Patients with Chiari malformation Type I presenting with acute neurological deficits: case series

2011 ◽  
Vol 7 (3) ◽  
pp. 244-247 ◽  
Author(s):  
Chester K. Yarbrough ◽  
Alexander K. Powers ◽  
Tae Sung Park ◽  
Jeffrey R. Leonard ◽  
David D. Limbrick ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Posterior Fossa ◽  
Neurological Deficit ◽  
Chiari Malformation ◽  
Acute Onset ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Sensory Deficits ◽  
Operative Notes

Object A subset of patients with Chiari malformation Type I (CM-I) presented with acute onset of a neurological deficit. In this study the authors summarize their experience with these patients' clinical presentation, imaging results, timing of surgery, and outcome following decompression. Methods The authors reviewed clinical records, imaging studies, and operative notes from all patients undergoing posterior fossa decompression for CM-I at St. Louis Children's Hospital from 1990 to 2008. Of the 189 patients who underwent surgery, 6 were identified with the acute onset of a neurological deficit at presentation. Results All 6 children (age range 3–14 years, 3 boys and 3 girls) had either syringomyelia (5 patients) or T2 signal changes in the spinal cord (1 patient) and CM-I on initial MR imaging. Three patients presented after minor trauma (1 with paraparesis, 2 with sensory deficits). Three patients presented without a clear history of trauma (1 with abrupt onset of spontaneous dysphagia and ataxia, 2 with sensory deficits). Decompression was performed at a mean 7.7 ± 4.9 days after symptom onset (7.0 ± 1.6 days after neurosurgical evaluation). In 1 patient, symptoms had resolved by the time of surgery; in the remainder of the patients, clear improvements were noted within 2 weeks of surgery, with complete resolution of symptoms by 12 months postoperatively. Follow-up MR images were obtained in 4 patients, demonstrating improvement in the extent of the syrinx in each patient. Conclusions Children with CM-I and syringomyelia can develop acute spinal cord or bulbar deficits with relatively minor head or neck injuries. The prognosis for symptomatic improvement in the observed deficit is good, with each patient in our series showing resolution of deficits over time. However, based on this relatively limited experience, the authors suggest that patients who present with an acute neurological deficit and are found to have CM-I be managed with early posterior fossa decompression. Patients with CM-I and syringomyelia may be at higher risk of acute neurological deficit than those without a syrinx.

scholarly journals Syrinx resolution after posterior fossa decompression in patients with scoliosis secondary to Chiari malformation type I

2011 ◽  
Vol 21 (6) ◽  
pp. 1143-1150 ◽  
Author(s):  
Tao Wu ◽  
Zezhang Zhu ◽  
Jian Jiang ◽  
Xin Zheng ◽  
Xu Sun ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I

Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis

2008 ◽  
Vol 2 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Susan R. Durham ◽  
Kristina Fjeld-Olenec
Keyword(s):  
Cerebrospinal Fluid ◽  
Surgical Treatment ◽  
Clinical Improvement ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Meta Analysis ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Patient Age

Object Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children. Methods The authors searched Medline–Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000–2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years. Results Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08–0.69) and a higher rate of cerebrospinal fluid–related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53–23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95–1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91–2.25) were noted between PFDD and PFD. Conclusions Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid–related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.

scholarly journals The significance of occipitocervical dura angulation in selection of surgery procedures for Chiari malformation type I

2019 ◽  
Vol 7 (3) ◽  
pp. 109-115
Author(s):  
Bo Xiu ◽  
Rui Zhang
Keyword(s):  
Posterior Fossa ◽  
Dura Mater ◽  
Chiari Malformation ◽  
Sagittal Plane ◽  
Atlantoaxial Instability ◽  
Obtuse Angle ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Selection Of

At present, the common surgical procedures for the Chiari malformation type I are comprised of posterior fossa decompression, duraplasty and tonsillectomy. Some neurosurgeons prefer these so called minimally invasive surgeries. However, there are still some failures for patients undergoing the above surgeries in clinical practice. Analyzing causes of many surgical failures, the author put forward the anatomical concept of occipitocervical dura angulation (ODA). The ODA is defined as the included angle between the cerebral dura mater and spinal dura mater at the posterior foramen magnum on the median sagittal plane. For Chiari malformation type I without atlantoaxial instability, the selection of appropriate surgeries and accurate evaluation on the effect of the decompression can be realized after the comprehensive analysis both on the severity of tonsil herniation and the ODA. Tonsillectomy may be needed to add to posterior fossa decompression (PFD) and duraplasty for Chiari malformation type I with the ODA being the larger obtuse angle and/or the tonsil herniation to the level of arcus posterior atlantis.

scholarly journals Improvement of Hydrocephalus in Patients with Chiari Malformation After Posterior Fossa Decompression

2018 ◽  
Vol 27 (1) ◽  
pp. 71-73
Author(s):  
Lindolfo Carlos Heringer ◽  
Matheus Fernandes De Oliveira ◽  
Ulysses Oliveira De Sousa ◽  
Wanderley Cerqueira De Lima ◽  
Ricardo Vieira Botelho
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Clinical Symptoms ◽  
Foramen Magnum ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Cisterna Magna ◽  
Long Time ◽  
Small Posterior Fossa

Introduction. The association between hydrocephalus and Chiari malformation (CM) has not been described frequently. Ventricular dilation affects 7% to 10% of patients with CM, but the ideal choice of surgical treatment is controversial. Wereport a case of a patient with Chiari malformation and hydrocephalus with improvement in clinical symptoms and ventricular dilatation. Case Description. A 19-year-old male complaining of frontal headache when coughing, laughing and during valsalva maneuvers, associated with dizziness for 2 months. Magnetic resonance (MR) showed hydrocephalus and small posterior fossa with overcrowding of contents, characterizing Chiari malformation type I, with cerebellar tonsils protruding through magnum foramen. Patient underwent surgery with posterior fossa decompression in a semi-sitting position and removal of the arc C1.After 3 months of follow-up, headache disappeared becoming asymptomatic. Control MR showed improvement of hydrocephalus with restoration of the cisterna magna and CSF flow. Discussion. Hydrocephalus has been related to CM for a long time. In our case, we performed treatment with intradural and intra-arachnoidal approach with bilateral tonsillectomy without placing ventricular shunt. The cisterna magna was “recreated”. There was improvement of hydrocephalus with decreased Evans ratio index and symptoms disappearance. Although there is no other studies addressing such matter, in this case, the improvement suggests that the CSF compression at the foramen magnum was the cause of associated hydrocephalus with Chiari malformation. 

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

2011 ◽  
Vol 8 (5) ◽  
pp. 430-437 ◽  
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.

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