Chiari Malformation Presenting in Adults: A Surgical Experience in 127 Cases

Levy, Walter J.; Mason, Laura; Hahn, Joseph F.

doi:10.1227/00006123-198304000-00003

Cited by 271 publications

(84 citation statements)

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“…20,98,99 Among patients with CM-I, associated syringomyelia is frequent and has been reported to range from 30% to 70%. 90,98,99,127 Between 15% and 30% of adult patients with CM-I are reportedly asymptomatic. 20 Suboccipital craniectomy and foramen magnum decompression, with or without dural opening and with or without arachnoid opening and dissection, have been used most commonly for operative treatment; however, there is no clear idea of the frequency of their use in the reported series or whether a difference exists between pediatric and adult patients.…”

Section: Discussionmentioning

confidence: 99%

“…Combined series, which included both pediatric and adult patients, constituted 61 reports (42%). [5][6][7]9,11,12,[25][26][27]34,37,38,40,43,44,46,47,52,53,55,63,66,67,70,73,74,76,83,87,90,91,94,96,100,102,103,106,110,111,114,115,117,121,126,127,129,130,132,135,[138][139][140]…”

Section: General Informationmentioning

confidence: 99%

“…There seems to be a slight female predominance of cases (1.3:1), and the association with syringomyelia has been reported to range widely, from 30% to 70%. 90,127 Suboccipital craniectomy and foramen magnum decompression, with or without dural opening and with or without arachnoid opening or dissection, have been used most commonly for surgical treatment but without a clear idea of the frequency of use of either procedure in the reported series. Furthermore, it has not been noted whether a difference exists in the operative techniques used for pediatric and adult patients.…”

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confidence: 99%

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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

205

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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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Section: Discussionmentioning

confidence: 99%

Section: General Informationmentioning

confidence: 99%

mentioning

confidence: 99%

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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

205

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“…These have never been entirely reliable in demonstrating the syrinx cavity and its relationship to other intracranial abnormalities. Myelography probably misses approximately one-fourth of syrinxes [16]. Highresolution CT scanning with metrizamide offers an improvement over this, but may still miss some syrinxes [13].…”

Section: Discussionmentioning

confidence: 99%

“…There have been several reports of the results of various surgical procedures, including suboccipital craniectomy with or without exposure of the fourth ventricle and plugging of the obex with muscle; upper cervical laminectomy for foramen magnum decompression; shunting from the syrinx to the subarachnoid space, pleura or peritoneum; intermittent percutaneous aspiration of the syrinx; terminal ventriculostomy; and ventriculoperitoneal and lumboperitoneal shunting [3, 9, 10, 18-20, 23-27, 31]. Although posterior fossa decompression remains the traditional surgical approach, performing the additional steps, such as plugging of the obex, shunting of the fourth ventricle, using lumboperitoneal devices or performing terminal ventriculostomy does not enhance the surgical treatment of this disorder [14,16,17,25].…”

Section: Discussionmentioning

confidence: 99%

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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The chiari type I malformation in two monozygotic twins and first‐degree relatives

Stovner

Cappelen

Nilsen³

et al. 1992

Annals of Neurology

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The presence of the Chiari type I malformation in 2 adult monozygotic female twins, their mother, and possibly in 2 of their 4 daughters is reported. The diagnosis was made by magnetic resonance imaging and confirmed at the time of surgery in 1 twin. Monozygosity of the twins was proved by DNA typing. The disorder in the present family should probably be classified together with the autosomal dominant craniocervical malformations. Nongenetic factors also seem to be important because the twins were discordant for the extent of herniation of the cerebellar tonsils and the presence of syringomyelia.

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Chiari Malformation Presenting in Adults: A Surgical Experience in 127 Cases

Cited by 271 publications

References 0 publications

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

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

Surgical management of syringomyelia-Chiari complex

The chiari type I malformation in two monozygotic twins and first‐degree relatives

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