Ascites, Non-cirrhotic portal hypertension

Thanks Michael for a great case! This is a case of a middle age person with an extensive malignancy history (cardiac tumor, non-hodgkin lymphoma) and ITP presenting with incidentally found ascites thought possibly secondary to idiopathic noncirrhotic portal hypertension from chemotherapy.


Main points:

  1. ) Thanks to Sven Olson for this: An autologous stem cell transplant is a misnomer, as individuals are not actually getting a transplant in the sense they are getting someone else's tissue, and a better name is "high dose chemotherapy with stem cell rescue." See this previous blog post about this.
  2. ) An evidence-based approach to diagnosing ascites includes:
    1. a. Physical exam tips: lower extremity edema (negative LR 0.1), shifting dullness (positive LR 2.7) and fluid wave (LR 6.0). Here's a great Stanford 25 webpage about how the physical exam can help.
    2. b. Bedside ultrasound is excellent for looking for ascites and even 100 cc's can be located pretty easily
  3. ) One way to think about the differential of ascites is to break it down into:
    1. a. SAAG > 1.1 (elevated hydrostatic pressure)
      1. i. Elevated JVP suggests cardiac etiology (eg heart failure)
      2. ii. Normal JVP suggests portal HTN (80% have cirrhosis)
    2. b. SAAG < 1.1
      1. i. Low albumin (ascites protein < 2.5): protein losing enteropathy, nephrotic etc.
      2. ii. Peritoneal disease (ascites protein >2.5): malignancy, infection, serosits, pancreatitis
      3. iii. TIP: if your pretest for portal HTN is very high but SAAG < 1.1, you are probably correct (see this study from American Journal of Gastroenterology)

Approach to Ascites from Non-cirrhotic Portal Hypertension

Another way to think about ascites from portal hypertension is:

  1. ) Prehepatic - portal vein/ splenic vein thromobsis, splanchnic AVM
  2. ) Intrahepatic - presinusoidal/ sinusoidal/ postsinusoidal (eg. AV fistula, PBC, neoplastic occlusion, schistosomiasis, infiltrative diseases, toxins, high dose vitamin A etc)
  3. ) Posthepatic - budd chiari, IVC obstruction, cardiac disease

The differential is even more extensive than this and should prompt hepatology consultation

First a liver biopsy should be done to exclude cirrhosis

See this great NEJM case that goes over these concepts.