Spinal neurological complications of achondroplasia. Results of surgical treatment.
Spinal neurological complications caused the admission of 17 patients with achondroplasia to the UCLA affiliated hospitals between 1955 and 1979. These patients constituted 41% of all achondroplastic patients admitted during that period. The spinal stenotic syndromes could be divided into three groups: Group I: thoracolumbar stenosis (10 patients); Group II: foramen magnum and upper cervical stenosis (five patients); and Group III: generalized spinal stenosis (two patients). Eleven patients underwent a total of 18 decompressive operative procedures for treatment of paraparesis, quadriparesis, sensory deficits, and sphincter dysfunction. Excellent results were obtained with patients in Group I and II, 77% of whom were ambulatory and continent postoperatively. Two patients in Group III fared less well, showing steady neurological deterioration despite multiple operative procedures. The spectrum of spinal neurological manifestations secondary to achondroplasia is reviewed. Problems with conventional radiological studies and the potential role of computerized tomographic analysis of such patients are discussed. Recommendations for surgical technique are made. Early recognition, prompt clinical evaluation, and safe and accurate radiological analysis of spinal neurological complications of achondroplasia will allow appropriate decompressive surgical procedures to be performed. Excellent results may be anticipated in the reversal and prevention of neurological deficit secondary to achondroplasia with such an approach.
D000130:Achondroplasia / Q000150:complications* / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000368:Aged; D002574:Cervical Vertebrae; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D005539:Foramen Magnum; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007403:Intervertebral Disc / Q000002:abnormalities; D008159:Lumbar Vertebrae; D008297:Male; D008875:Middle Aged; D009408:Nerve Compression Syndromes / Q000209:etiology* / Q000601:surgery; D013117:Spinal Cord Compression / Q000209:etiology* / Q000601:surgery; D013122:Spinal Diseases / Q000150:complications*; D013126:Spinal Nerve Roots; D013577:Syndrome; D013904:Thoracic Vertebrae