Upload
sadbad6
View
220
Download
0
Embed Size (px)
Citation preview
7/27/2019 Diaphragm Disorders_ [Print..
1/16
7/27/2019 Diaphragm Disorders_ [Print..
2/16
7/27/2019 Diaphragm Disorders_ [Print..
3/16
Di h Di d [P i t] M di i P l l htt // di i d / ti l /298107 i
7/27/2019 Diaphragm Disorders_ [Print..
4/16
Congenital hernias
Respiratory distress and/or cyanosis may occur within the first 24 hours of life.
If the defect is small enough, patients often remain asymptomatic for years or even decades.
Traumatic rupture
The acute phase manifests with abdominal pain, concurrent intra-abdominal and intrathoracic injuries, respiratory distress, and cardiac dysfunction.
Latent-phase symptoms include upper GI complaints, pain in the left-upper quadrant or chest, pain in the left shoulder, dyspnea, and orthopnea.
The GI obstructive phase manifests with nausea and vomiting with unrelenting abdominal pain, prostration, and respiratory distress.
Neurologic causes
Most patients with unilateral paralysis are asymptomatic. Manifestations include mild exertional dyspnea, generalized muscle fatigue, chest wall pain, and resting
dyspnea while lying with the paralyzed side down. Symptoms are generally more severe in patients with concomitant lung disease.
Bilateral paralysis manifests with shortness of breath, severe exertional dyspnea, and marked orthopnea.8
The orthopnea of bilateral diaphragmatic paralysis is
dramatic and occurs within minutes after assuming the recumbent position. Orthopnea is associated with tachypnea and rapid, shallow breathing. Patients have a poor
quality of s leep, which may cause fatigue. Significant orthopnea sometimes triggers a cardiac workup.
Physical
Physical findings upon examination vary depending on the etiology.
Congenital hernias
Right-sided heart
Decreased breath sounds on affected side
Scaphoid abdomen
Auscultation of bowel sounds in the thorax
Traumatic rupture
Marked respiratory distress
Decreased breath sounds on affected side
Palpation of abdominal contents in the chest upon insertion of chest tube
Auscultation of bowel sounds in chest
Paradoxical movement of abdomen with breathing
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine.medscape.com/article/298107-prin
4 of 16 23/02/1430 06:20
Diaphragm Disorders: [Print] eMedicine Pulmonology http://emedicine medscape com/article/298107 prin
7/27/2019 Diaphragm Disorders_ [Print..
5/16
Neurologic causes
Decreased breath sounds
Generalized or focal neurologic deficits
Dullness on lower chest upon percussion on the involved side
Excursion of involved hemithorax decreased compared with healthy side
Paralysis
Paradoxical abdominal wall retraction during inspiration (best appreciated on supine position)
Hypoxemia, secondary to atelectasis-induced ventilation-perfusion mismatch, exacerbated in supine position
Signs of cor pulmonale occasionally present
Causes
The etiology of diaphragmatic dysfunction is most easily separated into anatomic or neurologic causes.
Anatomic defects
Congenital defects - Bochdalek hernia, Morgagni hernia, eventration of the diaphragm, and diaphragmatic agenesis
Acquired defects - Traumatic rupture, penetrating injuries, idiopathic etiologies, and iatrogenic responses to surgery or other invasive procedures
Innervation defects
Brain stem stroke
Spinal cord disorders - Trauma to the cervical spinal cord, syringomyelia, poliomyelitis, anterior horn cell disease, amyotrophic lateral sclerosis, and motor neuron
disease
Phrenic nerve neuropathy9
- Trauma to the phrenic nerve from surgery,10
radiation,11
or tumor; Guillain-Barr syndrome; diabetic, nutritional, and alcoholic neuropathy;
vasculitic neuropathy; lead and poison neuropathy; and infection-related nerve injury (eg, diphtheria, tetanus, typhoid, measles, botulism)
Myasthenia gravis
Muscular disorders - Myotonic dystrophies, Duchenne muscular dystrophy, and metabolic myopathies
Idiopathic etiologies
Postpolio syndrome presenting as isolated diaphragmatic paralysis
Phrenic nerve injury due to cold cardioplegia during cardiac surgery10
Thyroid disorders
Postviral neuropathy
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine.medscape.com/article/298107-prin
5 of 16 23/02/1430 06:20
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine medscape com/article/298107-prin
7/27/2019 Diaphragm Disorders_ [Print..
6/16
Connective-tissue disease (eg, systemic lupus erythematosus, rheumatoid arthritis) - Can lead to progressive shrinking lung syndrome
Acid maltase deficiency
Malnutrition12
Neurologic causes of diaphragmatic paralysis
Spinal cord transaction13
Multiple sclerosis
Amyotrophic lateral sclerosis
Cervical spondylosis
Poliomyelitis
Guillain-Barr syndrome
Phrenic nerve dysfunction
Compression by tumor
Cardiac surgery cold injury10
Blunt trauma14, 15
Idiopathic phrenic neuropathy
Postviral phrenic neuropathy
Radiation therapy11
Cervical chiropractic manipulation16
Myopathic causes of diaphragmatic paralysis
Limb-girdle dystrophy
Hyperthyroidism or hypothyroidismMalnutrition
Acid maltase deficiency
Connective-tissue diseases
Systemic lupus erythematosus
Dermatomyositis
Mixed connective-tissue disease
Amyloidosis
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine.medscape.com/article/298107-prin
6 of 16 23/02/1430 06:20
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine.medscape.com/article/298107-prin
7/27/2019 Diaphragm Disorders_ [Print..
7/16
Infection
Herpes zoster
Idiopathic myopathy
Differential Diagnoses
Decreased pulmonary or abdominal compliance
Pleural adhesions
Other Problems to Be Considered
Fractures, Cervical Spine
Guillain-Barr Syndrome
Myasthenia Gravis
Cervical fracture
Cerebral hemorrhage or ischemia
Numerous neuropathies
Injury to phrenic nerve from trauma
Injury to phrenic nerve from neoplasm
Injury to phrenic nerve from surgery (most commonly from cardiac surgery due to cold cardioplegia)
Alveolar hypoventilation caused by brainstem or higher cervical spinal injury17
Anterior horn cell or neuromuscular junction disease to differentiate from phrenic nerve dysfunction
Workup
Laboratory Studies
Laboratory studies are limited to discovery of neuropathic causes of diaphragmatic dysfunction.
Arterial blood gas determinations may show hypoxemia with underlying V/Q mismatch and progressive hypercapnia as respiratory failure develops.
Diaphragm Disorders: [Print] eMedicine Pulmonology http://emedicine.medscape.com/article/298107 prin
7 of 16 23/02/1430 06:20
7/27/2019 Diaphragm Disorders_ [Print..
8/16
7/27/2019 Diaphragm Disorders_ [Print..
9/16
7/27/2019 Diaphragm Disorders_ [Print..
10/16
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine.medscape.com/article/298107-prin
7/27/2019 Diaphragm Disorders_ [Print..
11/16
Medication is limited to the etiology of neurologic involvement.
Follow-up
Further Outpatient Care
Once an anatomic defect is corrected, the patient should undergo periodic chest radiography and assessment of pulmonary function. Although the rate of spontaneous
recurrence of a repaired diaphragmatic hernia is low, small defects in the repair site have been reported. Therefore, surveillance is important.
If dysfunction was secondary to a tumor encroaching on the phrenic nerve, maintaining close follow-up contact with the patient is important to ensure that the neoplasm has not
recurred.
Complications
Anatomic defects may lead to respiratory failure, incarceration or strangulation of bowel, or hypoplasia of the lung in congenital defects.
Neurologic problems may lead to respiratory failure.
Prognosis
Patients with anatomic repairs
The prognosis for patients with anatomic repairs from traumatic rupture directly correlates with the extent of concomitant injuries.
Neonates generally have a good prognosis after repair of congenital diaphragmatic hernias, but the prognosis is directly related to the development of the lung on the
affected side.
Patients with neurologic conditions
The prognosis for patients with neurologic conditions generally correlates with etiology.
Persons with high cervical spine fractures generally fare worse than individuals with transient neuropathies such as Guillain-Barr syndrome.
Idiopathic diaphragmatic disease has a variable prognosis, with some patients recovering spontaneously.
Miscellaneous
Medicolegal Pitfalls
Failure to conduct periodic chest radiography and assessment of pulmonary function once an anatomic defect is corrected
Failure to maintain close follow-up contact if dysfunction was secondary to a tumor encroaching on the phrenic nerve
11 of 16 23/02/1430 06:20
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine.medscape.com/article/298107-prin
7/27/2019 Diaphragm Disorders_ [Print..
12/16
In patients with bilateral diaphragmatic dysfunction, failing to recognize the diagnosis until the patient presents with cor pulmonale and/or cardiorespiratory failure
Multimedia
Media file 1: Radiograph of a man who fell 45 ft from scaffolding, through plate glass windows, and onto the ground. Intraoperatively, he had a
completely avulsed diaphragm on the left side. The patient subsequently recovered after a 45-day hospital course of treatment.
References
Rochester DF. The diaphragm: contractile properties and fatigue. J Clin Invest. May 1985;75(5):1397-402. [Medline].1.
Wiseman NE, MacPherson RI. "Acquired" congenital diaphragmatic hernia. J Pediatr Surg. Oct 1977;12(5):657-65. [Medline].2.
Shah R, Sabanathan S, Mearns AJ, Choudhury AK. Traumatic rupture of diaphragm.Ann Thorac Surg. Nov 1995;60(5):1444-9. [Medline].3.
Sharma OP. Traumatic diaphragmatic rupture: not an uncommon entity--personal experience with collective review of the 1980's. J
Trauma. May 1989;29(5):678-82. [Medline].
4.
Grmoljez PF, Lewis JE Jr. Congenital diaphragmatic hernia: Bochdalek type.Am J Surg. Dec 1976;132(6):744-6. [Medline].5.
12 of 16 23/02/1430 06:20
7/27/2019 Diaphragm Disorders_ [Print..
13/16
7/27/2019 Diaphragm Disorders_ [Print..
14/16
7/27/2019 Diaphragm Disorders_ [Print..
15/16
Diaphragm Disorders: [Print] - eMedicine Pulmonology http://emedicine.medscape.com/article/298107-prin
7/27/2019 Diaphragm Disorders_ [Print..
16/16
All Rights Reserved
(http://www.medscape.com/public/copyright)
16 of 16 23/02/1430 06:20