Diaphragm Disorders Treatment & Management
- Author: Ryland P Byrd, Jr, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more...
Patients with unilateral diaphragmatic paralysis are typically asymptomatic and do not need treatment. Patients may have some dyspnea in situations associated with exertion or if they have an underlying pulmonary disease.
Treatment should be considered when dyspnea is out of proportion to the physical exertion. Medical care should be focused on the etiology of the dysfunction. In anatomic causes and defects, the only treatment option is surgical repair. Once an anatomic etiology is considered, the most important next step is to discover the cause. Neurologic processes, depending on the etiology, can generally be treated medically.
Many patients with severe diaphragmatic dysfunction require ventilatory support. Depending on the etiology of the disease, some patients only require short-term support, while others may require life-long ventilatory breathing.
Most patients with bilateral diaphragmatic paralysis eventually develop progressive ventilatory failure resulting from fatigue of the accessory muscles. Patients may have progressive disease with carbon dioxide retention and irreversible ventilatory failure. These patients require ventilatory assistance.
In one study, improvement was demonstrated after ventilatory support with external negative-pressure respirators in patients with overt carbon dioxide retention and decreased central ventilatory drive. Some patients who have decreased ventilatory strength as measured by PI-max and transdiaphragmatic pressures had these measures of respiratory function normalize after a period of negative-pressure ventilatory assistance.
Patients with cor pulmonale also may manifest improvement in function and correction of blood gas abnormalities with nighttime or intermittent daytime non-invasive ventilation.
If the patient does not respond to nasal or oral positive-pressure ventilation, alternative forms of therapy such as negative-pressure cuirass or pulmo-wrap, rocking bed, or positive-pressure pulmo-belt can be used.
Tracheotomy with positive-pressure intermittent or permanent ventilation is reserved for patients with life-threatening disease.
Patients should undergo a sleep study if he or she is considered for negative-pressure ventilation because it can precipitate or exacerbate pre-existing upper airway obstruction. Positive-pressure ventilation can minimize the need for a sleep study.
If the phrenic nerve is intact and the problem lies in actually transmitting an impulse to the nerve, phrenic nerve or diaphragmatic pacing may be useful modalities in the treatment of this subset of patients.
Progressive reconditioning is recommended when using a diaphragmatic pacer. High stimulating frequencies and a prolonged period of pacing may lead to irreversible muscle dysfunction. Patients with diaphragmatic pacing require tracheotomies because pacer-induced breathing is not synchronized with the upper airway. Investigations with diaphragmatic pacers and upper airway sensors are ongoing.[25, 26]
Once a diagnosis of neurologic dysfunction is made, ordering studies to determine the cause is vital. A number of neurologic etiologies can be managed medically, but discovering the cause is often a challenge.
Surgery in indicated in the management of anatomic defects in the diaphragm. The type of surgical intervention depends on the anatomic defect or problem.
Manage congenital diaphragmatic defects through transabdominal primary surgical repair.
Acquired diaphragmatic defects (ie, traumatic rupture, late-onset congenital diaphragmatic defect) are typically managed by thoracoscopic plication of the hemidiaphragm. Plication usually results in improved lung function and exercise endurance and less dyspnea. Plication of the diseased diaphragm improves ventilation to the well-perfused lung and improves gas exchange, which improves static lung mechanics.
In a selected group of patients, plication of the diaphragm improved vital capacity by 10-20% and improved the partial pressure of arterial oxygen (PaO2) by 10%.
A surgical series reported improvement in forced tidal volume from 216 mm/GHz to 415 mm/GHz postplication, and mechanical ventilatory support could be discontinued 2-12 days after plication. Plication can also be achieved by video-assisted thoracoscopy.
Phrenic nerve injury
Primary repair of phrenic nerve damage from trauma can be attempted but does not generally restore function. With expectant treatment, few patients regain phrenic nerve function. Manage injury from a tumor by resection of the tumor encasing the phrenic nerve. Most patients regain function of the nerve. Cold phrenic nerve injury during cardiac surgery generally resolves with expectant management.
Spinal cord injury or phrenic nerve injury
Diaphragmatic pacing is an investigational technology that allows the placement electrodes within the diaphragm that stimulate the diaphragm to contract. This can be performed either transthoracically or transabdominally. The more recent studies support the use of laparoscopy and thoracoscopy.
Rochester DF. The diaphragm: contractile properties and fatigue. J Clin Invest. 1985 May. 75(5):1397-402. [Medline].
Wiseman NE, MacPherson RI. "Acquired" congenital diaphragmatic hernia. J Pediatr Surg. 1977 Oct. 12(5):657-65. [Medline].
Shah R, Sabanathan S, Mearns AJ, Choudhury AK. Traumatic rupture of diaphragm. Ann Thorac Surg. 1995 Nov. 60(5):1444-9. [Medline].
Sharma OP. Traumatic diaphragmatic rupture: not an uncommon entity--personal experience with collective review of the 1980's. J Trauma. 1989 May. 29(5):678-82. [Medline].
Keller RL, Tacy TA, Hendricks-Munoz K, et al. Congenital diaphragmatic hernia: endothelin-1, pulmonary hypertension, and disease severity. Am J Respir Crit Care Med. 2010 Aug 15. 182(4):555-61. [Medline].
Grmoljez PF, Lewis JE Jr. Congenital diaphragmatic hernia: Bochdalek type. Am J Surg. 1976 Dec. 132(6):744-6. [Medline].
Rees JR, Redo SF, Tanner DW. Bochdalek's hernia. A review of twenty-one cases. Am J Surg. 1975 Mar. 129(3):259-61. [Medline].
Asano S, Arvapalli R, Manne ND, et al. Cerium oxide nanoparticle treatment ameliorates peritonitis-induced diaphragm dysfunction. Int J Nanomedicine. 2015. 10:6215-25. [Medline].
Greer JJ, Babiuk RP, Thebaud B. Etiology of congenital diaphragmatic hernia: the retinoid hypothesis. Pediatr Res. 2003 May. 53(5):726-30. [Medline].
Kumar N, Folger WN, Bolton CF. Dyspnea as the predominant manifestation of bilateral phrenic neuropathy. Mayo Clin Proc. 2004 Dec. 79(12):1563-5. [Medline].
Zifko U, Auinger M, Albrecht G, et al. Phrenic neuropathy in chronic renal failure. Thorax. 1995 Jul. 50(7):793-4. [Medline].
Efthimiou J, Butler J, Woodham C, Benson MK, Westaby S. Diaphragm paralysis following cardiac surgery: role of phrenic nerve cold injury. Ann Thorac Surg. 1991 Oct. 52(4):1005-8. [Medline].
Brander PE, Jarvinen V, Lohela P, Salmi T. Bilateral diaphragmatic weakness: a late complication of radiotherapy. Thorax. 1997 Sep. 52(9):829-31. [Medline].
Murciano D, Rigaud D, Pingleton S, Armengaud MH, Melchior JC, Aubier M. Diaphragmatic function in severely malnourished patients with anorexia nervosa. Effects of renutrition. Am J Respir Crit Care Med. 1994 Dec. 150(6 Pt 1):1569-74. [Medline].
Lemons VR, Wagner FC Jr. Respiratory complications after cervical spinal cord injury. Spine. 1994 Oct 15. 19(20):2315-20. [Medline].
Iverson LI, Mittal A, Dugan DJ, Samson PC. Injuries to the phrenic nerve resulting in diaphragmatic paralysis with special reference to stretch trauma. Am J Surg. 1976 Aug. 132(2):263-9. [Medline].
van Vugt AB, Schoots FJ. Acute diaphragmatic rupture due to blunt trauma: a retrospective analysis. J Trauma. 1989 May. 29(5):683-6. [Medline].
Heffner JE. Diaphragmatic paralysis following chiropractic manipulation of the cervical spine. Arch Intern Med. 1985 Mar. 145(3):562-4. [Medline].
Gierada DS, Slone RM, Fleishman MJ. Imaging evaluation of the diaphragm. Chest Surg Clin N Am. 1998 May. 8(2):237-80. [Medline].
Summerhill EM, El-Sameed YA, Glidden TJ, McCool FD. Monitoring recovery from diaphragm paralysis with ultrasound. Chest. 2008 Mar. 133(3):737-43. [Medline].
Zanforlin A, Smargiassi A, Inchingolo R, Valente S, Ramazzina E. Ultrasound in obstructive lung diseases: the effect of airway obstruction on diaphragm kinetics. A short pictorial essay. J Ultrasound. 2015 Dec. 18 (4):379-84. [Medline].
Bellemare F, Bigland-Ritchie B. Assessment of human diaphragm strength and activation using phrenic nerve stimulation. Respir Physiol. 1984 Dec. 58(3):263-77. [Medline].
Hill NS. Noninvasive ventilation. Does it work, for whom, and how?. Am Rev Respir Dis. 1993 Apr. 147(4):1050-5. [Medline].
DiMarco AF, Onders RP, Kowalski KE, Miller ME, Ferek S, Mortimer JT. Phrenic nerve pacing in a tetraplegic patient via intramuscular diaphragm electrodes. Am J Respir Crit Care Med. 2002 Dec 15. 166(12 Pt 1):1604-6. [Medline].
Glenn WW. The treatment of respiratory paralysis by diaphragm pacing. Ann Thorac Surg. 1980 Aug. 30(2):106-9. [Medline].
Glenn WW, Hogan JF, Loke JS, Ciesielski TE, Phelps ML, Rowedder R. Ventilatory support by pacing of the conditioned diaphragm in quadriplegia. N Engl J Med. 1984 May 3. 310(18):1150-5. [Medline].
Ciccolella DE, Daly BD, Celli BR. Improved diaphragmatic function after surgical plication for unilateral diaphragmatic paralysis. Am Rev Respir Dis. 1992 Sep. 146(3):797-9. [Medline].
Freeman RK, Wozniak TC, Fitzgerald EB. Functional and physiologic results of video-assisted thoracoscopic diaphragm plication in adult patients with unilateral diaphragm paralysis. Ann Thorac Surg. 2006 May. 81(5):1853-7; discussion 1857. [Medline].
Davis J, Goldman M, Loh L, Casson M. Diaphragm function and alveolar hypoventilation. Q J Med. 1976 Jan. 45(177):87-100. [Medline].
Baum GL, Crapo JD, Celli BR, eds. Textbook of Pulmonary Diseases. 6th ed. Boston, Mass: Little Brown & Company; 1995.
Murray JF, Nadel JA, eds. Textbook of Respiratory Medicine. Philadelphia, Pa: WB Saunders; 1997.