Search

In 1891, Hans Chiari described a group of congenital hindbrain anomalies, which were eventually named after him. He classified these malformations into three types (Chiari malformations I, II, and III), and four years later added the Chiari IV malformation. However, numerous reports across the literature do not seem to fit Chiari's original descriptions of these malformations, so researchers have been encouraged to propose new classifications to encompass these variants (e.g., Chiari 0, Chiari1.5, and Chiari 3.5 malformations). Moreover, there is a continued misunderstanding and misuse of the term "Chiari IV malformation." Therefore, the current review intended to describe anatomical, pathophysiological, and clinical aspects of the newer classifications with clarifications of the Chiari malformations. We reviewed available literature about Chiari malformations and their variants using "PubMed" and "Google Scholar." We also looked into the term Chiari IV, clarifying its original description and citing examples where the term has been used erroneously. References in the reviewed articles were searched manually. Variants of the originally described Chiari malformations are termed Chiari 0, Chiari 1.5, and Chiari 3.5. Each has distinct anatomical characteristics and some of these are extremely rare and incompatible with life (e.g. Chiari 3.5). Chiari IV malformation has been further clarified. Some physicians might be unfamiliar with the newer classifications of Chiari malformations because these conditions are rare or even unique. Furthermore, care is needed in using the term "Chiari IV malformation", which must be consistent with Chiari's original description, i.e. an occipital encephalocele containing supratentorial contents. Clin. Anat. 31:314-322, 2018. © 2018 Wiley Periodicals, Inc., (© 2018 Wiley Periodicals, Inc.)

Although the Chiari malformations are well-studied and described developmental anomalies, there remains some incongruity in regards to their underlying etiologies. A number of theories have been proposed with the purpose of accounting for the embryology and pathogenesis of the Chiari I and II malformations and their associated complications and clinical syndromes. The present review aims to review the pertinent literature for all of the main theories that have been proposed, and outline their validity and relevance to our contemporary understanding of these anomalies. Clin. Anat. 31:202-215, 2018. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Objectives: To characterise the symptomatic phenotype of Chiari-like malformation (CM), secondary syringomyelia (SM) and brachycephaly in the Cavalier King Charles Spaniel using morphometric measurements on mid-sagittal Magnetic Resonance images (MRI) of the brain and craniocervical junction., Methods: This retrospective study, based on a previous quantitative analysis in the Griffon Bruxellois (GB), used 24 measurements taken on 130 T1-weighted MRI of hindbrain and cervical region. Associated brachycephaly was estimated using 26 measurements, including rostral forebrain flattening and olfactory lobe rotation, on 72 T2-weighted MRI of the whole brain. Both study cohorts were divided into three groups; Control, CM pain and SM and their morphometries compared with each other., Results: Fourteen significant traits were identified in the hindbrain study and nine traits in the whole brain study, six of which were similar to the GB and suggest a common aetiology. The Control cohort had the most elliptical brain (p = 0.010), least olfactory bulb rotation (p = 0.003) and a protective angle (p = 0.004) compared to the other groups. The CM pain cohort had the greatest rostral forebrain flattening (p = 0.007), shortest basioccipital (p = 0.019), but a greater distance between the atlas and basioccipital (p = 0.002) which was protective for SM. The SM cohort had two conformation anomalies depending on the severity of craniocervical junction incongruities; i) the proximity of the dens (p <0.001) ii) increased airorhynchy with a smaller, more ventrally rotated olfactory bulb (p <0.001). Both generated 'concertina' flexures of the brain and craniocervical junction., Conclusion: Morphometric mapping provides a diagnostic tool for quantifying symptomatic CM, secondary SM and their relationship with brachycephaly. It is hypothesized that CM pain is associated with increased brachycephaly and SM can result from different combinations of abnormalities of the forebrain, caudal fossa and craniocervical junction which compromise the neural parenchyma and impede cerebrospinal fluid flow., Competing Interests: SPK is a post-graduate research student and CC an undergraduate at the University of Surrey where CR and RMLR are employed. ZK is employed by Montreal University. JJ, CD, AT and CR are employed by Fitzpatrick Referrals Ltd, Surrey, GU7 Q22. SG is employed by the Goddard Veterinary Group, Stone Lion Veterinary Hospital, SW19 5AU. AKM is an independent statistical consultant operating professionally as a sole trader under the company name of akm-stats and did not receive any salary or other remittance for his contribution in this investigation. The University of Surrey, Montreal University, Fitzpatrick Referrals Ltd, Goddard Veterinary Group did not play a role in the study design, data collection and analysis, decision to publish, or preparation of the manuscript and only provided financial support in the form of authors' salaries and/or research materials. None of the authors have personal or financial relationships with other people or organizations that might inappropriately influence or bias the content of the paper. There are no patents, products in development, or marketed products to declare. This does not alter the authors' adherence to PLOS ONE policies on sharing data and materials.

It is proposed that negative central nervous system (CNS) pressure is one of the filling mechanisms of the fluid spaces of the CNS. Negative CNS pressure is caused by the combination of gravitational force and body movement. The venous system imposes pressure fluctuations on the CNS due to changes in posture and body cavity pressure. It is proposed here that filling of veins, arteries and cerebrospinal (CSF) spaces are all assisted by negative CNS pressure. Hyperemia in the CNS in response to pressure changes with movement was described in the first part of this hypothesis. By this means parenchyma water levels may increase (Williams, 2008). In the developmental forms of hydrocephalus expansion of CSF spaces is a more prominent feature than parenchyma water changes. This feature is explained by this second part of the hypothesis where the negative pulsatility of pressure that can accompany positive pressure pulsatility, which occurs with body movement, is described as the pathological force that leads to cavity filling. When CNS compliance is lost there is overrepresentation of low and well as high pressure pulsations in response to body movements. Pressure that leads to the development of hydrocephalus can be described as being abnormally labile. Negative CNS pressure causes cavity filling in an analogous way to pleural cavity filling, with water passing from parenchyma tissue. Positive pressure within the pressure profile may cause expansion of regions of the CNS skeletal system that are able to grow such as the cranial vault so that large head size is a frequent accompaniment to hydrocephalus that is caused by this mechanism. Hydrocephalic disorders that are characterised by negative pressure filling mechanism often have a skeletal anatomical abnormality that causes reduced CNS compliance and adversely affect neural development. This is often in the form of obstruction to CSF flow around the base of the brain that then leads to vault expansion by means of high pressure pulsation and ventricle enlargement by means of low pressure pulsation. In health pressure pulsatility does not lead to enduring changes in water distribution within the CNS compartments but it assists physiological water balance., (Copyright © 2016 The Author. Published by Elsevier Ltd.. All rights reserved.)

Hindbrain herniations come in many forms and have been further subdivided as their original descriptions. For cerebellar tonsillar ectopia, they can be divided into two categories, acquired and congenital. Acquired hindbrain herniations are due to increased intracranial pressure caused by certain conditions such as trauma or brain tumor. Although the mechanism for their formation is not clear, congenital hindbrain herniation makes up the majority of these congenital malformations. Furthermore, these malformations are often found to harbor additional anatomical derailments in addition to the hindbrain herniation whether it be the cerebellar tonsils (Chiari I malformation) or the cerebellar vermis (Chiari II malformation). This article reviews these forms of cerebellar ectopia and describes the details of their anomalous anatomy. Moreover, this article compares and contrasts the differing embryological theories found in this literature., (© 2014 Wiley Periodicals, Inc.)

Purpose: To evaluate bSSFP (balanced steady state free precession) and half-Fourier RARE (rapid acceleration with relaxation enhancement) MRI sequences in their ability to demonstrate fetal anatomic landmarks, quantify the degree of cerebellar herniation in Chiari II malformations and level and length of the associated open neural tube defects, as well as evaluate interobserver reliability of these measurements., Materials and Methods: Two independent observers retrospectively reviewed MRIs of 37 fetuses with Chiari II malformations and associated open neural tube defects (mean gestational age: 27 weeks 2 days). Comparison of bSSFP and RARE included: (i) Ability to confidently identify anatomic landmarks of the posterior fossa and spine; (ii) Measurements of the foramen magnum, cerebellar tonsillar herniation length, intervertebral disc space level of tonsillar herniation, open neural tube defect length, and disc space start and end level of the open neural tube defect; (iii) Observed conspicuity of anatomic landmarks., Results: There was no significant difference in assessment of cerebellar tonsillar herniation or open neural tube defect level between bSSFP and RARE for either observer. Intervertebral discs were more conspicuous on bSSFP while cerebellar tonsils were more conspicuous on RARE (P < 0.05). Interobserver reliability was strong for both sequences in assessing the foramen magnum (r = 0.95, 0.94), tonsillar herniation length (r = 0.93, 0.95), and open neural tube defect length (r = 0.97, 0.96)., Conclusion: Despite improved conspicuity of the intervertebral discs with bSSFP and cerebellar tonsils with RARE, there is no significant difference in measurement of hindbrain herniation or open neural tube defect level; interobserver reliability is excellent for both sequences., (Copyright © 2013 Wiley Periodicals, Inc.)

Object: Chiari malformation Type I (CM-I) is characterized by hindbrain deformity. We investigated the effects of craniocervical decompression surgery on the anatomical features of hindbrain deformity with a prospective MRI study of patients with CM-I., Methods: A prospective longitudinal study was conducted in 48 patients with CM-I (39 with syringomyelia) treated with craniocervical decompression. Clinical examinations and cervical MRI were performed before surgery and 1 week, 3-6 months, and annually after surgery. Hindbrain deformity was defined by tonsillar ectopia, pointed cerebellar tonsils, and/or cervicomedullary protuberance. The length of the clivus, basiocciput (sphenooccipital synchondrosis to basion), supraocciput (internal occipital protuberance to opisthion), and anteroposterior (AP) width of CSF pathways at the foramen magnum were measured and compared with those from 18 healthy volunteers (control group)., Results: Before surgery, the patients' posterior fossa bones were short and their CSF pathways were narrow. All patients had tonsillar ectopia (mean [± SD] 12.3 ± 5.1 mm; normal 0.3 ± 1.0). The majority of patients had pointed tonsils and more than two-thirds exhibited a cervicomedullary protuberance. Clivus and basiocciput lengths were significantly shorter than the values obtained in the control group. However, the supraocciput length did not differ significantly from control measurements. The mean bulbopontine sulcus distance superior to the basion was 9.5 ± 2.6 mm (vs 13.6 ± 2.8 mm in controls; p < 0.0001). The AP widths of the CSF pathways at the level of the foramen magnum were significantly narrowed. After surgery, CSF pathways significantly expanded both ventrally and dorsally. By 3-6 months after surgery, pointed tonsils became round, cervicomedullary protuberance disappeared, and tonsillar ectopia diminished by 51% (to 6.0 ± 3.3 mm; p < 0.0001)., Conclusions: The cerebellar tonsils and brainstem assumed a normal appearance within 6 months after craniocervical decompression. These findings support the concept that the CM-I is not a congenital malformation of the neural elements but rather an acquired malformation that arises from pulsatile impaction of the cerebellar tonsils into the foramen magnum. Clinical trial registration no.: NCT00001327.

Partial rhombencephalosynapsis (PRECS) has been recently reported in association with Chiari II (CII). However, its existence as a true malformation is challenged due to the anatomical changes potentially induced by CII. The aim of this report was to investigate the contribution of midbrain/hindbrain tractography in this setting. A 13-year-old boy with a known CII malformation and operated myelomeningocele was referred for brain imaging after a first complex partial seizure. In addition to the classical features of CII, MRI showed partially fused cerebellar hemispheres and multiple supratentorial abnormalities. Diffusion tensor imaging (DTI) color map and tractography showed absent transverse fibers on the midsection of the cerebellum, scarce fibers of the middle cerebellar peduncle (MCP), absence of the middle pontine crossing tract, and fibers running vertically in the medial part of the cerebellum. Vertical mediocerebellar fibers are a feature of classical RECS and the paucity or absence of MCP fibers is mainly described in CII. In our patient, DTI and FT therefore demonstrated structural characteristics of both RECS and CII confirming their potential coexistence and suggesting possible shared embryological pathway.

Object: Hindbrain herniation syndrome, or Chiari malformation Type I (CM-I), occurs frequently with craniovertebral junction (CVJ) abnormalities when there is reduction in the posterior fossa volume. Syringomyelia is often present. Posterior fossa dorsal decompression (PFDD) is typically performed but has adverse results when ventral bone abnormality exists. This paper presents the results of a prospective study on CVJ abnormalities in patients with CM-I and syringomyelia., Methods: Between 1984 and 2008 (the MR imaging era), 298 patients with CVJ abnormalities and CM-I underwent ventral cervicomedullary decompression. Eighty-four patients had associated syringomyelia (15 with secondary invagination and 69 with primary basilar invagination, os odontoideum, or malunion of fractures). Of these 84 patients with CVJ abnormalities, CM-I, and syringomyelia, 46 had previously undergone PFDD, and 28 had previously undergone PFDD combined with fusion procedures or shunt placements. Of the 84 patients, a cervicothoracic syrinx was observed in 57, thoracic syrinx in 14, and holocord syrinx in 13. Studies included CT, MR imaging, and cine flow studies. All 298 patients who underwent ventral CVJ decompression had irreducible or partially reducible pathology. All 84 with syringomyelia showed brainstem dysfunction, lower cranial nerve symptoms, or myelopathy., Results: Brainstem signs improved in 66 of the 84 patients, myelopathy improved in 58, and syringomyelia regressed in 64., Conclusions: Neurological improvement and syringomyelia resolution can occur using only ventral cervicomedullary junction decompression in patients with basilar invagination and basilar impression. This is likely due to the relief of neural encroachment and reestablishment of CSF pathways.

Background and Objectives: The cerebellar tonsillar herniation occurring in Chiari malformation Type I (CMI) mainly results from overcrowding of a normally developing hindbrain within a primary small posterior cranial fossa (PCF) due to an anomaly in the embryological development of the occipital bone. In the present study, the lengths of PCF parameters were studied in adult CMI patients., Patients and Methods: The authors retrospectively examined 15 adult patients with CMI. Multiple measurements were made on magnetic resonance images (MRIs). The results were compared with the findings in 25 controls and 30 dry skulls., Results: Length of the neural structures did not significantly differ between the CMI and the control groups. The average length of the basiocciput was significantly shorter in the CMI group as compared with the control group. The mean length of the supraocciput was significantly shorter and the average diameter of the foramen magnum was significantly longer in the CMI group when compared to the control group and dry skulls., Conclusions: The morphometric data suggest that, in CMI, a hypoplastic occipital bone, possibly due to the paraxial mesodermal defect of the parachordal plate, causes overcrowding in PCF, which contains the normally developed neural structures., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Purpose: Foramen magnum dimensions and intracranial volume in Chiari I malformations in children were studied, and the statistical relationship between patient demographics, radiological features and foramen magnum morphometry was investigated., Methods: Linear measurements were used to calculate the intracranial volume using preoperative magnetic resonance images and computed tomogram images. The area of the foramen magnum was obtained independently using computer imaging software and a regression formula. The result of 21 pediatric patients was compared with a matched control group., Results: The area of the foramen magnum was within the range of the expected value deduced using a formula based on the intracranial volume. There was no statistical difference in the area and linear dimensions of the foramen magnum in the study and control groups. Six patients (28%) had a foramen magnum in close proximity to a spherical shape., Conclusion: The authors provide a simple, accurate and reproducible method of estimating foramen magnum area in the pediatric Chiari I group. The irregular shape of the foramen magnum is accentuated by developmental bony and soft tissue anomalies at the cranio-vertebral junction in Chiari malformation. Consequently, an individualized cross-sectional assessment of the foramen magnum in relation to the hindbrain tissue in the same plane is required to study the initiation and propagation of the Chiari I symptomatology.

Introduction: Chiari type II malformation (CII) is a developmental deformity of the hindbrain. We have previously reported that many patients with CII have impaired smooth pursuit, while few make inaccurate saccades or have an abnormal vestibuloocular reflex. In contrast, saccadic adaptation and visual fixation are normal. In this report, we correlate results from several eye movement studies with neuroimaging in CII. We present a model for structural changes within the cerebellum in CII., Methods: Saccades, smooth pursuit, the vestibulo-ocular reflex, and visual fixation were recorded in 21 patients with CII, aged 8-19 years and 39 age-matched controls, using an infrared eye tracker. Qualitative and quantitative MRI data were correlated with eye movements in 19 CII patients and 28 controls., Results: Nine patients with CII had abnormal eye movements. Smooth pursuit gain was subnormal in eight, saccadic accuracy abnormal in four, and vestibulo-ocular reflex gain abnormal in three. None had fixation instability. Patients with CII had a significantly smaller cerebellar volume than controls, and those with normal eye motion had an expanded midsagittal vermis compared to controls. However, patients with abnormal eye movements had a smaller (non-expanded) midsagittal vermis area, posterior fossa area and medial cerebellar volumes than CII patients with normal eye movements., Conclusions: The deformity of CII affects the structure and function of the cerebellum selectively and differently in those with abnormal eye movements. We propose that the vermis can expand when compressed within a small posterior fossa in some CII patients, thus sparing its ocular motor functions.

Object: The chronic tonsillar herniation defining Chiari malformation Type I (CMI) is thought to result from overcrowding of a normally developing hindbrain within a congenitally small posterior cranial fossa (PCF) due to occipital hypoplasia. The goals in the present study were to authenticate the cranioencephalic disproportion in a group of patients with CMI and to discuss new developmental aspects according to which part of the occipital bone was underdeveloped., Methods: The authors retrospectively examined a group of 17 patients with CMI. Measurements of osteotentorial and neural structures of the PCF were made on MR images of the brain. The results were compared with findings in 30 healthy controls by using the Mann-Whitney U-test., Results: Dimensions of the neural structures did not differ between the 2 groups of patients. The mean length of the basiocciput was significantly shorter in the CMI group (19.4 mm) compared with the control group (25.7 mm; p = 0.0003). The mean diameter of the foramen magnum was larger in the CMI group, but this difference was not statistically significant. The dimensions of the supraocciput and the mean angle of the cerebellar tentorium were identical in the 2 groups., Conclusions: Data in this study support the idea that occipital hypoplasia is the main cause of overcrowding within the PCF. Basioccipital shortness is a cardinal feature of the resultant shallow PCF and could proceed from a congenital disorder of the cephalic mesoderm of the parachordal plate or occur later in the infancy because of premature stenosis of the sphenooccipital synchondrosis.

Objectives: The objective of this article is to probe the surgical procedures of treatment for Chiari I malformation (CMI) associated with syringomyelia and evaluate their efficacy based on clinical and neuroradiological results., Study Design: Retrospective study., Setting: Department of Neurosurgery in Qilu Hospital of Shandong University in China., Methods: We retrospectively reviewed 316 surgically treated patients with CMI. We classified our patients after surgery in four global outcome categories as follows: very good (complete remission or marked improvement), good (slight improvement), fair (stability), poor (slight or marked deterioration). We simply describe the postoperative diameter of the syrinx (collapse, decreased, unchanged or increased)., Results: At the time of discharge, the result was very good in 163 cases (51.58%), good in 92 cases (29.11%), fair in 49 cases (15.51%) and poor in 12 cases (3.80%). In the follow-up period, neurological status improved and was then sustained in 94.49% of patients (206/218). At more than 2 years after surgery, we were able to compare pre- and postoperative MR images in 218 patients. A collapse of the syrinx was seen in 34.86% (76/218) of patients. The size of the syrinx was decreased in 31.65% (69/218) of patients, remained unchanged in 26.15% (57/218) of patients and increased in 7.34%(16/218) of patients., Conclusion: Patients with CMI should be treated using various surgical procedures according to different magnetic resonance imaging types; surgical treatments may fully decompress the medulla oblongata and ameliorate the clinical syndromes.

The natural history of Chiari malformation and our ability to alter it is poorly understood, and reported results of hindbrain decompression show high recurrence rates. We report 11 years of experience of hindbrain decompression, to evaluate long-term outcome related to surgical technique. The results of patients who underwent hindbrain decompression between 1994 and 2005 were retrospectively analysed. We identified 96 patients from operative records and reviewed all clinical records for presenting symptomatology and examination findings. Decompression technique includes traditional decompression, duraplasty and bone--only decompression. Symptoms at the last available follow-up visit were defined by the assessing clinician as resolved, improved, unchanged or worse. There were 35 males and 61 females with a mean age of 33 years (range 6 - 62 years). The mean length of follow-up is 3.6 years with a range of 6 months to 9 years. Postoperative resolution or improvement in symptoms was seen in 75 patients (78%). Drop attacks and headaches were the most likely to respond to hindbrain decompression, showing improvement or resolution in 100 and 92% of cases. Dysaesthetic arm pain and weakness carried the worse prognosis with only 20% having symptom resolution. Sixteen patients had only bony decompression leaving the dura intact. In eight patients (66%) the headaches resolved following bony decompression alone. However the headaches were unchanged in 25% of cases. Dysaesthetic pain and weakness was unchanged in 60%. Restoration of CSF flow dynamics at the foramen magnum by surgical decompression does not consistently result in resolution of symptoms in all patients. Identification of predictors of successful outcome following decompression, coupled with early intervention and appropriate choice of procedure may result in improved outcomes. Although this is a retrospective study it suggests that bone only decompression should be reserved for patients with isolated headache.

Object: Many authors have concluded that the Chiari malformation Type I (CM-I) is due to a smaller than normal posterior cranial fossa. In order to establish this smaller geometry as the cause of hindbrain herniation in a family, the authors of this paper performed volumetric analysis in a family found to have this malformation documented in 4 generations., Methods: Members from this family found to have a CM-I by imaging underwent volumetric analysis of their posterior cranial fossa using the Cavalieri method., Results: No member of this family found to have CM-I on preoperative imaging had a posterior fossa that was significantly smaller than that of age-matched controls., Conclusions: The results of this study demonstrate that not all patients with a CM-I will have a reduced posterior cranial fossa volume. Although the mechanism for the development of hindbrain herniation in this cohort is unknown, this manifestation can be seen in multiple generations of a familial aggregation with normal posterior fossa capacity.

Introduction: The authors report a case of three brothers., Case Descriptions: Two of these siblings presented with congenital growth hormone deficiency (GHD) and Chiari I malformation (CIM). The third younger brother has been found not to have GHD or the CIM., Discussion: Sparse cases of these two clinical occurrences have been reported. Further, the posterior cranial fossa (PF) has been determined to be altered in patients with CIM and GHD., Conclusion: Our current case reports strengthen the association between these two pathological entities and, to our knowledge, is the first description of both defects in siblings.

Object: The goal of this study was to establish whether children with Chiari malformation Type I (CM-I) have abnormal skull base geometry., Methods: Distances and angles between skull base landmarks were measured on preoperative magnetic resonance images obtained in 30 children (age range 36-204 months) with symptomatic isolated CM-I; 16 of them (53%) had syringomyelia. Comparisons were made with 42 children of similar age who comprised the control group by using one-way analysis of variance., Results: The angle formed by the crista galli (CG), dorsum sellae (DS), and foramen magnum (FM) was larger than normal in individuals with CM-I than in those without (145 degrees in patients with CM-I but no syringomyelia and 151 degrees in those with CM-I and syringomyelia compared with 135 degrees in controls; p = 0.000). The angle formed by the left internal auditory meatus (IAM), FM, and right IAM was also larger than normal in the patients (122 degrees in patients with CM-I but no syringomyelia and 123 degrees in those with CM-I and syringomyelia compared with 110 degrees in controls; p = 0.001). The angle formed by the anterior clinoid process (ACP), CG, and right ACP was smaller than normal (29 degrees in all patients with CM-I compared with 34 degrees in controls; p = 0.000). The distance between the two IAMs was longer than normal (75 mm in patients with CM-I but no syringomyelia and 63 mm in those with CM-I and syringomyelia compared with 58 mm in controls; p = 0.000). The distance between the two ACPs was shorter than normal in the syringomyelia group (31 mm in patients with CM-I but no syringomyelia and 27 mm in those with CM-I and syrinx compared with 32 mm in controls; p = 0.001). Within the group of patients with CM-I, the DS-FM and left ACP-right ACP distances were smaller in the syringomyelia group (p = 0.009 and p = 0.037, respectively)., Conclusions: Children with CM-I have abnormal geometrical measurements of their entire skull base, not only the posterior fossa, irrespective of presence of syringomyelia. This may indicate a mesodermal defect as a possible cause of the malformation.

Objective: Some have proposed that the calvarial thickening seen in patients with rickets results in an increased rate of Chiari I malformation (CIM) in these patients. The present study measures the posterior fossa volume in children with rickets to verify previous case reports indicting a small posterior fossa as the cause for an increased rate of CIM in children with rickets., Methods: Patients were chosen by use of a computer database to search for individuals diagnosed with rickets. Nineteen patients were identified with this diagnosis. Seven patients were found from this cohort to have imaging of the head. Axial computed tomographic and magnetic resonance images were analyzed by use of the Cavalieri method to define posterior fossa volumes. These data were then compared with those from age-matched control subjects., Results: Mean volumes of the posterior fossa were significantly reduced in all patients compared with age-matched control subjects (P < 0.0001)., Conclusion: We have found that the volume of the posterior fossa is significantly smaller in children with rickets versus age-matched control subjects. Furthermore, 29% of our study group had an associated CIM. We may hope that these data will aid in the further understanding of the pathophysiology of CIM in cases of metabolic bone disease.

Study Design: Magnetic resonance imaging (MRI) was used to compare quantitatively the position of the cerebellar tonsil in neurologically normal adolescents with that in idiopathic scoliosis (AIS) patients and age-matched healthy controls., Objectives: To redefine the normal reference level of the cerebellar tonsil in healthy adolescents and to compare the differences in tonsillar level with AIS of different severities., Summary of Background Data: Asymptomatic Chiari I malformation has been reported not infrequently in children and adolescents with AIS. The definition of tonsillar herniation and its variations with sex, age, and curve severities have not been clarified., Methods: Sagittal MRI of hindbrain was performed on 170 adolescents, including 117 patients with AIS and 53 age-matched healthy controls., Results: The cerebellar tonsillar tip in healthy patients was found at an average of 2.8 mm above the basion-opisthion reference line (BO line) (range, 0-10.5 mm), in contrast to the finding that 17.9% of the AIS patients had tonsillar tip below the BO line. The position of tonsil in the AIS group was lower than that in the healthy controls (P < 0.05), and the differences also increased with the severity of the curve (P = 0.027)., Conclusions: Using the current MRI reference standards, the incidence of tonsillar herniation could be significantly underestimated. According to our experience, any inferior displacement of a tonsil below the BO line in adolescents should be regarded as abnormal. Scoliosis could be an important manifestation of subclinical tonsillar herniation.

There has been almost no evaluation or discussion of the symmetry of tonsillar ectopia in the medical literature. We measured the degree of left and right tonsillar herniation in 42 pediatric patients with a symptomatic Chiari I malformation and made clinical/radiological correlations. 18% of all patients with tonsillar asymmetry had clinical symptoms or physical findings referable to the inequality of their hindbrain herniation. In addition, 95% of patients with a coexisting syringomyelia had a right cerebellar tonsillar herniation greater than the left. We hypothesize that slight differences in posterior cranial fossa morphology may physically allow for greater unilateral herniation of the left or right cerebellar tonsil, which may be manifested in a patient's clinical presentation., (Copyright 2002 S. Karger AG, Basel)

Chiari I malformation is a well-described entity characterized by hindbrain herniation through the foramen magnum. Although the exact origin of congenital Chiari I malformation is unknown, it appears to be caused by a mismatch between the volume of the posterior fossa neural elements and the posterior fossa cranial content. Several theories have been proposed to describe the resultant pathophysiology of this mismatch. It is clear, however, that abnormal cerebrospinal fluid flow and velocity play a role in the symptoms and signs associated with this disorder. The authors will review the pathophysiology, clinical presentation, and treatment options for patients with Chiari I malformation.

The spontaneous resolution of syringomyelia has been reported infrequently. In patients with Chiari I malformations, resolution of the syringomyelia has sometimes been associated with improvement of their malformation. The authors present a case of spontaneous resolution followed by recurrence of syringomyelia and a corresponding change in the Chiari malformation. This case is of interest in light of the theories postulated to explain spontaneous resolution of syringomyelia.

Background: It has been theorized that fetal myelomeningocele repair may reduce ongoing intrauterine injury and perhaps allow healing and regeneration of dysplastic neural tissue. We report on the postnatal imaging studies of the first 4 patients to have undergone intrauterine myelomeningocele repair at our institution., Methods: Each of the 4 patients underwent postnatal sonographic and MRI. In addition, the postnatal ultrasounds of these 4 were compared to a group of retrospective controls., Results: MRI scans of the 4 experimental subjects revealed no evidence of hindbrain herniation while other stigmata of the Chiari-II malformation persisted. In comparison to the retrospective controls this absence of herniation was distinctly unusual., Conclusion: Intrauterine myelomeningocele repair may reduce the degree of hindbrain herniation normally seen in patients with myelomeningocele. This raises the possibility that intrauterine repair may decrease the morbidity associated with the Chiari type-II malformation including brainstem dysfunction, hydrocephalus and syringomyelia.

Firstly, 14 patients are described who developed either an acquired Chiari malformation (ACM) alone (7 cases) or ACM and syringomyelia (7 cases) after lumbar subarachnoid space (SAS) shunting or in one case, epidural anaesthesia with SAS penetration. Four groups are considered: 3 cases with craniofacial dysostosis and communicating hydrocephalus (CH), 4 cases with CH alone, 3 cases with pseudotumour cerebri (PTC) and a miscellaneous group (4 cases). Initial treatment was varied: resiting the shunt to ventricle or cisterna magna [6], adding an H-V valve [1], syrinx shunting [4] and posterior fossa decompression [3]. Further treatment was required in 6 cases. Secondly, incidence was examined in 87 patients with PTC initially treated either by lumbar SAS shunting [70] or cisterna magna shunting [17]. In the first sub-group, 11 cases (15.7 per cent) developed an ACM, 3 symptomatic (as above) and eight asymptomatic with 1 case also having syringomyelia whereas 1 case occurred in the second group with a questionanably symptomatic ACM. While accurate for symptomatic lesions, these figures are tentative with respect to asymptomatic lesions due to inadequate pre-treatment radiology and detailed MR follow-up. The main conclusions are, first, that the incidence of symptomatic ACM and/or syringomyelia is not high enough to warrant abandoning SAS shunting; second that asymptomatic lesions need not necessarily be treated and third, that when treatment is required, shunt resiting is the first choice.

Hindbrain hernias with or without hydrosyringomyelia were difficult diagnostic problems before the availability of magnetic resonance imaging. Today, the problem seems not to be in evaluating the anatomical extent of the caudal herniation of the cerebellum, but in determining which patient should be considered for operative intervention and the extent of the surgery. Chiari I patients are presenting at younger ages, occasionally with irritability as their only symptom. Should all of these children be submitted to an operation? Chiari II patients are now operated on with the first detectable symptom or evidence of a syrinx, and yet medullary dysfunction from the Chiari II malformation remains the leading cause of death in treated myelomeningoceles today. Our knowledge of the natural history of the untreated conditions and the increased safety of the operation has made surgical intervention a much more viable option for this group of patients.

Cine-MRI with presaturation bolus tracking was used in patients with syringomyelia associated with a Chiari malformation to study pulsatile movement of the hindbrain, cervical spinal cord, cerebrospinal fluid and the fluid within the syrinx. Nine patients had 13 examinations, 6 preoperative, 3 after syringosubarachnoid shunting and 4 after posterior fossa decompression. Five controls were also examined. Dynamic display of the acquired images demonstrated downward displacement of the presaturation bolus on the cerebellar tonsils and medulla oblongata (or upper cervical cord) at the C1 level in all preoperative examinations and in two patients after syringosubarachnoid shunting but with residual foramen magnum obstruction. Downward displacement of the bolus on the cervical spinal cord was also demonstrated in 7 examinations, but not observed in the controls. Thus, the hind-brain-spinal cord axis showed larger pulsatile movements in patients with foramen magnum obstruction. Based on these observations and a review of the literature, a new theory on the mode of extension of syringomyelia, emphasising the role of increased pulsatile movement of the hind-brain-spinal cord axis is proposed: that the pulsatile movements, together with a one-way valve mechanism in the syrinx cavity act as a "vacuum-pump" to enlarge the syrinx.