Megaoesophagus

Intorduction

Megaoesophagus describes a dilated, hypomotile oesophagus that is not able to effectively transport food from the the pharnyx to the stomach. The condition may be congenital or acquired and, although it occurs in most of the major domestic species, it is most common in dogs and cats. The following sections describe the causes of megaoesophagus.

Congenital Megaoesophagus

Congenital Idiopathic Megaoesophagus

Megaoesophagus (Copyright Alun Williams 2009 (RVC))

Congenital megaoesophagus is sometimes called 'congenital achalasia' but this term implies a defect of the cardiac sphincter of the stomach (as in man) whereas the canine condition affects the whole oesophagus. This disease is inherited in a number of breeds of dog, including the fox terrier (in an autosomal recessive manner), miniature Schnauzer (dominantly with 60% penetrance), great Dane, Irish setter, Chinese Shar-pei, Newfoundland and German shepherd. It is also an inherited disorder of the Siamese cat where it may occur with concurrent pyloric stenosis.

The disease is thought to result from a delay in the maturation of either upper motor neurones in the central swallowing centre in the brainstem or of the afferent sensory arm of the peristaltic reflex. The resultant hypomotility of the oesophagus leads to a functional obstruction and boluses of food do not reach the stomach and are eventually regurgitated. The condition is seen most commonly at 6-7 months during a period of rapid growth. Affected animals eat, regurgitate shortly afterwards and may then attempt to eat again. As with any disease that results in chronic regurgitation, affected animals lose weight due to malnutrition and may also develop aspiration pneumonia.

Myasthenia Gravis

Myasthenia gravis is a disease of the neuromuscular junction that occurs due to a defect or absence of nicotinic acetylcholine receptors on the post-synaptic membrane (in the congenital form) or due to the presence of antibodies that bind to the receptors and prevent them from functioning normally (in the acquired form). The classical focal form of myasthenia gravis affects only the extraocular muscles, the cranial oesophagus and the muscles innervated by cranial nerves V, VII and IX. 80% of animals diagnosed with myasthenia gravis have megaoesophagus at presentation.

Acquired Megaoesophagus

Megaoesophagus may occur in any animal in which there is a disruption of the brain centres, reflex arcs or muscles responsible for normal peristalsis and tone along the oesophagus. Potential causes include:

• Brain Centres
  • Viral encephalitides including those syndromes caused by canine distemper virus.
  • Presence of toxins, including ingestion of lead, mercury, organophosphates and botulinum toxin and exposure to tetanus toxin
• Reflex arcs
  • Peripheral neuropathies, including labrador retriever myopathy and giant axonal neuropathy of German Shepherd dogs
  • Feline dysautonomia, also know as Key-Gaskell Syndrome.
• Muscle
  • Myositis and polymyositis/polyneuritis
  • Glycogen storage disease type II.
  • Muscle weakness as occurs with severe hypokalaemia and hypoadrenocorticism (Addison's disease).
• Any cause of prolonged oesophageal obstruction may result in megaoesophagus which eventually becomes irreversible if the underlying cause is not removed. Examples include foreign bodies, neoplasia and strictures.

The result is the presence of a hypotonic oesophagus which is distended by accumulated food material. The thoracic oesophagus from the thoracic inlet to the diaphragm is usually dilated and affected animals regurgitate repeatedly, lose weight and may develop aspiration pneumonia.

Diagnosis

Diagnostic Imaging

Most cases of megaoesophagus can be diagnosed on plain radiographs of the chest. The following features may be observed:

• A large radiolucent structure dorsal to the trachea on lateral radiographs. It should be noted that it is normal for the oesophagus to contain air if the animal is under general anaesthesia.
• The tissue between the trachea and oesophagus may be compressed producing a radio-opaque tracheal stripe sign along the dorsal surface of the trachea on a lateral radiograph. The trachea may also appear to deviate ventrally. On a dorso-ventral radiograph of the chest, the trachea may be pushed to the right by the enlarged oesophagus.
• Chronic regurgitation increases the risk of developing aspiration pneumonia. If it is present, it produces an alveolar pattern in the cranial and ventral lung lobes.
• With vascular ring anomalies, the oesophagus is usually only dilated cranial to the base of the heart.

The presence of megaoesophagus can be confirmed by feeding barium contrast medium, either as a liquid or mixed with meat (a 'barium meal'). The advantage of the barium meal is that it distends the oesophagus to show the extent of its dilation and it provides better definition of the point of constriction in animals with a vascular ring anomaly.

Endoscopy allows the operator to visualise the dilated oesophagus directly but this is rarely required to reach a diagnosis.

Diagnostic Tests

Hematological and biochemical analysis of blood samples may be useful to diagnose several underlying causes of acquired megaoesophagus, such as myositis with an elevated creatine kinase (CK) concentration. Further tests, such as an ACTH stimulation test, may also be indicated depending on the suspected cause. Congenital idiopathic megaoesophagus is diagnosed in young animals (often of a predisposed breed) in which all other causes have been discounted.

Treatment

If the underlying cause is found then this should be treated.

• Small boluses of food OR a liquid gruel should be fed to the animal little and often, depending on preference of the dog and owner, as this reduces the risk of aspiration.
• The forelimbs of the animal should be raised for 10-15 minutes after each to meal to allow the food to reach the stomach.
• The animal should be monitored constantly by its owner for the development of signs of aspiration pneumonia, a frequent recurrent problem in animals with congenital megaoesophagus. This condition should be treated aggressively with intra-venous bactericidal antibiotics (preferably selected on the basis of results from a culture of a broncho-alveolar lavage), coupage and nebulisation.
  

Prognosis

Good to poor; depending on underlying cause.

Sudden death due to aspiration pneumonia is a risk that may occur at any time.

References

Small animal internal medicine