Difference between revisions of "Megaoesophagus"

Revision as of 11:24, 28 May 2010

Congenital Achalasia - Megaoesophagus

Megaoesophagus (Courtesy of Alun Williams (RVC))

This condition has been termed achalasia but this implies (in man) a cardiac sphincter defect, which is not found in the dog.
A functional rather than a physical blockage

Inherited disorder in several breeds:
- Autosomal recessive in Fox Terrier.
- Also in Miniature Schnauzer, Great Dane, German Shepherds and others.
- Also in Siamese cats.

Clinical

See at approx. 6-7 months of age when growing quickly.
Eats food, vomits, eats again and vomits again because oesophagus has no tone to it.
Oesophagus may become extremely dilated producing a megaoesophagus.
Can affect the whole length of oesophagus.

Pathogenesis

Appears to be due to due to delay in maturation of oesophageal innervation, either:
- in upper motor neurones of central swallowing centre or
- in the afferent sensory arm of the reflex involved with peristalsis.

If dog eats from raised bowel may be satisfactory as food goes down by gravity.
Once in stomach it is passed on normally.

Megoesophagus Secondary to Dextra-Aorta

Dextra-aorta (Courtesy of Alun Williams (RVC))

Vascular rings are congenital abnormalities of the aortic arch system which interfere with oesophageal function. Normally, the aortic arch, pulmonary artery and ductus arteriosus (ligamentum arteriosum) all lie on the left side of the oesophagus.
However, when there is anomalous development of the aortic arch, for example in the right-sided arch, the oesophagus becomes enclosed within a ring formed by the heart base, aorta, ductus arteriosus and pulmonary artery.
The compression and stricture produced by the “ring” prevents passage of solid food beyond the heart base and food accumulates rostral (cranial) to the obstruction, resulting in dilatation or megaoesophagus.
In most cases food is eventually regurgitated undigested.
The condition is seen in pups just after weaning and if these are presented for treatment before severe dilatation occurs, then surgical correction of the ring (if possible) offers a good prognosis.

Secondary to Myasthenia Gravis

Secondary to Key-Gaskell Syndrome

Acquired megaoesophagus

Occurs spontaneously in adult in any disorder that disrupts normal reflex involved in swallowing either peripheral or central.
These can include:
- Viral encephalitides
- Peripheral neuropathies
  - Degeneration or inactivity of local myenteric nerve plexuses (Auerbach’s plexuses),
  - or to lesions in the vagus nerve or the central nucleus in the medulla of the brain which supply the oesophagus.
- Poisonings such as:
  - Lead
  - Tetanus
  - Botulism
- Myopathies.

The result is the presence of a hypotonic oesophagus in which food accumulates causing distension
Usually the thoracic oesophagus from the thoracic inlet to the diaphragm is affected and grossly the oesophagus is dilated, thin-walled and hypotonic.

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-{{OpenPagesTop}}
+===Congenital Achalasia - Megaoesophagus===
-== Introduction  ==
+[[Image:Megaoes.gif|left|thumb|125px|<small><center>Megaoesophagus (Courtesy of Alun Williams (RVC))</center></small>]]
+*This condition has been termed achalasia but this implies (in man) a cardiac sphincter defect, which is not found in the dog.
+*A functional rather than a [[#Traumatic Pathology|physical blockage]]
-Megaoesophagus describes a dilated, hypomotile oesophagus that is not able to effectively transport food from the [[Pharynx - Anatomy & Physiology|pharnyx]] to the [[Monogastric Stomach - Anatomy & Physiology|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.
+*Inherited disorder in several breeds:
+**Autosomal recessive in Fox Terrier.
+**Also in Miniature Schnauzer, Great Dane, German Shepherds and others.
+**Also in Siamese cats.
-== Congenital Megaoesophagus  ==
+====Clinical====
+*See at approx. 6-7 months of age when growing quickly.
+*Eats food, [[Control of Feeding - Anatomy & Physiology#The Vomit Reflex|vomits]], eats again and [[Control of Feeding - Anatomy & Physiology#The Vomit Reflex|vomits]] again because [[Oesophagus - Anatomy & Physiology|oesophagus]] has no tone to it.
+*[[Oesophagus - Anatomy & Physiology|Oesophagus]] may become extremely dilated producing a megaoesophagus.
+*Can affect the whole length of [[Oesophagus - Anatomy & Physiology|oesophagus]].
-=== Congenital Idiopathic Megaoesophagus  ===
+====Pathogenesis====
+*Appears to be due to due to delay in maturation of [[Oesophagus - Anatomy & Physiology|oesophageal]] innervation, either:
+**in upper motor neurones of central swallowing centre or
+**in the afferent sensory arm of the reflex involved with peristalsis.
-[[Image:Megaoes.gif|thumb|left|125px|<small><center>Megaoesophagus (Alun Williams 2009 (RVC))</center></small>]] 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.
+*If dog eats from raised bowel may be satisfactory as food goes down by gravity.
+*Once in [[Forestomach - Anatomy & Physiology|stomach]] it is passed on normally.
-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'''.
+===Megoesophagus Secondary to Dextra-Aorta===
+[[Image:Praa.gif|right|thumb|125px|<small><center>Dextra-aorta (Courtesy of Alun Williams (RVC))</center></small>]]
+*Vascular rings are congenital abnormalities of the aortic arch system which interfere with [[Oesophagus - Anatomy & Physiology|oesophageal]] function. Normally, the aortic arch, pulmonary artery and ductus arteriosus (ligamentum arteriosum) all lie on the left side of the [[Oesophagus - Anatomy & Physiology|oesophagus]].
+*However, when there is anomalous development of the aortic arch, for example in the [[right-sided arch]], the [[Oesophagus - Anatomy & Physiology|oesophagus]] becomes enclosed within a ring formed by the '''heart base''', '''aorta''', '''ductus arteriosus''' and '''pulmonary artery'''.
+*The compression and stricture produced by the “ring” prevents passage of solid food beyond the heart base and food accumulates rostral (cranial) to the obstruction, resulting in dilatation or megaoesophagus.
+*In most cases food is eventually regurgitated undigested.
+*The condition is seen in pups just after weaning and if these are presented for treatment before severe dilatation occurs, then surgical correction of the ring (if possible) offers a good prognosis.
-=== [[Myasthenia Gravis]]  ===
+===Secondary to [[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 congential form is primarily seen in breeds such as the Minitaure Daschund and Jack Russell Terrier and signs are usually seen from 6-8weeks of age. The acquired form occurs from 6+ months and is seen in breeds such as Labrador, Golden Retreiver and German Shepherd Dogs. 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 and occurs in around 30% of cases. The generalised, exercise intolerance plus megaoesophagus form, is seen in 60% of cases. 80% of animals diagnosed with myasthenia gravis have megaoesophagus at presentation.
+===Secondary to [[Key-Gaskell Syndrome]]===
-== Acquired Megaoesophagus  ==
+===Acquired megaoesophagus===
+*Occurs spontaneously in adult in any disorder that disrupts normal reflex involved in swallowing either peripheral or central.
+*These can include:
+**Viral encephalitides
+**Peripheral neuropathies
+***Degeneration or inactivity of local myenteric nerve plexuses (Auerbach’s plexuses),
+***or to lesions in the vagus nerve or the central nucleus in the medulla of the brain which supply the [[Oesophagus - Anatomy & Physiology|oesophagus]].
+**Poisonings such as:
+***Lead
+***Tetanus
+***Botulism
+**Myopathies.
-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:
+*The result is the presence of a hypotonic [[Oesophagus - Anatomy & Physiology|oesophagus]] in which food accumulates causing distension
+*Usually the thoracic oesophagus from the thoracic inlet to the diaphragm is affected and grossly the [[Oesophagus - Anatomy & Physiology|oesophagus]] is dilated, thin-walled and hypotonic.
-'''Brain Centres'''
-*'''Viral encephalitides''' including those syndromes caused by '''[[Canine Distemper Virus|canine distemper virus]]'''.
-*'''Presence of toxins''', including ingestion of '''lead''', '''mercury''', '''organophosphates''' and '''botulinum toxin''' and exposure to '''tetanus toxin'''
-'''Reflex arcs'''
+[[Category:Oesophagus - Pathology]]
-*'''Peripheral neuropathies''', including Labrador Retriever myopathy and giant axonal neuropathy of German Shepherd dogs
+[[Category:Cat]][[Category:Dog]]
-*'''Feline dysautonomia''', also know as [[Oesophageal Dysautonomia|Key-Gaskell Syndrome]].
-'''Muscle'''
-*'''Myositis''' and '''polymyositis/polyneuritis'''
-*'''Glycogen storage disease''' type II.
-'''Muscle weakness''' as occurs with severe '''hypokalaemia''' and '''[[Hypoadrenocorticism|hypoadrenocorticism]]''' (Addison's disease).
-Any cause of '''prolonged [[Impaction of the Oesophagus|oesophageal obstruction]]''' may result in megaoesophagus which eventually becomes irreversible if the underlying cause is not removed. Examples include [[Oesophageal Foreign Body|foreign bodies]], [[Oesophageal Neoplasia|neoplasia]] and [[Oesophageal Stricture|strictures]].
-The result is the presence of a hypotonic [[Oesophagus - Anatomy & Physiology|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  ==
-Primarily, a full history and clinical examination is required to assess that regurgitation rather than vomiting is occurring.
-=== 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  ===
-<br>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 or T4/ TSH levels, may also be indicated if hypoadrenocortiscm is 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 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.
-*Gastrostomy tubes have been shown to decreased the amount of regurgitation, however, some animals may still regurgitate saliva.<br>
-== Prognosis  ==
-Good to poor; depending on underlying cause.
-Sudden death due to '''aspiration pneumonia''' is a risk that may occur at any time.
-{{Learning
-|Vetstream = [https://www.vetstream.com/canis/Content/Disease/dis00989.asp, Megaoesophagus]
-}}
-== References  ==
-Blood, D.C. and Studdert, V. P. (1999)''' Saunders Comprehensive Veterinary Dictionary''' (2nd Edition)'' Elsevier Science ''
-Ettinger, S.J. and Feldman, E. C. (2000) '''Textbook of Veterinary Internal Medicine Diseases of the Dog and Cat Volume 2''' (Fifth Edition) ''W.B. Saunders Company ''
-Ettinger, S.J, Feldman, E.C. (2005) '''Textbook of Veterinary Internal Medicine '''(6th edition, volume 2) ''W.B. Saunders Company''
-Fossum, T. W. et. al. (2007) '''Small Animal Surgery''' (Third Edition) ''Mosby Elsevier''
-Nelson, R.W. and Couto, C.G. (2009)''' Small Animal Internal Medicine '''(Fourth Edition) ''Mosby Elsevier''
-{{review}}
-{{OpenPages}}
-[[Category:Oesophagus_-_Pathology]] [[Category:Oesophageal_Diseases_-_Cat]] [[Category:Oesophageal_Diseases_-_Dog]][[Category:Expert Review - Small Animal]][[Category:Expert Review - Farm Animal]][[Category:Expert Review - Horse]] [[Category:Oesophageal_Diseases_-_Cattle]] [[Category:Oesophageal_Diseases_-_Horse]]

Difference between revisions of "Megaoesophagus"

Revision as of 11:24, 28 May 2010

Contents

Congenital Achalasia - Megaoesophagus

Clinical

Pathogenesis

Megoesophagus Secondary to Dextra-Aorta

Secondary to Myasthenia Gravis

Secondary to Key-Gaskell Syndrome

Acquired megaoesophagus

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