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Ascites, Pediatric


Basics


Description


  • Ascites is defined as a pathologic accumulation of intraperitoneal fluid.
  • Peritoneal fluid formation is a dynamic process of production and absorption.
  • In children, ascites is usually the result of liver or renal disease.
  • In adults, ascites is most often due to portal hypertension from cirrhosis.
  • Ascites is the most common of the three major complications of cirrhosis; the other two complications of cirrhosis are hepatic encephalopathy and variceal hemorrhage.

Pathophysiology


  • Normal circulation
    • Blood enters the liver from the hepatic artery and portal vein, perfuses the hepatic sinusoids, and exits the liver via the hepatic veins.
    • Hepatic lymph, formed by the filtration of sinusoidal plasma into the space of Disse, drains from the liver via the transdiaphragmatic lymphatic vessels to the thoracic duct.
    • Hepatic lymph is isosmotic to plasma, as the sinusoidal endothelium is highly permeable to albumin.
    • In the intestine, the mesenteric capillary membrane is impermeable to albumin. The osmotic gradient favors the return of interstitial fluid/lymph into the capillary.
    • Intestinal lymph from regional lymphatics combines with hepatic lymph in the thoracic duct.
  • Portal hypertension
    • Ascitic fluid production is due to a net transfer of fluid that exceeds the drainage capacity of the lymphatics.
  • Cirrhotic ascites results from three pathophysiologic process:
    • Portal hypertension
    • Vasodilation: mediated predominantly by nitric oxide
    • Hyperaldosteronism: Decreased effective volume sensed by the kidneys stimulate the renin-angiotensin-aldosterone system, leading to increased sympathetic activity and antidiuretic hormone secretion.
  • Noncirrhotic ascites can be the result of the following:
    • Proteinaceous material produced by malignant cells or by inflammation of visceral and/or parietal peritoneum: peritoneal carcinomatosis, tuberculous ascites
    • Obstruction of lymphatic flow by mass, tumor, or external pressure
    • Impaired portal flow: right-sided heart failure, Budd-Chiari syndrome, portal venous malformations
    • Decreased effective arterial blood volume: heart failure
    • Decreased oncotic pressure/ hypoalbuminemia: nephrotic syndrome, protein-losing enteropathy, severe malnutrition
    • Primary (congenital) abnormalities of the lymphatics, metabolic disorders (lysosomal storage diseases including sialidosis, Wolman disease, GM1 gangliosidosis, Gaucher disease, and Niemann-Pick type C)
    • Rupture of intra-abdominal viscus or peritoneal/mesenteric cyst, bowel perforation, ureteral rupture

Etiology


Accumulation of fluid occurs with the following:  
  • Inflammatory conditions (e.g., mesenteric adenitis, tuberculosis, pancreatitis, secondary to inflammation of visceral, and/or parietal peritoneum)
  • Portal hypertension or obstruction of portal vein flow and/or lymphatic flow by mass, tumor, or external pressure; tumors of abdominal viscera, retroperitoneum, thorax, or mediastinum (often characterized by chylous ascites)
  • Infectious processes: abscess, tuberculosis, Chlamydia infection, schistosomiasis
  • Gastrointestinal: infarcted bowel/perforation, pancreatitis, ruptured pancreatic duct, parenchymal liver disease
  • Gynecologic: ovarian tumors, torsion, or rupture
  • Renal: nephrotic syndrome, obstructive uropathy, perforated urinary tract, peritoneal dialysis
  • Cardiac: CHF, constrictive pericarditis, inferior vena cava web
  • Neoplastic: lymphoma, neuroblastoma
  • Miscellaneous: systemic lupus erythematous, eosinophilic ascites, chylous ascites, hypothyroidism, ventriculoperitoneal shunt

Diagnosis


History


  • The etiology for acute decompensation in hepatocellular function (e.g., massive bleeding, sepsis, superimposed infections) should be investigated.
  • Weight gain
  • Use of umbilical catheters in newborn period (increased risk of portal vein thrombosis)
  • Evidence of chronic liver disease
  • Respiratory distress
  • Exposure to hepatotoxins
  • Developmental delay or growth failure suggestive of metabolic disease

Physical Exam


  • Vital signs:
    • Increased heart rate (increased cardiac output)
    • Lower blood pressure seen in cirrhotics
  • General appearance: cachexia
  • Abdominal exam
    • Protuberant abdomen, bulging flanks (fluid wave or shifting dullness)
    • Caput medusae, umbilical hernia,
    • Dullness to percussion
    • Peritoneal signs
    • Abdominal pain
    • Splenomegaly
  • Auscultation of the pericardium: pericardial friction rub (pericarditis), cor pulmonale
  • Neurologic exam: hepatic encephalopathy
  • Skin
    • Jaundice
    • Spider angioma
    • Palmar erythema
    • Scratch marks
    • Striae
    • Xanthomas
  • Extremities
    • Clubbing
    • Edema

Diagnostic Evaluation


Laboratory
  • Complete blood count
  • Electrolytes
  • Liver test: transaminases, prothrombin time/international normalized ratio, total protein, albumin, total and fractionated bilirubin
  • Amylase and lipase (to exclude pancreatitis)
  • Creatinine and blood urea nitrogen
  • Fluid cultures: blood, urine, ascitic fluid
  • Urinalysis
  • Specific testing for etiologies of ascites from chronic liver disease and other causes as deemed appropriate

Imaging
  • Ultrasound of the abdomen with Doppler study
    • Study of choice to differentiate between free and loculated fluid collection and the presence of intra-abdominal masses
    • Can evaluate patency of hepatic and portal vasculature and directionality of flow
  • Plain radiography (centralized bowel loops)
  • Abdominal computed axial tomography
  • Abdominal magnetic resonance imaging

Abdominal Paracentesis
  • Ascitic fluid analysis is essential:
    • Cell count and differential
    • Albumin, total protein
    • Culture, Gram stain
    • Glucose (low in infection)
    • Lactate dehydrogenase concentration (high in infection, bowel perforation, or tumor)
    • Other optional tests include amylase (high in perforated viscus or pancreatitis), triglycerides (high in chylous ascites), and cytology (peritoneal carcinomatosis).
  • Serum to ascites albumin gradient (SAAG)
    • (Serum albumin) - (ascites albumin)
    • Blood and ascitic fluid analysis should be obtained on same day.
    • SAAG ≥1.1 g/dL suggests presence of portal hypertension.
    • If SAAG <1.1 g/dL, suspect other causes.

Differential Diagnosis


  • Organomegaly: enlarged liver or spleen
  • Mesenteric cyst or ovarian cyst: does not have shifting dullness when position is changed
  • Bowel obstruction
  • Cancer
  • Heart failure
  • Nephrotic syndrome

Treatment


  • The management of the ascites should be directed toward the underlying etiology.
  • Benefits of treatment of ascites should always be weighed against risks and complications of treatment.
  • Mobilization of cirrhotic ascitic fluid is best accomplished by creating a negative sodium balance and then maintaining the balance.
  • In patients with cirrhosis, causes of decompensation should be sought, such as sodium and fluid overload, infection, esophageal hemorrhage, spontaneous bacterial peritonitis.
  • In adults, dietary sodium intake is restricted to 44-88 mEq (1-2 g/24 h) or approximately 17-35 mEq (0.4-0.8 g) per thousand calories.
  • In pediatrics, restricting dietary sodium intake is recommended: diet with no extra salt to a maximum 2 mEq/kg/24 h (to be balanced against palatability of food and nutritional needs).
  • Water is only restricted in patients with profound hyponatremia (<125 mEq/L): 50-75% of maintenance requirements.
  • Goal of diuretic therapy is reduction of bodyweight by 0.5-1% daily until ascites is resolved.
  • Spironolactone (PO)
    • Most effective single diuretic, as it counteracts the hyperaldosteronism present in cirrhotic ascites
    • Acts on the distal collecting system, hence, inhibits reabsorption of 2% of filtered sodium
    • Bioactive metabolites have long half-lives, hence, need >5 days to achieve steady state.
    • Start at 2-3 mg/kg/24 h as a single morning (max 100 mg initial dose). In adults, typical starting dose is up to 100 mg/24 h and can be increased to max 400 mg/24 h.
    • Most effective in combination with furosemide
  • Adequacy of spironolactone therapy can be monitored with urinary sodium excretion (desired >50 mEq/L). If no response, furosemide is added.
  • Furosemide (PO)
    • Loop diuretic: can increase sodium excretion by 30%
    • Start at 1 mg/kg (max initial dose 40 mg), may increase every few days if needed.
    • Adults maintain ratio of 100 mg of spironolactone to 40 mg of furosemide to maintain eukalemia (max 400 mg spironolactone to 160 mg of furosemide daily)
  • When diuretics are used, urine output and serum electrolytes should be closely monitored to prevent prerenal azotemia and decreased effective blood flow to the kidneys.
  • Albumin: Supplementation may aid fluid mobilization if albumin is <2.5 g/dL (use 1 g/kg of 25% albumin until level >2.5 g/dL).
  • Refractory ascites: Diuretic-refractory ascites derives from a lack of response to dietary sodium restriction and maximal diuretic therapy. Treatment options are the following:
    • Therapeutic abdominal paracentesis (large-volume paracentesis) is used in adults with refractory ascites.
    • In children, this approach is used to relieve respiratory distress or other sequelae of rapidly increasing intra-abdominal pressure.
    • Paracentesis of volumes >1 L should be accompanied by IV infusion of 25% albumin during the procedure.
    • Transjugular intrahepatic portosystemic shunting may be valuable in cases where portal hypertension is felt to be the underlying etiology of ascitic accumulation.
    • Orthotopic liver transplantation is the only curative therapy for refractory ascites from liver disease and the only definitive treatment that has been shown to improve survival.
  • When diuretics are used, urine output and serum electrolytes should be closely monitored to prevent prerenal azotemia and decreased effective blood flow to the kidneys.

Alert


  • Ultrasonography should be the initial diagnostic imaging of choice for evaluation of ascites.
  • With congenital ascites, evaluate for lysosomal storage diseases.
  • Diagnostic paracentesis is crucial in the evaluation of new-onset ascites.
  • Calculate SAAG to differentiate between portal hypertension and other causes.
  • In patients with liver disease and new-onset ascites, evaluate for etiologies to explain acute decompensation.

Prognosis


  • Development of ascites in the setting of cirrhosis is a landmark in the natural history of cirrhosis: 15% of adult patients succumb in 1 year and 44% in 5 years.
  • Liver transplantation dramatically improves survival.
  • Prognosis depends on etiology of ascites: nephrotic syndrome (ascites will regress as proteinuria clears), infection, hepatic decompensation (prognosis improves if able to reverse cause of liver injury).

Complications


  • Spontaneous bacterial peritonitis (SBP)
    • Spontaneous ascitic fluid infection: Infection of the peritoneal fluid may occur in the absence of secondary cause (e.g., bowel perforation or intra-abdominal abscess).
    • Fever, irritability, abdominal tenderness, and distention are common signs, vomiting and diarrhea may occur.
    • Abdominal paracentesis must be performed and ascitic fluid must be analyzed before a confident diagnosis of ascitic fluid infection can be made. The blood culture bottle should be injected with peritoneal fluid at the bedside in order to increase the culture yield.
    • Diagnosis: ascitic fluid absolute polymorphonuclear leukocytes ≥250 cells/mm3 without evidence of an intra-abdominal process
    • Bacterial organisms commonly identified are the following: Streptococcus pneumoniae, Escherichia coli, Klebsiella pneumoniae, enterococci, and Haemophilus influenzae.
    • Broad-spectrum antibiotics with gram-negative coverage such as third generation cephalosporin can be used until identification of bacterial pathogen allows narrower coverage.
    • Prophylaxis for recurrent SBP has been recommended in certain situations.
  • Other complications:
    • Respiratory distress from decreased lung volume and diaphragmatic limitation: hepatic hydrothorax (large symptomatic pleural effusion that occurs in a cirrhotic patient in the absence of primary cardiopulmonary disease), abdominal wall hernias with rupture, tense ascites with leakage (especially after paracentesis)
    • Conservative management consists of appropriate initial therapy for most of these except hernia rupture, which requires surgical reduction.

Additional Reading


  • European Association for the Study of the Liver. EASL clinical practice guideline on the management of ascites, spontaneous bacterial peritonitis, and hepatorenal syndrome in cirrhosis. J Hepatol.  2010,53(3):397-417.  [View Abstract]
  • Griefer  MJ, Murray  KF, Colleti  RB. Pathophysiology, diagnosis, and management of pediatric ascites. J Pediatr Gastroenterol Nutr.  2011;52(5):503-513.  [View Abstract]
  • Hou  W, Sanyal  AJ. Ascites: diagnosis and management. Med Clin North Am.  2009;93(4):801-817.  [View Abstract]
  • Runyon  BA. Management of adult patients with ascites due to cirrhosis: update 2012. Hepatology.  2013(6):2087-2107.  [View Abstract]

Codes


ICD09


  • 789.59 Other ascites
  • 789.51 Malignant ascites
  • 014.00 Tuberculous peritonitis, unspecified

ICD10


  • R18.8 Other ascites
  • R18.0 Malignant ascites
  • A18.31 Tuberculous peritonitis

SNOMED


  • 389026000 Ascites (disorder)
  • 236005001 Malignant ascites (disorder)
  • 236004002 Hepatic ascites (disorder)
  • 4501007 Tuberculous ascites (disorder)

FAQ


  • Q: What etiologies are likely in cases of congenital ascites?
  • A: Lysosomal storage disorders and/or other metabolic diseases should be excluded. If hepatic function is impaired, causes of neonatal liver failure should also be investigated.
  • Q: What is the best test to discriminate the type of ascites?
  • A: Analysis of the peritoneal fluid collected by abdominal paracentesis is required for this purpose. The SAAG is helpful to discriminate ascites due to portal hypertension from other etiologies.
  • Q: Where does the word "ascites" come from?
  • A: It is thought that the word ascites is derived from the Greek word "askos" which refers to a container of wine or a wineskin.
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