| Literature DB >> 20796294 |
Takahito Fujimori1, Motoki Iwasaki, Yukitaka Nagamoto, Hironobu Sakaura, Kazuya Oshima, Hideki Yoshikawa.
Abstract
BACKGROUND: With increasing use of magnetic resonance imaging (MRI), underlying syringomyelia is increasingly found in patients with presumed idiopathic scoliosis. To determine the indications for MRI in the differential diagnosis of scoliosis, several clinical characteristics of syringomyelia have been reported. Neurological signs, particularly abnormal superficial abdominal reflex (SAR), are important in establishing the initial diagnosis of scoliosis. However, the prevalence of abnormal SAR in patients with scoliosis and the sensitivity of this sign in predicting syringomyelia are not well known. We aimed to determine the diagnostic utility of SAR and other characteristics of syringomyelia in patients with scoliosis.Entities:
Year: 2010 PMID: 20796294 PMCID: PMC2939502 DOI: 10.1186/1748-7161-5-17
Source DB: PubMed Journal: Scoliosis ISSN: 1748-7161
Demographic data of 9 patients with syringomyelia
| Case | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 |
|---|---|---|---|---|---|---|---|---|---|
| Age (years) | 28 | 8 | 12 | 9 | 14 | 16 | 6 | 11 | 12 |
| Gender | M | M | F | F | F | M | F | M | F |
| Abnormality | Chiari | Syrinx | Chiari | Syrinx | Chiari | Chiari | Chiari | Chiari | Chiari |
| Cobb angle | 90 | 60 | 105 | 85 | 71 | 56 | (38) | (23) | (30) |
| Flexibility (%) | 52 | 78 | 55 | 50 | 56 | 58 | None | None | None |
| SAR | Bil hypo | R abs | L abs | Bil hypo | L abs | L hypo | L abs | L hypo | Normal |
| Convex side | rt T | rt T | rt T | rt T | lt T | rt T+ | lt T-L | lt L | rt T |
| Corrective surgery | ASF | ASF | ASF + PSF | ASF + PSF | PSF | PSF | None | None | None |
| Neurosurgery | None | SS | FMD | SS | FMD | FMD+ SS | FMD | FMD | None |
ASF, anterior spinal fusion; Chiari, Arnold-Chiari malformation with syrinx; FMD, foramen magnum decompression; lt, left; L, lumbar; PSF, posterior spinal fusion; rt, right; SAR, superficial abdominal reflex; SS, syringo-subarachnoid shunt; T, thoracic; T-L, thoracolumbar
Abnormality of superficial abdominal reflex (20 of 93 cases; 22%)
| Abnormal SAR | Number of cases | Diagnosis |
|---|---|---|
| A: Bilateral absence | 6 | 6 myopathic |
| B: Unilateral absence | 6 | 4 syringomyelia |
| 1 thoracogenic (after abdominal surgery) | ||
| 1 myelomeningocele (after abdominal surgery) | ||
| C: Bilateral hyporeflexia | 5 | 2 syringomyelia |
| 1 Duchenne muscular dystrophy | ||
| 2 idiopathic | ||
| D: Unilateral hyporeflexia | 3 | 2 syringomyelia |
| 1 thoracogenic (after abdominal surgery) |
SAR, superficial abdominal reflex
Abnormal SAR as a predictor of non-idiopathic scoliosis in all patients
| Non-idiopathic | Idiopathic | Total | ||
|---|---|---|---|---|
| Abnormal SAR | 18 | 2 | 20 | PPV 90% |
| Normal SAR | 29 | 44 | 73 | NPV 60% |
| Total | 47 | 46 | 93 | |
| Sensitivity 38% | Specificity 96% |
NPV, negative predictive value; PPV, positive predictive value; SAR, superficial abdominal reflex
Figure 1Posteroanterior radiograph (A) and sagittal MRI (B) in Case 9. The patient was neurologically normal. The syrinx was spindle type and the space of the cisterna magna was preserved. This patient was not suitable for FMD and was treated using an underarm brace.
Abnormal SAR as a predictor of scoliosis with syringomyelia in all patients
| Syringomyelia | No syringomyelia | Total | ||
|---|---|---|---|---|
| Abnormal SAR | 8 | 12 | 20 | PPV 40% |
| Normal SAR | 1 | 72 | 73 | NPV 99% |
| Total | 9 | 84 | 93 | |
| Sensitivity 89% | Specificity 86% |
NPV, negative predictive value; PPV, positive predictive value; SAR, superficial abdominal reflex
Abnormal SAR as a predictor of scoliosis with syringomyelia in patients with "idiopathic" scoliosis
| Syringomyelia | No syringomyelia | Total | ||
|---|---|---|---|---|
| Abnormal SAR | 8 | 2 | 10 | PPV 80% |
| Normal SAR | 1 | 44 | 45 | NPV 98% |
| Total | 9 | 46 | 55 | |
| Sensitivity 89% | Specificity 95% |
"idiopathic" included idiopathic scoliosis and scoliosis with syringomyelia
Curve flexibility of syringomyelia
| Syringomyelia | Idiopathic scoliosis | P | |
|---|---|---|---|
| Gender (% male) | 44 | 4.3 | 0.02*† |
| Age (years) | 14.5 (8-28) | 14.1 (7-21) | 0.44 |
| Risser sign | 2.8 (0-5) | 3.4 (0-5) | 0.27 |
| Curve magnitude(°) | 78 (56-105) | 69 (45-128) | 0.17 |
| Curve flexibility (%) | 58 (50-78) | 44 (11-96) | 0.02* |
Values are expressed as mean (range).
*Significant difference compared to the idiopathic scoliosis group
†Fisher's exact test
Figure 2Distribution of curve flexibility between syringomyelia and idiopathic scoliosis (Cobb angle ≥60°).
Flexibility ≥ 50% as a predictor of scoliosis with syringomyelia in "idiopathic" scoliosis underwent operation
| Syringomyelia | No syringomyelia | Total | ||
|---|---|---|---|---|
| Flexibility ≥ 50% | 6 | 17 | 23 | PPV 26% |
| Flexibility < 50% | 0 | 29 | 29 | NPV 100% |
| Total | 6 | 46 | 52 | |
| Sensitivity 100% | Specificity 63% |
"idiopathic" included idiopathic scoliosis and scoliosis with syringomyelia
Relationship between syrinx type and neurological abnormality
| Case | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 |
|---|---|---|---|---|---|---|---|---|---|
| Syrinx level | C3-7 | Th6-9 | C5-7 | C5-8 | C5-9 | C2-7 | C1-L1 | C2-Th11 | C5-8 |
| Syrinx length | 5 | 4 | 3 | 4 | 5 | 6 | 20 | 17 | 4 |
| SAR | Bil hypo | R abs | L abs | Bil hypo | L abs | L hypo | L abs | L hypo | Normal |
| Other neurological findings | Bil Babinski reflex | Hyper of L/E | None | Hyper of L/E | Hypo of L/E | Hyper of ATR | Hypo of L/E | Headache | None |
abs, absence; ATR, Achilles tendon reflex; Bil, bilateral; hyper, hyperreflexia; hypo, hyporeflexia; L, left; L/E, lower extremities; R, right; SAR, superficial abdominal reflex; SD, sensory disturbance
Figure 3Posteroanterior radiograph (A) and sagittal MRI (B) in Case 1. This patient had bilateral hyporeflexia of SAR and declined neurological decompression surgery, but anterior fusion was performed without any neurological complication. The Cobb angle was 90° and curve flexibility was 52%. The syrinx was slit type.
Diagnostic utility of SAR in "idiopathic" scoliosis
| Sensitivity (%) | Specificity (%) | PPV (%) | NPV (%) | N | |
|---|---|---|---|---|---|
| Inoue (2004) * | 34 | 97 | 71 | 87 | 250 |
| Saifuddin (2005) * | 22 | 90 | 25 | 89 | 73 |
| Present study | 89 | 95 | 80 | 98 | 55 |
N, number of patients; NPV, negative predictive value; PPV, positive predictive value; SAR, superficial abdominal reflex * Abnormal SAR means asymmetry