DİYAFRAM TONUS BOZUKLUKLARI

Öz

Diyafram, toraks ve abdominal kaviteyi ayıran kubbe biçimli muskulofibröz yapı olarak, solunumun temel kası ve yaşam için vazgeçilmez bir organ olarak kabul edilir. Kas liflerinin özgün yapısı ve sinirsel innervasyonu sayesinde, hem istirahat hem de zorlanmış solunumda kritik rol oynar. Diyaframın elektriksel aktivitesi, nörofizyolojik tekniklerle izlenebilmekte ve ventilasyon desteğinin kişiye özel ayarlanmasında önemli bir gösterge olmaktadır. Ancak çeşitli patolojik durumlar -sinir, kas veya nöromüsküler düzeydeki hasarlar- diyafram disfonksiyonuna yol açar; bu durumlar dispne, egzersiz intoleransı ve solunum yetmezliği gibi ciddi klinik tabloları beraberinde getirir. Konjenital evantrasyon, paralizi, spastisite ve myoklonus gibi farklı disfonksiyon formları, solunum fonksiyonlarının bozulmasına ve yaşam kalitesinde düşüşe neden olur. Özellikle nörolojik hastalıklarda diyafram spastisitesi, kas kasılmalarının kısıtlanması yoluyla ventilasyon etkinliğini olumsuz etkiler. Bu derleme, diyaframın anatomik ve fizyolojik özelliklerini, elektriksel aktivite değerlendirme yöntemlerini ve disfonksiyonlarının klinik etkilerini kapsamlı biçimde ele alarak, diyafram sağlığının korunması ve hastalıkların etkin yönetimi için multidisipliner yaklaşımların önemini vurgulamaktadır.

Anahtar Kelimeler

• Diyafram
• Diyafram eventrasyonu
• Diyafragmatik flutter
• Elektrofizyoloji
• Spastisite

DIAPHRAGM TONE DISORDERS

Öz

The diaphragm, a dome-shaped musculo-fibrous structure separating the thoracic and abdominal cavities, is recognized as the primary muscle of respiration and an indispensable organ for life. Thanks to the unique composition of its muscle fibers and neural innervation, it plays a critical role in both resting and forced breathing. The electrical activity of the diaphragm can be monitored using neurophysiological techniques, serving as an important indicator for personalized ventilatory support adjustments. However, various pathological conditions—affecting the nerves, muscles, or neuromuscular junction—lead to diaphragmatic dysfunction, which may cause dyspnea, exercise intolerance, and respiratory failure. Different forms of dysfunction such as congenital eventration, paralysis, spasticity, and myoclonus contribute to impaired respiratory functions and reduced quality of life. Particularly in neurological diseases, diaphragmatic spasticity negatively impacts ventilatory efficiency by limiting muscle contraction capacity. This review comprehensively discusses the anatomical and physiological features of the diaphragm, methods for assessing its electrical activity, and the clinical implications of its dysfunctions. It highlights the importance of a multidisciplinary approach for preserving diaphragm health and effectively managing related diseases.

Anahtar Kelimeler

• Diaphragm
• Diaphragmatic eventration
• Diaphragmatic flutter
• Electrophysiology
• Spasticity

Kaynakça

1. 1. Tsimouris D, Arvanitidis M, Moutzouri M, Koumantakis GA, Gioftsos G, Papandreou M, Grammatopoulou E. Is manual therapy of the diaphragm effective for people with obstructive lung diseases? A systematic review. Respiratory Medicine and Research 2023;83:101002.
2. 2. Liberati C, Byrne BJ, Fuller DD, Croft C, Pitts T, Ehrbar J, Leon-Astudillo C, Smith BK. Diaphragm pacing and independent breathing in individuals with severe Pompe disease. Front Rehabil Sci. 2023;4:1184031.
3. 3. Criner GJ, Gayen S, Zantah M, Dominguez Castillo E, Naranjo M, Lashari B, Pourshahid S, Gangemi A. Clinical review of non-invasive ventilation. Eur Respir J. 2024;64(5):2400396.
4. 4. Campbell NA, Reece JB, Urry LA, Cain ML, Wasserman SA, Minorsky PV, Jackson RB, eds. Biology. 8th ed. Sydney: Pearson/Benjamin Cummings Co; 2008:334.
5. 5. Arora NS, Rochester DF. Effect of body weight and muscularity on human diaphragm muscle mass, thickness, and area. J Appl Physiol Respir Environ Exerc Physiol. 1982;52(1):64-70.
6. 6. Downey R. Anatomy of the normal diaphragm. Thorac Surg Clin. 2011;21:273-9.
7. 7. Dubé BP, Dres M. Diaphragm dysfunction: diagnostic approaches and management strategies. J Clin Med. 2016;5(12):113.
8. 8. Shields TW. Embryology and anatomy of the diaphragm. In: Shields TW, LoCicero J, Reed CE, Feins RH (ed). General Thoracic Surgery. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2009:690-2.

9. 9. Hussain SN, Magder S. Diaphragmatic intramuscular pressure in relation to tension, shortening, and blood flow. J Appl Physiol. 1991;71(1):159-67.
10. 10. Özkan S. Diyafram evantrasyonu ve cerrahi tedavisi. In: Yücel O, Genç (ed). Torasik Konjenital Anomaliler ve Cerrahisi. Ankara: Derman Tıbbi Yayıncılık; 2011:88-98.
11. 11. Geiger PC, Cody MJ, Macken RL, Sieck GC. Maximum specific force depends on myosin heavy chain content in rat diaphragm muscle fibers. J Appl Physiol. 2000;89(2):695–703.
12. 12. Polla B, D’Antona G, Bottinelli R, Reggiani C. Respiratory muscle fibres: specialisation and plasticity. Thorax. 2004;59:808-17.
13. 13. Pacia EB, Aldrich TK. Assessment of diaphragm function. Chest Surg Clin N Am. 1998;8(2):225-36.
14. 14. McCool FD, Manzoor K, Minami T. Disorders of the diaphragm. Clin Chest Med. 2018;39(2):345-60.
15. 15. De Troyer A, Sampson M, Sigrist S, Macklem PT. Action of costal and crural parts of the diaphragm on the rib cage in dog. J Appl Physiol Respir Environ Exerc Physiol. 1982;53(1):30-9.
16. 16. Rochester DF. The diaphragm: contractile properties and fatigue. J Clin Invest. 1985;75(5):1397-402.
17. 17. Gibson GJ. Diaphragmatic paresis: pathophysiology, clinical features, and investigation. Thorax. 1989;44:960-70.
18. 18. Nason LK, Walker CM, McNeeley MF, Burivong W, Fligner CL, Godwin JD. Imaging of the diaphragm: anatomy and function. Radiographics. 2012;32(1):51-70.
19. 19. Roberts HC. Imaging the diaphragm. Thorac Surg Clin. 2009;19(3):431-50.
20. 20. Wright CD, Williams JG, Ogilvie CM, Donnelly RJ. Results of diaphragmatic plication for unilateral diaphragmatic paralysis. J Thorac Cardiovasc Surg. 1985;90(2):195-8.
21. 21. McCool FD, Tzelepis GE. Dysfunction of the diaphragm. N Engl J Med. 2012;366(10):932-42.
22. 22. Kharma N. Dysfunction of the diaphragm: imaging as a diagnostic tool. Curr Opin Pulm Med. 2013;19(4):394-8.
23. 23. Salvatierra A. Trastornos del diafragma. In: Martín P, Ramos G, Sanchís J (ed). Medicina Respiratoria. 2nd ed. Madrid: Aula Médica Ediciones; 2006:1243-53.
24. 24. Ricoy J, Rodríguez-Núñez N, Álvarez-Dobaño JM, Toubes ME, Riveiro V, Valdés L. Diaphragmatic dysfunction. Pulmonology. 2019;25(4):223-35.
25. 25. Muller N, Volgyesi G, Becker L, Bryan MH, Bryan AC. Diaphragmatic muscle tone. J Appl Physiol. 1979;47(2):279-84.
26. 26. Wu XY, Huang YZ, Liu HG, Huang DY, Tang R, Qiu HB. Effects of pulmonary stretch reflex on lung injury in rabbits with acute respiratory distress syndrome. World J Emerg Med. 2011;2(4):296-301.
27. 27. Kraaijenga JV, de Waal CG, Hutten GJ, de Jongh FH, van Kaam AH. Diaphragmatic activity during weaning from respiratory support in preterm infants. Arch Dis Child Fetal Neonatal Ed. 2017;102(4):307–11.
28. 28. Stein H, Firestone K, Rimensberger PC. Synchronized mechanical ventilation using electrical activity of the diaphragm in neonates. Clin Perinatol. 2012;39(3):525–42.
29. 29. O’Brien MJ, van Eykern LA, Prechtl HF. Monitoring respiratory activity in infants: a non-intrusive diaphragm EMG technique. In: Rolfe P (ed). Non-invasive physiological measurements. London: Academic Press Inc; 1983:131–77.
30. 30. Stein H, Beck J, Dunn M. Non-invasive ventilation with neurally adjusted ventilatory assist in newborns. Semin Fetal Neonatal Med. 2016;21(3):154–161.
31. 31. Patel N, Chong K, Baydur A. Methods and applications in respiratory physiology: respiratory mechanics, drive and muscle function in neuromuscular and chest wall disorders. Front Physiol. 2022;13:838414.
32. 32. Bellemare F, Grassino A. Effect of pressure and timing of contraction on human diaphragm fatigue. J Appl Physiol. 1982;53(5):1190–5.
33. 33. Bellemare F. Strength of the respiratory muscles. In: Roussos C (ed). The Thorax. Lung Biology in Health and Disease. 1995; 85:1161–97.
34. 34. Sarmento A, Fregonezi G, Lira M, Marques L, Pennati F, Resqueti V, et al. Changes in electromyographic activity, mechanical power, and relaxation rates following inspiratory ribcage muscle fatigue. Sci Rep. 2021;11:12475.
35. 35. Ducharme-Crevier L, Du Pont-Thibodeau G, Emeriaud G. Interest of monitoring diaphragmatic electrical activity in the pediatric intensive care unit. Crit Care Res Pract. 2013;2013:384210.
36. 36. Wu M, Yuan X, Liu L, Yang Y. Neurally adjusted ventilatory assist vs. conventional mechanical ventilation in adults and children with acute respiratory failure: a systematic review and meta-analysis. Frontiers in Medicine, 2022;9:814245.
37. 37. Shah SD, Shah M, Anjum F. Neurally adjusted ventilatory assist (NAVA). In StatPearls [Internet]. StatPearls Publishing. 2024 [cited 20 Dec 2024] Avaliable from: https://www.ncbi.nlm.nih.gov/books/NBK572111/
38. 38. Al-Ayyad M, Owida HA, De Fazio R, Al-Naami B, Visconti P. Electromyography monitoring systems in rehabilitation: A review of clinical applications, wearable devices and signal acquisition methodologies. Electronics, 2023;12(7):1520.
39. 39. Campanini I, Cosma M, Manca M, Merlo A. Added value of dynamic EMG in the assessment of the equinus and the equinovarus foot deviation in stroke patients and barriers limiting its usage. Frontiers in neurology, 2020;11:583399.
40. 40. Guslits BG, Gaston SE, Bryan MH, et al. Diaphragmatic work of breathing in premature human infants. J Appl Physiol. 1987;62(4):1410–5.
41. 41. Pham TM, O’Malley L, Mayfield S, et al. The effect of high flow nasal cannula therapy on the work of breathing in infants with bronchiolitis. Pediatr Pulmonol. 2015;50(7):713–20.
42. 42. Van Leuteren RW, de Waal CG, de Jongh FH, Bem RA, van Kaam AH, Hutten G. Diaphragm activity pre and post extubation in ventilated critically ill infants and children measured with transcutaneous electromyography. Pediatr Crit Care Med. 2021;22(11):950–9.
43. 43. Emeriaud G, Beck J, Tucci M, et al. Diaphragm electrical activity during expiration in mechanically ventilated infants. Pediatr Res. 2006;59(5):705–10.
44. 44. Larouche A, Massicotte E, Constantin G, et al. Tonic diaphragmatic activity in critically ill children with and without ventilatory support. Pediatr Pulmonol. 2015;50(12):1304–12.
45. 45. Allo JC, Beck JC, Brander L, et al. Influence of neurally adjusted ventilatory assist and positive end-expiratory pressure on breathing pattern in rabbits with acute lung injury. Crit Care Med. 2006;34(11):2997–3004.
46. 46. Chen Y, Li P, Wang J, Wu W, Liu X. Assessments and targeted rehabilitation therapies for diaphragmatic dysfunction in patients with chronic obstructive pulmonary disease: A narrative review. COPD: Journal of Chronic Obstructive Pulmonary Disease, 2022;17:457–473.
47. 47. Woo A, Tchoe HJ, Shin HW, Shin CM, Lim CM. Assisted Breathing with a Diaphragm Pacing System: A Systematic Review. Yonsei Med J. 2020;61(12):1024-1033.
48. 48. Le Pimpec-Barthes F, Legras A, Arame A, Pricopi C, Boucherie JC, Badia A, Panzini CM. Diaphragm pacing: the state of the art. J Thorac Dis. 2016;8(Suppl 4):S376-86.
49. 49. Vashisht R, Chowdhury YS. Diaphragmatic pacing. In StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2023 [20 Dec 2024] Available from: https://www.ncbi.nlm.nih.gov/books/NBK557793/
50. 50. Bedarf JR, Nitsch L, Malotki R, Andrié R, Reimann J. Diaphragmatic myoclonus owing to electrode dislocation of implantable cardioverter defibrillator. Parkinsonism Relat Disord. 2019;63:232–4.
51. 51. Caviness JN, Brown P. Myoclonus: current concepts and recent advances. Lancet Neurol. 2004;3(10):598–607.
52. 52. Kothiala A, Shankar V, Desai S. Myoclonus associated with infections: a narrative review. Ann Mov Disord. 2022;5(3):137–52.
53. 53. Ghosh R, León-Ruiz M, Bandyopadhyay S, Roy D, Benito-León J. Scrub typhus presenting as diaphragmatic myoclonus. Neurol Sci. 2022;43(6):4023–4.
54. 54. Panigrahy N, Kumar PP, Chirla DK. Respiratory flutter syndrome in a neonate. Indian Pediatr. 2013;50(2):242–3.
55. 55. Borroni B, Gazzina S, Dono F, et al. Diaphragmatic myoclonus due to SARS-CoV-2 infection. Neurol Sci. 2020;41(12):3471–4.
56. 56. He YJ, Li B, Pan YG, Zhang XL, Jin LJ. Botulinum toxin A for treatment of diaphragmatic myoclonus. Chin Med J (Engl). 2017;130(6):753–4.
57. 57. Krastev G, Daniš M, Haring J. Severe diaphragmatic myoclonus treated with unconventional therapy: a case report. Respir Med Case Rep. 2024;51:102074.
58. 58. Ramírez JD, Gonzales M, Hoyos JA, Grisales L. Diaphragmatic flutter: a case report and literature review. Neurologia. 2015;30(4):249–51.
59. 59. Koul R, Alfutaisi A. Diaphragmatic myoclonus. Indian Pediatr. 2017;54:326.
60. 60. Hernández-Fernández F, Pardal-Fernández JM, García-Martínez E, Segura T. Respiratory myoclonus, a side effect of galantamine. Farm Hosp. 2011;35(2):97–9.
61. 61. Onesti E, Ceccanti M, Rubino A, Frasca V, Inghilleri M. Atypical case of diaphragmatic pseudo myoclonus. Parkinsonism Relat Disord. 2017;43:118–9.
62. 62. Katz ES, Gauda E, Crawford T, Ogunlesi F, Lefton-Greif MA, McGrath-Morrow S, Marcus CL. Respiratory flutter syndrome: an underrecognized cause of respiratory failure in neonates. Am J Respir Crit Care Med. 2001;164(7):1161–4.
63. 63. Miranda B, Pinto S, de Carvalho M. The impact of spasticity on diaphragm contraction: electrophysiological assessment. Clin Neurophysiol. 2018;129(8):1544–50.
64. 64. Ersöz M, Selcuk B, Gündüz R, Kurtaran A, Akyüz M. Decreased chest mobility in children with spastic cerebral palsy. Turk J Pediatr. 2006;48(4):344–50.
65. 65. Laffont I, Durand MC, Rech C, De La Sotta AP, Hart N, Dizien O, Lofaso F. Breathlessness associated with abdominal spastic contraction in a patient with C4 tetraplegia: a case report. Arch Phys Med Rehabil. 2003;84(6):906–8.
66. 66. Konstantinidi A, Liakou P, Kopanou Taliaka P, Lampridou M, Kalatzi N, Loukas I, Sokou R. Congenital diaphragmatic eventration in the neonatal period: Systematic review of the literature and report of a rare case presenting with gastrointestinal disorders. Pediatric Reports. 2023;15(3): 442-51.
67. 67. O’Connor BR, Teague WJ. What Is the Best Approach to Evaluation and Treatment of Children with Diaphragmatic Eventration? In Difficult Decisions in Pediatric Surgery Cham: Springer Nature Switzerland; 2025:215-29.
68. 68. Cheng J, Parmar T, Smyth J, Bolisetty S, Lui K, Schindler T. Non-invasive neurally adjusted ventilatory assist (NIV-NAVA) in the neonatal intensive care unit (NICU): an Australian NICU experience. BMC pediatrics. 2024;24(1):514.
69. 69. Ramos VFML, Considine E, Karp BI, Lungu C, Alter K, Hallett M. Ultrasound as diagnostic tool for diaphragmatic myoclonus. Movement disorders clinical practice, 2016;3(3):282-4.
70. 70. Chen KHS, Keng LT, Kuo YT, Tai YC, Chen R. Ultrasound‐Guided Needle Electromyography for Assessing Diaphragmatic Myoclonus. Movement Disorders Clinical Practice, 2020;7(7):870.
71. 71. Whitlock WL, Young CR. Treatment strategies for diaphragmatic myoclonus. Chest, 1995;108(5):1471-2.
72. 72. Panelli A, Verfuß MA, Dres M, Brochard L, Schaller SJ. Phrenic nerve stimulation to prevent diaphragmatic dysfunction and ventilator-induced lung injury. Intensive Care Medicine Experimental, 2023;11(1):94.