Patient with Chiari malformation Type I presenting with inducible hemifacial weakness

2011 ◽  
Vol 8 (6) ◽  
pp. 620-624 ◽  
Author(s):  
Cordelie E. Witt ◽  
Anthony C. Wang ◽  
Cormac O. Maher ◽  
Khoi D. Than ◽  
Hugh J. L. Garton ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Head Rotation ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Suboccipital Craniectomy ◽  
History Of ◽  
Hypernasal Speech ◽  
Eye Opening ◽  
The Right

In this report, the authors describe the first known case of inducible hemifacial weakness in a patient with Chiari malformation Type I (CM-I). The patient was a 14-year-old girl with a 1-year history of right facial paresis induced by sustained leftward head rotation. These episodes were characterized by weak activation of her right facial muscles with preserved eye opening and closure. Additionally, she had hypernasal speech, persistent headaches, and intermittent left arm twitching. Magnetic resonance imaging demonstrated a CM-I. A suboccipital craniectomy and C-1 laminectomy were performed for decompression of the CM-I, with duraplasty and coagulation of the pial surface of the cerebellar tonsils. At the 9-month follow-up, the patient's inducible hemifacial weakness had completely resolved. Her symptoms were thought to have resulted from the CM-I, perhaps due to traction on the right facial nerve by the ectopic tonsils with head rotation.

Nonoperative management of enlarging syringomyelia in clinically stable patients after decompression of Chiari malformation type I

2021 ◽  
Vol 28 (1) ◽  
pp. 28-33
Author(s):  
Nicholas S. Szuflita ◽  
Tiffany N. Phan ◽  
Jason H. Boulter ◽  
Robert F. Keating ◽  
John S. Myseros
Keyword(s):  
Chiari Malformation ◽  
Nonoperative Management ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Suboccipital Craniectomy ◽  
Close Observation ◽  
Lost To Follow Up ◽  
Serial Mri

OBJECTIVE The authors aimed to describe the natural history and optimal management of persistent syringomyelia after suboccipital craniectomy for Chiari malformation type I (CM-I). METHODS A cohort of all patients who presented to a tertiary pediatric hospital with newly diagnosed CM-I between 2009 and 2017 was identified. Patients with persistent or worsened syringomyelia were identified on the basis of a retrospective review of medical records and imaging studies. The management of these patients and their clinical courses were then described. RESULTS A total of 153 children with CM-I and syringomyelia were evaluated between 2009 and 2017. Of these, 115 (68.8%) patients underwent surgical intervention: 40 patients underwent posterior fossa decompression (PFD) alone, 43 underwent PFD with duraplasty, and 32 underwent PFD with duraplasty and fourth ventricle stent placement. Eleven (7.19%) patients had increased syringomyelia on subsequent postoperative imaging. Three of these patients underwent revision surgery because of worsening scoliosis or pain, 2 of whom were lost to follow-up, and 4 were managed nonoperatively with close surveillance and serial MRI evaluations. The syringes decreased in size in 3 patients and resolved completely in 1 patient. CONCLUSIONS Persistent or worsened syringomyelia after CM-I decompression is uncommon. In the absence of symptoms, nonoperative management with close observation is safe for patients with persistent syrinx.

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

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.

Natural history of Chiari malformation Type I following decision for conservative treatment

2011 ◽  
Vol 8 (2) ◽  
pp. 214-221 ◽  
Author(s):  
Jennifer Strahle ◽  
Karin M. Muraszko ◽  
Joseph Kapurch ◽  
J. Rajiv Bapuraj ◽  
Hugh J. L. Garton ◽  
...  
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Foramen Magnum ◽  
Type I ◽  
Csf Flow ◽  
Chiari Malformation Type I ◽  
Nonsurgical Management ◽  
Cerebellar Tonsillar Herniation ◽  
History Of

Object The natural history of the Chiari malformation Type I (CM-I) is incompletely understood. The authors report on the outcome of a large group of patients with CM-I that were initially selected for nonsurgical management. Methods The authors identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis. Results Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not. Conclusions In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen.

scholarly journals Interlayer Dural Split Technique for Chiari I Malformation Treatment in Adult -- Technical Note

2021 ◽  
Author(s):  
Ioan Alexandru Florian ◽  
Mihaela Maria Pop ◽  
Teodora Larisa Timis ◽  
Ioan Stefan Florian
Keyword(s):  
Chiari Malformation ◽  
Chiari I Malformation ◽  
Sensory Loss ◽  
Posterior Arch ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Control Series ◽  
Suboccipital Craniectomy ◽  
Chiari I ◽  
Split Technique

Objective: To present an alternative surgical technique in treating cases of Chiari I Malformation with mild-to-moderate syringomyelia after decompressive suboccipital craniectomy: incising only the outer layer of the dura mater, then dissecting it from the inner layer without opening the latter. Methods and Results: We utilized this technique in a short series of three cases who were admitted in our department for mild symptoms such as intermittent headache and dissociated sensory loss in the upper limbs, caused by a Chiari Malformation Type I. The patients were placed in the sitting position. We performed a reduced median suboccipital craniectomy and resection of the posterior arch of C1 adapted to the level of tonsil descent, from a limited superior half to a complete resection. Afterwards, we incised the outer dural layer, while sparing the inner one. Using a fine dissector, we then split apart the outer and inner layers to the margin of the craniectomy. Through the transparency of the inner layer and the arachnoid, the cerebellum and the medulla were visible and pulsating. An autologous fascia duraplasty was then performed. The postoperative course was favorable in all cases, patients being discharged without any deficits and with complete symptom resolution. Conclusions: Interlayer dural split technique can be used effectively in treating symptomatic cases of type I Chiari malformation in adults, with mild-to-moderate syringomyelia. It is less invasive than opening the dura and possibly more effective than decompressive craniectomy and C1 laminectomy alone. This technique must be validated in a larger case-control series.

Quadriparesis due to the delayed formation of a compressive epidural cerebrospinal fluid collection following suboccipital craniectomy with duraplasty for Chiari malformation Type I

2007 ◽  
Vol 7 (4) ◽  
pp. 450-453 ◽  
Author(s):  
Daniel M. Oberer ◽  
Oran S. Aaronson ◽  
Joseph S. Cheng
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Chiari Malformation ◽  
Fluid Collection ◽  
Spontaneous Intracranial Hypotension ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Suboccipital Craniectomy ◽  
Neurological Insult

✓ The authors describe a previously undocumented complication of suboccipital craniectomy combined with duraplasty for the treatment of Chiari malformation Type I and propose techniques to prevent its occurrence. Although there have been reports of epidural pseudomeningoceles in the setting of spontaneous intracranial hypotension and intracranial hygromas following suboccipital craniectomy with duraplasty, the authors believe this case to be the first instance of quadriparesis caused by the delayed formation of a compressive epidural cerebrospinal fluid collection after suboccipital craniectomy with duraplasty. This complication is significant and must be recognized given the potential severity of neurological insult and the number of these procedures performed yearly in both the pediatric and adult populations.

Bony Decompression for Chiari Malformation Type I: Long-Term Follow-Up

2019 ◽  
pp. 119-124 ◽  
Author(s):  
Luca Massimi ◽  
Paolo Frassanito ◽  
Daniela Chieffo ◽  
Gianpiero Tamburrini ◽  
Massimo Caldarelli
Keyword(s):  
Chiari Malformation ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Long Term Follow Up

scholarly journals Breaking Barriers for Cerebrospinal Fluid Flow in Chiari Malformation Type I: “What and How Much Is Enough?” A Retrospective Analysis of 74 Cases

2018 ◽  
Vol 07 (03) ◽  
pp. 196-208
Author(s):  
Manish Jaiswal ◽  
Amit Patil ◽  
Radhey Mittal
Keyword(s):  
Cerebrospinal Fluid ◽  
Natural History ◽  
Retrospective Analysis ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Common Finding ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Compression Syndrome ◽  
History Of

Abstract Introduction: Chiari malformation type I is a collection of hindbrain abnormalities, for which natural history of the disease process is not clear. The challenge is to identify which patients will benefit most from posterior fossa decompression. Objectives: To identify important surgical implications that most likely benefit patients with Chiari malformation type I by analyzing and reviewing various operative interventions in these patients with appropriate symptoms and then following their course. Subjects & Methods: Retrospective analysis of 74 operated Chiari malformation type I with syrinx adult patients was done. Results: No definite pattern of progression in natural history of disease was noted. Most of the patients who were symptomatically stable for months to years presented with recent rapid progression. The most common symptom was suboccipital pain. The most common finding was lower extremity weakness. On clinical presentation basis, patients were divided into three categories: foramen magnum compression syndrome, central cord syndrome, and cerebellar syndrome. Most patients in our study fall in first category. Foramen magnum decompression with atlas posterior arch removal and sometimes partial C2 laminectomy depending on extent of tonsillar descent as well as augmentation duraplasty was done in most patients. Improvement was seen in foramen magnum compression syndrome group more significantly. Conclusions: Individualized surgical techniques for breaking the barriers of cerebrospinal fluid (CSF) flow in Chiari malformation type I with syrinx to restore normal CSF dynamics across craniocervical junction provide the pragmatic solution. The trend is toward balance between optimum wide decompression as compared with long craniocaudal decompression and preserving normal integrity.

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

2011 ◽  
Vol 7 (4) ◽  
pp. 375-379 ◽  
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.

scholarly journals The impact of depression on surgical outcome in Chiari malformation type I: an assessment based on the Chicago Chiari Outcome Scale

2020 ◽  
Vol 33 (3) ◽  
pp. 273-280
Author(s):  
James Feghali ◽  
Yuxi Chen ◽  
Yangyiran Xie ◽  
Christopher Chen ◽  
Judy Huang
Keyword(s):  
Surgical Outcome ◽  
Chiari Malformation ◽  
Type I ◽  
Univariable Analysis ◽  
Chiari Malformation Type I ◽  
Depressed Patients ◽  
Significant Difference ◽  
Pain Symptoms ◽  
The Impact

OBJECTIVEThe effect of depression on outcomes in Chiari malformation type I (CM-1) is unclear. The authors sought to determine whether depression affects outcome in a surgical cohort of CM-1 patients by using a validated outcome assessment tool, the Chicago Chiari Outcome Scale (CCOS).METHODSThe authors performed a retrospective analysis of a prospectively maintained database of 149 adult CM-1 patients undergoing suboccipital decompression with duraplasty and cranioplasty. Baseline presentation characteristics and composite as well as subcomponent CCOS scores at last follow-up were compared between depressed and nondepressed patients. Outcome comparisons included both a univariable analysis and a logistic regression model adjusting for several covariates.RESULTSThe prevalence of depression in the study cohort was 28% (41/149). Baseline demographic and imaging characteristics were similar between the 2 patient groups. Dizziness (p = 0.019) and imbalance (p = 0.015) were significantly more common among depressed patients, but clinical symptoms and severity were otherwise comparable. On univariable analysis, depressed patients were significantly less likely to experience improvement in pain symptoms (OR 0.14, 95% CI 0.03–0.61, p = 0.003) and functionality (OR 0.17, 95% CI 0.03–0.99, p = 0.049). No significant difference was identified in complications, nonpain symptom improvement, or overall composite CCOS improvement. Similar results were obtained on multivariable analysis controlling for several covariates.CONCLUSIONSDepression is independently associated with poor surgical outcome in adult CM-1 patients, namely when evaluating improvement in pain symptoms and functionality. Optimizing the management of depression preoperatively and ensuring follow-up for psychiatric comorbidity in the postoperative period may possibly lead to improved outcomes.

Heterotopic ossification following suboccipital craniectomy decompression surgery for Chiari malformation type I: case report

2019 ◽  
Vol 23 (6) ◽  
pp. 704-707 ◽  
Author(s):  
Christopher E. Louie ◽  
Jennifer Hong ◽  
David F. Bauer
Keyword(s):  
Heterotopic Ossification ◽  
Chiari Malformation ◽  
Bone Growth ◽  
Low Cost ◽  
Rare Complication ◽  
Decompression Surgery ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Suboccipital Craniectomy

Suboccipital craniectomy with duraplasty is a commonly performed procedure for children with symptomatic Chiari malformation type I (CM-I). Several dural substitutes are used for duraplasty, ranging from pericranium to synthetic materials. When available, autologous pericranium is often preferred due to its low cost, performance in obtaining a watertight closure, ease of suturing, and absence of immune reaction. Long-term follow-up data on the durability of various dural substitutes are lacking. The authors report a rare, long-term complication of duraplasty performed using an autologous pericranial graft, and they conduct a literature review of similar complications. Heterotopic ossification of an autologous pericranial graft is a rare complication of duraplasty. This dystrophic bone growth can be symptomatic due to compression of neural structures, and it requires reoperation for removal. Surgeons should consider this rare long-term complication in patients presenting with unusual symptoms after duraplasty with pericranium.

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