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Chiari II Malformation: MR Imaging Evaluation

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Chiari II Malformation: MR Imaging Evaluation 783 Chiari II Malformation: MR Imaging Evaluation Samuel M. Wolpert' The purpose of this study was to explore the value of high-detail MR imaging in the Mary Anderson' diagnosis of the Chiari II malformation. Twenty-four patients with known Chiari II R. Michael Scott2 malformation as diagnosed by CT scanning were evaluated with cranial MR scans. Two Eddie S. K. Kwan' patients also had spine scans. The sagittal-plane images were the most informative, Val M. Runge3 and abnormalities of the telencephalon, diencephalon, mesencephalon, rhomboen­ cephalon, upper spinal cord, and mesencephalon were shown extremely well. We found MR to be an easy and accurate method for demonstrating the abnormalities of the Chiari II malformation, and it is our procedure of choice. The Chiari II malformation is a complex developmental deformity characterized by an elongated small cerebellum and brainstem with caudal displacement of the medulla, parts of the cerebellum, and pons through an enlarged foramen magnum into the cervical spinal canal [1]. A meningomyelocele is a nearly constant accom­ panying feature, as is hydrocephalus. In addition, numerous other malformations of the neuraxis including polymicrogyria, subependymal heterotopias, beaked col­ licular plate, aqueduct stenosis, diastematomyelia, diplomyelia, hydromyelia, and syringomyelia have been reported [1]. We had the opportunity to evaluate 24 patients with Chiari II malformation with high-detail MR imaging. Most abnormalities previously described as seen by CT [2-5] were also seen by MR. In this article we review these abnormalities and report on other associated features of the Chiari malformation that either have a greater frequency than has previously been reported or that have not been described before. Subjects and Methods Twenty-four patients with meningomyeloceles and known Chiari II malformations as diagnosed by CT scanning were investigated with MR imaging. Three patients were less than 1 year old, five were 1-5 years old , five were 6-10 years old, and 11 were 10-20 years old. This article appears in the September/Octo­ In 21 of the patients ventricular shunts had been inserted previously; in one patient a lumbar ber 1987 issue of AJNR and the November 1987 subarachnoid-peritoneal shunt had been placed . Sagittal and axial head images were obtained issue of AJR. in all patients. The majority of the sagittal images (16) were 3 mm thick; the rest were either Received December 16, 1986; accepted after 4 or 5 mm thick. Eighteen patients had coronal scans (5-10 mm thick). Spin-echo techniques revision March 13, 1987. were used. Since the anatomy of the lesions was the prime consideration , mild T1-weighted , Department of Radiology, Section of Neurora­ images were obtained in all cases with a repetition time of 500, 600, or 800 msec and an diology, New England Medical Center Hospitals, 750 Washington St. , Boston, MA 02111 . Address echo time of 17 msec. T2-weighted images were not obtained routinely. In all patients, an reprint requests to S. M. Wolpert. attempt was made to include the upper cervical spine on the sagittal images. In addition, two 2 Department of Neurosurgery, New England patients had separate T1-weighted sagittal images of the spine, and one of the two had T2- Medical Center Hospitals, Boston, MA 02111 . weighted sequences as well . 3 Department of Radiology, Section of MR Im­ aging, New England Medical Center Hospitals, Bos­ ton , MA 02111 . Results AJNR 8:783-792, September/October 1987 The results are categorized by the involvement of the different embryologic parts 0195-6108/87/0805-0783 © American Society of Neuroradiology of the brain and are summarized in Table 1. 784 WOLPERT ET AL. AJNR:8, September/October 1987 TABLE 1: MR Abnormalities in Chiari II Malformations about 50% of patients with the Chiari II malformation [6, 7], according to Muller [8] the cerebral cortex in patients with No. of Patients Location: Abnormality the Arnold-Chiari malformation is normal and should not be (n = 24) confused with true polymicrogyria, in which the cellular layers Telencephalon: of the cortex are deficient. Muller used the term "stenogyria" Stenogyria 7 Frontal horn beakinga 11 to describe a histologically normal cortex thrown into innu­ Corpus callosum (partial agenesis) 8 merable small , closely spaced folds. Although we have no Supracerebellar CSF-containing spaces 10 histologic verification of normal cortical architecture in our Third ventri cle diverticulum 1 patients, this term appears to be more appropriate for the Diencephalon: Enlarged third ventricle 3 MR appearances of the cortical gyral pattern in these patients. Large massa intermedia 16 Anterior horns.-Anteroinferior pointing of the anterior Elevation of hypothalamus 2 horns was seen in 11 of the 12 patients in this series in whom Mesencephalon: coronal scans had been obtained through the frontal horns Aqueduct not seen 17 (Fig . 2) . In one series these appearances were seen on Bulbous tectum 14 Beaked tectum 10 ventriculograms in 32 of 62 patients with the Arnold-Chiari R homboencephalon malformation [9]. The cause of the findings was thought to Absent cerebellar folia 12 be either a medially directed force causing undue juxtaposition Superior vermis 9 of the two cerebral hemispheres or marked inward bulging of Inferior vermis 10 Overlapping anterior cerebellar margins 18 the caudate nucleus [4, 9]. Cerebellar vermial pegs 23 Corpus callosum .-Partial agenesis of the corpus callosum b Cervicomedullary deformities : was seen in eight (33%) of the 24 patients (Figs. 1, 3, and 4). Type 1 6 Six of these patients had medial radiating sulci on the inner Type 2 7 Type 3a 8 surface of the adjacent parietal lobes. In another four patients Type 3b 3 marked thinning of the posterior portion of the body and the Pyramidal hypogenesis 4 splenium of the corpus callosum was seen , possibly a result Spinal cord: of previous severe hydrocephalus. Other associated features Wide anterior space 9 of agenesis of the corpus callosum , such as dysplasia or Syrin x 3 Mesoderm al: absence of the cingulate gyrus [10], were seen in six of the Clival scalloping 19 eight patients. Failure of the parietooccipital and calcarine Gyral interdigitations (falx hypoplasia) 9 fissures to converge was seen in six patients. Wide incisurac 15 Agenesis of the corpus callosum in association with the Gyral indentations (into skull) 8 Arnold-Chiari malformation has been described but not with • Twelve acceptable coronal scans of frontal horns. this great a frequency. Gilbert et al. [11] described three b See text for elaboration. cases of complete or nearly complete agenesis of the corpus c Ali 15 patients had overlapping anterior cerebellar margins. callosum in a series of 25 autopsies of children with Arnold­ Chiari malformations. Venes et al. [12] reported a case of partial agenesis of the corpus callosum demonstrated by MR, Discussion but the anomaly was not mentioned in the article . Two cases MR imaging is known to be superior to CT in demonstrating of the Chiari II malformation demonstrated by MR were found the craniovertebral junction , particularly the spinal cord , me­ in a series of 11 patients with agenesis of the corpus callosum dulla, cerebellum , pons, and surrounding subarachnoid described by Atlas et al. [13]. The association between the spaces , and MR is perhaps the best technique for demon­ two anomalies further emphasizes the fact that the Chiari II strating structural abnormalities of the Chiari II malformation. malformation involves not only the hindbrain but also other Although additional manifestations of the malformation such fiber tracts in the human brain . as aqueduct stenosis and massa intermedia enlargement are The finding of a deformed corpus callosum on MR may be usually well defined by CT, MR demonstrates superior ana­ of some value in predicting intelligence in children with mye­ tomic detail in these areas as well . lomeningoceles. Of the eight children with the anomaly, two The MR images of our patients, in addition to duplicating are severely retarded and two require special schooling. One previous CT findings, highlighted other components of the child appears to have a normal mental state, but is so disabled malformation that previously have not been emphasized or by extremity deficits and breathing difficulties that he cannot described in the radiologic or neuropathologic literature. Each be tested easily. Only three appear to be developing normally of the areas is discussed separately. with normal intelligence. The other 16 children without severe deformations of the corpus callosum all have normal or nearly Telencephalon normal intelligence. Supracerebellar CSF-containing spaces (CCS).-Wide su­ Stenogyria.-Small , closely spaced gyral folds were seen pracerebellar CCS involving to varying degrees the quadri­ in seven patients, five of whom also had partial agenesis of geminal plate cistern , the cistern velum interpositum, the the corpus callosum (Fig. 1). Although this finding is usually interhemispheric fissure, and the ambient cistern were seen termed "polymicrogyria" and is described as occurring in in 10 of the 24 patients (Figs. 3-5). In one other patient the AJNR:8, September/October 1987 MR OF CHIARI II MALFORMATION 785 A B Fig, 1.-Multiple, closely spaced gyri (open arrows) are Fig. 2.-A, Inferior pointing of lateral ventricular floor (arrows) is associated with radially oriented. Posterior portion of body and splenium of enlarged anterior horns. corpus callosum are absent (arrowhead). Rostrum of corpus B, Heads of caudate nuclei (arrows) bulge medially into anterior horns. Note enlarged callosum is absent (short solid arrow). Aqueduct is not seen lateral ventricles and occipital horn. Third ventricle is unusually large. and inferior colliculus (curved arrow) has beaked appearance. Fourth ventricle is at level of foramen magnum and inferiorly is seen extending through foramen magnum anterior to inferior vermial peg (long solid arrow) (group 2a in text). Clivus is concave. An insufficient amount of upper cervical canal is included on scan to determine whether cervicomedullary kink is present also.
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