Sudden onset of Chiari malformation Type I in previously asymptomatic patients

Chiari malformation Type I (CM-I) is usually suspected in patients with slowly progressing neurological symptoms. However, in some instances, especially if syringomyelia is associated, an abrupt clinical onset is reported and is accompanied by an acknowledged risk of potentially severe clinical signs or even sudden death. Little is known about such a critical course in CM-I/syringomyelia complex. The authors describe 3 challenging cases of the abrupt onset of CM-I/syringomyelia to reveal more information on the clinical presentation and pathogenetic mechanisms of this sudden and potentially severe clinical phenomenon: a 38-year-old man experienced acute respiratory failure requiring intubation following acute decompensation of hydrocephalus associated with Noonan syndrome, a 1-year-old boy had sudden hemiparesis and Horner syndrome after a minor head/neck injury, and a 2.5-year-old boy presented with quickly progressing tetraplegia and dyspnea after a mild flexion and extension neck injury a few hours before. All 3 patients showed a CM-I/syringomyelia complex at diagnosis, and all of them had a good neurological outcome after surgery despite the ominous clinical presentation.

Download Full-text

Treatment practices for Chiari malformation Type I with syringomyelia: results of a survey of the American Society of Pediatric Neurosurgeons

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.8.peds10427 ◽

2011 ◽

Vol 8 (5) ◽

pp. 430-437 ◽

Cited By ~ 52

Author(s):

Brandon G. Rocque ◽

Timothy M. George ◽

John Kestle ◽

Bermans J. Iskandar

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Multicenter Trial ◽

Posterior Fossa Decompression ◽

Type I ◽

First Line ◽

Chiari Malformation Type I ◽

Asymptomatic Patients ◽

Clinical Scenarios ◽

American Society

Object The purpose of this study was to report the results of a survey of the American Society of Pediatric Neurosurgeons (ASPN) on treatment of Chiari malformation Type I (CM-I) with syringomyelia. Methods A questionnaire was circulated during the 2006 meeting of the ASPN, in which surgeons were surveyed on their management of patients with CM-I and syringomyelia. The survey consisted of questions about 4 clinical scenarios, common causes of surgical failures, and complications. Results There were 72 respondents, representing more than 90% of attendees at the 2006 ASPN meeting and approximately half of the society's members. The majority of respondents (85%) reported that they perform posterior fossa decompression as first-line treatment for CM-I with syringomyelia. Seven percent perform bony decompression alone, 36% open the dura, and 27% shrink the tonsils. Very few respondents indicated that they offer syrinx drainage as first-line therapy (< 3%). Although all respondents reported that they treat symptomatic CM-I/syringomyelia patients surgically, 15% of respondents indicated that they do not operate on asymptomatic patients. Finally, respondents stated that their most common complications are pseudomeningocele and chemical meningitis. Conclusions This survey, given to a representative group of experienced North American pediatric neurosurgeons, confirms that posterior fossa decompression is still the preferred treatment modality in children with CM-I and syringomyelia, regardless of symptoms. Although most surgeons open the dura, preferred techniques for decompression vary. In contrast to the results of past surveys, conservative follow-up is now only used by a minority of respondents and only in the asymptomatic patient, and primary syrinx drainage seems to have lost popularity. A multicenter trial of surgical outcomes has been designed based on the information from this survey.

Download Full-text

Outcomes in pediatric patients with Chiari malformation Type I followed up without surgery

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.1.peds10341 ◽

2011 ◽

Vol 7 (4) ◽

pp. 375-379 ◽

Cited By ~ 47

Author(s):

David Benglis ◽

Derek Covington ◽

Ritwik Bhatia ◽

Sanjiv Bhatia ◽

Mohamed Samy Elhammady ◽

...

Keyword(s):

Chiari Malformation ◽

Pediatric Patients ◽

Neurological Deficits ◽

Type I ◽

Imaging Studies ◽

Chiari Malformation Type I ◽

Presenting Symptoms ◽

Asymptomatic Patients ◽

Number Of Patients

Object The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention. Methods The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up. Results The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5–22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up. Conclusions The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.

Download Full-text

Morphometric and volumetric comparison of 102 children with symptomatic and asymptomatic Chiari malformation Type I

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.8.peds17345 ◽

2018 ◽

Vol 21 (1) ◽

pp. 65-71 ◽

Cited By ~ 13

Author(s):

Siri Sahib S. Khalsa ◽

Ndi Geh ◽

Bryn A. Martin ◽

Philip A. Allen ◽

Jennifer Strahle ◽

...

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Pediatric Patients ◽

Incidental Finding ◽

Foramen Magnum ◽

Cerebellar Tonsil ◽

Type I ◽

Chiari Malformation Type I ◽

Asymptomatic Patients ◽

Volumetric Measurements

OBJECTIVEChiari malformation Type I (CM-I) is typically defined on imaging by a cerebellar tonsil position ≥ 5 mm below the foramen magnum. Low cerebellar tonsil position is a frequent incidental finding on brain or cervical spine imaging, even in asymptomatic individuals. Nonspecific symptoms (e.g., headache and neck pain) are common in those with low tonsil position as well as in those with normal tonsil position, leading to uncertainty regarding appropriate management for many patients with low tonsil position and nonspecific symptoms. Because cerebellar tonsil position is not strictly correlated with the presence of typical CM-I symptoms, the authors sought to determine if other 2D morphometric or 3D volumetric measurements on MRI could distinguish between patients with asymptomatic and symptomatic CM-I.METHODSThe authors retrospectively analyzed records of 102 pediatric patients whose records were in the University of Michigan clinical CM-I database. All patients in this database had cerebellar tonsil position ≥ 5 mm below the foramen magnum. Fifty-one symptomatic and 51 asymptomatic patients were matched for age at diagnosis, sex, tonsil position, and tonsil morphology. National Institutes of Health ImageJ software was used to obtain six 2D anatomical MRI measurements, and a semiautomated segmentation tool was used to obtain four 3D volumetric measurements of the posterior fossa and CSF subvolumes on MRI.RESULTSNo significant differences were observed between patients with symptomatic and asymptomatic CM-I related to tentorium length (50.3 vs 51.0 mm; p = 0.537), supraoccipital length (39.4 vs 42.6 mm; p = 0.055), clivus-tentorium distance (52.0 vs 52.1 mm; p = 0.964), clivus-torcula distance (81.5 vs 83.3 mm; p = 0.257), total posterior fossa volume (PFV; 183.4 vs 190.6 ml; p = 0.250), caudal PFV (152.5 vs 159.8 ml; p = 0.256), fourth ventricle volume to caudal PFV ratio (0.0140 vs 0.0136; p = 0.649), or CSF volume to caudal PFV ratio (0.071 vs 0.061; p = 0.138).CONCLUSIONSNo clinically useful 2D or 3D measurements were identified that could reliably distinguish pediatric patients with symptoms attributable to CM-I from those with asymptomatic CM-I.

Download Full-text

313 Morphometric and Volumetric Comparison of Symptomatic and Asymptomatic Chiari Malformation Type I

Neurosurgery ◽

10.1093/neuros/nyx417.313 ◽

2017 ◽

Vol 64 (CN_suppl_1) ◽

pp. 267-267

Author(s):

Siri Sahib S Khalsa ◽

Ndi Geh ◽

Bryn A Martin ◽

Philip A Allen ◽

Jennifer Strahle ◽

...

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Pediatric Patients ◽

Foramen Magnum ◽

Cerebellar Tonsil ◽

Type I ◽

Chiari Malformation Type I ◽

Asymptomatic Patients ◽

Specific Symptoms ◽

Volumetric Measurements

Abstract INTRODUCTION Chiari malformation type I (CMI) is typically defined by a cerebellar tonsil position 5 mm or more below foramen magnum. Low cerebellar tonsil position is a frequent incidental finding, even in asymptomatic individuals. Non-specific symptoms such as headache and neck pain are common in those with low tonsil position and in those with normal tonsil position, leading to uncertainty regarding appropriate management for many patients with low tonsil position and non-specific symptoms. Since cerebellar tonsil position is not strictly correlated with typical CMI symptoms, we sought to determine if other 2D morphometric or 3D volumetric measurements on MRI could distinguish asymptomatic from symptomatic CMI patients. METHODS We retrospectively analyzed 102 pediatric patients from the University of Michigan clinical CMI database. All patients in this database have cerebellar tonsil position at least 5 mm below foramen magnum. Fifty-one symptomatic and 51 asymptomatic patients were matched for age at diagnosis, sex, tonsil position, and tonsil morphology. Five 2D anatomic MRI measurements and four 3D volumetric measurements of posterior fossa and CSF sub-volumes on MRI were measured. RESULTS >There were no significant differences observed between symptomatic and asymptomatic CMI patients related to basilar impression (−5.9 mm vs. −7.0 mm; P = 0.026), tentorium length (50.3 mm vs. 51.0 mm; P = 0.537), supraoccipital length (39.4 mm vs. 42.6 mm; P = 0.055), clivus-tentorium distance (52.0 mm vs. 52.1 mm; P = 0.964), clivus-torcula distance (81.5 mm vs. 83.3 mm; P = 0.257), total posterior fossa volume (PFV; 183.4 mL vs. 190.6 mL; P = 0.250), caudal PFV (152.5 mL vs. 159.8 mL; P = 0.256), fourth ventricle volume to caudal PFV ratio (0.0140 vs. 0.0136; P = 0.649), or CSF volume to caudal PFV ratio (0.071 vs. 0.061; P = 0.138). CONCLUSION We did not identify any 2D or 3D measurements that could reliably distinguish pediatric patients with symptoms attributable to CMI from those with asymptomatic CMI.

Download Full-text

Syringobulbia in Patients with Chiari Malformation Type I: A Systematic Review

BioMed Research International ◽

10.1155/2019/4829102 ◽

2019 ◽

Vol 2019 ◽

pp. 1-8 ◽

Cited By ~ 1

Author(s):

Jian Shen ◽

Jie Shen ◽

Kaiyuan Huang ◽

Yixin Wu ◽

Jianwei Pan ◽

...

Keyword(s):

Posterior Fossa ◽

Clinical Features ◽

Chiari Malformation ◽

Initial Therapy ◽

Foramen Magnum ◽

Sudden Onset ◽

Posterior Fossa Decompression ◽

Type I ◽

Limb Weakness ◽

Chiari Malformation Type I

This study aimed to summarize the clinical features, diagnosis, and treatment of Chiari malformation type I- (CM-1-) associated syringobulbia. We performed a literature review of CM-1-associated syringobulbia in PubMed, Ovid MEDLINE, and Web of Science databases. Our concerns were the clinical features, radiologic presentations, treatment therapies, and prognoses of CM-1-associated syringobulbia. This review identified 23 articles with 53 cases. Symptoms included headache, neck pain, cranial nerve palsy, limb weakness/dysesthesia, Horner syndrome, ataxia, and respiratory disorders. The most frequently involved area was the medulla. Most of the patients also had syringomyelia. Surgical procedures performed included posterior fossa decompression, foramen magnum decompression, cervical laminectomy, duraplasty, and syringobulbic cavity shunt. Most patients experienced symptom alleviation or resolution postoperatively. A syringobulbic cavity shunt provided good results in refractory cases. Physicians should be aware of the possibility of syringobulbia in CM-1 patients, especially those with symptoms of sudden-onset brain-stem involvement. The diagnosis relies on the disorder’s specific symptomatology and magnetic resonance imaging. Our review suggests that the initial therapy should be posterior fossa decomposition with or without duraplasty. In refractory cases, additional syringobulbic cavity shunt is the preferred option.

Download Full-text

The natural history of the Chiari Type I anomaly

Journal of Neurosurgery Pediatrics ◽

10.3171/ped/2008/2/9/179 ◽

2008 ◽

Vol 2 (3) ◽

pp. 179-187 ◽

Cited By ~ 131

Author(s):

Federica Novegno ◽

Massimo Caldarelli ◽

Antonio Massa ◽

Daniela Chieffo ◽

Luca Massimi ◽

...

Keyword(s):

Mr Imaging ◽

Chiari Malformation ◽

Clinical Signs ◽

Type I ◽

Conservative Approach ◽

Tonsillar Herniation ◽

Chiari Malformation Type I ◽

Lack Of Information ◽

Mild Reduction

Object Since the advent of MR imaging, an increasing number of asymptomatic or oligosymptomatic patients have been diagnosed with Chiari malformation Type I (CM-I). The decision of whether or not to operate is more difficult in these patients than in those with clear symptoms because of the lack of information about the natural course of this disease. Methods The authors report on their experience in a series of 22 patients with CM-I who were evaluated at the authors' institution, and for whom a conservative approach to treatment was adopted. The patients ranged in age from 1 to 16 years (mean 6.3 years) at diagnosis. Neuroradiological and complete clinical examinations were performed in all patients 6 months after the first observation and every year thereafter. The follow-up period ranged from 3 to 19 years (mean 5.9 years). Results Chiari malformation Type I was incidentally detected on MR images in 11 of 22 patients. The remaining 11 patients had minimal clinical signs at presentation that were not regarded as necessitating immediate surgical treatment. Seventeen patients (77.3%) showed progressive improvement in their symptoms or remained asymptomatic at the last follow-up whereas 5 patients (22.7%) experienced worsening, which was mild in 2 cases and required surgical correction in the remaining 3 cases. On MR imaging a mild reduction in tonsillar herniation was appreciated in 4 patients (18.18%), with complete spontaneous resolution in 1 of these. In 16 patients, tonsillar herniation remained stable during follow-up. Conclusions The authors' data confirm the common impression that in both asymptomatic and slightly symptomatic patients with CM-I, a conservative approach to treatment should be adopted with periodic clinical and radiological examinations.

Download Full-text

Intraoperative ultrasonography used to determine the extent of surgery necessary during posterior fossa decompression in children with Chiari malformation Type I

Yearbook of Diagnostic Radiology ◽

10.1016/s0098-1672(08)70086-9 ◽

2007 ◽

Vol 2007 ◽

pp. 116-117

Author(s):

A.E. Oestreich

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Intraoperative Ultrasonography ◽

Posterior Fossa Decompression ◽

Type I ◽

Chiari Malformation Type I ◽

Extent Of Surgery

Download Full-text

Management of Chiari Malformation Type I with Posterior Fossa Decompression without Dural Opening

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0035-1566365 ◽

2015 ◽

Vol 76 (S 02) ◽

Author(s):

Walid Elhalaby

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Posterior Fossa Decompression ◽

Type I ◽

Chiari Malformation Type I ◽

Dural Opening

Download Full-text

Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale

Journal of Neurosurgery Spine ◽

10.3171/2020.6.spine20544 ◽

2020 ◽

pp. 1-7

Author(s):

Michael Lumintang Loe ◽

Tito Vivas-Buitrago ◽

Ricardo A. Domingo ◽

Johan Heemskerk ◽

Shashwat Tripathi ◽

...

Keyword(s):

Pilot Study ◽

Chiari Malformation ◽

Prognostic Significance ◽

Foramen Magnum ◽

Bivariate Analysis ◽

Type I ◽

Foramen Magnum Decompression ◽

Swallowing Function ◽

Chiari Malformation Type I

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

Download Full-text