eMedicine Specialties > Pulmonology > Diaphragmatic Disorders
Diaphragmatic Paralysis
Updated: Jul 23, 2009
Introduction
Background
The respiratory system functions as a vital pump that moves air in and out of the lung gas-exchange units. The respiratory pump consists of central respiratory centers, the spinal cord, peripheral nerves, neuromuscular junctions, and respiratory muscles.
The diaphragm, the most important muscle of ventilation, develops negative intrathoracic pressure to initiate ventilation. Innervated by cervical motor neurons C3-C5 via the phrenic nerves, the cone-shaped muscle by means of contraction decreases intrapleural pressure during inspiration, expands the rib cage, and thereby facilitates movement of gases into the lungs.
Pathophysiology
Diaphragmatic paralyses encompass a spectrum of disease involving a single leaflet, known as unilateral diaphragmatic paralysis (UDP), and that involving both leaflets, known as bilateral diaphragmatic paralysis (BDP).
Although the diaphragm performs most of the work, normal ventilation also requires the simultaneous contraction of respiration accessory muscles (ie, scalene, parasternal portion of the internal and external intercostal muscles, sternocleidomastoid, trapezius). In bilateral diaphragmatic paralysis, respiration accessory muscles assume some or all of the work of breathing by contracting more intensely. An increased effort in the struggle to breathe may fatigue the accessory muscles and lead to ventilatory failure.
Frequency
United States
Incidence is unknown.
Mortality/Morbidity
Unilateral diaphragmatic paralysis
The morbidity of the unilateral paralysis is mainly based on the underlying pulmonary functional status and the etiology of the paralysis. Because most cases of unilateral diaphragmatic paralysis are found incidentally during imaging studies, many patients have no symptoms. The patients that do have symptoms and decreased quality of life are those who have underlying lung disease.
Bilateral diaphragmatic paralysis
Patients with bilateral diaphragmatic paralysis are usually symptomatic and, when symptoms are severe or in the presence of underlying lung pathology, may develop ventilatory failure without medical intervention.
Clinical
History
Bilateral diaphragmatic paralysis is characterized by profound abnormalities of pulmonary and respiratory muscle function. Patients develop severe restrictive ventilatory impairment, and the vital capacity and total lung capacity frequently are below 50% of predicted for that patient. Lung capacity is reduced further when the patient assumes the supine position. Symptoms depend on whether the paralysis is unilateral or bilateral, how rapid the paralysis occurs, and the presence of underlying pulmonary disease.
- Unilateral diaphragmatic paralysis
- Unilateral diaphragmatic paralysis is often discovered incidentally in patients undergoing chest radiography for some other reason.
- Patients usually are asymptomatic at rest but experience dyspnea upon exertion and have a decrease in exercise performance.
- If the patient has an underlying lung disease, dyspnea may occur at rest.
- Some patients may develop orthopnea, which is less intense than bilateral diaphragmatic paralysis.
- Bilateral diaphragmatic paralysis
- Patients typically present with respiratory failure or dyspnea (may be misinterpreted as a sign of heart failure) that worsens in the supine position.1 Tachypnea and rapid, shallow breathing occur when the patient adopts the recumbent position.
- Patients also report anxiety, insomnia, morning headache, excessive daytime somnolence and fatigue, and poor sleep habits.
Physical
Physical examination findings depend on whether the paralysis is unilateral or bilateral. Generally, a breathing pattern of paradoxical abdominal wall retraction during inspiration occurs. The physician can evaluate the patient further by palpating under the costal margin and feeling for the descending hemidiaphragms during inspiration.
- Unilateral diaphragmatic paralysis
- Patients reveal dullness to percussion and absent breath sounds over the lower chest on the involved side.
- Excursion on the involved hemithorax is decreased when compared with the healthy side.
- Bilateral diaphragmatic paralysis
- Patients report morning headaches, confusion, and signs of cor pulmonale.
- Chest examination reveals limitation of diaphragmatic excursions and bilateral lower chest dullness with absent breath sounds.
- Patients are tachypneic and use accessory respiration muscles.
- The diagnostic finding is a paradoxical inward movement of the abdomen with inspiration.
Causes
- Unilateral diaphragmatic paralysis
- The most common diagnosed cause is a malignant (ie, metastatic lung cancer) lesion leading to nerve compression (approximately 30% of patients).
- If malignancy is not the cause, many times the etiology is not diagnosed.
- Other causes in the differential include surgical trauma, herpes zoster, cervical spondylosis, trauma, or secondary to surgery (mainly thoracic).
- Bilateral diaphragmatic paralysis
- The most common causes are secondary to motor neuron disease, including amyotrophic lateral sclerosis and postpolio syndrome.
- Other causes include thoracic trauma (including cardiac surgery),2 multiple sclerosis, myopathies, and muscular dystrophy (acid maltase deficiency).
More on Diaphragmatic Paralysis |
Overview: Diaphragmatic Paralysis |
| Differential Diagnoses & Workup: Diaphragmatic Paralysis |
| Treatment & Medication: Diaphragmatic Paralysis |
| Follow-up: Diaphragmatic Paralysis |
| Multimedia: Diaphragmatic Paralysis |
| References |
| Further Reading |
| Next Page » |
References
Kumar N, Folger WN, Bolton CF. Dyspnea as the predominant manifestation of bilateral phrenic neuropathy. Mayo Clin Proc. Dec 2004;79(12):1563-5. [Medline].
Ulku R, Onat S, Balci A, Eren N. Phrenic nerve injury after blunt trauma. Int Surg. Apr-Jun 2005;90(2):93-5. [Medline].
Gierada DS, Slone RM, Fleishman MJ. Imaging evaluation of the diaphragm. Chest Surg Clin N Am. May 1998;8(2):237-80. [Medline].
Miller JM, Moxham J, Green M. The maximal sniff in the assessment of diaphragm function in man. Clin Sci (Lond). Jul 1985;69(1):91-6. [Medline].
Lloyd T, Tang YM, Benson MD, King S. Diaphragmatic paralysis: the use of M mode ultrasound for diagnosis in adults. Spinal Cord. Aug 2006;44(8):505-8. [Medline].
Ciccolella DE, Daly BD, Celli BR. Improved diaphragmatic function after surgical plication for unilateral diaphragmatic paralysis. Am Rev Respir Dis. Sep 1992;146(3):797-9. [Medline].
Graham DR, Kaplan D, Evans CC, Hind CR, Donnelly RJ. Diaphragmatic plication for unilateral diaphragmatic paralysis: a 10-year experience. Ann Thorac Surg. Feb 1990;49(2):248-51; discussion 252. [Medline].
Versteegh MI, Braun J, Voigt PG, et al. Diaphragm plication in adult patients with diaphragm paralysis leads to long-term improvement of pulmonary function and level of dyspnea. Eur J Cardiothorac Surg. Sep 2007;32(3):449-56. [Medline].
Kuniyoshi Y, Yamashiro S, Miyagi K, Uezu T, Arakaki K, Koja K. Diaphragmatic plication in adult patients with diaphragm paralysis after cardiac surgery. Ann Thorac Cardiovasc Surg. Jun 2004;10(3):160-6. [Medline].
Alkofer B, Le Roux Y, Coffin O, Samama G. Thoracoscopic plication of the diaphragm for postoperative phrenic paralysis: a report of two cases. Surg Endosc. May 2004;18(5):868-70. [Medline].
Bach JR, Penek J. Obstructive sleep apnea complicating negative-pressure ventilatory support in patients with chronic paralytic/restrictive ventilatory dysfunction. Chest. Jun 1991;99(6):1386-93. [Medline].
DiMarco AF, Onders RP, Ignagni A, Kowalski KE, Mortimer JT. Phrenic nerve pacing via intramuscular diaphragm electrodes in tetraplegic subjects. Chest. Feb 2005;127(2):671-8. [Medline].
Easton PA, Fleetham JA, de la Rocha A, Anthonisen NR. Respiratory function after paralysis of the right hemidiaphragm. Am Rev Respir Dis. Jan 1983;127(1):125-8. [Medline].
Xu WD, Gu YD, Lu JB, Yu C, Zhang CG, Xu JG. Pulmonary function after complete unilateral phrenic nerve transection. J Neurosurg. Sep 2005;103(3):464-7. [Medline].
[Guideline] Consortium for Spinal Cord Medicine, Paralyzed Veterans of America. Respiratory management following spinal cord injury: a clinical practice guideline for health-care professionals. National Guideline Clearinghouse. Jan 2005.
Celli BR. Respiratory management of diaphragm paralysis. Semin Respir Crit Care Med. Jun 2002;23(3):275-81. [Medline].
Chuang ML, Chuang DC, Lin IF, Vintch JR, Ker JJ, Tsao TC. Ventilation and exercise performance after phrenic nerve and multiple intercostal nerve transfers for avulsed brachial plexus injury. Chest. Nov 2005;128(5):3434-9. [Medline].
Gibson GJ. Diaphragmatic paresis: pathophysiology, clinical features, and investigation. Thorax. Nov 1989;44(11):960-70. [Medline].
Glenn WW. The treatment of respiratory paralysis by diaphragm pacing. Ann Thorac Surg. Aug 1980;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. May 3 1984;310(18):1150-5. [Medline].
Kreitzer SM, Feldman NT, Saunders NA, Ingram RH Jr. Bilateral diaphragmatic paralysis with hypercapnic respiratory failure. A physiologic assessment. Am J Med. Jul 1978;65(1):89-95. [Medline].
Lisboa C, Pare PD, Pertuze J, et al. Inspiratory muscle function in unilateral diaphragmatic paralysis. Am Rev Respir Dis. Sep 1986;134(3):488-92. [Medline].
Moxham J, Shneerson JM. Diaphragmatic pacing. Am Rev Respir Dis. Aug 1993;148(2):533-6. [Medline].
Piehler JM, Pairolero PC, Gracey DR, Bernatz PE. Unexplained diaphragmatic paralysis: a harbinger of malignant disease?. J Thorac Cardiovasc Surg. Dec 1982;84(6):861-4. [Medline].
Sandham JD, Shaw DT, Guenter CA. Acute supine respiratory failure due to bilateral diaphragmatic paralysis. Chest. Jul 1977;72(1):96-8. [Medline].
Smith PE, Edwards RH, Calverley PM. Mechanisms of sleep-disordered breathing in chronic neuromuscular disease: implications for management. Q J Med. Dec 1991;81(296):961-73. [Medline].
Further Reading
Clinical guideline summary
Respiratory management following spinal cord injury: a clinical practice guideline for health-care professionals. 15
Keywords
diaphragmatic paralysis, diaphragm paralysis, diaphragm paresis, paralyzed diaphragm, diaphragmatic disorder, diaphragmatic dysfunction, diaphragm dysfunction, respiratory pump, central respiratory centers, spinal cord, peripheral nerves, neuromuscular junctions, respiratory muscles, diaphragm, bilateral diaphragmatic paralysis, unilateral diaphragmatic paralysis, ventilatory support, diaphragm tumor, diaphragm mass, breathing disorder, diaphragm, diaphragm pacing, diaphragmatic pacing, diaphragm plication, diaphragmatic plication
Overview: Diaphragmatic Paralysis