Abstract
Chiari malformation (CM) is primarily characterised by herniation of the cerebellar tonsils through the foramen magnum. Clinically, two main types of CM represent the vast majority of cases: type I (in adults) and type II (in infants). CM may result in neuronal impairment of the brainstem, upper spinal cord and cranial nerves. Part of the afferent and efferet systems and the central respiratory controlling system are located in the cranium-cervical transition and may be damaged in these pathologies, leading to respiratory disorders, such as respiratory failure and death. The ventilatory responses to exogenous and endogenous stimuli, such as responses to hypoxia and hypercapnia, are usually diminished, and apnea may be manifested and detected during sleep, allowing for the diagnosis. This study is a review of the relationship between sleep apnoea and adult CM.
Similar content being viewed by others
References
Aboulezz AO, Sartor K, Geyer CA, Gado MH (1985) Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr 9:1033–1036
Ali MM, Russell N, Awada A, McLean D (1996) A cranio-cervical malformation presenting as acute respiratory failure. J Emerg Med 14:569–572
Alvarez D, Pequena I, Arias M, Valdez L, Pereiro I, De la Torre R (1995) Acute respiratory failure as the first sign of Arniold-Chiari malformation associated with Syringomyelia. Eur Respir J 8:661–663
American Academy of Sleep Medicine (1999) Sleep related breathing disorders in adults: recommendations for syndrome definitions and measurements techniques in clinical research. Sleep 22:667–689
Arcaya J, Cacho J, Del Campo F, Grande J, Maillo A (1993) Arnold Chiari malformation associated with sleep apnea and central dysregulation of arterial pressure. Acta Neurol Scand 88:224–261
Armonda RA, Citrin CM, Foley KT, Ellenbogen RG (1994) Quantitative cinemode magnetic resonance imaging of Chiari I malformations: an analysis of cerebral fluid dynamics. Neurosurgery 35:214–224
Balk R, Hiller C, Lucas EA, Scrima L, Wilson FJ, Wooten V (1985) Sleep apnea and the Arnold-Chiari malformation. Am Rev Respir Dis 132:929–930
Barkowich AJ, Wippold FJ, Sherman JL, Citrin MC (1986) Significance of cerebellar tonsillar position on MRI. Am J Neuroradiol 7:759–799
Batzdorf U (1984) Chiari I malformation with syringomyelia. Evaluation of surgical terapy by magnetic resonance imaging. J Neurosurg 68:726–730
Beijani GK. Adult Chiari malformation. Neurosuurgical focus. Available in URL: http://www.org/education/journal/neurosurgical/july01/11-1-nsf-toc.asp
Birns JW (1988) An anusual form of laryngeal paralysis associated with Arnold-Chiari malformation. Ann Otol Rhinol Laryngol 93:447–451
Bittencourt LRA, Moura SMT, Palombini LO, Botelho RV, Nery LE (1996) Síndrome da apnéia do sono e dor cervical. J Pneumol 22(2):93–94
Bloch S, Van Rensburg MJ, Danziger J (1974) The Arnold-Chiari malformation. Clin Radiol 25:335–341
Blumental DT, Riggs JE (1997) Please don’t call me Arnold-Chiari unless you mean it. Arch Neurol 54(1):16
Bokinsky GE, Hudson LD, Weil JV (1973) Impaired peripheral chemosensitivity and acute respiratory failure in Arnold-Chiari malformation and syringomyelia. N Engl J Med 288:947–948
Botelho RV, Bittencourt LRA, Rotta JM, Nery LE, Tuffik S (1998) Apneia do sono central em paciente com malformação de Chiari tipo I e siringomielia: tratamento com cirurgia descompressiva. Relato de caso. J Bras Neurocir 9(3):111–114
Botelho RV, Bittencourt LRA, Rotta JM, Tufik S (2000) Polysomnographic respiratory findings in patients with Arnold-Chiari type I malformation and Basilar Invagination, with or without syringomyelia: preliminary report of a series of cases. Neurosurg Rev 23(3):151–155
Botelho RV, Bittencourt LRA, Rotta JMR, Tuffik S (2003) A prospective controlled study of sleep respiratory events in patients with craniovertebral junction malformation. J Neurosurg 99:1004–1009
Bullock R, Todd NV, Easton J, Hadley D (1988) Isolated central respiratory failure due3 to syringomyelia and Arnold-Chiari malformation. BMJ 5:1451–1460
Cahan LD, Bentson JR (1982) Considerations in the diagnosis and treatement of syringomyelia and the Chiari malformation. J Neurosurg 57:24–31
Campbell EJM (1965) Respiratory failure. BMJ 1451–1460
Caruana-Montaldo C, Glesson K, Zwillich CW (2000) The control of breathing in clinical practice. Chest 117:205–225
Chartier A, Martinot A, Dhellemmes P, Vallee L, Lamblin MD, Goran C, Leclerc F (2002) Chiari type I malformation in children: presentation in 34 cases. Arch Pediatr 9(8):789–796
Choi SS, Tran LP, Zalal LP (1999) Airway abnormalities in patients with Arnold-Chiari malformations. Otolaryngol Head Neck Surg 121(6):720–724
da Silva JAG, Holanda MMA (2003) Basilar impression, Chiari malformation and syringomyelia. A retrospective study of 53 surgically treated patients. Arch Neuropsiquiatr 61:368–375
Doerty MJ, Spence DP, Young C, Calverley PM (1995) Obstructive sleep apnea with Arnold-Chiari malformation. Thorax 50(6):607–696
Elster AD, Chen MY (1992) Chiari malformations: clinical and radiological reappraisal. Radiology 183:347–353
Ely EW, McCall WV, Haponik EF (1994) Multifatorial obstructive sleep apnea in a patient with Chiari malformation. J Neurol Sci 126(2):232–236
Fanfulla F, Eleotherion D, Patruno V, Bruschi C, Rampulla C (1998) Chronic respiratory failure in a patient with type I Arnold-Chiari malformation (ACM1) and syringomyelia. Monaldi Arch Chest Dis 53(2):138–141
Fish DR, Howard RS, Wiles CM, Simon L (1988) Respiratory arrest: a complication of cerebellar ectopia in adults. J Neurol Neurosurg Psychiatry 51:714–716
Gentry JB, Gonzalez JM, Blacklook JB (2001) Respiratory failure caused by Chiari I malformation with associated syringomyelia. Clin Neurol Neurosurg 103(1):43–45
Goel A, Batjiwale A, Desai K (1998) Basilar invagination: a study of 190 treated patients. J Neurosurg 88:962–968
Guilleminault C, Robinson A (1999) Central sleep apnea. Neurol Clin 14:611–628
Gupta R, Oh U, Spessot AL, Anderson RCE, Kaiser MG, Fitzsimmons BFM (2003) Resolution of Ondine’s curse after subocciptal decompression in a 72-year-old woman. Neurology 61(2):275–276
Halpin DM, Trend P, Symon L, Harding AE (1990) Type 1 Arnold-Chiari malformation in a 77 year old woman. J Neurol Neurosurg Psychiatry 53(1):88–89
Haouzi P, Marchal J, Allioui EM, Hannahart B, Chalon B, Braun M (2000) Corticospinal pathway and exercise hyperpnea. Lessons from a patient with Arnold-Chiari malformation. Respir Physiol 123(1–2):13–22
Hershberger ML, Chidekel A (2003) Arnold-Chiari malformation type I and sleep-disordered breathing: an uncommon manifestation of an important pediatric problem. J Pediatr Health Care 17(4):190–197
Hollinger PC, Holinger LD, Reichart TJ, Holliger PH (1978) Respiratory obstruction and apnea in infants with bilateral abductor vocal cord paralysis, meningocele, hydrocephalus and Arnold-Chiari malformation. J Pediatr 92:368–373
Ishikawa M, Kikuchi H, Fujisawa I, Yonekawa Y (1988) Tonsillar herniation on magnetic resonance imaging. Neurosurgery 22:77–81
Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ (1998) The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 89:212–216
Juan G, Ramon M, Ciscar MA, Garcia B, Lioret T, Cervello MA, Marin J (1999) Acute respiratory insufficiency as initial manifestation of brain stem lesions. Arch Bronchopneumol 35(11):560–563
Kirsch WM, Duncan BR, Black FO, Stears JC (1968) Laryngeal palsy in association with myelomeningocele, hydrocephalus and the Arnold-Chiari malformation. J Neurosurg 28(3):207–214
Lam B, Ryan CF (2000) Arnold-Chiari malformation presenting as sleep apnea syndrome. Sleep Med 1(2):139–144
Lee JH, Chung CK, Kim HJ (2001) Cerebral dissection from syringomyelia demonstrated using cine magnetic resonance imaging. Case report. J Neurosurg 94(2):318–321
Levitt P, Cohn MA (1988) Sleep apnea and the Arnold-Chiari malformation: case report. Neurosurgery 23(4):508–510
Linder A, Lindholm CE (1997) Laryngologic management of infants with the Chiari II syndrome. Int J Pediatr Otorhinolaryngol 39(3):187–197
List CF (1941) Neurologic syndromes accompanying developmental anomalies of occiptal bone, atlas and axis. Arch Neurol 45:577–616
Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50(1):29–55
Marin-Padilla M (1991) Cephalic axial skeletal-neural dysraphic disorders: embryology and pathology. Can J Neurol Sci 18:153–169
Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BS (2000) Asymptomatic Chiari type I malformation identified on magnetic resonance imaging. J Neurosurg 92:920–926
Mikulis DJ, Diaz O, Egglin TK, Sanchez R (1992) Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:725–728
Milhorat TH, Chow MW, Trinidad E, Kula RW, Mandell M, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44(5):1005–1017
Miyamoto M, Miyamoto T, Hirata K, Katayama S (1998) A case of Arnold-Chiari type I malformation presenting with dysrhythmic breathing during sleep. Psychiatr Clin Neurosci 52:212–216
Montserrat JM, Picado C, Agusti-Vidal A (1988) Arnold-Chiari malformation and paralysis of the diaphragm. Respiration 53:128–131
Moro ERP, Teive HAG, de Souza SMP, Lambrecht F, Werneck LC (1999) Malformação de Chiari tipo I. Relato de dois casos com apresentações clínicas pouco usuais. Arq Neuro-psiquiatr 57(3-A):666–671
Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86(1):40–47
Nogués MA, Gené R, Encabo H (1992) Risk of sudden death during sleep in syringomyelia and syringobulbia. J Neurol Neurosurg Psychiatry 55:585–589
Nogues MA, Roncoroni AJ, Benarroch E (2002) Breathing control in neurological diseases. Clin Auton Res 12:440–449
Noseda A, Devriendt J, Hoffmann G, Schmerber J (1996) Insuffisance respiratoire isolee sur malformatio d’Arnold-Chiari. Ver Mal Respir 13:440–442
O’Connor S, du Boulay G, Logue V (1973) The normal position of the cerebellar tonsils as demonstrated by myelography. J Neurosurg 39(3):387–389
Omer S, al-Kawi MZ, Bohlega SS, Bouchama A, Mclean D (1996) Respiratory arrest: a complication of Arnold-Chiari malformation in adults. Eur Neurol 36:36–38
Paul KS, Lye RH, Strang FA, Dutton J (1983) Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 58:183–187
Pillay PK, Awad IA, Little JR, Hahn JF (1991) Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery 28(5):639–645
Reybet-Degat O (2001) Pathology of craniocervical junction and sleep disorders. Rev Neurol 157:S156–S160
Rodman T, Resnik ME, Berkowitx RD, Fenelly JF, Olivia J (1962) Alveolar hypoventilation due to involvement of the respiratory center by obscure disease of the central nervous system. Am J Med 32:208–217
Ruff ME, Oakes WJ, Fisher S, Spock A (1987) Sleep apnea and vocal cord paralysis secondary to type I Chiari malformation. Pediatrics 80(2):231–234
Sahuquillo J, Rubio E, Poça M, Rovira A, Rodriguez-Baeza A, Cervera C (1994) Posterior fossa reconstruction: a surgical technique for the treatment of Chiari I-Syringomyelia complex. Preliminary results and magnetic resonance imaging quantitative assessment. Neurosurgery 35(5):874–884
Shiihara T, Shimizu Y, Mitsui T, Saitoh E, Sato S (1995) Isolated sleep apnea due to Chiari type I malformation and syringomyelia. Pediatr Neurol 13(3):266–267
Smoker WRK (1994) Craniovertebral junction. Normal anatomy, craniometry and congenital anomalies. Radiographics 14:255–277
Tsao TC, Juang YC, Chiang YC, Tsay YH, Lan RS, Lee CH (1991) Pneumonia preceding respiratory failure. A rare misleading clinical manifestation in adult Arnold-Chiari malformation. Chest 99(5):1294–1295
Tubbs RS, Elton S, Grabb P, Dockery SE, Bartolucci AA, Oakes WJ (2001) Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery 48:1050–1055
Wolf DA, Veasey SP III, Wilson SK, Adame J, Korndorffer WE (1998) Death following minor head trauma in two adult individuals with the Chiari I deformity. J Forens Sci 43(6):1241–1243
Yoshimi A, Nomura K, Furune S (2002) Sleep apnea syndrome associated with a type I Chiari malformation. Brain Dev 24(1):49–51
Zager EL, Ojemann RG, Poletti CE (1990) Acute presentations of syringomyelia. Report of three cases. J Neurosurg 72:133–138
Zolty P, Sanders MH, Pollack IF (2000) Chiari Malformation and sleep-disordered breathing: a review of diagnostic and management issues. Sleep 23(5):637–643
Author information
Authors and Affiliations
Corresponding author
Additional information
A commentary on this paper is available at http://dx.doi.org/10.1007/s10143-005-0401-x
Rights and permissions
About this article
Cite this article
Botelho, R.V., Bittencourt, L.R.A., Rotta, J.M. et al. Adult Chiari malformation and sleep apnoea. Neurosurg Rev 28, 169–176 (2005). https://doi.org/10.1007/s10143-005-0400-y
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10143-005-0400-y