Adult Scoliosis in Syringomyelia Associated with Chiari I Malformation

Ono, Atsushi; Ueyama, Kazumasa; Okada, Akihiro; Echigoya, Naoki; Yokoyama, T.; Harata, Seiko

doi:10.1097/00007632-200201150-00011

Cited by 58 publications

(33 citation statements)

References 35 publications

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“…Zadeh et al reported that in all ten children they studied with IS and syringomyelia, the superficial abdominal reflex was consistently absent on the same side as the convexity of the curve [19]. In a study by Ono et al, hyperreflexia appeared in all patients with Cobb angle >20°who had syringomyelia with CM-1 [14]. However, we could not confirm this finding.…”

Section: Discussionmentioning

confidence: 62%

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Preoperative radiological and electrophysiological evaluation in 100 adolescent idiopathic scoliosis patients

Hausmann

Böni

Pfirrmann

et al. 2003

European Spine Journal

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This is a prospective study of spinal magnetic resonance imaging (MRI), electrophysiological recordings, and neurological examinations of 100 patients admitted for surgery for adolescent idiopathic scoliosis (AIS), which was conducted to assess the prevalence of structural and functional abnormalities within the spinal cord in patients with clinically normal neurologic condition. In all patients the clinical diagnosis and intact neurological condition was ascertained by a spinal orthopedic surgeon. Full-length spinal axis MRI studies (T1/T2 sequences) and somato-sensory evoked potentials of the tibial nerves (tSSEPs) were preoperatively assessed by independent evaluators blinded to the patients' medical histories. Structural spinal cord abnormalities were found in three of 100 AIS patients on MR imaging. In one patient a Chiari malformation type 1 with an accompanying syringomyelia was diagnosed, which required a suboccipital decompression. In the other two patients small thoracic syringomyelias were diagnosed. Abnormalities of spinal cord function were detected in 68% of the 100 patients: tSSEP latencies corrected for body height were increased in 56% of the patients; pathological differences between tSSEPs on the left and right sides were present in 17% (12% in combination with a prolongation of the latency). The findings of this study indicate that MRI and electrophysiological examinations are essential to assess spinal cord abnormalities that are clinically not detectable in AIS patients. Even in patients with intact neurologic condition and clinically typical right-curved thoracic scoliosis, the possibility of intraspinal pathologies should be ruled out by MRI. It is especially important to detect structural pathologies like syringomyelia and Chiari malformation before proceeding with scoliosis surgery, as these conditions are associated with a higher neurological risk during scoliosis surgery. The electrophysiological recordings made in the present study, with the high number of pathological tSSEPs, are indicative of functional abnormalities with a subclinical involvement of the recorded neuronal pathways. The relevance of the latter findings is not yet clear, but pre-operative tSSEP examinations offer the possibility of assessing alterations in spinal cord function that are undetectable by clinical examination.

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Section: Discussionmentioning

confidence: 62%

“…The partial resection of the cerebellar tonsils (as in case 2) is necessary only in exceptional cases. Improvement of the scoliosis after surgery of the syringomyelia has been reported in children [14].…”

Section: Discussionmentioning

confidence: 96%

Preoperative radiological and electrophysiological evaluation in 100 adolescent idiopathic scoliosis patients

Hausmann

Böni

Pfirrmann

et al. 2003

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

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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“…The extent of cerebellar tonsillar descent was further classified into three grades ( Fig. 2) [14]: grade I, in which the tonsil descended beyond the foramen magnum but did not reach the C-1 arch; grade II, in which the tonsil reached the C-1 arch; and grade III, in which the tonsil descended beyond the C-1 arch. Syrinx configuration was categorized into distended, moniliform, slender, and circumscribed types ( Fig.…”

Section: Radiographic Evaluationmentioning

confidence: 99%

“…Syrinx configuration was categorized into distended, moniliform, slender, and circumscribed types ( Fig. 3) [14]. The maximal anteroposterior diameter of the syrinx (S) divided by the anteroposterior diameter of the spinal cord (C) at the same level provided the maximal S/C ratio [15].…”

Section: Radiographic Evaluationmentioning

confidence: 99%

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

Zhu

Jiang

et al. 2011

Eur Spine J

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Introduction Description of syrinx resolution after posterior fossa decompression (PFD) in patients with scoliosis secondary to Chiari malformation type I (CMI) and syringomyelia (SM) has been rarely reported in the literature. This study was performed to investigate the outcome of PFD in patients with scoliosis secondary to CMI and to identify potential predictive factors for better outcome after PFD. Material and methods Patients with scoliosis secondary to CMI and SM, who had undergone PFD during the period 2000 through 2009, were recruited. Inclusion criteria were (1) age B 18 years, (2) diagnosis of SM associated with CMI, (3) scoliosis as the first complaint, (4) having undergone preoperative and follow-up magnetic resonance imaging (MRI). Patients with acquired CMI anomalies or who had received syringosubarachnoid shunting were excluded. The maximal S/C ratio and syrinx length were measured to evaluate syrinx resolution after PFD. A 20% decrease in S/C ratio or length at the latest follow-up was defined as a significant radiographic improvement and complete resolution was used to describe the syrinx disappearing after PFD.Results 44 patients were recruited. Follow-up MRI was conducted for all 44 patients at 6 ± 3 months postoperatively, for 37 patients at 2 years ± 3 months, for 26 patients at 4 years ± 3 months, and for 15 patients at 6 years ± 3 months. 97.7% (43 of 44) of patients showed significant radiographic improvement by MRI. The distance of tonsillar descent (mm) was correlated significantly with the surgical outcome (r = 0.116, P = 0.013). Significant improvement was observed within 6 months postoperatively, with continued slow improvement after that. ConclusionSyringes showed significant improvement after PFD in most patients with scoliosis secondary to CMI. Resolution generally occurred within 6 months follow-up and continued at a slow rate for several years. In addition, the severity of tonsillar descent is a potential predictor for better improvement after standard PFD.

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Adult Scoliosis in Syringomyelia Associated with Chiari I Malformation

Abstract: In adult syringomyelia with scoliosis, the morbidity period is long, the syrinx is long, the neurologic symptoms are aggravated, and the surgical outcomes tend to be poor.

Cited by 58 publications

References 35 publications

Preoperative radiological and electrophysiological evaluation in 100 adolescent idiopathic scoliosis patients

Preoperative radiological and electrophysiological evaluation in 100 adolescent idiopathic scoliosis patients

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

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

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