Difference between revisions of "Megaoesophagus"

From WikiVet English
Jump to navigation Jump to search
Line 1: Line 1:
===Congenital Achalasia - Megaoesophagus===
+
{{unfinished}}
[[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]]
 
  
*Inherited disorder in several breeds:
+
==Description==
**Autosomal recessive in Fox Terrier.
+
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.
**Also in Miniature Schnauzer, Great Dane, German Shepherds and others.
 
**Also in Siamese cats.
 
  
====Clinical====
+
===Congenital Causes of Megaoesophagus===
*See at approx. 6-7 months of age when growing quickly.
+
====Congenital Achalasia====
*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.
+
[[Image:Megaoes.gif|left|thumb|125px|<small><center>Megaoesophagus (Copyright Alun Williams 2009 (RVC))</center></small>]]
*[[Oesophagus - Anatomy & Physiology|Oesophagus]] may become extremely dilated producing a megaoesophagus.   
+
This condition 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 oesophagusThis 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.
*Can affect the whole length of [[Oesophagus - Anatomy & Physiology|oesophagus]].
 
  
====Pathogenesis====
+
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.
*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.
 
  
*If dog eats from raised bowel may be satisfactory as food goes down by gravity.
+
Diagnosis, treatment, prognosis.
*Once in [[Forestomach - Anatomy & Physiology|stomach]] it is passed on normally.
 
  
===Megoesophagus Secondary to Dextra-Aorta===
+
 
 +
 
 +
 
 +
====[[Vascular Ring Anomaly|Vascular Ring Anomalies]]====
 
[[Image:Praa.gif|right|thumb|125px|<small><center>Dextra-aorta (Courtesy of Alun Williams (RVC))</center></small>]]
 
[[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]].
+
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. 
*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.
+
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.
*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]]===
+
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.
  
===Secondary to [[Key-Gaskell Syndrome]]===  
+
====[[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===
+
===Acquired Megaoesophagus===
*Occurs spontaneously in adult in any disorder that disrupts normal reflex involved in swallowing either peripheral or central.
+
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:
*These can include:
+
*'''Brain Centres'''
**Viral encephalitides
+
**'''Viral encephalitides''' including those syndromes caused by '''canine distemper virus'''.
**Peripheral neuropathies
+
**'''Ingestion of toxins''', including '''lead''', '''mercury''', '''botulinum toxin''' and absorption of '''tetanus toxin'''
***Degeneration or inactivity of local myenteric nerve plexuses (Auerbach’s plexuses),
+
*'''Reflex arcs'''
***or to lesions in the vagus nerve or the central nucleus in the medulla of the brain which supply the [[Oesophagus - Anatomy & Physiology|oesophagus]].
+
**'''Peripheral neuropathies''', including labrador retriever myopathy and giant axonal neuropathy of German Shepherd dogs
**Poisonings such as:
+
**'''Feline dysautonomia''', also know as [[Key-Gaskell Syndrome]].
***Lead
+
*'''Muscle'''
***Tetanus
+
**'''Myositis''' and '''polymyositis/polyneuritis'''
***Botulism
+
**'''Muscle weakness''' as occurs with severe '''hypokalaemia''' and '''hypoadrenocorticism''' (Addison's disease).
**Myopathies.
 
  
*The result is the presence of a hypotonic [[Oesophagus - Anatomy & Physiology|oesophagus]] in which food accumulates causing distension
+
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.
*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.
 
  
  

Revision as of 14:22, 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))

This condition 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.