Abstract
Chiari I malformation is characterized by downward herniation of the cerebellar tonsils
through the foramen magnum. Scant data are available on the clinical course, relationship
to the extent of herniation on magnetic resonance imaging in Chiari I malformation
and the presence of sleep-disordered breathing on polysomnography. Retrospective analysis
was performed looking at polysomnographic findings of children diagnosed with Chiari
I malformation. Details on how Chiari I malformation was diagnosed, brainstem magnetic
resonance imaging findings, and indications for obtaining the polysomnogram in these
patients were reviewed. We also reviewed available data on children who had decompression
surgery followed by postoperative polysomnography findings. Twenty-two children were
identified in our study (11 males, median age 10 years, range 1 to 18). Three had
central sleep apnea, five had obstructive sleep apnea, and one had both obstructive
and central sleep apnea. Children with sleep-disordered breathing had excessive crowding
of the brainstem structures at the foramen magnum and were more likely to have a greater
length of herniation compared with those children without sleep-disordered breathing
(P = 0.046). Patients with central sleep apneas received surgical decompression, and
their conditions were significantly improved on follow-up polysomnography. These data
suggest that imaging parameters may correlate with the presence of sleep-disordered
breathing in children with Chiari I malformation.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Pediatric NeurologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Concerning alterations in the cerebellum resulting from cerebral hydrocephalus. 1891.Pediatr Neurosci. 1987; 13: 3-8
- Review Article: Chiari type I malformation with or without syringomyelia: Prevalence and genetics.J Genetic Counseling. 2003; 12: 297-311
- Evaluation and management of the Chiari malformation type 1 for the primary care pediatrician.Pediatr Clin North Am. 2004; 51: 477-490
- Can Chiari malformation negatively affect higher mental functioning in developmental age?.Neurol Sci. 2011; 32: S307-S309
- Orthostatic intolerance and syncope associated with Chiari type I malformation.J Neurol Neurosurg Psychiatry. 2005; 76: 1034-1036
- The effects of posterior fossa decompressive surgery in adult patients with Chiari malformation and sleep apnea.J Neurosurg. 2010; 112: 800-807
- Chiari malformation and sleep related breathing disorders.J Neurol Neurosurg Psychiatry. 2007; 78: 1344-1348
- Sleep apnea and REM sleep behavior disorder in patients with Chiari malformations.Arq Neuropsiquiatr. 2008; 66: 344-349
- The International Classification of Sleep Disorders Diagnostic and Coding Manual. American Academy of Sleep Medicine, Westchester, IL2005
- Sleep-disordered breathing in children.Chang Gung Med J. 2009; 32: 247-257
- A prospective controlled study of sleep respiratory events in patients with craniovertebral junction malformation.J Neurosurg. 2003; 99: 1004-1009
- Sleep-disordered breathing in Chiari malformation type 1.Pediatr Neurol. 2008; 39: 207-208
- Multifactorial obstructive sleep apnea in a patient with Chiari malformation.J Neurol Sci. 1994; 126: 232-236
- Obstructive sleep apnoea with Arnold-Chiari malformation.Thorax. 1995; 50 (discussion 696-7): 690-691
- Arnold-Chiari malformation presenting as sleep apnea syndrome.Sleep Med. 2000; 1: 139-144
- Sleep apnea and the Chiari I malformation: Case report.Neurosurgery. 1988; 23: 508-510
- A case of Arnold-Chiari Type I malformation presenting with dysrhythmic breathing during sleep.Psychiatry Clin Neurosci. 1998; 52: 212-216
- Nocturnal apnea in Chiari type I malformation.Eur J Pediatr. 2011; 170: 1349-1352
- Secondary nocturnal enuresis caused by central sleep apnea from Chiari malformation type 1.J Pediatr Urol. 2010; 6: 265-269
- Arnold Chiari type 1 malformation presenting with sleep disordered breathing in well children.Arch Dis Child. 2006; 91: 342-343
- Central sleep apnoea in Arnold-Chiari malformation: evidence of pathophysiological heterogeneity.Eur Respir J. 1998; 12: 1482-1485
- Isolated sleep apnea due to Chiari type I malformation and syringomyelia.Pediatr Neurol. 1995; 13: 266-267
- Isolated central sleep apnea in type I Chiari malformation: Improvement after surgery.Pediatr Pulmonol. 2010; 45: 1141-1144
- Chiari type I malformation causing central apnoeas in a 4-month-old boy.Eur J Paediatr Neurol. 2009; 13: 463-465
- [Central sleep apnea syndrome as the first sign of type I Chiari malformation].An Pediatr (Barc). 2008; 68: 277-281
- The natural history of the Chiari Type I anomaly.J Neurosurg Pediatr. 2008; 2: 179-187
- Variance of the position of the cerebellar tonsils with age: preliminary report.Radiology. 1992; 183: 725-728
- Obstructive and central sleep apnoea in Arnold-Chiari malformation: resolution following surgical decompression.Sleep Breath. 2011; 15: 611-613
- Defining the signs and symptoms of Chiari malformation type I with and without syringomyelia.Neurol Res. 2011; 33: 240-246
- Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients.Neurosurgery. 1999; 44: 1005-1017
- Relationship of cine phase-contrast magnetic resonance imaging with outcome after decompression for Chiari I malformations.Neurosurgery. 2006; 59: 140-146
Article info
Publication history
Accepted:
December 17,
2012
Received:
August 22,
2012
Footnotes
Both Umakanth Khatwa, MD and Sriram Ramgopal, MD contributed equally to this manuscript.
Disclosures: Drs. Khatwa, Ramgopal, Mylavarapu, Prabhu, Smith, Pai, Proctor, Scott and Zarowski have nothing to disclose.
Dr. Kothare is funded by the following grants: National Institutes of Health: 1 RC1 HL099749-01 (R21), and RFA-HL-09-001, and Investigator Initiated Grant from Eisai Pharma, Inc, to assess safety and efficacy of rufinamide in children, and the Harvard Catalyst to assess cardiorespiratory abnormalities during seizures in children.
Identification
Copyright
© 2013 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
ScienceDirect
Access this article on ScienceDirectLinked Article
- Response to Losurdo et al.Pediatric NeurologyVol. 49Issue 5
- PreviewWe would like to thank Losurdo et al. for their interest and comments on our article on magnetic resonance imaging findings and sleep apnea in patients with Chiari type 1 malformations,1 in addition to furnishing the relevant literature on sleep studies in Chiari type 1 malformation, which they have so elegantly summarized in their accompanying Table. This table supplies additional information on the topic to the journal's readership.
- Full-Text
- Preview
- What Causes Sleep-Disordered Breathing in Chiari I Malformation? Comment on: “MRI Findings and Sleep Apnea in Children With Chiari I Malformation”Pediatric NeurologyVol. 49Issue 5
- PreviewKhatwa et al.,1 in their article entitled “MRI Findings and Sleep Apnea in Children With Chiari I Malformation,” addressed sleep-disordered breathing (SDB) in a cohort of children with Chiari type I malformation (CM-I), and delineated the relationships between clinical symptoms, polysomnographic findings, and neuroimaging. Moreover, the authors included in their cohort a subset of patients who underwent surgical decompression of the posterior fossa.
- Full-Text
- Preview
