformation: a case series and review of the literature‡

Zakaria, Rasheed; Kandasamy, Jothy; Khan, Yousaf Aziz; Jenkinson, Michael D.; Hall, Samuel; Brodbelt, Andrew; Pigott, T.; Mallucci, Conor

doi:10.3109/02688697.2011.650738

Cited by 44 publications

(24 citation statements)

References 11 publications

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“…8,36 The pathophysiological mechanisms behind CMI and IIH are still poorly understood, and current treatment strategies are rather empirical, not addressing the primary cause of the disease, which in many cases can result in treatment failure. 7,13,19,31,45 Even though CMI and IIH differ with regard to clinical presentation, radiological findings, and treatment, common underlying mechanisms have been proposed 6 based on observations that both CMI and IIH occur frequently in young, often obese women; 9,24 that the 2 entities share similar symptoms; 19 and, most importantly, that some IIH patients present with radiological evidence of tonsillar ectopy. 1,4,40 In addition, some investigators have described patients diagnosed with CMI as having IIH-like symptoms and not responding to FMD.…”

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confidence: 97%

Comparative observational study on the clinical presentation, intracranial volume measurements, and intracranial pressure scores in patients with either Chiari malformation Type I or idiopathic intracranial hypertension

Frič

Eide

2017

JNS

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OBJECTIVE Several lines of evidence suggest common pathophysiological mechanisms in Chiari malformation Type I (CMI) and idiopathic intracranial hypertension (IIH). It has been hypothesized that tonsillar ectopy, a typical finding in CMI, is the result of elevated intracranial pressure (ICP) combined with a developmentally small posterior cranial fossa (PCF). To explore this hypothesis, the authors specifically investigated whether ICP is comparable in CMI and IIH and whether intracranial volumes (ICVs) are different in patients with CMI and IIH, which could explain the tonsillar ectopy in CMI. The authors also examined whether the symptom profile is comparable in these 2 patient groups. METHODS The authors identified all CMI and IIH patients who had undergone overnight diagnostic ICP monitoring during the period from 2002 to 2014 and reviewed their clinical records and radiological examinations. Ventricular CSF volume (VV), PCF volume (PCFV), and total ICV were calculated from initial MRI studies by using volumetric software. The static and pulsatile ICP scores during overnight monitoring were analyzed. Furthermore, the authors included a reference (REF) group consisting of patients who had undergone ICP monitoring due to suspected idiopathic normal-pressure hydrocephalus or chronic daily headache and showed normal pressure values. RESULTS Sixty-six patients with CMI and 41 with IIH were identified, with comparable demographics noted in both groups. The occurrence of some symptoms (headache, nausea, and/or vomiting) was comparable between the cohorts. Dizziness and gait ataxia were significantly more common in patients with CMI, whereas visual symptoms, diplopia, and tinnitus were significantly more frequent in patients with IIH. The cranial volume measurements (VV, PCFV, and ICV) of the CMI and IIH patients were similar. Notably, 7.3% of the IIH patients had tonsillar descent qualifying for diagnosis of CMI (that is, > 5 mm). The extent of tonsillar ectopy was significantly different between the CMI and IIH cohorts (p < 0.001) but also between these 2 cohorts and the REF group. Pulsatile ICP was elevated in both cohorts without any significant between-group differences; however, static ICP was significantly higher (p < 0.001) in the IIH group. CONCLUSIONS This study showed comparable and elevated pulsatile ICP, indicative of impaired intracranial compliance, in both CMI and IIH cohorts, while static ICP was higher in the IIH cohort. The data did not support the hypothesis that reduced PCFV combined with increased ICP causes tonsillar ectopy in CMI. Even though impaired intracranial compliance seems to be a common pathophysiological mechanism behind both conditions, the mechanisms explaining the different clinical and radiological presentations of CMI and IIH remain undefined.

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confidence: 97%

Comparative observational study on the clinical presentation, intracranial volume measurements, and intracranial pressure scores in patients with either Chiari malformation Type I or idiopathic intracranial hypertension

Frič

Eide

2017

JNS

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“…Although the pulsatile CSF stroke volume is only $1 cc with each cardiac cycle [10,11], the partial stenosis near the CVJ causes elevated CSF pressure gradients [12,13] that can result in severe neurologic consequences. At present, MRI techniques are being developed to measure CSF pressure gradients noninvasively [14], but these methods are not in standard use and have not been validated.…”

Section: Introductionmentioning

confidence: 99%

Cerebrospinal Fluid Flow Impedance is Elevated in Type I Chiari Malformation

Shaffer

Martin

Rocque

et al. 2014

Journal of Biomechanical Engineering

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Diagnosis of Type I Chiari malformation (CMI) is difficult because the most commonly used diagnostic criterion, cerebellar tonsillar herniation (CTH) greater than 3-5 mm past the foramen magnum, has been found to have little correlation with patient symptom severity. Thus, there is a need to identify new objective measurement(s) to help quantify CMI severity. This study investigated longitudinal impedance (LI) as a parameter to assess CMI in terms of impedance to cerebrospinal fluid motion near the craniovertebral junction. LI was assessed in CMI patients (N ¼ 15) and age-matched healthy controls (N ¼ 8) using computational fluid dynamics based on subject-specific magnetic resonance imaging (MRI) measurements of the cervical spinal subarachnoid space. In addition, CTH was measured for each subject. Mean LI in the CMI group (551 6 66 dyn/cm 5 ) was significantly higher than in controls (220 6 17 dyn/cm 5 , p < 0.001). Mean CTH in the CMI group was 9.0 6 1.1 mm compared to À0.4 6 0.5 mm in controls. Regression analysis of LI versus CTH found a weak relationship (R 2 ¼ 0.46, p < 0.001), demonstrating that CTH was not a good indicator of the impedance to CSF motion caused by cerebellar herniation. These results showed that CSF flow impedance was elevated in CMI patients and that LI provides different information than a standard CTH measurement. Further research is necessary to determine if LI can be useful in CMI patient diagnosis.

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“…Prospective analysis of this topic is needed, but a prospective study is difficult because of the rarity of post-CM-I decompression hydrocephalus necessitating CSF diversion; we found an incidence of 7.4%, which is comparable to similarly low rates reported in other studies. 15 In our study we did not examine radiographic risk factors, which likely also play an important role in the prediction of which patients will develop post-decompression hydrocephalus. Future studies evaluating radiographic variables in these patients, preferably in conjunction with clinical variables, would likely offer more robust associations.…”

Section: Discussionmentioning

confidence: 98%

Chiari-related hydrocephalus: assessment of clinical risk factors in a cohort of 297 consecutive patients

Guan

Riva-Cambrin

Brockmeyer

2016

FOC

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OBJECTIVE Patients treated for Chiari I malformation (CM-I) with posterior fossa decompression (PFD) may occasionally and unpredictably develop postoperative hydrocephalus. The clinical risk factors predictive of this type of Chiari-related hydrocephalus (CRH) are unknown. The authors' objective was to evaluate their experience to identify risk factors that may predict which of these patients undergoing PFD will develop CRH after surgery. METHODS The authors performed a retrospective clinical chart review of all patients who underwent PFD surgery and duraplasty for CM-I at the Primary Children's Hospital in Utah from June 1, 2005, through May 31, 2015. Patients were dichotomized based on the need for long-term CSF diversion after PFD. Analysis included both univariate and multivariable logistic regression analyses. RESULTS The authors identified 297 decompressive surgeries over the period of the study, 22 of which required long-term postoperative CSF diversion. On multivariable analysis, age < 6 years old (OR 3.342, 95% CI 1.282–8.713), higher intraoperative blood loss (OR 1.003, 95% CI 1.001–1.006), and the presence of a fourth ventricular web (OR 3.752, 95% CI 1.306–10.783) were significantly associated with the need for long-term CSF diversion after decompressive surgery. CONCLUSIONS Younger patients, those with extensive intraoperative blood loss, and those found during surgery to have a fourth ventricular web were at higher risk for the development of CRH. Clinicians should be alert to evidence of CRH in this patient population after PFD surgery.

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Raised intracranial pressure and hydrocephalus following hindbrain decompression for Chiari I malformation: a case series and review of the literature‡

Cited by 44 publications

References 11 publications

Comparative observational study on the clinical presentation, intracranial volume measurements, and intracranial pressure scores in patients with either Chiari malformation Type I or idiopathic intracranial hypertension

Comparative observational study on the clinical presentation, intracranial volume measurements, and intracranial pressure scores in patients with either Chiari malformation Type I or idiopathic intracranial hypertension

Cerebrospinal Fluid Flow Impedance is Elevated in Type I Chiari Malformation

Chiari-related hydrocephalus: assessment of clinical risk factors in a cohort of 297 consecutive patients

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