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==Introduction==
 
==Introduction==
Pericardio-Peritoneo-Diaphragmatic Hernia (PPDH) is a congenital abnormality characterised by incomplete formation of the septum transversum during early embryonic development, meaning that the peritoneal cavity and pericardial sac remain continuous in later life.  The defect may also result from a failure of the septum transversum to fuse with the pleuroperitoneal folds.  Since the diaphragm (which is formed by the septum transversum) does not separate the two cavities, abdominal organs are able to move into the pericardial sac. Many animals are diagnosed as adults and show few clinical signs on presentation.  
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Pericardio-Peritoneo-Diaphragmatic Hernia (PPDH) is a congenital abnormality characterised by incomplete formation of the septum transversum during early embryonic development, meaning that the peritoneal cavity and pericardial sac remain '''continuous''' in later life.  The defect may also result from a failure of the septum transversum to fuse with the pleuroperitoneal folds.  Since the diaphragm (which is formed by the septum transversum) does not separate the two cavities, abdominal organs are able to move '''into the pericardial sac'''. '''Liver''' is most commonly herniated, followed by small intestine, spleen and stomach.
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Clinical signs may appear at any age, but cases are usually diagnosed before the age of 4. Some animals never develop clinical signs, and PPDH is an '''incidental finding'''.
    
==Signalment==
 
==Signalment==
 
The defect may be inherited in the Weimaraner breed and it occurs most commonly in Himalayan and Domestic Long Hair cats.  It is often accompanied by other congenital defects, such as umbilical hernias, sternal malformations, cardiac septal defects and pulmonary vascular anomalies.   
 
The defect may be inherited in the Weimaraner breed and it occurs most commonly in Himalayan and Domestic Long Hair cats.  It is often accompanied by other congenital defects, such as umbilical hernias, sternal malformations, cardiac septal defects and pulmonary vascular anomalies.   
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==Diagnosis==
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==Clinical Signs==
===Clinical Signs===
   
The disease is frequently asymptomatic but clinical signs may include:
 
The disease is frequently asymptomatic but clinical signs may include:
*'''Gastro-intestinal signs''', including vomiting, diarrhoea, abdominal pain, anorexia and weight loss.
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:'''Gastro-intestinal signs''', including vomiting, diarrhoea, abdominal pain, anorexia and weight loss.
*'''Respiratory distress''' may occur if abdominal contents occupy a large amount of the thoracic cavity.
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:'''Respiratory distress''' may occur if abdominal contents occupy a large amount of the thoracic cavity.
*'''Cardiac signs''' are unusual but [[Cardiac Tamponade|cardiac tamponade]] (with right-sided heart failure) may develop if abdominal organs are incarcerated in the pericardial sac.  The heart sounds will be muffled on auscultation and borborygmi may be apparent.  A heart murmur may be present if the animal has concurrent septal defects.
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:'''Cardiac signs''' are unusual but [[Cardiac Tamponade|cardiac tamponade]] (with right-sided heart failure) may develop if abdominal organs are incarcerated in the pericardial sac.  The heart sounds will be muffled on auscultation and borborygmi may be apparent.  A heart murmur may be present if the animal has concurrent septal defects.
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===Diagnosis===
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'''<u>Radiography</u>''':
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On plain radiographs of the chest, the cardiac silhouette is enlarged and continuous with that of the diaphragm.  If loops of small intestine have passed into the pericardial sac, areas of lucency may be apparent '''overlying''' the cardiac silhouette and, if necessary, this can be confirmed by a '''barium''' contrast study.  The trachea may also be displaced dorsally. The liver cannot be identified in the abdomen.
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In cats, an area of radio-opacity may be apparent in the area of the dorsal diaphragm on a lateral radiograph, referred to as the 'dorsal peritoneo-pericardial remnant.'
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===Radiography===
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'''<u>Ultrasonography</u>''':
On plain radiographs of the chest, the cardiac silhouette is enlarged and continuous with that of the diaphragm.  If loops of small intestine have passed into the pericardial sac, areas of lucency may be apparent overlying the cardiac silhouette and, if necessary, this can be confirmed by a barium contrast study.  The trachea may also be displaced dorsally.  In cats, an area of radio-opacity may be apparent in the area of the dorsal diaphragm on a lateral radiograph, referred to as the 'dorsal peritoneo-pericardial remnant.'  
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===Ultrasonography===
   
Definitive diagnosis generally relies on the identification of abdominal organs within the pericardial sac.  Septal defects may also be diagnosed during the scan.
 
Definitive diagnosis generally relies on the identification of abdominal organs within the pericardial sac.  Septal defects may also be diagnosed during the scan.
    
==Treatment==
 
==Treatment==
If the animal is showing few clinical signs or if the hernia is discovered incidentally, '''conservative treatment''' may be all that is required.  In animals showing overt clinical signs, the abdominal organs can be retracted via a '''midline coeliotomy''' and the defect in the diaphragm can then be closed surgically. This procedure may trap air within the pericardium so it is important not to close the pericardium and to perform '''pericardiocentesis''' before closure of the abdominal incision.  Even though organs may have been present in the pericardial sac for prolonged periods, it is rare for adhesions to form as these structures are in constant motion.  
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If the animal is showing few clinical signs or if the hernia is discovered incidentally, '''conservative treatment''' may be all that is required.   
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In animals showing overt clinical signs, the abdominal organs can be retracted via a '''midline coeliotomy''' and the defect in the diaphragm can then be closed surgically. This is usually done with a single layer of sutures, starting dorsally and continuing ventrally. This procedure may trap air within the pericardium so it is important not to close the pericardium and to perform '''pericardiocentesis''' before closure of the abdominal incision.  Even though organs may have been present in the pericardial sac for prolonged periods, it is rare for adhesions to form as these structures are in constant motion.  
    
==Prognosis==
 
==Prognosis==
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Generally, the prognosis after surgery is excellent.
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A study assessing treatment regimes used in cats showed a post-operative mortality rate of 14%, whereas progressive disease was reported in 9% of those managed conservatively.  This result may largely reflect the criteria used for determining whether a cat was a surgical candidate <ref>'''Reimer SB, Kyles AE, Filipowicz DE, Gregory CR''' Long-term outcome of cats treated conservatively or surgically for peritoneopericardial diaphragmatic hernia: 66 cases (1987-2002) ''J Am Vet Med Assoc. 2004 Mar 1;224(5):728-32''</ref>.   
 
A study assessing treatment regimes used in cats showed a post-operative mortality rate of 14%, whereas progressive disease was reported in 9% of those managed conservatively.  This result may largely reflect the criteria used for determining whether a cat was a surgical candidate <ref>'''Reimer SB, Kyles AE, Filipowicz DE, Gregory CR''' Long-term outcome of cats treated conservatively or surgically for peritoneopericardial diaphragmatic hernia: 66 cases (1987-2002) ''J Am Vet Med Assoc. 2004 Mar 1;224(5):728-32''</ref>.   
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==Test yourself with the Developmental Pathology Flashcards==
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{{Learning
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|flashcards = [[Small Animal Soft Tissue Surgery Q&A 06]]
    
[[Cardiovascular Developmental Pathology Flashcards]]
 
[[Cardiovascular Developmental Pathology Flashcards]]
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}}
    
==Literature Search==
 
==Literature Search==
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Use these links to find recent scientific publications via CAB Abstracts (log in required unless accessing from a subscribing organisation).
 
Use these links to find recent scientific publications via CAB Abstracts (log in required unless accessing from a subscribing organisation).
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http://www.cabdirect.org/search.html?q=title%3A%28%22PericardioPeritoneal%22%29+OR++title%3A%28%22Peritoneopericardial%22%29+++OR+title%3A%28PPDH%29 Peritoneopericardial Diaphragmatic Hernia publications]
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[http://www.cabdirect.org/search.html?q=title%3A%28%22PericardioPeritoneal%22%29+OR++title%3A%28%22Peritoneopericardial%22%29+++OR+title%3A%28PPDH%29 Peritoneopericardial Diaphragmatic Hernia publications]
    
==References==
 
==References==
'''Reimer SB, Kyles AE, Filipowicz DE, Gregory CR''' Long-term outcome of cats treated conservatively or surgically for peritoneopericardial diaphragmatic hernia: 66 cases (1987-2002) ''J Am Vet Med Assoc. 2004 Mar 1;224(5):728-32''
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Reimer SB, Kyles AE, Filipowicz DE, Gregory CR '''Long-term outcome of cats treated conservatively or surgically for peritoneopericardial diaphragmatic hernia: 66 cases (1987-2002)''' ''J Am Vet Med Assoc. 2004 Mar 1;224(5):728-32''
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Gorman N (1997) '''Canine Medicine and Therapeutics''' ''Blackwell Science'' Fourth Edition
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'''Gorman N''' 1997 Canine Medicine and Therapeutics ''Blackwell Science'' Fourth Edition
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Ware, W. (2007) '''Cardiovascular disease in small animal medicine''' ''Manson Publishing''
    
<references/>
 
<references/>
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[[Category:To Do - Helen]]
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[[Category:To Do - Review]]
    
[[Category:Peritoneal_Cavity_-_Developmental_Pathology]]
 
[[Category:Peritoneal_Cavity_-_Developmental_Pathology]]
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