Abstract
Background
With the advent of MRI, the diagnosis of Chiari I malformation is being made in younger patients, often with minimal or no neurologic symptoms.
Clinical features
The symptoms and signs of a Chiari I malformation may be related to the compression of neural and/or dural structures at the cranio-cervical junction or may be related to the associated syringomyelia. Scoliosis is a particularly important and common finding in those children with syringomyelia.
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References
Albers FW, Ingels KJ (1993) Otoneurological manifestations in Chiari-I malformation. J Laryngol Otol 107:441–443
Anwer UE, Fisher M (1996) Acute and atypical presentations of syringomyelia. Eur Neurol 36:215–218
Bronstein AM, Miller DH, Rudge P, Kendall BE (1987) Down beating nystagmus: magnetic resonance imaging and neuro-otological findings. J Neurol Sci 81:173–184
Cahan LD, Bentson JR (1982) Considerations in the diagnosis and treatment of syringomyelia and the Chiari malformation. J Neurosurg 57:24–31
Chopra JS, Sawhney IM, Kak VK, Khosla VK (1988) Craniovertebral anomalies: a study of 82 cases. Br J Neurosurg 2:455–464
Combarros O, Alvarez de Arcaya A, Berciano J (1998) Isolated unilateral hypoglossal nerve palsy: nine cases. J Neurol 245:98–100
Da Silva JA (1992) Basilar impression and Arnold-Chiari malformation. Surgical findings in 209 cases. Neurochirurgia 35:189–195
Dauser RC, DiPietro MA, Venes JL (1988) Symptomatic Chiari I malformation in childhood: a report of 7 cases. Pediatr Neurosci 14:184–190
Dyste GN, Menezes AH (1988) Presentation and management of pediatric Chiari malformations without myelodysplasia. Neurosurgery 23:589–597
Dyste GN, Menezes AH, VanGilder JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159–168
Emery E, Redondo A, Rey A (1997) Syringomyelia and Arnold Chiari in scoliosis initially classified as idiopathic: experience with 25 patients. Eur Spine J 6:158–162
Gingold SI, Winfield JA (1991) Oscillopsia and primary cerebellar ectopia: case report and review of the literature. Neurosurgery 29:932–936
Hampton F, Williams B, Loizou LA (1982) Syncope as a presenting feature of hindbrain herniation with syringomyelia. J Neurol Neurosurg Psychiatry 45:919–922
Hanieh A, Sutherland A, Foster B, Cundy P (2000) Syringomyelia in children with primary scoliosis. Childs Nerv Syst 16:200–202
Hida K, Iwasaki Y, Koyanagi I, Abe H (1999) Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes. Surg Neurol 51:383–390; discussion 390–391
Hoffman HJ, Neill J, Crone KR, Hendrick EB, Humphreys RP (1987) Hydrosyringomyelia and its management in childhood. Neurosurgery 21:347–351
Levy WJ, Mason L, Hahn JF (1983) Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery 12:377–390
Listernick R, Tomita T (1991) Persistent crying in infancy as a presentation of Chiari type I malformation. J Pediatr 118:567–569
Loft LM, Ward RF (1992) Hiccups. A case presentation and etiologic review. Arch Otolaryngol Head Neck Surg 118:1115–1119
Menezes AH (1991) Chiari I malformations and hydromyelia—complications. Pediatr Neurosurg 17:146–154
Menezes AH (1995) Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis. Pediatr Neurosurg 23:260–269
Nohria V, Oakes WJ (1990) Chiari I malformation: a review of 43 patients. Pediatr Neurosurg 16:222–227
Palma V, Sinisi L, Andreone V, Fazio N, Serra LL, Ambrosio G, De Michele G (1993) Hindbrain hernia headache and syncope in type I Arnold-Chiari malformation. Acta Neurol 15:457–461
Pascual J, Oterino A, Berciano J (1992) Headache in type I Chiari malformation. Neurology 42:1519–1521
Pedersen RA, Troost BT, Abel LA, Zorub D (1980) Intermittent downbeat nystagmus and oscillopsia reversed by suboccipital craniectomy. Neurology 30:1239–1242
Ramirez N, Johnston CE, Browne RH (1997) The prevalence of back pain in children who have idiopathic scoliosis. J Bone Joint Surg 79:364–368
Rekate HL, Nadkarni TD, Teaford PA, Wallace D (1999) Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and conjecture as to mechanism. Neurosurgery 45:386–391
Ruff ME, Oakes WJ, Fisher SR, Spock A (1987) Sleep apnea and vocal cord paralysis secondary to type I Chiari malformation. Pediatrics 80:231–234
Saez RJ, Onofrio BM, Yanagihara T (1976) Experience with Arnold-Chiari malformation, 1960 to 1970. J Neurosurg 45:416–422
Schlesinger EB, Antunes JL, Michelsen WJ, Louis KM (1981) Hydromyelia: clinical presentation and comparison of modalities of treatment. Neurosurgery 9:356–365
Sclafani AP, DeDio RM, Hendrix RA (1991) The Chiari-I malformation. Ear Nose Throat J 70:208–212
Selmi F, Davies KG, Weeks RD (1995) Type I Chiari deformity presenting with profound sinus bradycardia: case report and literature review. Br J Neurosurg 9:543–545
Wisoff JH, Epstein F (1989) Management of hydromyelia. Neurosurgery 25:562–571
Yeow YK, Tjia TL (1989) The localizing value of downbeat nystagmus. Singapore Med J 30:273–276
Yoshimi A, Nomura K, Furune S (2002) Sleep apnea syndrome associated with a type I Chiari malformation. Brain Dev 24:49–51
Zadeh HG, Sakka SA, Powell MP, Mehta MH (1995) Absent superficial abdominal reflexes in children with scoliosis. An early indicator of syringomyelia. J Bone Joint Surg Br 77:762–767
Zager EL, Ojemann RG, Poletti CE (1990) Acute presentations of syringomyelia. Report of three cases. J Neurosurg 72:133–138
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Steinbok, P. Clinical features of Chiari I malformations. Childs Nerv Syst 20, 329–331 (2004). https://doi.org/10.1007/s00381-003-0879-x
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DOI: https://doi.org/10.1007/s00381-003-0879-x