Symptomatic Chiari Malformation in Adults: A New Classification Based on Magnetic Resonance Imaging with Clinical and Prognostic Significance

Pillay, Prem K.; Awad, Issam A.; Little, John R.; Hahn, Joseph F.

doi:10.1227/00006123-199105000-00001

Cited by 172 publications

(84 citation statements)

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“…More recently, MRI has become the procedure of choice for diagnosis of cranial and spinal malformations [13,20]. Myelography and myelography assisted with computed tomography (CT), which were the techniques used in detecting syrinxes before MRI, have been the most commonly used radiological methods in the study of syringomyelia.…”

Section: Discussionmentioning

confidence: 99%

“…However, there are no objective criteria to suggest which procedure will produce the best surgical results in such cases, and comparisons are difficult because of the differences in surgical techniques, indications for operation, and criteria for the evaluation of results, including length of follow-up period. It should be pointed out that in the most successful surgical series, posterior fossa decompression relieves the symptoms of syringomyelia in only 50% of the cases [1,18,20]. The other group of authors have advocated syrinx shunting procedures as the initial approach, as they are easy to perform, are generally well tolerated and are associated with results that are easily the equal of those achieved by foramen magnum decompression [2][3][4].…”

Section: Discussionmentioning

confidence: 99%

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Surgical management of syringomyelia-Chiari complex

Ergün¹,

Akdemir²,

Gezici³

et al. 2000

European Spine Journal

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IntroductionMagnetic resonance imaging (MRI) has facilitated the diagnosis of syringomyelia in recent years, providing sharp delineation of syrinxes and accurate identification of malformations at the cranio-cervical junction. What is the mechanism of origin and maintenance of syringomyelia? Syringomyelia cannot be ascribed to a single pathophysiological mechanism, and its etiology and natural history are quite variable. It occurs in relation to intramedullary tumors, compressive myelopathy, spinal trauma, hydrocephalus and anomalies such as the Chiari malformation. Syringomyelia, is most frequently associated with Chiari malformation, and several hypotheses have been proposed to explain its pathophysiology in this particular manifestation.One explanation for syrinx formation, which has recently been reported by Oldfield et al. [18], requires no Abstract Great variety exists in the indications and techniques recommended for the surgical treatment of syringomyelia-Chiari complex. More recently, magnetic resonance (MR) imaging has increased the frequency of diagnosis of this pathology and offered a unique opportunity to visualize cavities inside the spinal cord as well as their relationship to the cranio-cervical junction. This report presents 18 consecutive adult symptomatic syringomyelia patients with Chiari malformation who underwent foramen magnum decompression and syringosubarachnoid shunting. The principal indication for the surgery was significant progressive neurological deterioration. All patients underwent preoperative and postoperative MRI scans and were studied clinically and radiologically to assess the changes in the syrinx and their neurological picture after surgical intervention. All patients have been followed up for at least 36 months. No operative mortality was encountered; 88.9% of the patients showed improvement of neurological deficits together with radiological improvement and 11.1% of them revealed collapse of the syrinx cavity but no change in neurological status. None of the patients showed further deterioration of neurological function. The experience obtained from this study demonstrates that foramen magnum decompression to free the cerebro-spinal fluid (CSF) pathways combined with a syringosubarachnoid shunt performed at the same operation succeeds in effectively decompressing the syrinx cavity, and follow-up MR images reveal that this collapse is maintained. In view of these facts, we strongly recommend this technique, which seems to be the most rational surgical procedure in the treatment of syringomyeliaChiari complex.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Surgical management of syringomyelia-Chiari complex

Ergün¹,

Akdemir²,

Gezici³

et al. 2000

European Spine Journal

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“…[1][2][3][4][5][6][7][8][9][11][12][13][14][15][16][17][18][19][65][66][67]70,71,100,[102][103][104][105][106][107][108][109][110][111][113][114][115][116][117][118][119][120][121][122][123][124][125][126][127][128][129][130][132]…”

Section: General Informationunclassified

Pediatric and adult Chiari malformation Type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes

Arnautovic

Splavski

Boop

et al. 2015

PED

199

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OBJECT Chiari malformation Type I (CM-I) is a hindbrain disorder associated with elongation of the cerebellar tonsils, which descend below the foramen magnum into the spinal canal. It occurs in children and adults. Clinical symptoms mainly develop from alterations in CSF flow at the foramen magnum and the common subsequent development of syringomyelia. METHODS The authors reviewed English-language reports of pediatric, adult, and combined (adult and pediatric) surgical series of patients with CM-I published from 1965 through August 31, 2013, to investigate the following: 1) geographical distribution of reports; 2) demographics of patients; 3) follow-up lengths; 4) study durations; 5) spectrum and frequency of surgical techniques; 6) outcomes for neurological status, syrinx, and headache; 7) frequency and scope of complications; 8) mortality rates; and 9) differences between pediatric and adult populations. Research and inclusion criteria were defined, and all series that contained at least 4 cases and all publications with sufficient data for analysis were included. RESULTS The authors identified 145 operative series of patients with CM-I, primarily from the United States and Europe, and divided patient ages into 1 of 3 categories: adult (> 18 years of age; 27% of the cases), pediatric (≤ 18 years of age; 30%), or unknown (43%). Most series (76%) were published in the previous 21 years. The median number of patients in the series was 31. The mean duration of the studies was 10 years, and the mean follow-up time was 43 months. The peak ages of presentation in the pediatric studies were 8 years, followed by 9 years, and in the adult series, 41 years, followed by 46 years. The incidence of syringomyelia was 65%. Most of the studies (99%) reported the use of posterior fossa/foramen magnum decompression. In 92%, the dura was opened, and in 65% of these cases, the arachnoid was opened and dissected; tonsillar resection was performed in 27% of these patients. Postoperatively, syringomyelia improved or resolved in 78% of the patients. Most series (80%) reported postoperative neurological outcomes as follows: 75% improved, 17% showed no change, and 9% experienced worsening. Postoperative headaches improved or resolved in 81% of the patients, with a statistical difference in favor of the pediatric series. Postoperative complications were reported for 41% of the series, most commonly with CSF leak, pseudomeningocele, aseptic meningitis, wound infection, meningitis, and neurological deficit, with a mean complication rate of 4.5%. Complications were reported for 37% of pediatric, 20% of adult, and 43% of combined series. Mortality was reported for 11% of the series. No difference in mortality rates was seen between the pediatric and adult series. CONCLUSIONS Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications. Furthermore, surgeons may benefit from comparing published data with their own. In the future, operative CM-I reports should provide all details of each case for the purpose of comparison.

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“…139 Lower position of the cerebellar tonsils in CM is associated with a greater likelihood of syringomyelia. [139][140][141] It is probable that Chiari symptoms and the formation of spinal syringes are the result of crowding at the foramen magnum that leads to abnormal movement of CSF at the craniocervical junction. 134 Children with CM often have a smaller than normal posterior fossa volume, resulting in crowding of the posterior fossa and foramen magnum contents.…”

Section: Chiari and Syringomyeliamentioning

confidence: 99%

Incidental Findings on Brain and Spine Imaging in Children

Maher¹,

Piatt²,

Ragheb³

et al. 2015

Pediatrics

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In recent years, the utilization of diagnostic imaging of the brain and spine in children has increased dramatically, leading to a corresponding increase in the detection of incidental findings of the central nervous system. Patients with unexpected findings on imaging are often referred for subspecialty evaluation. Even with rational use of diagnostic imaging and subspecialty consultation, the diagnostic process will always generate unexpected findings that must be explained and managed. Familiarity with the most common findings that are discovered incidentally on diagnostic imaging of the brain and spine will assist the pediatrician in providing counseling to families and in making recommendations in conjunction with a neurosurgeon, when needed, regarding additional treatments and prognosis.

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Symptomatic Chiari Malformation in Adults: A New Classification Based on Magnetic Resonance Imaging with Clinical and Prognostic Significance

Cited by 172 publications

References 0 publications

Surgical management of syringomyelia-Chiari complex

Surgical management of syringomyelia-Chiari complex

Pediatric and adult Chiari malformation Type I surgical series 1965–2013: a review of demographics, operative treatment, and outcomes

Incidental Findings on Brain and Spine Imaging in Children

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