Difference between revisions of "Lymphangiectasia"

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{{dog}}
 
{{dog}}
 
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{{cat}}
  
 
==Signalment==
 
==Signalment==
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'''Lymphangiectasia''' is characterised by dilation and dysfunction of the lymphatic vessels of the intestines.  Consequently, protein rich lymph leaks into the intestinal lumen, causing a protein-losing enteropathy and severe lipid malabsorption.  It is relatively common in dogs but rare in cats.   
 
'''Lymphangiectasia''' is characterised by dilation and dysfunction of the lymphatic vessels of the intestines.  Consequently, protein rich lymph leaks into the intestinal lumen, causing a protein-losing enteropathy and severe lipid malabsorption.  It is relatively common in dogs but rare in cats.   
  
Lymphangiectasia can be classified as primary or secondary.  '''Primary lymphangiectasia''' may form part of of a localised or a more widespread lymphatic abnormality.   '''Secondary lymphangiectasia''' is the result of lymphatic obstruction.  The obstruction may be caused by:
+
Lymphangiectasia can be classified into a primary or a secondary lymphangiectasia.  '''Primary lymphangiectasia''' may form part of a localised or a more widespread lymphatic abnormality. '''Secondary lymphangiectasia''' results from lymphatic obstruction, which may be caused by:
*inflammation, neoplastic infiltration o fibrosis
+
*inflammation, neoplastic infiltration or fibrosis
 
*thoracic duct obstruction
 
*thoracic duct obstruction
 
*right sided cardiac failure
 
*right sided cardiac failure
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*hepatic disease
 
*hepatic disease
  
Lymphagiectasia often accompanies a lipogranulomatous inflammation, but it is not clear which is the primary event.  Lymphangitis can cause lymphatic obstruction but the leakage of lymph can cause a granuloma to form.
+
Lymphangiectasia often accompanies a lipogranulomatous inflammation, but it is not clear which is the primary event.  Lymphangitis can cause lymphatic obstruction but the leakage of lymph can also cause a granuloma to form.
  
  
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===Clinical Signs===
 
===Clinical Signs===
 
*Weight loss
 
*Weight loss
*Chronic diarrhoea, steatorrhoea
+
*Chronic diarrhoea; steatorrhoea
 
*Ascites, oedema or chylothorax may result if there is severe hypoproteinaemia or lymphatic obstruction
 
*Ascites, oedema or chylothorax may result if there is severe hypoproteinaemia or lymphatic obstruction
 
*Increased appetite
 
*Increased appetite
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====Other Tests====
 
====Other Tests====
*Faeacal α1-proteinase inhibitor concentrations or chromium 51-labelled albumin may be used to confirm protein-losing enteropathy.
+
*Faecal α1-proteinase inhibitor concentrations or chromium 51-labelled albumin may be used to confirm protein-losing enteropathy.
  
  
 
===Diagnostic Imaging===
 
===Diagnostic Imaging===
 
====Ultrasound====
 
====Ultrasound====
Abdominal ultrasonography may reveal pleural fluid or ascites as well as help to narrow down other differential diagnoses.  Mucosa of intestinal loops may appear thickened due to oedema.
+
Abdominal ultrasonography may reveal pleural fluid or ascites as well as helping to narrow down other differential diagnoses.  Mucosa of intestinal loops may appear thickened due to oedema.
  
 
====Endoscopy====
 
====Endoscopy====
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Refer to [[Intestines Inflammatory Bowel Disease And Related Conditions - Pathology #Lymphangiectasia|Lymphangiectasia]] for pathology
 
Refer to [[Intestines Inflammatory Bowel Disease And Related Conditions - Pathology #Lymphangiectasia|Lymphangiectasia]] for pathology
  
It is essential to distinguish a true lymphangiectasia from a secondary lacteal dilation due to [[Inflammatory Bowel Disease - WikiClinical|Inflammatory Bowel Disease ]] (IBD).  In the case of IBD, inflammtory infiltrate will be seen in the lamina propria, but the degree of infiltration may be underestimated if oedema is present.
+
It is essential to distinguish a true lymphangiectasia from a secondary lacteal dilation due to [[Inflammatory Bowel Disease - WikiClinical|Inflammatory Bowel Disease ]] (IBD).  In the case of IBD, inflammatory infiltrate will be seen in the lamina propria, but the degree of infiltration may be underestimated if oedema is present.
  
  
 
==Treatment==
 
==Treatment==
*Identify and treat the underlying cause if it is caused by secondary lymphangiectasia
+
*Identify and treat the underlying cause if it is a secondary lymphangiectasia
 +
*Fat-restricted diet
 +
**The diet needs to be calorific and highly digestible
 
*Supplementation of fat soluble vitamins
 
*Supplementation of fat soluble vitamins
 
*Anecdotal report of glutamine supplementation
 
*Anecdotal report of glutamine supplementation
*Prednisolone at 0.5-1.0 mg/kg PO BID
+
*Prednisolone at 1-2 mg/kg/day PO
 
**Anti-inflammatory and immunosuppressive effect may be beneficial
 
**Anti-inflammatory and immunosuppressive effect may be beneficial
**This is particularly true if there is associated lymphangitis, lipogranulomas and a lymphocytic-plasmacytic infiltration of the lamina propria.
+
**This is particularly true if there is associated lymphangitis, lipogranulomas or a lymphocytic-plasmacytic infiltration of the lamina propria.
 +
**Azathioprine at 2.2 mg/kg q48 hours or Ciclosporin at 3-5 mg/kg q24 to 12 hours can also be considered
 
*Antimicrobials such as metronidazole or tylosin  
 
*Antimicrobials such as metronidazole or tylosin  
**This may be beneficial due to their potential immunomodulatory effect and modulation of enteric flora
+
**This may be beneficial due to their potential immunomodulatory effect and modulation of the enteric flora
*Diuretics, preferably combinations, such as frusemide and spironolactone are used to treat effusions.
+
*Diuretics such as frusemide and spironolactone are used to manage effusions.
*Short term treatment with plasma or colloids can be given for plasma expandsion.
+
*Short term treatment with plasma or colloids can be given for plasma expansion.
  
  
 
==Prognosis==
 
==Prognosis==
Guarded.  The response to treatment is generally poor although some dogs may do well.  Dogs in may be in remission for several years but the disease eventually progress to fulminant hypoproteinaemia.
+
Guarded.  The response to treatment is generally poor although some dogs may do well.  Dogs may be in remission for several years but the disease eventually progress to fulminant hypoproteinaemia.
  
  

Revision as of 13:42, 12 August 2009



Category:WikiClinical CanineCow
Category:WikiClinical FelineCow

Signalment

  • Breed predisposition:
    • Yorkshire Terrier
    • Lundehund
    • Rottweiler
    • Soft Coated Wheaten Terriers


Description

Lymphangiectasia is characterised by dilation and dysfunction of the lymphatic vessels of the intestines. Consequently, protein rich lymph leaks into the intestinal lumen, causing a protein-losing enteropathy and severe lipid malabsorption. It is relatively common in dogs but rare in cats.

Lymphangiectasia can be classified into a primary or a secondary lymphangiectasia. Primary lymphangiectasia may form part of a localised or a more widespread lymphatic abnormality. Secondary lymphangiectasia results from lymphatic obstruction, which may be caused by:

  • inflammation, neoplastic infiltration or fibrosis
  • thoracic duct obstruction
  • right sided cardiac failure
  • caval obstruction
  • hepatic disease

Lymphangiectasia often accompanies a lipogranulomatous inflammation, but it is not clear which is the primary event. Lymphangitis can cause lymphatic obstruction but the leakage of lymph can also cause a granuloma to form.


Diagnosis

Clinical Signs

  • Weight loss
  • Chronic diarrhoea; steatorrhoea
  • Ascites, oedema or chylothorax may result if there is severe hypoproteinaemia or lymphatic obstruction
  • Increased appetite
  • Vomiting, lethargy and anorexia (less common)


Laboratory Tests

Haematology

  • Panhypoproteinaemia
  • Lymphopaenia

Biochemistry

  • Hypocholesterolaemia
  • Hypocalcaemia due to hypoproteinaemia, vitamin D and calcium malabsorption
  • Hypomagnesaemia

Other Tests

  • Faecal α1-proteinase inhibitor concentrations or chromium 51-labelled albumin may be used to confirm protein-losing enteropathy.


Diagnostic Imaging

Ultrasound

Abdominal ultrasonography may reveal pleural fluid or ascites as well as helping to narrow down other differential diagnoses. Mucosa of intestinal loops may appear thickened due to oedema.

Endoscopy

Grossly, multiple white lipid droplets with prominent mucosal blebs can be seen.


Histopathology

Preferably, a full thickness biopsy is needed for a definitive diagnosis.

Refer to Lymphangiectasia for pathology

It is essential to distinguish a true lymphangiectasia from a secondary lacteal dilation due to Inflammatory Bowel Disease (IBD). In the case of IBD, inflammatory infiltrate will be seen in the lamina propria, but the degree of infiltration may be underestimated if oedema is present.


Treatment

  • Identify and treat the underlying cause if it is a secondary lymphangiectasia
  • Fat-restricted diet
    • The diet needs to be calorific and highly digestible
  • Supplementation of fat soluble vitamins
  • Anecdotal report of glutamine supplementation
  • Prednisolone at 1-2 mg/kg/day PO
    • Anti-inflammatory and immunosuppressive effect may be beneficial
    • This is particularly true if there is associated lymphangitis, lipogranulomas or a lymphocytic-plasmacytic infiltration of the lamina propria.
    • Azathioprine at 2.2 mg/kg q48 hours or Ciclosporin at 3-5 mg/kg q24 to 12 hours can also be considered
  • Antimicrobials such as metronidazole or tylosin
    • This may be beneficial due to their potential immunomodulatory effect and modulation of the enteric flora
  • Diuretics such as frusemide and spironolactone are used to manage effusions.
  • Short term treatment with plasma or colloids can be given for plasma expansion.


Prognosis

Guarded. The response to treatment is generally poor although some dogs may do well. Dogs may be in remission for several years but the disease eventually progress to fulminant hypoproteinaemia.


References

  • 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.
  • Hall, E.J, Simpson, J.W. and Williams, D.A. (2005) BSAVA Manual of Canine and Feline Gastroenterology (2nd Edition) BSAVA
  • Nelson, R.W. and Couto, C.G. (2009) Small Animal Internal Medicine (Fourth Edition) Mosby Elsevier.