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Diaphragmatic paralysis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Synonyms and keywords:Phrenic palsy, Diaphragmatic palsy, Phrenic paralysis, Diaphragmatic paresis.

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2] [3] [4] [5]

Overview

Diaphragmatic paralysis was first suggested by Steurtz, in 1911 during a simple phrenicotomy for treatment of lung disease. Diaphragmatic paralysis may be classified according to involvement of leaflets into unilateral or bilateral. It is thought that diaphragmatic paralysis is the result of paralysis of cervical nerve roots( C3-C5). Common causes of unilateral diphragmatic paralysis include idiopathicphrenic nerve injury in cardiac surgery and viral infection. Common causes of bilateral diphragmatic paralysis include idiopathic, cervical spinal cord disease and motor neuron disease. Unilateral diaphragmatic paralysis must be differentiated from eventration of the diaphragm. Eventration of the diaphragm is an abnormal elevation of the hemidiaphragm. Bilateral diaphragmatic paralysis must be differentiated from other diseases that cause elevation of the diaphragm such as pleural adhesions, subpulmonic effusions, obesityascites, abdominal organomegaly and ileus. Other differential diagnosis are dermatomyositispolymyositisrib fracturepleural effusionsamyotrophic lateral sclerosis.The incidence of diaphragmatic paralysis is unknown, but incidence of diaphragmatic paralysis after cardiac surgery is approximately 30,000 to 75,000 per 100,000 individuals. Prevalence of diaphragmatic paralysis after cardiac surgery is 1,600 per 100,000 in children. The most potent risk factor in the development of diaphragmatic paralysis is cooling or stretching in cardiac surgery. Common complication of diaphragmatic paralysis includes severe pulmonary dysfunction in bilateral diaphragmatic paralysis. Studies of choice for unilateral diaphragmatic paralysis include upright chest radiograph and fluoroscopic sniff test. Studies of choice for bilateral diaphragmatic paralysis include measurement of transdiaphragmatic pressure (Pdi). The majority of patients with unilateral diaphragmatic paralysis are asymptomatic.  Dyspnea in supine position and orthopnea are common symptoms of bilateral diphragmatic paralysis. Laboratory findings consistent with the diagnosis of diaphragmatic paralysis include reduced oxygen saturation in the supine position in unilateral and bilateral diaphragmatic paralysis and elevated arterial partial pressure of carbon dioxide (PaCO2) in bilateral diaphragmatic paralysis. An x-ray and ultrasound are helpful in the diagnosis of diaphragmatic paralysis. No treatment is required for unilateral diaphragmatic paralysis because most patients are asymptomatic. In bilateral diaphragmatic paralysis treatment options are ventilatory support and diaphragmatic pacing. Surgery is usually reserved for patients with either dyspnea in strenous physical activity and patients with underlying severe pulmonary disease. Surgical plication of the involved hemidiaphragm can be considered for unilateral diaphragmatic paralysis. surgery in bilateral diaphragmatic paralysis can be done via neurolysis and nerve grafting.

Historical Perspective

Diaphragmatic paralysis was first suggested by Steurtz, in 1911 during simple phrenicotomy for treatment of lung disease. In 1946, a case of poliomyelitiswith respiratory paralysis was explained in Romania.

Classification

Diaphragmatic paralysis may be classified according to involvement of leaflets into unilateral or bilateral. Bilateral diaphragmatic paralysis is a medical emergency. Unilateral diaphragmatic paralysis is often discovered incidentally on chest x-ray for other reasons.

Pathophysiology

It is thought that diaphragmatic paralysis is the result of paralysis of cervical nerve roots( C3-C5 ). Diaphragmatic paralyses can be unilateral or bilateral according to involvemnet of one or two leaflets of diaphragm. In the case of unilateral diaphragm paralysis, it is compensated by other hemidiaphragm or accessory muscles of respiration. In bilateral diaphragmatic paralysis, accessory muscles do all of the work of breathing and finally it may lead to ventilatory failure. Early diaphragmatic paralysis may be one of the manifestations of genetic neuromuscular disorders such as spinal muscular atrophy (Werdnig-Hoffmann disease) and acid maltase deficiency. On gross and microscopic pathology, there are no characteristic findings of diaphragmatic paralysis. left untreated, patients with unilateral diaphragmatic paralysis may recover fully or partially.

Causes

Common causes of unilateral diphragmatic paralysis include idiopathicphrenic nerve injury in cardiac surgery and viral infection and less common causes include cervical spondylosis, cervical compressive tumors and blunt neck trauma. Common causes of bilateral diphragmatic paralysis include idiopathic, cervical spinal cord disease and motor neuron disease. Less common causes of bilateral diphragmatic paralysis include parsonage turner syndrome and malnutrition.

Differentiating Diaphragmatic Paralysis from Other Diseases

Unilateral diaphragmatic paralysis must be differentiated from eventration of the diaphragm. Eventration of the diaphragm is an abnormal elevation of the hemidiaphragm. Bilateral diaphragmatic paralysis must be differentiated from other diseases that cause elevation of the diaphragm such as pleural adhesions, subpulmonic effusions, obesityascites, abdominal organomegaly and ileus. Diaphragmatic paralysis must be differentiated from other disease that cause dyspnea such as dermatomyositispolymyositisrib fracturepleural effusions, and amyotrophic lateral sclerosis.

Epidemiology and Demographics

The incidence of diaphragmatic paralysis is unknown, but incidence of diaphragmatic paralysis after cardiac surgery is approximately 30,000 to 75,000 per 100,000 individuals. Prevalence of diaphragmatic paralysis after cardiac surgery is 1,600 per 100,000 in children.The morbidity and mortality of the unilateral diaphragmatic paralysis is related to underlying pulmonary function and etiology. Most of the bilateral diaphragmatic paralysis are symptomatic and may develop ventilatory failure. Patients of all age groups may develop diaphragmatic paralysis. There is no racial predilection to diaphragmatic paralysis. Men are more commonly affected.

Risk Factors

The most potent risk factor in the development of diaphragmatic paralysis is cooling or streching in cardiac surgery. Other risk factors include viruses, spinal cord transection and malnutrition.

Screening

There is insufficient evidence to recommend routine screening for diaphragmatic paralysis.

Natural History, Complications, and Prognosis

 If left untreated, patients with unilateral diaphragmatic paralysis may recover fully or partially. Common complications of diaphragmatic paralysis includes severe pulmonary dysfunction in bilateral diaphragmatic paralysis. Prognosis is generally excellent in unilateral diaphragmatic paralysis. Bilateral diaphragmatic paralysis is a medical emergency.

Diagnosis

Diagnostic Study of Choice

Studies of choice for unilateral diaphragmatic paralysis include upright chest radiograph and fluoroscopic sniff test. Studies of choice for bilateral diaphragmatic paralysis include measurement of transdiaphragmatic pressure (Pdi).

History and Symptoms

The majority of patients with unilateral diaphragmatic paralysis are asymptomatic. Exertional dyspnea and decreased exercise performance are common symtoms of unilateral diaphragmati paralysis. Less common symtoms of unilateral diaphragmatic paralysis include dyspnea at rest and orthopnea. Dyspnea in supine position and orthopnea are common symtoms of bilateral diphragmatic paralysis. Less common symtoms of bilateral diaphragmatic paralysis include daytime fatigue and confusion.

Physical Examination

Patients with unilateral diphragmatic paralysis usually appear normal. Patients with bilateral diaphragmatic paralysis usually are in respiratory distress. The severe forms of bilateral diaphragmatic paralysis would lead to pulmonary hypertension.

Laboratory Findings

Laboratory findings consistent with the diagnosis of diaphragmatic paralysis include reduced oxygen saturation in the supine position in unilateral and bilateral diaphragmatic paralysis and elevated the arterial partial pressure of carbon dioxide (PaCO2) in bilateral diaphragmatic paralysis. Hypoxemia may be seen in arterial blood gas in bilateral diaphragmatic paralysis.

Electrocardiogram

There are no ECG findings associated with diaphragmatic paralysis.

X-ray

An x-ray is helpful in the diagnosis of diaphragmatic paralysis. Findings on an x-ray suggestive of unilateral diaphragmatic paralysis include elevated hemidiaphragm on the paralysed side and small lung volumes. Findings on an x-ray suggestive of bilateral diaphragmatic paralysis include smooth elevation of the hemidiaphragms and atelectasis at the lung base.

Echocardiography and Ultrasound

Ultrasound may be helpful in the diagnosis of diaphragmatic paralysis. Findings on an ultrasound suggestive of diaphragmatic paralysis include abnormal paradoxical movement during inspiration and Less than 20% thickening of the diaphragm.

CT scan

Chest CT scan may be helpful in the diagnosis of tumors as causes of diaphragmatic paralysis. Findings on CT scan suggestive of diaphragmatic paralysis include patchy areas of atelectasis and elevation of one or both hemidiaphragm.

MRI

Cervical spine MRI may be helpful in the diagnosis of diaphragmatic paralysis. Findings on MRI suggestive of diaphragmatic paralysis include spinal column or nerve roots pathologic conditions as causes of diaphragmatic paralysis.

Other Imaging Findings

Fluoroscopic sniff test may be helpful in the diagnosis of diaphragmatic paralysis. Findings on sniff test suggestive of diaphragmatic paralysis include paradoxical elevation of the paralyzed hemidiaphragm during inspiration.

Other Diagnostic Studies

Other diagnostic studies for diphragmatic paralysis include pulmonary function test which demonstrates decrease in vital capacity in diaphragmatic paralysis. Maximal inspiratory pressure (MIP) can be decreaed. Electromyography and polysomnography are other diagnostic studies.

Treatment

Medical Therapy

No treatment is required for unilateral diaphragmatic paralysis because most patients are asymptomatic. In bilateral diaphragmatic paralysis treatment options are ventilatory support and diaphragmatic pacing.

Surgery

Surgery is not the first-line treatment option for patients with unilateral diaphragmatic paralysis. Surgery is usually reserved for patients with either dyspnea in strenous physical activity and patients with underlying severe pulmonary disease. Surgical plication of the involved hemidiaphragm can be considered for unilateral diaphragmatic paralysis. surgery in bilateral diaphragmatic paralysis can be done via neurolysis and nerve grafting.

Primary Prevention

There are no established measures for the primary prevention of diaphragmatic paralysis. However, the incidence of diaphragmatic paralysis is less in off-pump coronary artery bypass grafting (OPCAB) compared to conventional CABG.

Secondary Prevention

Effective measures for the secondary prevention of diaphragmatic paralysis include chest physiotherapy following post cardiac surgery diaphragmatic paralysis.

References


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Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

Diaphragmatic paralysis was first suggested by Steurtz, in 1911 during simple phrenicotomy for treatment of lung disease. In 1946, a case of poliomyelitis with respiratory paralysis was explained in Rumania.

Historical Perspective

Discovery

  • Diaphragmatic paralysis was first suggested by Steurtz, in 1911 during simple phrenicotomy for treatment of lung disease.[1]
  • Thomas Harris, in 1892, stated that depression of the epigastric and hypochondriac regions on deep inspiration not always indicative of diaphragmatic paralysis.
  • In 1946, a case of poliomyelitis with respiratory paralysis was explained in Rumania [2]


References

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Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

Diaphragmatic paralysis may be classified according to involvement of leaflets into unilateral or bilateral. Bilateral diaphragmatic paralysis is a medical emergency. Unilateral diaphragmatic paralysis is often discovered incidentally on chest x-ray for other reasons.

Classification

  • Diaphragmatic paralysis may be classified according to involvement of leaflets into unilateral or bilateral.[1][2][3]
  • Unilateral diaphragmatic paralysis is more common than bilateral diaphragmatic paralysis. It is often discovered incidentally on chest x-ray for other reasons.[3]
  • Bilateral diaphragmatic paralysis can be considered a medical emergency.

References

  1. Dubé BP, Dres M (2016). “Diaphragm Dysfunction: Diagnostic Approaches and Management Strategies”. J Clin Med. 5 (12). doi:10.3390/jcm5120113. PMC 5184786. PMID 27929389.
  2. Sánchez J, Medrano G, Debesse B, Riquet M, Derenne JP (1985). “Muscle fibre types in costal and crural diaphragm in normal men and in patients with moderate chronic respiratory disease”. Bull Eur Physiopathol Respir. 21 (4): 351–6. PMID 4041660.
  3. 3.0 3.1 Lieberman DA, Faulkner JA, Craig AB, Maxwell LC (1973). “Performance and histochemical composition of guinea pig and human diaphragm”. J Appl Physiol. 34 (2): 233–7. doi:10.1152/jappl.1973.34.2.233. PMID 4265565.

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Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

It is thought that diaphragmatic paralysis is the result of paralysis of cervical nerve roots( C3-C5 ). Diaphragmatic paralyses can be unilateral or bilateral according to involvemnet of one or two leaflets of diaphragm. In the case of unilateral diaphragm paralysis, it is compensated by other hemidiaphragm or accessory muscles of respiration. In bilateral diaphragmatic paralysis, accessory muscles do all of the work of breathing and finally it may lead to ventilatory failure. Early diaphragmatic paralysis may be one of the manifestations of genetic neuromuscular disorders such as spinal muscular atrophy (Werdnig-Hoffmann disease) and acid maltase deficiency. On gross and microscopic pathology, there are no characteristic findings of diaphragmatic paralysis.

Pathophysiology

The main muscles of inspiration include:

The muscles of expiration:

Pathogenesis

  • The diaphragm is the musculo-fibrous membrane. It has two parts: non-contractile central fibrous  and peripheral muscular components. [1][2][3]
  • Peripheral muscular section has two fibers:
    • Type 1: slow and fatigue resistant fibers: play roles in low intensity, continual cycle of breathing
    • Type 2: fast fibers: play roles in rapid and intense situations such as:
      • Talking
      • Singing,
      • Sneezing,
      • Defecation
      • Acute hyperventilation

The diaphragm create negative intrathoracic pressure and facilitates movement of air into the lungs. It is innervated by cervical nerve roots ( C3-C5 ) via the phrenic nerves.[4][5]

Diaphragmatic paralysis can be unilateral or bilateral according to involvemnet of one or two leaflets of diaphragm.

Diaphragmatic paralysis is an uncommon cause of dyspnea.

It is understood that diaphragmatic paralysis is the result of paralysis of cervical nerve roots (C3-C5 ).

In the case of unilateral diaphragm paralysis, it is compensated by other hemidiaphragm or accessory muscles of respiration.

In bilateral diaphragmatic paralysis, accessory muscles do all of the work of breathing and finally it may lead to ventilatory failure.

Bilateral diaphragmatic paralysis is usually seen with generalized muscle weakness. In some cases, the diaphragm is the only muscle involved. 

Genetics

Early diaphragmatic paralysis may be one of the manifestations of genetic neuromuscular disorders such as spinal muscular atrophy (Werdnig-Hoffmann disease) and acid maltase deficiency.[6]

Gross Pathology

  • On gross pathology, there are no characteristic findings of diaphragmatic paralysis.

Microscopic Pathology

  • On microscopic histopathological analysis, there are no characteristic findings of diaphragmatic paralysis.

References

  1. Mizuno M (1991). “Human respiratory muscles: fibre morphology and capillary supply”. Eur. Respir. J. 4 (5): 587–601. PMID 1936230.
  2. Sánchez J, Medrano G, Debesse B, Riquet M, Derenne JP (1985). “Muscle fibre types in costal and crural diaphragm in normal men and in patients with moderate chronic respiratory disease”. Bull Eur Physiopathol Respir. 21 (4): 351–6. PMID 4041660.
  3. Roussos C, Macklem PT (1982). “The respiratory muscles”. N. Engl. J. Med. 307 (13): 786–97. doi:10.1056/NEJM198209233071304. PMID 7050712.
  4. Lieberman DA, Faulkner JA, Craig AB, Maxwell LC (1973). “Performance and histochemical composition of guinea pig and human diaphragm”. J Appl Physiol. 34 (2): 233–7. doi:10.1152/jappl.1973.34.2.233. PMID 4265565.
  5. Fell SC (1998). “Surgical anatomy of the diaphragm and the phrenic nerve”. Chest Surg. Clin. N. Am. 8 (2): 281–94. PMID 9619305.
  6. Sivan Y, Galvis A (1990). “Early diaphragmatic paralysis. In infants with genetic disorders”. Clin Pediatr (Phila). 29 (3): 169–71. doi:10.1177/000992289002900305. PMID 2407409.

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Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

Common causes of unilateral diphragmatic paralysis include idiopathic, phrenic nerve injury in cardiac surgery and viral infection and less common causes of it include cervical spondylosis, cervical compressive tumors and blunt neck trauma. Common causes of bilateral diphragmatic paralysis include idiopathic, cervical spinal cord disease and motor neuron disease. Less common causes of bilateral diphragmatic paralysis include parsonage turner syndrome and malnutrition.

Causes

Life-threatening Causes

  • Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated. There are no life-threatening causes of diaphragmatic paralysis

Common Causes

Unilateral diaphragmatic paralysis :[1]

Bilateral diaphragmatic paralysis:[3]

Less Common Causes

Unilateral diaphragmatic paralysis : [8]

Bilateral diaphragmatic paralysis :[3]

Less common causes of diaphragmatic paralysis include:

  • Parsonage Turner syndrome (brachial neuritis or neuralgic amyotrophy)
  • Malnutrition[10]

Genetic Causes

Causes by Organ System

Cardiovascular No underlying causes
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drug Side Effect No underlying causes
Ear Nose Throat No underlying causes
Endocrine Hypothyroidism, Hyperthyroidism
Environmental No underlying causes
Gastroenterologic Systemic botulism, Malnutrition
Genetic Acid maltase deficiency 
Hematologic No underlying causes
Iatrogenic Phrenic nerve injury in cardiac surgery, Neck surgery, Iatrogenic embolization
Infectious Disease Herpes zoster, Poliomyelitis
Musculoskeletal/Orthopedic Polymyositis, Dermatomyositis, Inclusion body myopathy, Limb-girdle muscular dystrophy, Acid maltase deficiency, Cervical spondylosis,
Neurologic Large tumors, Spinal cord transection, Amyotrophic lateral sclerosis, Guillain-Barré syndrome, Post-viral neuropathy, Myasthenia gravis, Systemic botulism, Neuralgic amyotrophy, Parsonage Turner syndrome 
Nutritional/Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic Cervical compressive tumors,
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary Pneumonia
Renal/Electrolyte No underlying causes
Rheumatology/Immunology/Allergy Autoimmune diseases,Lambert-Eaton myasthenic syndrome , Polymyositis, Dermatomyositis
Sexual No underlying causes
Trauma Blunt neck trauma,
Urologic No underlying causes
Miscellaneous Idiopathic

Causes in Alphabetical Order

List the causes of the disease in alphabetical order.

References

  1. Dubé, Bruno-Pierre; Dres, Martin (2016). “Diaphragm Dysfunction: Diagnostic Approaches and Management Strategies”. Journal of Clinical Medicine. 5 (12): 113. doi:10.3390/jcm5120113. ISSN 2077-0383.
  2. Canbaz S, Turgut N, Halici U, Balci K, Ege T, Duran E (2004). “Electrophysiological evaluation of phrenic nerve injury during cardiac surgery–a prospective, controlled, clinical study”. BMC Surg. 4: 2. doi:10.1186/1471-2482-4-2. PMC 320489. PMID 14723798.
  3. 3.0 3.1 Dubé BP, Dres M (2016). “Diaphragm Dysfunction: Diagnostic Approaches and Management Strategies”. J Clin Med. 5 (12). doi:10.3390/jcm5120113. PMC 5184786. PMID 27929389.
  4. Chen R, Grand’Maison F, Strong MJ, Ramsay DA, Bolton CF (1996). “Motor neuron disease presenting as acute respiratory failure: a clinical and pathological study”. J. Neurol. Neurosurg. Psychiatry. 60 (4): 455–8. PMC 1073907. PMID 8774419.
  5. Betensley AD, Jaffery SH, Collins H, Sripathi N, Alabi F (2004). “Bilateral diaphragmatic paralysis and related respiratory complications in a patient with West Nile virus infection”. Thorax. 59 (3): 268–9. PMC 1746955. PMID 14985569.
  6. Valadas A, de Carvalho M (2008). “Myasthenia gravis and respiratory failure related to phrenic nerve lesion”. Muscle Nerve. 38 (4): 1340–1. doi:10.1002/mus.21067. PMID 18785183.
  7. Nicolle, Michael W.; Stewart, Dwight J.; Remtulla, Hussein; Chen, Robert; Bolton, Charles F. (1996). “Lambert-Eaton myasthenic syndrome presenting with severe respiratory failure”. Muscle & Nerve. 19 (10): 1328–1333. doi:10.1002/(SICI)1097-4598(199610)19:10<1328::AID-MUS10>3.0.CO;2-Q. ISSN 0148-639X.
  8. Chapman SA, Holmes MD, Taylor DJ (2000). “Unilateral diaphragmatic paralysis following bronchial artery embolization for hemoptysis”. Chest. 118 (1): 269–70. PMID 10893396.
  9. Tsao BE, Ostrovskiy DA, Wilbourn AJ, Shields RW (2006). “Phrenic neuropathy due to neuralgic amyotrophy”. Neurology. 66 (10): 1582–4. doi:10.1212/01.wnl.0000216140.25497.40. PMID 16717226.
  10. Murciano, D; Rigaud, D; Pingleton, S; Armengaud, M H; Melchior, J C; Aubier, M (1994). “Diaphragmatic function in severely malnourished patients with anorexia nervosa. Effects of renutrition”. American Journal of Respiratory and Critical Care Medicine. 150 (6): 1569–1574. doi:10.1164/ajrccm.150.6.7952616. ISSN 1073-449X.
  11. Sivan Y, Galvis A (1990). “Early diaphragmatic paralysis. In infants with genetic disorders”. Clin Pediatr (Phila). 29 (3): 169–71. doi:10.1177/000992289002900305. PMID 2407409.
  12. Kishnani PS, Steiner RD, Bali D, Berger K, Byrne BJ, Case LE, Case L, Crowley JF, Downs S, Howell RR, Kravitz RM, Mackey J, Marsden D, Martins AM, Millington DS, Nicolino M, O’Grady G, Patterson MC, Rapoport DM, Slonim A, Spencer CT, Tifft CJ, Watson MS (2006). “Pompe disease diagnosis and management guideline”. Genet. Med. 8 (5): 267–88. doi:10.109701.gim.0000218152.87434.f3 Check |doi= value (help). PMC 3110959. PMID 16702877.

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Differentiating Diaphragmatic paralysis from Other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

Unilateral diaphragmatic paralysis must be differentiated from eventration of the diaphragm. Eventration of the diaphragm is an abnormal elevation of the hemidiaphragm. Bilateral diaphragmatic paralysis must be differentiated from other diseases that cause elevation of the diaphragm such as pleural adhesions, subpulmonic effusions, obesity, ascites, abdominal organomegaly and ileus. Diaphragmatic paralysis must be differentiated from other disease that cause dyspnea such as dermatomyositis, polymyositis, rib fracture, pleural effusions, amyotrophic lateral sclerosis.

Differentiating diaphragmatic paralysis from other Diseases

Unilateral diaphragmatic paralysis must be differentiated from eventration of the diaphragm. Eventration of the diaphragm is an abnormal elevation of the hemidiaphragm that some parts of hemidiaphragm are replaced by fibrous tissue. Clinical manifestations of eventration of the diaphragm include asymptomatic, infection and respiratory distress.[1]

Bilateral diaphragmatic paralysis must be differentiated from other diseases that cause elevation of the diaphragm such as:

Diaphragmatic paralysis must be differentiated from other disease that cause dyspnea such as:

Abbreviations: ABG (arterial blood gas); ACE (angiotensin converting enzyme); BMI (body mass index); CBC (complete blood count); CSF (cerebrospinal fluid); CXR (chest X-ray); DOE (dyspnea on exercise); ECG (electrocardiogram); FEF (forced expiratory flow rate); FEV1 (forced expiratory volume); FVC (forced vital capacity); JVD (jugular vein distention); MCV (mean corpuscular volume); Plt (platelet); RV (residual volume); SIADH (syndrome of inappropriate antidiuretic hormone); TSH (thyroid stimulating hormone); Vt (tidal volume); WBC (white blood cell);

Organ system Diseases Clinical manifestations Diagnosis Other features
Symptoms Physical exam
Loss of consciousness Agitation Weight loss Fever Chest pain Cough Orthopnea DOE Cyanosis Clubbing JVD Peripheral edema Auscultation CBC ABG Imaging Spirometry Gold standard
Acute Dyspnea Respiratory system Head and Neck,

Upper airway

Aspiration[2] + +/- + + Diminished breath sounds Normal Normal Atelectasis Vt, ↑RV Bronchoscopy Choking
Chest and Pleura,

Lower airway

Atelectasis +/- +/- +/- +/- Diminished breath sounds, Wheeze Normal O2, Normal/↓CO2 Collapsed lung lobe, fissuresdisplacement FVC Chest CT scan Surgical procedure, Aspiration,

Mechanical ventilation

Bronchitis[3] + + + Rhonchi  WBC Normal Normal Normal Physical exam Rhonchi relieved by cough
Bronchiolitis[4] + +/- + Wheeze and Crackles WBC Normal Bronchovascular markings Vt Clinical assessment Respiratory syncytial virus (RSV)
Lung carcinoma[5] + + + + Wheeze and Crackles Normal Normal Mass lesion, hilar lymphadenopathy Vt, ↑RV Bronchoscopy  Paraneoplastic syndromes, such as SIADH and lambert-Eaton
Pneumonia[6] + + + Wheeze, Rhonchi, and Crackles WBC, neutrophilia Normal Lobar consolidation Normal Chest X-ray and CT Scan productive cough
Pneumothorax[7] + +/- Diminished breath sounds Normal O2, ↑CO2 Radiolucency without lung marking Vt CXR and Chest CT scan Tracheal deviation
Pulmonary embolism[8] + +/- Normal Normal Respiratory alkalosis Normal Normal Pulmonary CT angiography Pleuritic chest pain
Rib fractures (flail chest)[9] + + Normal Normal Respiratory acidosis Fracture marks Normal Chest X-ray Pneumothorax
Cardiovascular system Pericardial tamponade[10] +/- + +/- +/- + Muffled heart sounds Normal Normal Water bottle appearance enlarged heart Normal Echocardiography Fluid accumulation in pericardium
Pulmonary edema[11] +/- + + + + + + + + + + Basal crackle Normal Respiratory alkalosis Bat wing pattern, air bronchograms Vt, ↑RV Cardiac Catheterization Tachypnea
Central nervous system Stroke + +/- Normal Normal Normal Intracranial infarct or hemorrhage Normal Brain MRI Paralysis or paresthesia
Encephalitis[12] + + + Normal WBC, neutrophilia Normal Normal Normal CSF PCR Confusion
Traumatic brain injury[13] + +/- Normal Normal Respiratory acidosis Intracerebral hemorrhage Normal Brain CT scan Lucid interval
Organ system Diseases Clinical manifestations Diagnosis Other features
Symptoms Physical exam
Loss of consciousness Agitation Weight loss Fever Chest pain Cough Orthopnea DOE Cyanosis Clubbing JVD Peripheral edema Auscultation CBC ABG Imaging Spirometry Gold standard
Chronic Dyspnea Respiratory system Head and Neck,

Upper airway

Goiter[14] + Normal Normal Normal Normal Normal Blood test (TSH, T4) Weight gain
Laryngeal adenocarcinoma[15] + +/- Stridor Normal O2, ↑CO2 Retropharyngeal tissue thickness Normal Laryngoscopy Choking sensation
Chest and Pleura,

Lower airway

COPD[16] +/- + + + + + + +/- Expiratory wheeze RBC Respiratory alkalosis, Metabolic acidosis ↑ Bronchovascular markings, Cardiomegaly FEV1/FVC Physical exam and

Spirometry

Heavy smoking history
Emphysema[17] +/- + + Expiratory wheeze, Hyperinflation Normal Respiratory alkalosis, Metabolic acidosis Flattening of diaphragm, vertical heart FEV1/FVC Physical exam and

Spirometry

Barrel chest
Pulmonary hypertension[18] +/- +/- +/- +/- + + Accentuated S2 Normal Hypoxia and acidosis Enlarged pulmonary arteries Physiologic RV Cardiac catheterization Syncope,

Ascites, Pleural effusion

Sarcoidosis[19] +/- +/- + + Crackles Normal O2, ↑CO2 Hilar adenopathy FEV1/FVC High resolution computed tomography (HRCT) Hypercalcemia, high ACE
Pleural effusion[20] +/- + + +/- +/- +/- Egophony (“E-to-A” change) Normal Normal Blunting of the costophrenic and cardiophrenic angle Vt, ↑RV Light’s criteria Tactile fremitus, Asymmetrical chest expansion
Diaphragmatic paralysis[21] +/- +/- +/- + + Normal Normal Normal Unilateral or bilateral diaphragmatic flattening Vt, ↑RV

(anatomical)

CXR confirmed by fluoroscopic sniff test Respiratory insufficiency
Tuberculosis[22] + + + + +/- Rhonchi, Wheezing, Crackles WBC O2, ↑CO2 Patchy consolidation or poorly defined linear and nodular opacities Restrictive, obstructive, or mixed IFN-γ release assay (IGRA)

Acid-fast staining

Night sweat
Cardiovascular system Constrictive pericarditis[10] + + + + Muffled heart sounds Normal Normal Calcifications  Normal Chest CT scan Syncope
Pericardial effusion[23] +/- + + +/- +/- + Muffled heart sounds Normal Normal Fluid density around the heart Normal M-mode and 2-dimensional Doppler echocardiography Hoarseness, Palpitation
Neuromuscular disease Amyotrophic lateral sclerosis[24] +/- +/- Normal WBC Normal Normal Vt, ↑RV Revised El Escorial criteria (clinical) Muscle weakness, Dysphagia
Polymyositis/dermatomyositis[25] +/- + +/- Normal WBC Normal Normal Vt, ↑RV Muscle biopsy Muscle weakness, Heliotrope
Mitochondrial diseases[26] +/- Wheeze WBC, Plt Normal Normal Vt, ↑RV Muscle biopsy Muscle pain
Glycolytic enzyme defects (e.g., McArdle)[27] +/- +/- Normal Normal Normal Normal Vt, ↑RV Muscle biopsy (ragged red fibers) Myoglobinuria,

Muscle weakness

Systemic Ascites[28] Normal Normal Normal Peritoneal fluid accumulation Vt, ↑RV Abdominal ultrasound Abdominal distention
Kyphoscoliosis[29] Wheeze Normal Normal Deviated vertebral column Vt, ↑RV

(anatomical)

Standing lateral spine radiograph Low back pain
Obesity[30] Normal Normal O2 Normal Vt, ↑RV

(anatomical)

BMI Low stamina,

Sweating

References

Template:WikiDoc Sources

  1. Ravisagar, Patel; Abhinav, Singh; Mathur, R.M.; Anula, Sisodia (2015). “Eventration of diaphragm presenting as recurrent respiratory tract infections – A case report”. Egyptian Journal of Chest Diseases and Tuberculosis. 64 (1): 291–293. doi:10.1016/j.ejcdt.2014.10.002. ISSN 0422-7638.
  2. O’Horo JC, Rogus-Pulia N, Garcia-Arguello L, Robbins J, Safdar N (2015). “Bedside diagnosis of dysphagia: a systematic review”. J Hosp Med. 10 (4): 256–65. doi:10.1002/jhm.2313. PMC 4607509. PMID 25581840.
  3. Cantin, Luce; Bankier, Alexander A.; Eisenberg, Ronald L. (2009). “Bronchiectasis”. American Journal of Roentgenology. 193 (3): W158–W171. doi:10.2214/AJR.09.3053. ISSN 0361-803X.
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References

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Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

The incidence of diaphragmatic paralysis is unknown, but the incidence of diaphragmatic paralysis after cardiac surgery, radiologically, is approximately 30,000 to 75,000 per 100,000 individuals. Prevalence of diaphragmatic paralysis after cardiac surgery is 1,600 per 100,000 in children. The morbidity and mortality of the unilateral diaphragmatic paralysis is related to underlying pulmonary function and etiology. Most of the bilateral diaphragmatic paralysis are symptomatic and may develop ventilatory failure. Patients of all age groups may develop diaphragmatic paralysis. There is no racial predilection to diaphragmatic paralysis. Men are more commonly affected.

Epidemiology and Demographics

Incidence

  • The incidence of diaphragmatic paralysis is unknown, but the incidence of diaphragmatic paralysis after cardiac surgery is approximately 30,000 to 75,000 per 100,000 individuals.[1]

Prevalence

The prevalence of diaphragmatic paralysis is unknown, but prevalence of diaphragmatic paralysis after cardiac surgery is 1,600 per 100,000 in children. [2]

Case-fatality rate/Mortality rate

  • Most of the unilateral diaphragmatic paralysis are asymptomatic and are discovered incidentally during imaging and mortality rate are low.
  • Most of the bilateral diaphragmatic paralysis are symptomatic and may develop ventilatory failure.

Age

  • Patients of all age groups may develop diaphragmatic paralysis.

Race

  • There is no racial predilection to diaphragmatic paralysis.

Gender

  • Men are more commonly affected by diaphragmatic paralysis than women.[4]

References

  1. Efthimiou J, Butler J, Woodham C, Benson MK, Westaby S (1991). “Diaphragm paralysis following cardiac surgery: role of phrenic nerve cold injury”. Ann. Thorac. Surg. 52 (4): 1005–8. PMID 1929616.
  2. de Leeuw M, Williams JM, Freedom RM, Williams WG, Shemie SD, McCrindle BW (1999). “Impact of diaphragmatic paralysis after cardiothoracic surgery in children”. J. Thorac. Cardiovasc. Surg. 118 (3): 510–7. doi:10.1016/S0022-5223(99)70190-X. PMID 10469969.
  3. Canbaz S, Turgut N, Halici U, Balci K, Ege T, Duran E (2004). “Electrophysiological evaluation of phrenic nerve injury during cardiac surgery–a prospective, controlled, clinical study”. BMC Surg. 4: 2. doi:10.1186/1471-2482-4-2. PMC 320489. PMID 14723798.
  4. Lagueny A, Ellie E, Saintarailles J, Marthan R, Barat M, Julien J (1992). “Unilateral diaphragmatic paralysis: an electrophysiological study”. J. Neurol. Neurosurg. Psychiatry. 55 (4): 316–8. PMC 489048. PMID 1583519.

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Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

The most potent risk factor in the development of diaphragmatic paralysis is cooling or streching in cardiac surgery. Other risk factors include viruses, spinal cord transection and malnutrition.

Risk Factors

Common Risk Factors

  • Common risk factors in the development of diphragmatic paralysis include:[1]

Less Common Risk Factors

  • Less common risk factors in the development of diaphragmatic paralysis include:

References

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Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

There is insufficient evidence to recommend routine screening for diaphragmatic paralysis.

Screening

There is insufficient evidence to recommend routine screening for diaphragmatic paralysis.

References

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Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2]

Overview

If left untreated, patients with unilateral diaphragmatic paralysis may recover fully or partially. Common complication of diaaphragmatic paralysis includes severe pulmonary dysfunction in bilateral diaphragmatic paralysis. Prognosis is generally excellent in unilateral diaphragmatic paralysis and bilateral diaphragmatic paralysis is a medical emergency.

Natural History, Complications, and Prognosis

Natural History

  • If left untreated, patients with unilateral diaphragmatic paralysis may recover fully or partially. Most of the bilateral diaphragmatic paralysis are symptomatic and may develop ventilatory failure. [1]

Complications

  • Common complication of diaphragmatic paralysis include:
    • Severe pulmonary dysfunction in bilateral diaphragmatic paralysis.

Prognosis

  • Prognosis is generally excellent in unilateral diaphragmatic paralysis.[2]
  • Bilateral diaphragmatic paralysis is a medical emergency

References

  1. Byron WA (1983). “Respiratory function after paralysis of the right hemidiaphragm”. Am. Rev. Respir. Dis. 127 (6): 788. doi:10.1164/arrd.1983.127.6.788. PMID 6859664.
  2. Qureshi A (2009). “Diaphragm paralysis”. Semin Respir Crit Care Med. 30 (3): 315–20. doi:10.1055/s-0029-1222445. PMID 19452391.

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Diagnosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

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