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Chiari Malformation in Adults: Relation of J Neurol Neurosurg Psychiatry: First Published As 10.1136/Jnnp.56.10.1072 on 1 October 1993

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Chiari Malformation in Adults: Relation of J Neurol Neurosurg Psychiatry: First Published As 10.1136/Jnnp.56.10.1072 on 1 October 1993 107212ournal ofNeurology, Neurosurgery, and Psychiatry 1993;56:1072-1077 Chiari malformation in adults: relation of J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.10.1072 on 1 October 1993. Downloaded from morphological aspects to clinical features and operative outcome J M Stevens, W A D Serva, B E Kendall, A R Valentine, J R Ponsford Abstract headache and neck pain, and those most To determine whether clinical features refractory were often ataxia and nystagmus. attributed to cerebellar ectopia could be Clearly not all patients with the adult related to the severity of the malforma- Chiari malformation benefit from operation. tion, and if morphological features could Attempts at risk stratification have been be related to operative outcome, a retro- made, and recently a careful study by spective study of 141 patients with the Menezes' group14 established three preopera- adult Chiari malformation was carried tive clinical features predictive of a poor oper- out, 81 receiving operative treatment. ative outcome-namely, ataxia, scoliosis, and Morphological parameters derived from muscle atrophy. Attempts at relating the preoperative clinical imaging were com- severity of the Chiari lesion to individual clin- pared with presenting clinical features ical features or syndromes have been few and and postoperative outcomes. Patients some of the conclusions conflicting.15-'7 There with the most severe cerebellar malfor- has been no formal attempt at operative risk mation, defined as descent of the cere- stratification based on morphological fea- bellar tonsils to or below the axis, had tures. disabling ataxia and nystagmus more The present study was begun several years frequently. Those with brainstem com- ago to investigate if the severity of the hind pression had limb weakness and muscle brain abnormality could be related to preop- wasting more frequently. Operative out- erative clinical features and postoperative out- come was significantly less favourable in come. Because the design of the investigation patients with severe cerebellar ectopia was retrospective, and large numbers of (12% improved, 690% deteriorated) than patients with detailed clinical data were in those with minor ectopia (50% required, it was necessary to use data mainly improved, 17% deteriorated). Patients from myelography and CT rather than MRI with a distended cervical syrinx had a which only became available at our institution more favourable outcome than those some time after the study began. Nevertheless, without. Morphological features help most of the criteria used in the study are predict operative risk. equally applicable to MRI. (jNeurol Neurosurg Psychiatry 1993;56:1072-1077) http://jnnp.bmj.com/ Materials and methods The study consisted of a retrospective review The term adult Chiari malformation is used of 141 symptomatic adult patients diagnosed to describe descent of the hind brain into the as having a Chiari malformation by myelogra- cervical canal, in which meningomyelocoele is phy alone in 91 or in combination with CT in absent and hydrocephalus rare, and patients 50 patients. At least five of these patients also usually present in adult life.' The clinical fea- had MRI performed on a Vista MR (Picker tures which may be associated are protean26 International) operating at 05 or 0-26 T. on September 28, 2021 by guest. Protected copyright. and a causal relationship in a given case may One patient only had hydrocephalus, and not be established with certainty, especially as none had a meningomyelocoele. Eighty one modern computed imaging suggests that min- patients were operated on in one of three sur- imal protrusion of parts of the cerebellar gical units; all were subjected to a posterior The National the foramen magnum Hospitals for hemispheres through is suboccipital and upper cervical decompres- Neurology and relatively common, and that even marked sion, 12 had partial excision of herniated Neurosurgery, Queen protusion is often asymptomatic.7 The issue is cerebellar tonsils, and eight had an occlusion Square and Maida important because it is often maintained that of the obex. Thirty one patients also had a Vale, London, UK to J M Stevens disability due the adult Chiari malforma- syringostomy. Sixty patients did not receive W A D Serva tion usually is curable by operation.' 5 operations at our institution: 32 were lost to B E Kendall In the nine operative series which have follow up and may have been on A R Valentine operated included more than in J R Ponsford 20 patients and which elsewhere, 18 had significant co-existing dis- the mean was at Correspondence to: follow up least 6 months, the ease such as multiple sclerosis (10 patients) Dr J M Stevens, mean proportion and patients with sustained and severe spondylotic spinal cord compres- Department of Radiology, Maida Vale Hospital, improvement has been 56% (range 29-82%), sion (eight patients), eight refused operation, London W9 ITL. the proportion who deteriorated 24% (range and it was unclear why no operation was per- Received 22 October 1992 6-31%) and the perioperative mortality 3% formed in two. and in revised form 8 January 1993. (range 0_5-6%).1 4 The clinical features Clinical data were gleaned from the case Accepted 19 January 1993 most likely to be alleviated have been notes and evaluated by one of two neurolo- Chiari malformation in adults: relation ofmorphological aspects to clinicalfeatures and operative outcome 1073 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.56.10.1072 on 1 October 1993. Downloaded from Table 1 Clinical groupings. arches of atlas and axis; and C, tonsils below the axis. The upper border of each neural (a) General 1 Headache: considered only if it was exacerbated by head arch was taken as the reference point for each movement, exercise, or coughing. group. 2 Drop attacks: unexplained falls with or without brief loss of consciousness. 3 Neck pain: with or without occipital radiation. FORAMEN MAGNUM (b) Functional anatomical The degree of obstruction at the foramen 1 Upper and brainstem (midbrain, pons): dysfunction of cranial nerves m, IV, V, VI, VII, and VIII. magnum was also assessed. Contrast medium 2 Medulla oblongata: dysfunction of cranial nerves IX, X, flowed freely into the head from the spinal XI, xII. 3 Brainstem-cerebelar connections: ataxia, vertigo, subarachnoid space via the anterior cere- nystagmnus. brospinal fluid pathway on myelography in all 4 Peripheral sensory disturbances: impaired pain and temperature sensation; loss of position and vibratory cases, although in some it was obstructed sense; subjective complaints of numbness of posteriorly. Two types of observation were parasthesiae in limb or trunk. 5 Peripheral motor disturbances: all forms of weakness in made: limbs or trunk, with or without muscle wasting. 1 The size of the cisterna magna was assessed 6 Syringomyelia: this category included only those patients in which there was a firm diagnosis of both above and at the level of the foramen syringomyelia in the case notes, based on clinical and magnum and its mid-sagittal size determined radiological findings. by measuring the minimum distance between (c) Postoperative clinical categories 1 Original clinicalfeatures: The preoperative symptoms/ the internal occipital crest and posterior mar- signs were classified postoperatively as either improved, gin of the foramen magnum and the cerebel- unchanged or worse. 2 New clinicalfeatures: any complaints or signs appearing lum. CT or midline sagittal tomograms and after operation which was not present before, excluding MRI were used wherever possible for this those directly related to the wound itself. 3 Headache: this feature was considered separately when assessment. Accurate measurement was not present in the preoperative clinical picture. possible, but the cisterna magna was scored as obliterated if it was not visible, small if its mid-sagittal width was less than 2-3 mm, and gists (WS and JP) not involved in the routine normal if it was greater than 2-3 mm. management of the patients. A large volume 2 Contrast medium visible in thefourth ventri- of data was obtained which, for the purposes cle either during the myelogram, or on CT of analysis, was simplified by arranging it into within 1 hour of the myelogram provided the categories and groups indicated in table 1. concentration was similar to that in the poste- Missing data in any individual case were rior fossa cisterns, was recorded. This was recorded as such, and the case excluded from taken as evidence that either one or each of analysis of that feature. Data sets were judged the foramena of Magendie or of Lushka were to be adequate for 127 patients. The postop- patent. Arachnoiditis was considered present erative assessments were divided into early if the cisterna magna was obliterated and the and late. All early assessments were made fourth ventricle failed to opacify on computed within 1 month of operation and features myelography. referable to the surgical wound were excluded, such as neck pain and stiffness. SYRINGOMYELIA The late assessments were made, as far as The upper spinal cord was also assessed for possible, from the last outpatient attendance. the presence of syringomyelia. Syringomyelia This varied from 3 months to 9 years after was considered probable if there was a central http://jnnp.bmj.com/ operation; but in only 18 did this period accumulation of contrast medium within the exceed 2 years and the mean follow up period spinal cord on early or delayed post- was 1 2 years. The available non-operated myelography CT, or a circumscribed central group was considered too small and heteroge- signal change was visible on MRI, or if there neous to be used for a meaningful parallel was a diffuse abnormality of cord size not group follow up study. explained by compression. A mid-sagittal The radiological features were assessed by diameter of the spinal cord of greater than 10 on September 28, 2021 by guest.
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