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Chapter 20 - Respiratory Issues in Children with Neurological Disorders

Published online by Cambridge University Press:  26 May 2025

Martin Groß
Affiliation:
MEDIAN Clinic Bad Tennstedt
Eelco F. M. Wijdicks
Affiliation:
Mayo Clinic
Maxwell S. Damian
Affiliation:
Basildon University Hospitals
Oliver Summ
Affiliation:
Evangelisches Krankenhaus Oldenburg
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Summary

Neurological disorders in children encompass a heterogeneous group of conditions, congenital or acquired, that are often associated with significant respiratory morbidity. In considering the impact on the respiratory system that are relevant in children with neurological disorders, the phenotypes can be broadly classified into (1) impaired respiratory drive; (2) loss of airway protection/patency; (3) neuromuscular weakness; and (4) reduced or elevated muscle tone. These are not mutually exclusive. In this chapter, the authors review the pathophysiology of respiratory impairment in children with neurological disorders, in the context of childhood development, and discuss the principles behind long-term management of respiratory issues, including monitoring and therapies available to optimize respiratory function. Acute management strategies, including in the pediatric intensive care unit are discussed, alongside the ethical dilemmas faced by clinicians treating this patient population. The lack of high-quality evidence makes management of respiratory issues in children with severe neurological impairments challenging.

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Publisher: Cambridge University Press
Print publication year: 2025

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References

Mahon, M, Kibirige, MS. Patterns of admissions for children with special needs to the paediatric assessment unit. Arch Dis Child. 2004 Feb;89(2):165–9.CrossRefGoogle Scholar
Plioplys, AV, Kasnicka, I, Lewis, S, Moller, D. Survival rates among children with severe neurologic disabilities. South Med J. 1998 Feb;91(2):161–72.CrossRefGoogle ScholarPubMed
Hollins, S, Attard, MT, von Fraunhofer, N, McGuigan, S, Sedgwick, P. Mortality in people with learning disability: risks, causes, and death certification findings in London. Dev Med Child Neurol. 1998 Jan;40(1):50–6.Google Scholar
Stocks, J, Hislop, A, Sonnappa, S. Early lung development: lifelong effect on respiratory health and disease. Lancet Respir Med. 2013 Nov;1(9):728–42.CrossRefGoogle ScholarPubMed
Burri, PH. Structural aspects of postnatal lung development: alveolar formation and growth. Biol Neonate. 2006;89(4):313–22.CrossRefGoogle ScholarPubMed
Narayanan, M, Owers-Bradley, J, Beardsmore, CS, et al. Alveolarization continues during childhood and adolescence: new evidence from helium-3 magnetic resonance. Am J Respir Crit Care Med. 2012 Jan 15;185(2):186–91.CrossRefGoogle ScholarPubMed
Gaultier, C. Respiratory muscle function in infants. Eur Respir J. 1995 Jan;8(1):150–3.CrossRefGoogle ScholarPubMed
Dassios, T, Vervenioti, A, Dimitriou, G. Respiratory muscle function in the newborn: a narrative review. Pediatr Res. 2022 Mar;91(4):795803.CrossRefGoogle ScholarPubMed
Blanco, CE. Maturation of fetal breathing activity. Biol Neonate. 1994;65(3–4):182–8.CrossRefGoogle ScholarPubMed
Estrellado-Cruz, WL, Beckerman, RC. Control of ventilation and apnea. In: Goldsmith, JP, Karotkin, EH, eds. Assisted Ventilation of the Neonate. ScienceDirect; 2011:4770.CrossRefGoogle Scholar
Huppi, P, Sizonenko, S, Amato, M. Lung disease and brain development. Biol Neonate. 2006;89(4):284–97.CrossRefGoogle ScholarPubMed
Newman, CJ, O’Regan, M, Hensey, O. Sleep disorders in children with cerebral palsy. Dev Med Child Neurol. 2006 Jul;48(7):564–8.CrossRefGoogle ScholarPubMed
McLaren, AT, Bin-Hasan, S, Narang, I. Diagnosis, management and pathophysiology of central sleep apnea in children. Paediatr Respir Rev. 2019 Apr;30:4957.Google ScholarPubMed
Szuhay, G, Rotenberg, J. Sleep apnea in pediatric neurological conditions. Curr Neurol Neurosci Rep. 2009 Mar;9(2):145–52.CrossRefGoogle ScholarPubMed
Tesoro, S, Robertis, EDE, Marturano, F, Van Den Hout, HJ, De Graaff, JC. Anesthesiological approach to the floppy child. Minerva Anestesiol. 2021 Aug;87(8):940–9.Google Scholar
McCrea, N, O’Donnell, R, Brown, R. Outpatient respiratory management of the child with severe neurological impairment. Arch Dis Child Educ Pract Ed. 2013 Jun;98(3):8491.CrossRefGoogle ScholarPubMed
Wilkinson, DJ, Baikie, G, Berkowitz, RG, Reddihough, DS. Awake upper airway obstruction in children with spastic quadriplegic cerebral palsy. J Paediatr Child Health. 2006 Jan–Feb;42(1-2):44–8.CrossRefGoogle ScholarPubMed
Arvedson, J, Rogers, B, Buck, G, Smart, P, Msall, M. Silent aspiration prominent in children with dysphagia. Int J Pediatr Otorhinolaryngol. 1994 Jan;28(2–3):173–81.CrossRefGoogle ScholarPubMed
Abrahams, P, Burkitt, BF. Hiatus hernia and gastro-oesophageal reflux in children and adolescents with cerebral palsy. Aust Paediatr J. 1970 Mar;6(1):41–6.Google ScholarPubMed
Casas, MJ, Kenny, DJ, McPherson, KA. Swallowing/ventilation interactions during oral swallow in normal children and children with cerebral palsy. Dysphagia. 1994 Winter;9(1):40–6.CrossRefGoogle ScholarPubMed
Arvedson, JC. Feeding children with cerebral palsy and swallowing difficulties. Eur J Clin Nutr. 2013 Dec;67(suppl 2):S912.CrossRefGoogle ScholarPubMed
Morton, RE, Wheatley, R, Minford, J. Respiratory tract infections due to direct and reflux aspiration in children with severe neurodisability. Dev Med Child Neurol. 1999 May;41(5):329–34.CrossRefGoogle ScholarPubMed
Marpole, R, Blackmore, AM, Gibson, N, Cooper, MS, Langdon, K, Wilson, AC. Evaluation and management of respiratory illness in children with cerebral palsy. Front Pediatr. 2020 Jun 24;8:333.CrossRefGoogle Scholar
Millman, AJ, Finelli, L, Bramley, AM, et al. Community-acquired pneumonia hospitalization among children with neurologic disorders. J Pediatr. 2016 Jun;173:188–95.e4.CrossRefGoogle ScholarPubMed
Ranucci, G, Buccigrossi, V, de Freitas, MB, Guarino, A, Giannattasio, A. Early-life intestine microbiota and lung health in children. J Immunol Res. 2017;2017:8450496.CrossRefGoogle ScholarPubMed
Seddon, PC, Khan, Y. Respiratory problems in children with neurological impairment. Arch Dis Child. 2003 Jan;88(1):75–8.CrossRefGoogle ScholarPubMed
Yang, S, Andras, LM, Redding, GJ, Skaggs, DL. Early-onset scoliosis: a review of history, current treatment, and future directions. Pediatrics. 2016 Jan;137(1):e20150709.CrossRefGoogle ScholarPubMed
Faria, J, Harb, J, Hilton, A, Yacobucci, D, Pizzuto, M. Salivary botulinum toxin injection may reduce aspiration pneumonia in neurologically impaired children. Int J Pediatr Otorhinolaryngol. 2015 Dec;79(12):2124–8.CrossRefGoogle ScholarPubMed
Blakeman, TC, Scott, JB, Yoder, MA, Capellari, E, Strickland, SL. AARC clinical practice guidelines: artificial airway suctioning. Respir Care. 2022 Feb;67(2):258–71.CrossRefGoogle ScholarPubMed
Fitzgerald, K, Dugre, J, Pagala, S, Homel, P, Marcus, M, Kazachkov, M. High-frequency chest wall compression therapy in neurologically impaired children. Respir Care. 2014 Jan;59(1):107–12.CrossRefGoogle ScholarPubMed
Reardon, CC, Christiansen, D, Barnett, ED, Cabral, HJ. Intrapulmonary percussive ventilation vs incentive spirometry for children with neuromuscular disease. Arch Pediatr Adolesc Med. 2005 Jun;159(6):526–31.CrossRefGoogle ScholarPubMed
Hov, B, Andersen, T, Toussaint, M, et al. User-perceived impact of long-term mechanical assisted cough in paediatric neurodisability. Dev Med Child Neurol. 2023 May;65(5):655–63.CrossRefGoogle ScholarPubMed
Hament, JM, Bax, NM, van der Zee, DC, De Schryver, JE, Nesselaar, C. Complications of percutaneous endoscopic gastrostomy with or without concomitant antireflux surgery in 96 children. J Pediatr Surg. 2001 Sep;36(9):1412–15.CrossRefGoogle ScholarPubMed
Hassall, E. Outcomes of fundoplication: causes for concern, newer options. Arch Dis Child. 2005 Oct;90(10):1047–52CrossRefGoogle ScholarPubMed
Martin, TR. The relationship between malnutrition and lung infections. Clin Chest Med. 1987 Sep;8(3):359-72.CrossRefGoogle ScholarPubMed
Sullivan, PB, Alder, N, Bachlet, AM, et al. Gastrostomy feeding in cerebral palsy: too much of a good thing? Dev Med Child Neurol. 2006 Nov;48(11):877–82.CrossRefGoogle ScholarPubMed
Ward, S, Chatwin, M, Heather, S, Simonds, AK. Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax. 2005 Dec;60(12):1019–24.CrossRefGoogle ScholarPubMed
Chatwin, M, Bush, A, Simonds, AK. Outcome of goal-directed non-invasive ventilation and mechanical insufflation/exsufflation in spinal muscular atrophy type I. Arch Dis Child. 2011 May;96(5):426–32.CrossRefGoogle ScholarPubMed
Dohna-Schwake, C, Podlewski, P, Voit, T, Mellies, U. Non-invasive ventilation reduces respiratory tract infections in children with neuromuscular disorders. Pediatr Pulmonol. 2008 Jan;43(1):6771.CrossRefGoogle ScholarPubMed
Galland, BC, Elder, DE, Taylor, BJ. Interventions with a sleep outcome for children with cerebral palsy or a post-traumatic brain injury: a systematic review. Sleep Med Rev. 2012 Dec;16(6):561–73.CrossRefGoogle ScholarPubMed
Schmidt, B, Roberts, RS, Davis, P, Doyle, LW, Barrington, KJ, Ohlsson, A, Solimano, A, Tin, W; Caffeine for Apnea of Prematurity Trial Group. Caffeine therapy for apnea of prematurity. N Engl J Med. 2006 May 18;354(20):2112–21.CrossRefGoogle ScholarPubMed
Davis, JS, Allais, L, Abdallah, C. Caffeine use in the anesthetic management of a patient with congenital central hypoventilation. Cureus. 2022 Jul 7;14(7):e26646.Google ScholarPubMed
Finkel, RS, Mercuri, E, Meyer, OH, et al; SMA care group. Diagnosis and management of spinal muscular atrophy: part 2: pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics. Neuromuscul Disord. 2018 Mar;28(3):197207.CrossRefGoogle ScholarPubMed
Milési, C, Baleine, J, Matecki, S, et al. Is treatment with a high flow nasal cannula effective in acute viral bronchiolitis? A physiologic study. Intensive Care Med. 2013 Jun;39(6):1088–94.Google ScholarPubMed
Fine-Goulden, MR, Puppala, NK, Durward, A. Mechanisms of ventilator dependence in children with neuromuscular and respiratory control disorders identified by monitoring diaphragm electrical activity. Intensive Care Med. 2012 Dec;38(12):2072–9.CrossRefGoogle ScholarPubMed
Rettig, JS, Duncan, ED, Tasker, RC. Mechanical ventilation during acute brain-injury in children. Paediatr Respir Rev. 2016 Sep;20:1723.Google ScholarPubMed
Larcher, V, Craig, F, Bhogal, K, Wilkinson, D, Brierley, J; Royal College of Paediatrics and Child Health. Making decisions to limit treatment in life-limiting and life-threatening conditions in children: a framework for practice. Arch Dis Child. 2015 May;100(suppl 2):s323.CrossRefGoogle ScholarPubMed
NICE National Institute for Health and Care Excellence. Transition from children’s to adults’ services for young people using health or social care services. 2016. Accessed May 2023. www.nice.org,uk/guidance/ng43.Google Scholar

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