Cerebrospinal fluid area and syringogenesis in Chiari malformation type I

J Neurosurg 134:825–830, 2021

Syringogenesis in Chiari malformation type I (CM-I) is thought to occur secondary to impaction of the cerebellar tonsils within the foramen magnum (FM). However, the correlation between the CSF area and syringogenesis has yet to be elucidated. The authors sought to determine whether the diminution in subarachnoid space is associated with syringogenesis. Further, the authors sought to determine if syrinx resolution was associated with the degree of expansion of subarachnoid spaces after surgery.

METHODS The authors performed a retrospective review of all patients undergoing posterior fossa decompression for CM-I from 2004 to 2016 at the University of Virginia Health System. The subarachnoid spaces at the FM and at the level of the most severe stenosis were measured before and after surgery by manual delineation of the canal and neural tissue area on MRI and verified through automated CSF intensity measurements. Imaging and clinical outcomes were then compared.

RESULTS Of 68 patients, 26 had a syrinx at presentation. Syrinx patients had significantly less subarachnoid space at the FM (13% vs 19%, p = 0.0070) compared to those without syrinx. Following matching based on degree of tonsillar herniation and age, the subarachnoid space was significantly smaller in patients with a syrinx (12% vs 19%, p = 0.0015). Syrinx resolution was associated with an increase in patients’ subarachnoid space after surgery compared with those patients without resolution (23% vs 10%, p = 0.0323).

CONCLUSIONS Syrinx development in CM-I patients is correlated with the degree to which the subarachnoid CSF spaces are diminished at the cranial outlet. Successful syrinx reduction is associated with the degree to which the subarachnoid spaces are increased following surgery.

One-year outcome in patients with idiopathic normal-pressure hydrocephalus: comparison of lumboperitoneal shunt to ventriculoperitoneal shunt

J Neurosurg 125:1483–1492, 2016

Idiopathic normal pressure hydrocephalus (iNPH) is treated with cerebrospinal fluid shunting, and implantation of a ventriculoperitoneal shunt (VPS) is the current standard treatment. The objective of this study was to compare the efficacy and safety of VPSs and lumboperitoneal shunts (LPSs) for patients with iNPH.

Methods The authors conducted a prospective multicenter study of LPS use for patients with iNPH. Eighty-three patients with iNPH (age 60 to 85 years) who presented with ventriculomegaly and high-convexity and medial subarachnoid space tightness on MR images were recruited from 20 neurological or neurosurgical centers in Japan between March 1, 2010, and October 19, 2011. The primary outcome was the modified Rankin Scale (mRS) score 1 year after surgery, and the secondary outcome included scores on the iNPH grading scale (iNPHGS). A previously conducted VPS cohort study with the same inclusion criteria and primary and secondary end points was used as a historical control.

Results: The proportion of patients who achieved a favorable outcome (i.e., improvement of at least 1 point in their mRS score) was 63% (95% CI 51%–73%) and was comparable to values reported with VPS implantation (69%, 95% CI 59%–78%). Using the iNPHGS, the 1-year improvement rate was 75% (95% CI 64%–84%) and was comparable to the rate found in the VPS study (77%, 95% CI 68%–84%). The proportion of patients experiencing serious adverse events (SAEs) and non-SAEs did not differ signi cantly between the groups at 1 year after surgery (SAEs: 19 [22%] of 87 LPS patients vs 15 [15%] of 100 VPS patients, p = 0.226; non-SAEs: 24 [27.6%] LPS patients vs 20 [20%] VPS patients, p = 0.223). However, shunt revisions were more common in LPS-treated patients than in VPS-treated patients (6 [7%] vs 1 [1%]).

Conclusions The efficacy and safety rates for LPSs with programmable valves are comparable to those for VPSs for the treatment of patients with iNPH. Despite the relatively high shunt failure rate, an LPS can be the treatment of choice because of its minimal invasiveness and avoidance of brain injury.

 

Benign external hydrocephalus: a review, with emphasis on management

Neurosurg Rev. DOI 10.1007/s10143-011-0327-4

Benign external hydrocephalus in infants, characterized by macrocephaly and typical neuroimaging findings, is considered as a self-limiting condition and is therefore rarely treated. This review concerns all aspects of this condition: etiology, neuroimaging, symptoms and clinical findings, treatment, and outcome, with emphasis on management. The review is based on a systematic search in the Pubmed and Web of Science databases. The search covered various forms of hydrocephalus, extracerebral fluid, and macrocephaly. Studies reporting small children with idiopathic external hydrocephalus were included, mostly focusing on the studies reporting a long-term outcome.

A total of 147 studies are included, the majority however with a limited methodological quality. Several theories regarding pathophysiology and various symptoms, signs, and clinical findings underscore the heterogeneity of the condition. Neuroimaging is important in the differentiation between external hydrocephalus and similar conditions. A transient delay of psychomotor development is commonly seen during childhood. A long-term outcome is scarcely reported, and the results are varying.

Although most children with external hydrocephalus seem to do well both initially and in the long term, a substantial number of patients show temporary or permanent psychomotor delay. To verify that this truly is a benign condition, we suggest that future research on external hydrocephalus should focus on the long-term effects of surgical treatment as opposed to conservative management.

Characterization of Cyclic CSF Flow in the Foramen Magnum and Upper Cervical Spinal Canal with MR Flow Imaging and Computational Fluid Dynamics

AJNR Am J Neuroradiol 31:997–1002.DOI 10.3174/ajnr.A1995

CSF flow has been shown to exhibit complex patterns in MR images in both healthy subjects and in patients with Chiari I. Abnormal CSF flow oscillations, according to prevailing opinion, cause syringomyelia and other clinical manifestations that affect some patients with the Chiari I malformation.

For this article, we reviewed the literature on PC MR of CSF flow, collected the published CFD studies relevant to CSF flow, and performed flow simulations. PC MR creates cine and still images of CSF flow and measurements of flow velocities. CFD, a technique used to compute flow and pressure in liquid systems, simulates the CSF flow patterns that occur in a specific geometry or anatomy of the SAS and a specific volume of flow.

Published PC MR studies show greater peak CSF velocities and more complex flow patterns in patients with Chiari I than in healthy subjects, with synchronous bidirectional flow one of the characteristic markers of pathologic flow. In mathematic models of the SAS created from high-resolution MR images, CFD displays complex CSF flow patterns similar to those shown in PC MR in patients. CFD shows that the pressure and flow patterns vary from level to level in the upper spinal canal and differ between patients with Chiari and healthy volunteers. In models in which elasticity and motion are incorporated, CFD displays CSF pressure waves in the SAS.

PC MR and CFD studies to date demonstrate significant alterations of CSF flow and pressure patterns in patients with Chiari I. CSF flow has nonlaminar complex spatial and temporal variations and associated pressure waves and pressure gradients. Additional simulations of CSF flow supplemented by PC MR will lead to better measures for distinguishing pathologic flow abnormalities that cause syringomyelia, headaches, and other clinical manifestations in Chiari I malformations.

CFD  computational fluid dynamics; PC MR  phase-contrast cardiac-gated MR studies; SAS  subarachnoid space