Tools
Phrenic Nerve Injury
Introduction
The phrenic nerve is among the most important nerves in the body due to its role in respiration. The phrenic nerve provides the primary motor supply to the diaphragm, the major respiratory muscle. Phrenic nerve injury, such as may occur from cardiothoracic surgery, can lead to diaphragmatic paralysis or dysfunction. The presentation of phrenic nerve injury is non-specific, and the diagnosis may easily be missed. Phrenic nerve injury can be identified by a number of imaging modalities including ultrasound, electromyography, and fluoroscopy[1].
Ultrasound is ideal and emerging as a diagnostic test for this condition as the modality is non-invasive, poses no risk of ionizing radiation, is easily reproducible, low cost and may be performed at the bedside. This is especially ideal for critical patients. Ultrasound is more commonly being used to evaluate phrenic nerve injury due to its ability to evaluate the function and structure of the diaphragm. Ultrasound of the diaphragm can help clinicians identify diseases caused by phrenic nerve injury including diaphragmatic paralysis and diaphragmatic dysfunction. Ultrasound can sometimes identify the etiology behind the disease and provide data for determining prognosis[2]. Diaphragmatic ultrasound is also useful in the assessment of prolonged mechanical ventilation and assessment of the failure of weaning from mechanical ventilation[3]. This modality can additionally help select patients for early surgical plication (which can decrease intensive care unit length of stay and ventilator-associated pneumonia)[4].
Etiology
Register For Free And Read The Full Article
Search engine and full access to all medical articles- 10 free questions in your specialty
- Free CME/CE Activities
- Free daily question in your email
- Save favorite articles to your dashboard
- Emails offering discounts
Learn more about a Subscription to StatPearls Point-of-Care
Etiology
Injury of the phrenic nerve can occur by multiple mechanisms. One common etiology of phrenic nerve injury is from surgery, primarily thoracic and cardiac surgery[5]. The left phrenic nerve descends anteriorly between the pericardium and mediastinal pleura. Therefore, it can be injured while dissecting near the area of an internal thoracic artery. Canbaz et al. identified that a major factor that causes injury during cardiac surgery is the icy slush that is used for myocardial protection. The phrenic nerve can also be damaged from blunt or penetrating trauma[6], metabolic diseases like diabetes[7], infectious causes such as Lyme disease and herpes zoster[8][9], direct invasion by tumor[10], neurological diseases such as cervical spondylosis and multiple sclerosis, myopathy (i.e., muscular dystrophy) and immunological disease (e.g., Guillain-Barre syndrome)[11].
Phrenic nerve injury may present as diaphragmatic dysfunction, unilateral diaphragmatic paralysis, or bilateral diaphragmatic paralysis. One of the most frequent causes of unilateral diaphragmatic paralysis is iatrogenic. In bilateral diaphragmatic paralysis, one of the most common causes is a motor neuron disease, including amyotrophic lateral sclerosis and post-polio syndrome[12].
Epidemiology
Few studies have shown that diaphragmatic paralysis occurs more often in male individuals. Scharf et al. found that 1% to 7% of patients with significant blunt trauma present with a diaphragmatic injury. Up to 3% of abdominal injuries also involve the diaphragm[13].
History and Physical
The diagnosis of phrenic nerve injury requires high suspicion due to nonspecific signs and symptoms including unexplained shortness of breath, recurrent pneumonia, anxiety, insomnia, morning headache, excessive daytime somnolence, orthopnea, fatigue, and difficulty weaning from mechanical ventilation. In unilateral diaphragmatic paralysis, the patient is often asymptomatic at rest and has dyspnea only during exertion. This unilateral diaphragmatic paralysis is typically found incidentally on chest radiograph[10][14][15]. In comparison, patients with bilateral diaphragmatic paralysis always present with shortness of breath[15][16][17][18][19].
On physical examinations, findings may include decreased breath sounds on the affected side, dullness to percussion of the affected side of the chest and inward movement of the epigastrium during inspiration.
Evaluation
Phrenic nerve injury can be evaluated with multiple modalities, for example, esophageal and gastric manometry, diaphragmatic electromyography or ultrasound. Diaphragmatic ultrasound is primarily performed with a low-frequency curvilinear transducer. A high-frequency linear transducer is the best choice for an intercostal view since it has a small footprint that can fit between the ribs.
Patients are typically examined in the supine position during spontaneous inspiration and expiration. The pleural and peritoneal membranes should be visualized during imaging of the diaphragm for thickness measurements. The supine position limits any compensatory active expiration by the anterior abdominal wall, which may mask paralysis[20].
On ultrasound, the diaphragm appears as a thick echogenic line[21]. The left hemidiaphragm can be evaluated through the splenic window. The right hemidiaphragm is visualized through the liver window. Interestingly, pathologic conditions such as a left upper quadrant mass, splenomegaly or hepatomegaly may make an evaluation of the left hemidiaphragm easier[22]. Diagnostic criteria for diaphragmatic paralysis include paradoxical movement or significantly decreased diaphragmatic excursion[23].
Diaphragm thickness and thickening during respiration can be assessed with ultrasound, typically with a high-frequency linear transducer. The ultrasound transducer is placed at the anterior axillary line between the seventh and eighth or eighth and ninth ribs, which is the Zone of Apposition (ZOA). Diaphragm thickness can be used to assess for atrophy, especially as might occur while on mechanical ventilation. Diaphragmatic thickness values less than 0.2 cm at the end of expiration are considered to define diaphragmatic atrophy. In healthy volunteers, the average thickness of the diaphragm is typically between 0.22 to 0.28 cm, however in a paralyzed diaphragm, the diaphragmatic thickness is reported to be less, between 0.13 to 0.19 cm. The Thickening Fraction can also be calculated and used as a predictor of success of weaning from mechanical ventilation[24][25][24].
Treatment / Management
Most patients with asymptomatic unilateral diaphragmatic paralysis do not require treatment. When identified, the underlying cause should be treated[26]. Surgical options are considered if the underlying cause is treated and the patient still has symptoms, or if the patient has bilateral diaphragmatic paralysis. There are various treatment options including plication and phrenic nerve stimulation. Plication of the affected site is a very useful treatment method that allows weaning from mechanical ventilation. Plication is preferably performed in unilateral diaphragmatic paralysis in non-morbidly obese patients[27]. Phrenic nerve stimulation is performed in intact phrenic nerve without evidence of myopathy. This procedure can be performed in patients with bilateral diaphragmatic paralysis with cervical spine injuries[28].(B2)
Differential Diagnosis
Since the symptoms of phrenic nerve injury are not specific, we have to consider all causes of shortness of breath including pulmonary, cardiac, metabolic, and hematologic causes. In bilateral diaphragmatic paralysis, the following additional diagnoses should be considered: anterior horn cells and neuromuscular junction diseases[29]. Hypoventilation secondary to cervical spine disease is also difficult to differentiate from phrenic nerve dysfunction.
Prognosis
The mortality and morbidity of phrenic nerve injury or diaphragmatic paralysis depend on the underlying causes and status of pulmonary function[26]. Generally speaking, unilateral diaphragmatic paralysis has a good prognosis unless the patient is experiencing severe shortness of breath. If recovery occurs in bilateral diaphragmatic paralysis, it usually takes more than one year for partial or full recovery[19][30].
Enhancing Healthcare Team Outcomes
There is potentially significant mortality and morbidity associated with phrenic nerve injury. Recovery is heavily dependent on an interprofessional effort of nurses, respiratory therapists, and clinicians educating and motivating the patients while providing quality care.
Media
(Click Image to Enlarge)
Elevated Right Hemidiaphragm. The image shows the chest x-ray of a patient who sustained a traumatic root avulsion brachial plexus injury. Notably, the elevated right hemidiaphragm indicates associated phrenic nerve injury. The patient also sustained fractures to the right midshaft clavicle and right first and second ribs.
Contributed by D Luo, MD
(Click Image to Enlarge)
CXR demonstrating right clavicle fracture, elevated right hemi-diaphragm associated with phrenic nerve injury Contributed by Zhongyu Li, MD PhD
References
Houston JG, Fleet M, Cowan MD, McMillan NC. Comparison of ultrasound with fluoroscopy in the assessment of suspected hemidiaphragmatic movement abnormality. Clinical radiology. 1995 Feb:50(2):95-8 [PubMed PMID: 7867276]
Yeh HC, Halton KP, Gray CE. Anatomic variations and abnormalities in the diaphragm seen with US. Radiographics : a review publication of the Radiological Society of North America, Inc. 1990 Nov:10(6):1019-30 [PubMed PMID: 2259759]
Kim WY, Suh HJ, Hong SB, Koh Y, Lim CM. Diaphragm dysfunction assessed by ultrasonography: influence on weaning from mechanical ventilation. Critical care medicine. 2011 Dec:39(12):2627-30. doi: 10.1097/CCM.0b013e3182266408. Epub [PubMed PMID: 21705883]
Kunovsky P,Gibson GA,Pollock JC,Stejskal L,Houston A,Jamieson MP, Management of postoperative paralysis of diaphragm in infants and children. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 1993; [PubMed PMID: 8396949]
Level 2 (mid-level) evidenceTripp HF, Bolton JW. Phrenic nerve injury following cardiac surgery: a review. Journal of cardiac surgery. 1998 May:13(3):218-23 [PubMed PMID: 10193993]
Level 3 (low-level) evidenceBell D, Siriwardena A. Phrenic nerve injury following blunt trauma. Journal of accident & emergency medicine. 2000 Nov:17(6):419-20 [PubMed PMID: 11104246]
Level 3 (low-level) evidenceWhite JE, Bullock RE, Hudgson P, Home PD, Gibson GJ. Phrenic neuropathy in association with diabetes. Diabetic medicine : a journal of the British Diabetic Association. 1992 Dec:9(10):954-6 [PubMed PMID: 1478044]
Level 3 (low-level) evidenceHemidiaphragmatic paresis after cervical herpes zoster., Derveaux L,Lacquet LM,, Thorax, 1982 Nov [PubMed PMID: 6298966]
Level 3 (low-level) evidenceAbbott RA, Hammans S, Margarson M, Aji BM. Diaphragmatic paralysis and respiratory failure as a complication of Lyme disease. Journal of neurology, neurosurgery, and psychiatry. 2005 Sep:76(9):1306-7 [PubMed PMID: 16107377]
Level 3 (low-level) evidencePiehler JM, Pairolero PC, Gracey DR, Bernatz PE. Unexplained diaphragmatic paralysis: a harbinger of malignant disease? The Journal of thoracic and cardiovascular surgery. 1982 Dec:84(6):861-4 [PubMed PMID: 6292583]
Gibson GJ. Diaphragmatic paresis: pathophysiology, clinical features, and investigation. Thorax. 1989 Nov:44(11):960-70 [PubMed PMID: 2688182]
Czapliński A, Strobel W, Gobbi C, Steck AJ, Fuhr P, Leppert D. Respiratory failure due to bilateral diaphragm palsy as an early manifestation of ALS. Medical science monitor : international medical journal of experimental and clinical research. 2003 May:9(5):CS34-6 [PubMed PMID: 12761460]
Level 3 (low-level) evidenceAl-Thani H, Jabbour G, El-Menyar A, Abdelrahman H, Peralta R, Zarour A. Descriptive Analysis of Right and Left-sided Traumatic Diaphragmatic Injuries; Case Series from a Single Institution. Bulletin of emergency and trauma. 2018 Jan:6(1):16-25. doi: 10.29252/beat-060103.. Epub [PubMed PMID: 29379805]
Level 2 (mid-level) evidenceLisboa C, Paré PD, Pertuzé J, Contreras G, Moreno R, Guillemi S, Cruz E. Inspiratory muscle function in unilateral diaphragmatic paralysis. The American review of respiratory disease. 1986 Sep:134(3):488-92 [PubMed PMID: 3752705]
Hart N, Nickol AH, Cramer D, Ward SP, Lofaso F, Pride NB, Moxham J, Polkey MI. Effect of severe isolated unilateral and bilateral diaphragm weakness on exercise performance. American journal of respiratory and critical care medicine. 2002 May 1:165(9):1265-70 [PubMed PMID: 11991876]
Level 2 (mid-level) evidenceLaroche CM,Carroll N,Moxham J,Green M, Clinical significance of severe isolated diaphragm weakness. The American review of respiratory disease. 1988 Oct [PubMed PMID: 3202460]
Sandham JD, Shaw DT, Guenter CA. Acute supine respiratory failure due to bilateral diaphragmatic paralysis. Chest. 1977 Jul:72(1):96-8 [PubMed PMID: 872664]
Level 3 (low-level) evidenceKumar N, Folger WN, Bolton CF. Dyspnea as the predominant manifestation of bilateral phrenic neuropathy. Mayo Clinic proceedings. 2004 Dec:79(12):1563-5 [PubMed PMID: 15595343]
Level 3 (low-level) evidenceHughes PD, Polkey MI, Moxham J, Green M. Long-term recovery of diaphragm strength in neuralgic amyotrophy. The European respiratory journal. 1999 Feb:13(2):379-84 [PubMed PMID: 10065685]
Houston JG, Angus RM, Cowan MD, McMillan NC, Thomson NC. Ultrasound assessment of normal hemidiaphragmatic movement: relation to inspiratory volume. Thorax. 1994 May:49(5):500-3 [PubMed PMID: 8016774]
Nason LK, Walker CM, McNeeley MF, Burivong W, Fligner CL, Godwin JD. Imaging of the diaphragm: anatomy and function. Radiographics : a review publication of the Radiological Society of North America, Inc. 2012 Mar-Apr:32(2):E51-70. doi: 10.1148/rg.322115127. Epub [PubMed PMID: 22411950]
Houston JG, Morris AD, Howie CA, Reid JL, McMillan N. Technical report: quantitative assessment of diaphragmatic movement--a reproducible method using ultrasound. Clinical radiology. 1992 Dec:46(6):405-7 [PubMed PMID: 1493655]
Fayssoil A, Behin A, Ogna A, Mompoint D, Amthor H, Clair B, Laforet P, Mansart A, Prigent H, Orlikowski D, Stojkovic T, Vinit S, Carlier R, Eymard B, Lofaso F, Annane D. Diaphragm: Pathophysiology and Ultrasound Imaging in Neuromuscular Disorders. Journal of neuromuscular diseases. 2018:5(1):1-10. doi: 10.3233/JND-170276. Epub [PubMed PMID: 29278898]
Wait JL,Nahormek PA,Yost WT,Rochester DP, Diaphragmatic thickness-lung volume relationship in vivo. Journal of applied physiology (Bethesda, Md. : 1985). 1989 Oct [PubMed PMID: 2676955]
Gottesman E, McCool FD. Ultrasound evaluation of the paralyzed diaphragm. American journal of respiratory and critical care medicine. 1997 May:155(5):1570-4 [PubMed PMID: 9154859]
Kokatnur L, Rudrappa M. Diaphragmatic Palsy. Diseases (Basel, Switzerland). 2018 Feb 13:6(1):. doi: 10.3390/diseases6010016. Epub 2018 Feb 13 [PubMed PMID: 29438332]
Simansky DA, Paley M, Refaely Y, Yellin A. Diaphragm plication following phrenic nerve injury: a comparison of paediatric and adult patients. Thorax. 2002 Jul:57(7):613-6 [PubMed PMID: 12096205]
Level 2 (mid-level) evidenceDiMarco AF, Phrenic nerve stimulation in patients with spinal cord injury. Respiratory physiology & neurobiology. 2009 Nov 30 [PubMed PMID: 19786125]
Level 3 (low-level) evidenceDubé BP, Dres M. Diaphragm Dysfunction: Diagnostic Approaches and Management Strategies. Journal of clinical medicine. 2016 Dec 5:5(12): [PubMed PMID: 27929389]
Summerhill EM, El-Sameed YA, Glidden TJ, McCool FD. Monitoring recovery from diaphragm paralysis with ultrasound. Chest. 2008 Mar:133(3):737-43. doi: 10.1378/chest.07-2200. Epub 2008 Jan 15 [PubMed PMID: 18198248]
Level 2 (mid-level) evidence