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      Timing to surgery of Chiari malformation type 1 affects complication types: An analysis of 13,812 patients

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          Abstract

          Background:

          Chiari malformations (CM) are congenital defects due to hypoplasia of the posterior fossa with cerebellar herniation into the foramen magnum and upper spinal canal. Despite the vast research done on this neurological and structural syndrome, clinical features and management options have not yet conclusively evolved. Quantification of proper treatment planning, can lead to potential perioperative benefits based on diagnoses and days to procedure. This study aims to identify if early operation produces better perioperative outcomes or if there are benefits to delaying CM surgery.

          Aims and Objective:

          Assess outcomes for Chiari type I.

          Methods:

          The KID database was queried for diagnoses of Chiari Malformation from 2003-2012 by icd9 codes (348.4, 741.0, 742.0, 742.2). Included patients: had complete time to procedure (TTP) data. Patients were stratified into 7 groups by TTP: Same-day as admission (SD), 1-day delay (1D), 2-day delay (2D), 3-day delay (3D), 4-7 days delay (4-7D), 8-14 days delay (8-14D), >14 days delay (>14D). Differences in pre-operative demographics (age/BMI) and perioperative complication rates between patient cohorts were assessed using Pearson's chi-squared tests and T-tests. Surgical details, perioperative complications, length of stay (LOS), total charges, and discharge disposition was compared. Binary logistic regressions determined independent predictors of varying complications (reference: same-day).

          Results:

          13,812 Chiari type I patients were isolated from KID (10.12 ± 6.3, 49.2F%, .063 ± 1.3CCI). CM-1 pts were older (10.12 yrs vs 3.62 yrs) and had a higher Charlson Comorbidity Score (0.62 vs 0.53; all P < 0.05). Procedure rates: 27.8% laminectomy, 28.3% decompression, and 2.2% spinal fusion. CM-1 experienced more complications (61.2% vs 37.9%) with the most common being related to the nervous system (2.8%), anemia (2.4%), acute respiratory distress disorder (2.1%), and dysphagia (1.2%). SD was associated with the low length of stay (5.3 days vs 9.5-25.2 days, P < 0.001), total hospital charges ($70,265.44 vs $90, 945.33-$269, 193.26, P < 0.001) when compared to other TTP groups. Relative to SD, all delay groups had significantly increased odds of developing postoperative complications (1D-OR: 1.29 [1.1- 1.6] → 8-14D-OR: 4.77[3.4-6.6]; all P < 0.05), more specifically, nervous system (1D-OR: 1.8 [1.2-2.5] → 8-14D-OR: 3.3 [1.8-6.2]; all P < 0.05).Sepsis complications were associated with a delay of at least 3D(2.5[1.4-4.6]) while respiratory complications (6.2 [3.1-12.3]) and anemia (2 [1.1-3.5]) were associated with a delay of at least 8-14D (all P < 0.05).

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          Most cited references25

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          Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients.

          Chiari malformations are regarded as a pathological continuum of hindbrain maldevelopments characterized by downward herniation of the cerebellar tonsils. The Chiari I malformation (CMI) is defined as tonsillar herniation of at least 3 to 5 mm below the foramen magnum. Increased detection of CMI has emphasized the need for more information regarding the clinical features of the disorder. We examined a prospective cohort of 364 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine, and some were evaluated using CINE-magnetic resonance imaging and other neurodiagnostic tests. For 50 patients and 50 age- and gender-matched control subjects, the volume of the posterior cranial fossa was calculated by the Cavalieri method. The families of 21 patients participated in a study of familial aggregation. There were 275 female and 89 male patients. The age of onset was 24.9+/-15.8 years (mean +/- standard deviation), and 89 patients (24%) cited trauma as the precipitating event. Common associated problems included syringomyelia (65%), scoliosis (42%), and basilar invagination (12%). Forty-three patients (12%) reported positive family histories of CMI or syringomyelia. Pedigrees for 21 families showed patterns consistent with autosomal dominant or recessive inheritance. The clinical syndrome of CMI was found to consist of the following: 1) headaches, 2) pseudotumor-like episodes, 3) a Meniere's disease-like syndrome, 4) lower cranial nerve signs, and 5) spinal cord disturbances in the absence of syringomyelia. The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (364 patients), tonsillar herniation of at least 5 mm (332 patients), and varying degrees of cranial base dysplasia. Volumetric calculations for the posterior cranial fossa revealed a significant reduction of total volume (mean, 13.4 ml) and a 40% reduction of cerebrospinal fluid volume (mean, 10.8 ml), with normal brain volume. These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterized by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible for headaches, pseudotumor-like episodes, endolymphatic hydrops, syringomyelia, and hydrocephalus) and direct compression of nervous tissue. The demonstration of familial aggregation suggests a genetic component of transmission.
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            Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging.

            Chiari Type I malformation (CMI) is a congenital disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Frequently, associated findings include abnormalities of nearby bony and neural elements as well as syringomyelia. Cerebellar tonsillar ectopia is generally considered pathological when greater than 5 mm below the foramen magnum. However, asymptomatic tonsillar ectopia is an increasingly recognized phenomenon, the significance of which is poorly understood. The authors retrospectively reviewed the records of all brain magnetic resonance (MR) images obtained at our hospital over a 43-month period in an attempt to ascertain the relative prevalence and MR imaging characteristics of asymptomatic CMIs. Of 22,591 patients who underwent MR imaging of the head and cervical spine, 175 were found to have CMIs with tonsillar herniation extending more than 5 mm below the foramen magnum. Of these, 25 (14%) were found to be clinically asymptomatic. The average extent of ectopia in this population was 11.4 +/- 4.86 mm, and was significantly associated with a smaller cisterna magna. Syringomyelia and osseous anomalies were found in only one asymptomatic patient. The authors suggest that the isolated finding of tonsillar herniation is of limited prognostic utility and must be considered in the context of all available clinical and radiographic data. Strategies for treating patients with asymptomatic CMIs are discussed.
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              Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome.

              The Chiari malformation is a condition characterized by herniation of the posterior fossa contents below the level of the foramen magnum, and is categorized into three types based on the degree of herniation. The authors review their surgical experience between 1975 and 1985 with 50 patients afflicted with symptomatic Chiari malformations. Any patient with associated myelomeningocele, tethered spinal cord, lipoma, or diastematomyelia was excluded from this series. Forty-one patients had Chiari I malformations, seven were classified as having Chiari II, and two as having Chiari III. The presentation of pediatric and adult patients was identical. Treatment was directed at the posterior fossa pathology. Seven patients with accompanying ventral bone compression underwent transoral decompression of the cervicomedullary junction, 42 had posterior decompressive procedures, and six received ventriculoperitoneal shunts. The posterior decompression included opening the outlet foramina of the fourth ventricle, occluding any communication between the spinal cord central canal and the obex, shunting the fourth ventricle, and placing a dural graft. Postoperatively, 20% of the patients are asymptomatic, 66% improved, and 8% stabilized; in 6% the disease has progressed in spite of multiple procedures. Preoperative signs that are predictive of a less favorable outcome include muscle atrophy, symptoms lasting longer than 24 months, ataxia, nystagmus, trigeminal hypesthesia, and dorsal column dysfunction (p less than 0.05, chi-square test). A model based on the presence or absence of atrophy, ataxia, and scoliosis at the time of the preoperative examination has been generated that allows prediction of long-term outcome at the 95% confidence level.
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                Author and article information

                Journal
                J Craniovertebr Junction Spine
                J Craniovertebr Junction Spine
                JCVJS
                Journal of Craniovertebral Junction & Spine
                Wolters Kluwer - Medknow (India )
                0974-8237
                0976-9285
                Jul-Sep 2020
                14 August 2020
                : 11
                : 3
                : 232-236
                Affiliations
                [1]Department of Orthopedics, NYU Langone Orthopedic Hospital, New York, USA
                [1 ]Department of Orthopedics, SUNY Downstate Medical Center, Brooklyn, NY, USA
                [2 ]Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA
                Author notes
                Address for correspondence: Dr. Peter Gust Passias, Department of Neurologic and Orthopaedic Surgery, Division of Spine, New York Spine Institute, NYU Langone Orthopedic Hospital, 301 East 17 th St, New York, NY, 10003, USA. E-mail: pgpassias@ 123456yahoo.com
                Article
                JCVJS-11-232
                10.4103/jcvjs.JCVJS_67_20
                7546060
                33100774
                2108c321-f6c8-47c7-98b8-68671e67f5f3
                Copyright: © 2020 Journal of Craniovertebral Junction and Spine

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 12 May 2020
                : 08 June 2020
                Categories
                Original Article

                Neurology
                chiari,complications,timing
                Neurology
                chiari, complications, timing

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