Abstract
Background
Scoliosis is a common finding in children with Chiari I malformation and syringomyelia. The development of scoliosis associated with a Chiari malformation typically has been ascribed to the presence of syringomyelia. Decompression of the hindbrain hernia will often alleviate the syringomyelia and, in many cases, stabilize or reverse the scoliosis.
Case report
We report a child with Chiari I malformation and scoliosis who presented with Valsalva-induced headache/neck pain. MRI revealed no syringomyelia or hydrocephalus. Radiographs revealed that her scoliotic curvature was approximately 13° and was a single levoscoliotic curve. This patient underwent a posterior fossa decompression with duraplasty for her Valsalva-induced pain. Postoperatively, she had resolution of her pain and there has been no progression of her scoliosis at 3 years follow-up.
Prognosis
Intriguingly, and scattered throughout the medical literature, many have noted cases of scoliosis in patients with only a Chiari I malformation and no syringomyelia. Moreover, experimental studies have induced scoliosis in animals after compression of the dorsal columns. After a review of the medical literature regarding a potential cause and effect of herniated hindbrain-induced scoliosis in the absence of syringomyelia, this association although rare, does seem plausible.
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References
Alexander MA, Bunch WH, Ebbeson SOE (1972) Can experimental dorsal rhizotomy produce scoliosis? J Bone Joint Surg 54A:1509–1513
Alzate JC, Kothbauer KF, Jallo GI et al (2001) Treatment of Chiari type I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurgical Focus 11:1–5
Arai S, Ohsuka Y, Moriya H et al (1993) Scoliosis associated with syringomyelia. Spine 18:1591–1592
Barrack RL, Whitecloud TS, Burke SW et al (1984) Proprioception in idiopathic scoliosis. Spine 9:681–685
Brockmeyer D, Gollogly S, Smith JT (2003) Scoliosis associated with Chiari I malformations: The effect of suboccipital decompression on scoliosis curve progression. A preliminary study. Spine 28:2505–2509
Cheng JCY, Guo X, Sher AHL et al (1999) Correlation between curve severity, somatosensory evoked potentials, and magnetic resonance imaging in adolescent idiopathic scoliosis. Spine 24:1679–1684
Dure LS, Percy AK, Cheek WR et al (1989) Chiari type I malformation in children. J Pediatrics 115:573–576
Dyste GN, Menezes AH, BVanGilder JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159–168
Farley FA, Puryear A, Hall JM et al (2002) Curve progression in scoliosis associated with chiari I malformation following suboccipital decompression. J Spinal Disorders Tech 15:410–414
Farley FA, Song KM, Birch JG et al (1995) Syringomyelia and scoliosis in children. J Pediatr Ortho 15:187–192
Fischbein NJ, Dillon WP, Cobbs C et al (1999) The “presyrinx” state: a reversible myelopathic condition that may precede syringomyelia. AJNR Am J Neuroradiol 20:7–20
Flynn JM, Sodha S, Lou JE et al (2004) Predictors of progression of scoliosis after decompression of an Arnold-Chiari I malformation. Spine 29:286–292
Ghanem IB, Londono C, Delalande O et al (1997) Chiari I malformation associated with syringomyelia and scoliosis. Spine 22:1313–1317
Glassman SD, Nazar GB, Dimar JR et al (1994) Neurogenic scoliosis in children. KMA Journal 92:19–24
Gupta R, Sharma R, Vashisht S et al (1999) Magnetic resonance evaluation of idiopathic scoliosis: a prospective study. Australas Radiol 43:461–465
Hida K, Iwasaki Y, Koyanagi I et al (1999) Pediatric syringomyelia with Chiari malformation: its clinical characteristics and surgical outcomes. Surg Neurol 51:383–391
Jacob P, Rhoton AL (1997) The Chiari I Malformation in Anson JA, Benzel EC, Awad IA (eds) Syringomyelia and the Chiari Malformations. Park Ridge, Illinois, American Association of Neurological Surgeons, pp 57–67
Kontio K, Davidson D, Letts M (2002) Management of scoliosis and syringomyelia in children. J Pediatr Ortho 22:771–779
Lewonowski K, King JD, Nelson MD (1992) Routine use of magnetic resonance imaging in idiopathic scoliosis patients less than eleven years of age. Spine 17:109–116
Lloyd-Roberts GC, Pincott JR, MacMeniman P et al (1978) Progression in idiopathic scoliosis: a preliminary report of a possible mechanism. J Bone Joint Surg 60:451–460
MacEwen GD (1973) Experimental scoliosis. Clin Orthop 93:69–74
Muhonen MG, Menezes AH, Swain PD et al (1992) Scoliosis in pediatric Chiari malformations without myelodysplasia. J Neurosurg 77:69–77
Nagib MG (1994) An approach to symptomatic children (ages 4–14 years) with chiari type I malformation. Pediatr Neurosurg 21:31–35
Nohria V, Oakes WJ (1990–1991) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:222–227
Nokes SR, Murtagh FR, Jones JD et al (1987) Childhood scoliosis: mr imaging. Radiology 164:791–797
Park JK, Gleason PL, Madsen JR et al (1997) Presentation and management of Chiari I malformation in children. Pediatr Neurosurg 26:190–196
Pincott JR, Feview JS, Tafts LF (1984) Scoliosis caused by section of dorsal spinal nerve roots. J Bone Joint Surg 66:27–29
Piper JG, Menezes AH (1996) Chiari Malformation in the Adult in Menezes AH and Sonntag VKH (eds) Principles of Spinal Surgery New York, McGraw-Hill pp 379–382
Sahlstrand T, Orrtengreen R, Nachemson A(1978) Postural equilibrium in adolescent idiopathic scoliosis. Acta Orthop Scand 49:354–365
Spiegel DA, Flynn JM, Stasikelis PJ et al (2003) Scoliotic curve patterns in patients with Chiari I malformation and/or syringomyelia. Spine 28:2139–2146
Suk SI, Song HS, Lee CK (1989) Scoliosis induced by anterior and posterior rhizotomy. Spine 14:692–697
Tubbs RS, Wellons JC, III, Oakes WJ (2002) Asymmetry of tonsillar ectopia in Chiari I malformation. Pediatr Neurosurg 37:199–202
Willimas B (1979) Orthopaedic features in the presentation of syringomyelia. J Bone Joint Surg 61:314–323
Winter RB, Lonstein JE, Denis F et al (1991) Prevalence of spinal canal or cord abnormalities in idiopathic, congenital, and neuromuscular scoliosis. Presented at the Scoliosis Research Society Meeting, Minneapolis, MN
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Tubbs, R.S., Doyle, S., Conklin, M. et al. Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature. Childs Nerv Syst 22, 1351–1354 (2006). https://doi.org/10.1007/s00381-006-0079-6
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DOI: https://doi.org/10.1007/s00381-006-0079-6