Diaphragm Disorders_ [Print

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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

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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


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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


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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.

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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

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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.

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Diaphragm Disorders_ [Print