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Key Features

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Essentials of Diagnosis
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  • Abdominal distention

  • Edema

  • Hypoalbuminemia

  • Hypogammaglobulinemia

  • Lymphopenia

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General Considerations
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  • Neonatal chylous ascites may be due to congenital infection or developmental abnormality of the lymphatic system (intestinal lymphangiectasia)

  • If the thoracic duct is involved, chylothorax may be present

  • Later in life, chylous ascites

    • May result from

      • Congenital lymphangiectasia

      • Retroperitoneal or lymphatic tumors

      • Peritoneal bands

      • Abdominal trauma

      • Intestinal malrotation

      • Infection

    • May occur after cardiac or abdominal surgery

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Clinical Findings

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Symptoms and Signs
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  • Both congenital and acquired lymphatic obstructions cause

    • Chylous ascites

    • Diarrhea

    • Failure to thrive

  • Abdomen is distended, with a fluid wave and shifting dullness

  • Unilateral or generalized peripheral edema may be present

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Differential Diagnosis
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  • Ascites due to liver disease

  • Constrictive pericarditis

  • Chronically elevated right heart pressure

  • Malignancy

  • Infection

  • Inflammatory diseases causing lymphatic obstruction

  • In the newborn, urinary ascites from anatomic abnormalities of the kidney or collecting system must be considered

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Diagnosis

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Laboratory Findings
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  • Hypoalbuminemia

  • Hypogammaglobulinemia

  • Lymphopenia

  • Ascitic fluid contains lymphocytes and has the biochemical composition of chyle if the patient has just been fed; otherwise, it is indistinguishable from ascites secondary to cirrhosis

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Treatment

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  • Supportive, consisting mainly of a high-protein diet and careful attention to infections

  • Shunting of peritoneal fluid into the venous system is sometimes effective

  • A fat-free diet supplemented with medium-chain triglycerides decreases the formation of chylous ascites

  • Total parenteral nutrition may rarely be necessary

  • Infusions of albumin generally provide only temporary relief and are rarely used for long-term management

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Outcome

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Complications
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  • Chylous ascites caused by intestinal lymphatic obstruction is associated with fat malabsorption and protein loss

  • Intestinal loss of albumin may lead to edema

  • Rapidly accumulating chylous ascites may cause respiratory complications

  • Primary infections and malignancies causing chylous ascites may be life threatening

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Prognosis
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  • In the neonate, congenital chylous ascites may spontaneously disappear following one or more paracenteses and a medium-chain triglyceride diet

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References

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Densupsoontorn  N  et al: Congenital chylous ascites: the roles of fibrin glue and CD31. Acta Paediatr 2009 Nov;98(11):1847–1849
[PubMed: 19627262] .
CrossRef
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Moreira  Dde A  et al: Congenital chylous ascites: a report of a case treated with hemostatic cellulose and fibrin glue. J Pediatr Surg 2013 Feb;48(2):e17–e19
[PubMed: 23414895] .
CrossRef
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Olivieri  C, Nanni  L, Masini  L, Pintus  C: Successful management of congenital chylous ascites with early octreotide and total parenteral nutrition in a newborn. BMJ Case Rep 2012 Sep 25;2012
[PubMed: 23010459] .

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