Management of Sleep Apnea in Children with Chiari I Malformation: A Retrospective Study

Introduction: The literature indicates that decompression of Chiari I malformations (CM-1) may resolve symptoms of sleep apnea. This study aims to identify the incidence of obstructive sleep apnea (OSA), central sleep apnea (CSA), and mixed sleep apnea in a cohort of pediatric CM-1 patients treated at our institution. We also assessed apnea-hypopnea index and symptomatology before and after surgery to investigate if Chiari decompression is a viable treatment for sleep apnea in CM-1 patients. Improvement relative to ENT surgical intervention was also considered. Methods: We identified 75 patients who underwent polysomnography (PSG) from our database of 465 CM-1 patients. Sleep apnea diagnosis was based on the sleep physician’s overall interpretation of the PSG. Symptomatology pre- and post-surgery was analyzed. Results: Of the 75 CM-1 patients that underwent PSG, 23 were diagnosed with sleep apnea. Sixteen had OSA, 6 had CSA, and 1 had mixed apnea. Twelve OSA patients received ENT intervention. Eight improved and 2 further improved after Chiari decompression. Of the 4 patients that did not improve, one of those later improved following Chiari decompression. Of the 6 CSA patients, 2 underwent Chiari decompression, but only one improved. The mixed apnea patient underwent several ENT interventions that did not relieve symptoms but improved following Chiari decompression. Discussion/Conclusions: Based on our results, sleep apnea in CM-1 patients may be obstructive, central, or mixed and is likely multifactorial. A multidisciplinary approach to the management of these patients is important, including neurosurgery, otolaryngology, and sleep medicine. Future prospective studies will lend further insight into this condition and its management.

[1]  T. Harkey,et al.  Natural history of Chiari I malformation in children: a retrospective analysis , 2020, Child's Nervous System.

[2]  R. Cavallazzi,et al.  Sleep Disordered Breathing and Magnetic Resonance Imaging Findings in Children With Chiari Malformation Type I. , 2017, Pediatric neurology.

[3]  M. Poca,et al.  Sleep-Related Breathing Disorders in Chiari Malformation Type 1: A Prospective Study of 90 Patients , 2017, Sleep.

[4]  L. Sacchetto,et al.  Evaluation of the central sleep apnea in asymptomatic children with Chiari 1 malformation: an open question , 2017, Child's Nervous System.

[5]  T. G. García,et al.  Reversible central sleep events in type I Chiari malformation , 2016 .

[6]  Pearl L. Yu,et al.  Surgical History of Sleep Apnea in Pediatric Patients with Chiari Type 1 Malformation. , 2015, Neurosurgery clinics of North America.

[7]  Ichiro Komada,et al.  Type I Chiari malformation presenting central sleep apnea. , 2014, Auris, nasus, larynx.

[8]  N. Wetjen,et al.  The role of nocturnal polysomnography in assessing children with Chiari type I malformation , 2013, Clinical Neurology and Neurosurgery.

[9]  E. Scarano,et al.  Sleep disordered breathing in children and adolescents with Chiari malformation type I. , 2013, Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine.

[10]  S. Prabhu,et al.  MRI findings and sleep apnea in children with Chiari I malformation. , 2013, Pediatric neurology.

[11]  R. Campisi,et al.  Type I Arnold-Chiari malformation with bronchiectasis, respiratory failure, and sleep disordered breathing: a case report , 2013, Multidisciplinary Respiratory Medicine.

[12]  C. Hukins,et al.  Obstructive and central sleep apnoea in Arnold–Chiari malformation: resolution following surgical decompression , 2011, Sleep and Breathing.

[13]  Rene van Gent,et al.  Nocturnal apnea in Chiari type I malformation , 2011, European Journal of Pediatrics.

[14]  H. Pasterkamp,et al.  Isolated central sleep apnea in type I Chiari malformation: Improvement after surgery , 2010, Pediatric pulmonology.

[15]  S. Tufik,et al.  The effects of posterior fossa decompressive surgery in adult patients with Chiari malformation and sleep apnea. , 2010, Journal of neurosurgery.

[16]  L. Servais,et al.  Chiari type I malformation causing central apnoeas in a 4-month-old boy. , 2009, European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.

[17]  J. Gosalakkal.  Sleep-disordered breathing in Chiari malformation type 1. , 2008, Pediatric neurology.

[18]  Michael J. Lyerly,et al.  The pediatric Chiari I malformation: a review , 2007, Child's Nervous System.

[19]  A. Schwartz,et al.  Neuromechanical control of upper airway patency during sleep. , 2007, Journal of applied physiology.

[20]  D. Fitzgerald,et al.  Arnold Chiari type 1 malformation presenting with sleep disordered breathing in well children , 2006, Archives of Disease in Childhood.

[21]  I. Svab,et al.  Sleep-disordered breathing in patients with Chiari malformation: Improvement after surgery , 2006, Neurology.

[22]  A. Kazis,et al.  Acute respiratory failure and sleep-disordered breathing in Arnold–Chiari malformation , 2005, Clinical Neurology and Neurosurgery.

[23]  A. Chidekel,et al.  Arnold-Chiari malformation type I and sleep-disordered breathing: an uncommon manifestation of an important pediatric problem. , 2003, Journal of pediatric health care : official publication of National Association of Pediatric Nurse Associates & Practitioners.

[24]  A. Yoshimi,et al.  Sleep apnea syndrome associated with a type I Chiari malformation , 2002, Brain and Development.

[25]  S. Tufik,et al.  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 , 2000, Neurosurgical Review.

[26]  I. Pollack,et al.  Chiari malformation and sleep-disordered breathing: a review of diagnostic and management issues. , 2000, Sleep.

[27]  J. Artieda,et al.  Chiari Type I Malformation, Glossopharyngeal Neuralgia and Central Sleep Apn'oea in a Child , 1996, Developmental medicine and child neurology.

[28]  S. Sato,et al.  Isolated sleep apnea due to Chiari type I malformation and syringomyelia. , 1995, Pediatric neurology.

[29]  J. Bodensteiner,et al.  Hypersomnolence and Pure Central Sleep Apnea Associated With the Chiari I Malformation , 1995, Journal of child neurology.

[30]  J. Grande,et al.  Arnold‐Chiari malformation associated with sleep apnea and central dysregulation of arterial pressure , 1993, Acta neurologica Scandinavica.

[31]  P. Levitt,et al.  Sleep apnea and the Chiari I malformation: case report. , 1988, Neurosurgery.

[32]  W. J. Oakes,et al.  Sleep apnea and vocal cord paralysis secondary to type I Chiari malformation. , 1987, Pediatrics.

[33]  R. Balk,et al.  Sleep apnea and the Arnold-Chiari malformation. , 1985, The American review of respiratory disease.

[34]  E. Esteller,et al.  Obstructive Sleep Apnea-Hypopnea Syndrome in Children: Beyond Adenotonsillar Hypertrophy , 2015 .

[35]  P. Adimi,et al.  Sleep-Disordered Breathing as Presenting Manifestation of Chiari Type I Malformation: A Case Report , 2014, Tanaffos.

[36]  M. Poca,et al.  Sleep disturbance: a forgotten syndrome in patients with Chiari I malformation. , 2014, Neurologia.

[37]  J. L. Peña Segura,et al.  [Central sleep apnea syndrome as the first sign of type I Chiari malformation]. , 2008, Anales de pediatria.

[38]  R. Gerardi,et al.  Sleep apneas, convulsive syncopes and autonomic impairment in type I Arnold-Chiari malformation. , 1991, European neurology.

[39]  F. Abroug,et al.  [Sleep apnea syndromes and Arnold-Chiari malformation]. , 1990, Revue des maladies respiratoires.

[40]  J. Haze,et al.  Obstructive sleep apnea. , 1987, Cranio : the journal of craniomandibular practice.