Difference between revisions of "Megaoesophagus"

Revision as of 14:38, 7 July 2010

Description

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 Causes of Megaoesophagus

Congenital Achalasia

Megaoesophagus (Copyright Alun Williams 2009 (RVC))

Congenital megaoesophagus is sometimes called 'congenital achalasia' but this 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.

Diagnosis, treatment, prognosis.

Vascular Ring Anomalies

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

Vascular rings are congenital abnormalities of the aortic arch system which interfere with oesophageal function. Many vascular anomalies (such as an aberrant right subclavian artery) are clinically silent and up to 20% of dogs and cats possess such an anomaly. Clinically significant anomalies are found most commonly in large breed dogs, particularly Irish setters and German Shepherd dogs.

The most common clinically significant anomaly is a dextra-aorta or persistent right aortic arch, in which the aorta is formed by the fourth right branchial arch rather than the fourth left arch. In the normal animal, the aorta, pulmonary artery and ductus arteriosus (which connects the former vessels) all lie of the left side of the oesophagus but, when the aorta develops on the right, the ductus arteriosus must pass across the oesophagus to reach the aorta. The oesophagus is then trapped within a ring formed by the aorta (on the right), the main pulmonary artery (on the left), the ductus arteriosus (or ligamentum arteriosum) dorsally and the heart base ventrally. The compression and stricture produced by the vascular ring prevents the passage of solid food beyond the heart base and food accumulates cranial to the obstruction. This results in dilation (megaoesophagus) which is usually confined to the cranial thoracic region but, in chronic cases, may extend along its whole length.

The condition usually becomes apparent at weaning when animals start to eat solid food that cannot pass through the constricted region. Affected animals tend to regurgitate undigested solid food but, if they are presented for treatment before severe dilation occurs, surgical correction of the ring (if possible) should result in a good prognosis.

Myasthenia Gravis

The classical focal form of myasthenia gravis affects 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. Myasthenia gravis may itself be either congenital or acquired.

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.
- Ingestion of toxins, including lead, mercury, botulinum toxin and absorption of 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
- Muscle weakness as occurs with severe hypokalaemia and hypoadrenocorticism (Addison's disease).

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 affected affected animals regurgitate repeatedly, lose weight and may develop aspiration pneumonia.

Diagnosis

Diagnostic Tests

These may useful to diagnose several underlying causes for acquired megaoesophagus.

Diagnostic Imaging

Any form of megaoesophagus can be diagnosed by various forms of diagnostic imaging.

Treatment

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-{{OpenPagesTop}}
+{{unfinished}}
-== Introduction  ==
-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.
+==Description==
+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 Causes of Megaoesophagus===
+====Congenital Achalasia====
+[[Image:Megaoes.gif|left|thumb|125px|<small><center>Megaoesophagus (Copyright Alun Williams 2009 (RVC))</center></small>]]
+Congenital megaoesophagus is sometimes called 'congenital achalasia' but this 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.
-=== Congenital Idiopathic Megaoesophagus  ===
+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.
-[[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.
+Diagnosis, treatment, prognosis.
-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'''.
+====[[Vascular Ring Anomalies|Vascular Ring Anomalies]]====
+[[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. Many vascular anomalies (such as an aberrant right subclavian artery) are clinically silent and up to 20% of dogs and cats possess such an anomaly.  Clinically significant anomalies are found most commonly in large breed dogs, particularly Irish setters and German Shepherd dogs.
-=== [[Myasthenia Gravis]]  ===
+The most common clinically significant anomaly is a '''dextra-aorta''' or '''persistent right aortic arch''', in which the aorta is formed by the fourth right branchial arch rather than the fourth left arch.  In the normal animal, the aorta, pulmonary artery and ductus arteriosus (which connects the former vessels) all lie of the left side of the oesophagus but, when the aorta develops on the right, the ductus arteriosus must pass across the oesophagus to reach the aorta.  The oesophagus is then trapped within a ring formed by the '''aorta''' (on the right), the '''main pulmonary artery''' (on the left), the '''ductus arteriosus''' (or ligamentum arteriosum) dorsally and the '''heart base''' ventrally.  The compression and stricture produced by the vascular ring prevents the passage of solid food beyond the heart base and food accumulates cranial to the obstruction.  This results in dilation (megaoesophagus) which is usually confined to the cranial thoracic region but, in chronic cases, may extend along its whole length.
-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.
+The condition usually becomes apparent at weaning when animals start to eat solid food that cannot pass through the constricted region.  Affected animals tend to regurgitate undigested solid food but, if they are presented for treatment before severe dilation occurs, surgical correction of the ring (if possible) should result in a good prognosis.
-== Acquired Megaoesophagus  ==
+====[[Myasthenia Gravis]]====
+The classical '''focal''' form of myasthenia gravis affects 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.  Myasthenia gravis may itself be either congenital or acquired.
-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:
+===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'''.
+**'''Ingestion of toxins''', including '''lead''', '''mercury''', '''botulinum toxin''' and absorption of '''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'''
+**'''Muscle weakness''' as occurs with severe '''hypokalaemia''' and '''hypoadrenocorticism''' (Addison's disease).
-'''Brain Centres'''
+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 affected affected animals regurgitate repeatedly, lose weight and may develop aspiration pneumonia.
-*'''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'''
+==Diagnosis==
-*'''Peripheral neuropathies''', including Labrador Retriever myopathy and giant axonal neuropathy of German Shepherd dogs
+===Diagnostic Tests===
-*'''Feline dysautonomia''', also know as [[Oesophageal Dysautonomia|Key-Gaskell Syndrome]].
+These may useful to diagnose several underlying causes for acquired megaoesophagus.
-'''Muscle'''
+===Diagnostic Imaging===
-*'''Myositis''' and '''polymyositis/polyneuritis'''
+Any form of megaoesophagus can be diagnosed by various forms of diagnostic imaging.
-*'''Glycogen storage disease''' type II.
-'''Muscle weakness''' as occurs with severe '''hypokalaemia''' and '''[[Hypoadrenocorticism|hypoadrenocorticism]]''' (Addison's disease).
+==Treatment==
-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.
+[[Category:Oesophagus - Pathology]]
+[[Category:Cat]][[Category:Dog]]
-== Diagnosis  ==
+[[Category:To_Do_-_James]]
-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]]