Expert Review of Gastroenterology & Hepatology

ISSN: 1747-4124 (Print) 1747-4132 (Online) Journal homepage: http://www.tandfonline.com/loi/ierh20

Management of ascites in children

Erin R Lane, Evelyn K Hsu & Karen F Murray

To cite this article: Erin R Lane, Evelyn K Hsu & Karen F Murray (2015) Management of
ascites in children, Expert Review of Gastroenterology & Hepatology, 9:10, 1281-1292, DOI:
10.1586/17474124.2015.1083419

To link to this article: http://dx.doi.org/10.1586/17474124.2015.1083419

Published online: 31 Aug 2015.

Submit your article to this journal

Article views: 514

View related articles

View Crossmark data

Citing articles: 1 View citing articles

Full Terms & Conditions of access and use can be found at

http://www.tandfonline.com/action/journalInformation?journalCode=ierh20

Download by: [University of Canberra] Date: 22 October 2017, At: 21:53

 Review

Management of ascites in
children
Expert Rev. Gastroenterol. Hepatol. 9(10), 1281–1292 (2015)

Erin R Lane1, Ascites is the pathologic accumulation of fluid within the peritoneal cavity. There are many
Evelyn K Hsu2 and causes of fetal, neonatal and pediatric ascites; however, chronic liver disease and subsequent
Karen F Murray*2 cirrhosis remain the most common. The medical and surgical management of ascites in
1
children is dependent on targeting the underlying etiology. Broad categories of management
Pediatric Gastroenterology, University
of Washington School of Medicine,
strategies include: sodium restriction, diuresis, paracentesis, intravenous albumin, prevention
Downloaded by [University of Canberra] at 21:53 22 October 2017

4800 Sand Point Way, NE, PO Box and treatment of infection, surgical and endovascular shunts and liver transplantation. This
5371/OB.9.640, Seattle, WA 98105, review updates and expands the discussion of the unique considerations regarding the
USA management of cirrhotic and non-cirrhotic ascites in the pediatric patient.
2
Division of Gastroenterology and
Hepatology Seattle Children’s and the
University of Washington, PO Box KEYWORDS: ascites . cirrhosis . diuresis . hypoalbuminemia . paracentesis . pediatrics . renin–angiotensin–aldosterone
5371/OB.9.640, Seattle, WA 98155, system . transjugular intrahepatic portosystemic shunt
USA
*Author for correspondence:
Tel.: +1 20 69 87 10 95 Management of ascites in infants and children effectively leads to a reduction in arterial blood
Fax: +1 20 69 87 27 21 is dependent on the identification of the spe- volume, resulting in activation of the sympa-
Karen.murray@seattlechildrens.org cific underlying etiology or pathologic mecha- thetic nervous system, renal sodium retention
nism. While there are a multitude of causes of via the renin–angiotensin–aldosterone system
fetal, neonatal and pediatric ascites (BOX 1), and secretion of antidiuretic hormone (ADH)
chronic liver disease and subsequent cirrhosis (FIGURE 2). The subsequent renal sodium reten-
remain the most common [1]. In adult tion in the setting of portal hypertension con-
patients, ascites most commonly accumulates tributes to the expansion of extracellular
secondary to cirrhosis from hepatitis C infec- volume and accumulation of ascites [3,4]. Portal
tion, alcoholic liver disease or non-alcoholic hypertension contributes to the elevation of
steatohepatitis. While the underlying etiology splanchnic capillary pressure, resulting in an
of ascites in children may differ from that of excess of lymph formation.
adults, many of the current management strat- Ascites also develops in the absence of liver
egies of ascites in the pediatric patient are disease or cirrhosis. Non-cirrhotic ascites is a
derived from evidence and experience with vast category and management is directed at
adults. The pathophysiology and diagnosis of identifying and targeting the underlying
pediatric ascites has previously been etiology.
reported [2]. The aim of this review is to In patients with ascites, the benefits of tar-
update and expand the discussion of the geted medical or surgical therapy must be
appropriate medical and surgical management measured against the potential risks of adverse
of cirrhotic and non-cirrhotic ascites in the clinical sequelae. Without prompt or adequate
pediatric patient. management, ascites may result in significant
Ascites is defined by the pathologic accumu- lung restriction and compromised ventilation,
lation of fluid within the peritoneal cavity. increased risk of infection, gastrointestinal
A delicate and highly regulated interplay of hemorrhage, encephalopathy, renal failure and
hydrostatic and oncotic forces regulate death. The initial diagnostic strategy should
splanchnic, portal and hepatic blood and lym- include a thorough physical examination and
phatic flow. Disruption of the balance of these laboratory evaluation of liver and renal disease
forces may result in the development of with measurement of transaminases, serum
ascites (FIGURE 1). In the setting of cirrhosis, albumin, coagulation studies, serum blood
ascites develops secondary to splanchnic vaso- urea nitrogen, creatinine, urinalysis and uri-
dilation, hyperaldosteronism and portal hyper- nary sodium excretion. Initial imaging evalua-
tension. Nitric oxide-mediated vasodilation tion should include abdominal sonographic

informahealthcare.com 10.1586/17474124.2015.1083419
2015 Informa UK Ltd ISSN 1747-4124 1281

 Review Lane, Hsu & Murray

Box 1. Non-cirrhotic causes of fetal and childhood imaging to evaluate hepatic and biliary anatomy, visualize liver
ascites. vasculature and assess for visible evidence of portal hyperten-
sion. It is possible to estimate the volume and character (sim-
. Hepatic non-cirrhotic causes
Budd–Chiari syndrome
ple vs loculated) of the ascitic fluid through non-invasive
imaging. Percutaneous or transjugular liver biopsy allow for
Congenital hepatic fibrosis
microscopic evaluation of liver parenchyma, assessing for struc-
Bile duct trauma or perforation
tural liver damage and possibly identifying an etiology. In the
Sinusoidal-obstruction syndrome
majority of children presenting with ascites, these tests will
Non-hepatic non-cirrhotic causes identify cirrhosis. However, when underlying etiology is in
. Peritoneal Infection question, a diagnostic paracentesis should be performed. Exam-
Tuberculosis ination of the ascitic fluid with cell counts, albumin, total pro-
Cytomegalovirus tein, bacterial culture, glucose, lactose dehydrogenase, amylase,
Ebstein–Barr Virus triglycerides, acid-fast smear and cytology aids in the identifica-
. Intestinal tion of etiology.
Appendicitis
Crohn’s disease Cirrhotic ascites
Downloaded by [University of Canberra] at 21:53 22 October 2017

Eosinophilic enteropathy In cirrhotic ascites, treatment strategies largely focus on mobi-

Intestinal atresia lizing intraperitoneal fluid and correcting the relative systemic
Meconium ileus hypovolemia.
Intestinal lymphangiectasia
Intestinal malrotation or perforation Sodium restriction
Pyloric duplication
Due to excessive renal sodium retention in cirrhosis, unre-
. Pancreatic stricted ingestion of dietary sodium can exacerbate the accumu-
Acute pancreatitis lation of ascites. Dietary restriction of sodium aids in the
Pancreatic pseudocyst
elimination of ascites as well as preventing re-accumulation
. Inflammatory disorders [5,6]. For children, restricting consumption to 1–2 mEq

Systemic lupus erythematosis

sodium/kg/day, and allowing for up to 1–2 g sodium a day in
Henoch–Schönlein purpura
adolescents is recommended and well tolerated. Sodium restric-
. Metabolic disease tion is not necessary for infants who consume breast milk
. Cardiac exclusively, as human breast milk typically has a very low
Heart failure sodium content [7]. For formula-fed infants, a low-sodium for-
. Genitourinary disorders mula may be utilized. Parents of infants consuming purees and
Nephrotic syndrome table foods, are counseled to avoid unnecessary addition of
Obstructive uropathy sodium. More stringent dietary restriction is unnecessary and
Posterior urethral valves generally poorly tolerated. While this strategy minimizes the
Bladder rupture expansion of total body sodium which aids in reduction of re-
Ureterocele accumulation of extracellular fluid, restriction of dietary
. Chylous ascites sodium alone is seldom sufficient in the management of pedi-
Thoracic duct trauma or ligation atric ascites Commonly, patients will require active diuresis.
Intestinal lymphangiectasia
Total parenteral nutrition extravasation Diuresis
. Malignancy Promoting a negative sodium balance is critical in the manage-
Lymphoma ment of ascites. Spironolactone, a potent natriuretic, is highly
Wilms’ tumor effective in the treatment of ascites as it counters the hyperal-
Germ cell tumors dosteronism that is characteristic in cirrhosis. As a competitive
Neuroblastoma inhibitor of aldosterone in the distal renal tubules, spironolac-
. Pseudo-ascites tone acts to increase excretion of sodium chloride and water
Celiac disease and conserve resorption of potassium and hydrogen ions. Spi-
Cystic mesothelioma ronolactone is metabolized in the liver and excreted in urine
Omental cyst and bile. It is administered to infants 0.5–1 mg/kg/day and in
Ovarian cyst older children generally 1–3 mg/kg/day in 2–3 divided doses
. Other up to maximum of 100 mg/day [2]. The clearance of spirono-
Abdominal trauma lactone is delayed in the setting of end-stage liver disease allow-
Hemoperitoneum ing for once-daily dosing of the medication. In clinical
Ventriculo-peritoneal shunt practice, monotherapy with spironolactone is seldom sufficient.

1282 Expert Rev. Gastroenterol. Hepatol. 9(10), (2015)

 Management of ascites in children Review

In those whom diuresis appears refractory

to spironolactone alone, there may be a
reduced fractional sodium delivery to the
distal renal tubule, due to enhanced Lymphatic
sodium re-absorption at the proximal IVC
flow
tubule. In these cases, adjunctive treat-
ment with the addition of a loop diuretic Space
of
is necessary. disse
Furosemide, a loop diuretic, directly Hepatic v.
inhibits reabsorption of sodium and chlo- Endothelial
ride in the ascending loop of Henle and cell
distal renal tubule. Additionally, it inter- Hepatic Hepatocyte
Sinusoidal
feres with the chloride-binding cotrans- sinusoid
lumen
port system which has the net effect of Osmotic
increasing excretion of water, sodium,
chloride, magnesium and calcium. In the
Downloaded by [University of Canberra] at 21:53 22 October 2017

treatment of ascites, furosemide is admin- Hydrostatic

istered between 0.5 and 2 mg/kg/day (up
to maximal dose of 40 mg/day) in Ascites
Hepatic a.
2–4 divided doses. In adults, it has been
suggested that more rapid mobilization of
moderate ascites may be achieved with a Portal v.
Lymphatic
combination of diuretics [8]. flow
Caution should be exercised when Osmotic
employing the use of diuretics in cir-
Mesenteric v.
rhotic ascites, especially in those with
Hydrostatic
pre-existing renal dysfunction, due to the
risk of pre-renal azotemia and renal fail-
ure with excessive intravascular volume
depletion. While criteria exist for diagno- Submucosa
Intestinal Capillary
sis of hepatorenal syndrome in adults, capillary lumen
similar criteria do not exist for chil-
dren [4]. Development or hepatorenal
syndrome in children is often marked by Endothelial
Ascites cell
progressive oliguria and a rise in serum
creatinine. In patients with end-stage liver
disease, active metabolites of both spiro-
nolactone and loop diuretics have pro-
longed half-lives. As a potassium-sparing
diuretic, spironolactone has the potential
to cause significant hyperkalemia, and
loop diuretics may lead to hyponatremia
and metabolic alkalosis.
Figure 1. Ascites formation. Hepatic lymph is formed by filtration of sinusoidal plasma
In the setting of cirrhotic ascites, grad-
into the space of Disse. It then drains from the liver via the transdiaphragmatic lymphatic
ual diuresis is generally preferred to vessels to the thoracic duct. The sinusoidal endothelium is highly permeable to albumin
reduce the probability of adverse clinical which results in no significant osmotic gradient across the sinusoid. Hydrostatic forces
effects and prevent re-accumulation of are the primary driver of hepatic lymph formation and are increased in portal hyperten-
ascitic fluid. Response to diuresis in chil- sion. Mesenteric capillaries are much less permeable to albumin. The osmotic force of
albumin in the capillary promotes the return of interstitial fluid to the capillary lumen
dren with ascites is best evaluated by
and counteracts the intralumenal hydrostatic forces. Ascites occurs when the net transfer
trending daily body weights. Adjunctive of fluid from blood vessels to lymphatic vessels exceeds the drainage capacity of the
physical examination findings should lymphatics.
assess for abdominal girth, peripheral Used with permission from Dudley FJ. Pathophysiology of ascites formation. Gastroenterol
edema and age-specific urine output, Clin North Am 1992;21:215-35 and Giefer MJ, Murray KF, Colletti RB. Pathophysiology,
Diagnosis and Management of Pediatric Ascites. J Pediatr Gastroenterol Nutr 2011;52
tachycardia and hypotension. Addition-
(5):503-513.
ally, spot measurement of renal excretion

informahealthcare.com 1283
 Review Lane, Hsu & Murray

No restricted to <1.5 l/day [3]. Enteral fluids should be used when-

ever possible, and intravenous crystalloid should be used with
Systemic vasodilation caution and close attention to the sodium content. Hypertonic
(–)
IV fluids will inevitably result in worsening ascites and fluid
Splanchnic vasodilation
Effective hypovolemia overload and hence only used when specifically indicated for
Renin-angiotensin- acute volume expansion. Hypotonic maintenance fluids should
aldosterone stimulation be utilized to avoid total body sodium overload. With the
exception of severe hyponatremia (serum sodium level
Free water retention
<120 mEq/l), use of hypertonic maintenance intravenous fluids
Plasma volume expansion will lead to worsening ascites and edema [11].

Figure 2. The renin–angiotensin–aldosterone system. Nitric Intravenous albumin

oxide (NO)-mediated systemic and splanchinic vasodilation results
in a reduction in circulating blood volume and effective hypovo-
Significant hepatic synthetic dysfunction in the setting of cir-
lemia. Renal juxtaglomerular cells sense this reduction, triggering rhosis leads to the development of hypoalbuminemia. As albu-
secretion of renin, which acts to convert angiotensin to angioten- min is integral in maintaining intravascular oncotic pressure,
sin I. Angiotensin I is converted to angiotensin II, which is nor- hypoalbuminemia often exacerbates or contributes to the devel-
Downloaded by [University of Canberra] at 21:53 22 October 2017

mally a potent vasoconstrictor but has a blunted response in the opment of ascites. However, the importance of intravenous
setting of cirrhosis. Angiotensin II also stimulates the release of
aldosterone from the adrenal cortex. Aldosterone acts on the
albumin in the management of ascites is not limited to its col-
distal renal tubules to increase the reabsorption of sodium and loidal osmotic function. In decompensated cirrhosis, there is
water, resulting in the net effect of plasma expansion. both a quantitative and qualitative deficiency of albumin.
Recently, the use of intravenous albumin has been shown to be
of sodium and potassium may be helpful in evaluating diuretic of benefit in the reduction of post-paracentesis circulatory dys-
response, with a goal ratio of urine sodium/potassium concen- function and renal complications, increased survival in sponta-
trations of >1 [9]. neous bacterial peritonitis and augmentation of pharmacologic
diuresis [13]. Nonetheless, the routine use of albumin in the
Fluid restriction management of cirrhotic ascites remains unclear [14]. Supple-
The development of hyponatremia in cirrhosis is an important mental albumin may be utilized to support intravascular vol-
prognostic indicator, and is associated with increased morbidity ume when serum concentrations fall below 2.5 g/dl. The use of
and mortality and decreased survival after transplantation [10,11]. 0.5–1 g/kg of dry weight of 5 or 25% albumin may be chosen
Perturbations in effective circulating blood volume in cirrhosis based on desired effect of intravascular volume expansion. To
impair renal elimination of solute-free water. In response to promote maximal diuresis, a loop diuretic is often given follow-
low arterial volumes, subsequent pathologic non-osmotic over- ing the administration of intravenous albumin.
secretion of ADH effectively promotes free water retention
leading to the development of hyponatremia. While reduction Paracentesis
in total body sodium supports a decrease in total body water, The utilization of paracentesis is both diagnostic and therapeu-
fluid restriction is generally not recommended unless serum tic. In adults, large-volume paracentesis (LVP) is recommended
sodium values are <125–130 mEq/l [12]. for the management of abdominal ascites unresponsive to
Meticulous enteral and intravenous fluid management is crit- diuretic therapy. The use of paracentesis in children with ascites
ical in patients with cirrhotic ascites, to avoid the development was first reported by Denzer in 1920 [15]. Historically, para-
of hypo- or hypervolemia. In infants whom are being fed enter- centesis in the pediatric population has been utilized as a diag-
ally, formula may need to be concentrated to maintain euvole- nostic approach to identify underlying etiology of ascites. LVP
mia while promoting adequate nutrition to promote growth. has been advocated as safe and efficacious in children when
Older adolescents with hypervolemia may need to be fluid using optimal technique [16]. When the etiology of ascites is in
question, the measurement of ascites albumin is critical in
determining whether ascites has developed secondary to cirrho-
Table 1. The serum-ascites albumin gradient sis. Specifically, the difference of simultaneously measured con-
differentiates ascites secondary to portal centrations of albumin in the serum and ascitic fluid, or the
hypertension from alternate etiologies. serum-ascites albumin gradient (SAAG), aids in the differentia-
tion of cirrhotic from non-cirrhotic ascites (TABLE 1). A SAAG
SAAG ratio of ‡1.1 g/dl SAAG ratio of £1.1 g/dl ratio of ‡1.1 g/dl is characteristic of ascites secondary to portal
Portal hypertension Malignancy-related ascites hypertension, whereas a SAAG <1.1 g/dl is seen in the absence
Budd–Chiari syndrome Tuberculosis-related ascites of portal hypertension. Using a cutoff of 1.1 g/dl predicts asci-
Portal vein thrombosis Bacterial peritonitis and serositis tes secondary to portal hypertension with 97% accuracy [3]. In
Heart failure Pancreatitis
the majority of cases, a SAAG of £1.1 g/dl effectively rules out
Nephrotic syndrome
portal hypertension as a cause of ascites [3,17].

1284 Expert Rev. Gastroenterol. Hepatol. 9(10), (2015)

 Management of ascites in children Review

The use of diagnostic paracentesis extends beyond the evalu- re-accumulation of ascites, development of circulatory dys-
ation of cirrhotic versus non-cirrhotic ascites. Samples of ascitic function, hepatorenal syndrome and shortened sur-
fluid should be used to inoculate blood culture bottles in the vival [4,14,23,24]. Several studies have examined the use of
clinical suspicion of bacterial peritonitis. Obtaining a complete plasma expanders to mitigate the potential side effects of
blood count with differential, including absolute neutrophil LVP, and have found that while they are effective in pre-
count can aid in the determination of traumatic ascites, infec- venting these complications, they have not been found to
tion or malignancy. An elevated ascitic concentration of amy- significantly improve survival [4,14,23,24]. Oncotic replacement
lase may indicate pancreatitis or intestinal perforation. In the of removed ascitic fluid with intravenous albumin has not
setting of uroascites, ascitic urea and creatinine are higher than been evaluated in the pediatric population. Additionally,
those concurrently measured in the serum. Elevated ascitic bili- LVP introduces the risk of intraperitoneal hemorrhage sec-
rubin indicates biliary or intestinal perforation. Chylous ascites ondary to bleeding mesenteric varices. This complication car-
often has a characteristic milky or turbid appearance, and is ries the highest mortality, estimated at 70% in the adult
defined by a greater concentration triglycerides in ascitic fluid literature [25].
than in serum.
Therapeutic paracentesis is often employed with diuretic- Infection
refractory ascites or when the volume of ascitic fluid causes The infection of ascitic fluid may result in sepsis, encephalop-
Downloaded by [University of Canberra] at 21:53 22 October 2017

significant pain or lung restriction. Anecdotally, removal of athy or renal failure and carries a high risk of mortality. The
ascitic fluid also increases appetite and oral intake in children, spontaneous infection of ascitic fluid, or spontaneous bacterial
which may be particularly helpful in the setting of higher peritonitis (SBP), results from translocation of intraluminal
nutritional requirements in children with cirrhosis. In adults, intestinal bacteria to mesenteric lymph nodes and subsequent
LVP, up to 6 l/day, has been demonstrated to be an effective bacteremia and infection of the ascitic fluid [26]. In hospital-
method to manage ascites and improve pulmonary func- ized adults with cirrhosis, the prevalence of SBP is 10–30%
tion [18,19]. To determine what volume of ascitic fluid may be but incidence or prevalence in the pediatric population is
safely and effectively removed in children, Kramer et al. com- unknown [27–29]. In a prospective case series, the most preva-
pleted a retrospective review of children aged 6 months to lent clinical markers suggestive of SBP, include: fever, volumi-
18 years with tense ascites unresponsive to medical manage- nous ascites and encephalopathy [28]. Other potential clinical
ment who underwent LVP. Fluid removed ranged from markers of SBP include: abdominal pain, altered intestinal
118 ± 56 ml/kg over approximately 3 h. No patient in this motility or ileus and renal failure [28]. Measuring the number
series developed the potential adverse clinical sequelae of of polymorphonuclear leukocytes (PMN) in ascitic fluid sup-
hypotension or hemorrhage indicating that LVP in children is ports the identification of SBP. Using a lower cutoff of
feasible and safe [16]. 250 PMN/mm3 provides the highest sensitivity for SBP,
The protocols for implementation of therapeutic or LVP in whereas 500 PMN/mm3 provides the highest specificity [28,29].
children are largely based on individual or institutional experi- The total protein concentration in ascitic fluid should also be
ence. Generally, baseline coagulation studies (prothrombin time measured to assess risk of SBP as patients with a protein con-
and international normalized ratio), platelet count and hemato- centration <15 g/l have been demonstrated to have increased
crit are obtained prior to the procedure. However, there are no risk of SBP [29].
data to support the implementation of cutoff of coagulation After the bedside inoculation of blood culture bottles with
parameters that precludes paracentesis [20–22] Furthermore, the removed ascitic fluid, the identification of a monomicrobial
routine administration of fresh frozen plasma or platelets before infection indicates SBP where a polymicrobial culture may
paracentesis in coagulopathic patients is not supported by indicate intestinal perforation. The most prevalent bacteria cul-
data [4,20,21]. tured from infected ascitic fluid in the setting of SBP are
Paracentesis may be safely completed without the routine uti- Gram-negative rods. In the pediatric population, the organisms
lization of ultrasound guidance. However, depending of opera- most frequently isolated include: Escherichia coli, Streptococcus
tor experience or institutional protocols, ultrasound may be pneumoniae, Klebsiella pneumoniae, Haemophilus influenzae,
utilized prior to the procedure to identify and mark the safest Streptococcus viridans, Enterococcus and non-typable Streptococcus
access point. Supine positioning is utilized, and a common nee- [30,31].
dle insertion site is two fingerbreadths medial and cephalad to Despite clinical suspicion of SBP, a negative ascites culture
the anterior superior iliac spine. In infants and children, both results in 40% of patients with suspicious clinical manifesta-
conscious sedation and general anesthesia may be necessary to tions and increased PMNs in ascitic fluid [28]. Due to the high
safely and successfully perform the procedure. The use of a par- morbidity and mortality associated with SBP, empiric treat-
acentesis needle (15 gauge fenestrated needle) versus intravascu- ment should not be delayed if clinical suspicion is high, despite
lar catheter (16 or 18 gauge) may result in faster flow rate of negative cultures or low PMN concentration. Empiric use of a
ascitic fluid and shorter duration of procedure [16]. third-generation cephalosporin or b-lactamase inhibitor with
There are several potential adverse clinical effects of penicillin such as piperacillin tazobactam is generally
therapeutic paracentesis. Specifically, LVP can result in acceptable.

informahealthcare.com 1285
 Review Lane, Hsu & Murray

complications, and in pediatrics is largely limited to palliative

care [32,33].
Transjugular intrahepatic portosystemic shunting (TIPS) is
commonly used to temporize or prevent complications of
portal hypertension, including: variceal bleeding, hypersplen-
ism and reduce refractory ascites in adult patients awaiting
liver transplantation [34–36]. With TIPS, an intrahepatic stent
is inserted between one hepatic vein and the portal vein by
a transjugular approach (FIGURES 3 & 4). TIPS attenuates the
pathologic sodium-retaining mechanisms and improves
response to diuresis [4]. Potential adverse outcomes after
TIPS include: shunt stenosis, portal vein thrombosis, hemor-
Figure 3. Transjugular intrahepatic portosystemic shunting rhage, encephalopathy and progressive hepatic failure. The
(TIPS). CT abdomen with intravenous and enteral contrast disadvantages of TIPS in the pediatric population include
demonstrating stent placement creating communication between
high cost, risk of procedure and limited availability to qua-
hepatic vein and portal vein.
ternary care centers. In pediatrics, the use of TIPS may be
Downloaded by [University of Canberra] at 21:53 22 October 2017

TIPS: Transjugular intrahepatic portosystemic shunt.

further limited by small patient size or the presence of vas-
cular malformations. In adult patients, the use of TIPS for
Surgical management the treatment of refractory ascites has been found to be
In patients with medically refractory ascites, the use of surgi- superior to paracentesis, however, there was no long-term
cal shunts are often utilized as a bridge to liver transplanta- survival benefit. Adult studies have demonstrated that
tion. Initially, the peritoneovenous shunt was developed to transplant-free survival is prolonged by TIPS, and there is
create a conduit for intraperitoneal fluid to return to the improved graft survival at 12 months as compared with sub-
intravascular space and potentially restore effective circulating jects in whom TIPS was not done prior to transplant [37].
blood volume. The Denver and LeVeen shunts consist of a However, TIPS has not been shown to increase long-term
perforated tube in the peritoneal cavity with a one-way pres- survival after liver transplantation [37].
sure-sensitive valve connected to a catheter that extends subcu-
taneously into a jugular vein. While these shunts were shown Non-cirrhotic ascites
to be effective in reducing volume of ascites, their use has Cirrhosis from chronic liver disease is the most common eti-
largely been abandoned because of significant rates of ology for ascites in the pediatric and adult population. In
fact, in adults, 85% of ascites is caused by cirrhosis, with less
than 5% attributable to heart failure or malignancy, the next
most common causes [38]. In pediatric patients, however, there
remain numerous other conditions that result in ascites (BOX
1). In those patients, determining the etiology can be difficult
and should be approached systematically. Medical and surgical
management of non-cirrhotic ascites is aimed at targeting
underlying etiology. Due to the wide differential encompassed
by the category ‘non-cirrhotic ascites’, the following discussion
is limited to pathophysiology and diagnosis of non-cirrhotic
causes of ascites.

Non-cirrhotic causes of fetal & childhood ascites

Chylous ascites
The lymphatic system acts as a unidirectional drainage system
to facilitate the mobilization and removal of interstitial fluid,
protein, lipids and bacteria. A majority of the total body vol-
ume of lymph originates from the abdominal viscera, most
notably the small intestine and liver [39]. In the gut, fluid
absorbed from the lumen enters lymphatic capillaries by hydro-
static and oncotic forces and vesicular transport (FIGURE 1). The
Figure 4. Transjugular intrahepatic portosystemic shunt primary mechanisms by which chylous ascites develop include:
(TIPS) placement. Endovascular stent placement over a guide- lymphatic trauma, obstruction or malformation [40–42]. Chylous
wire between the right hepatic vein and the right portal vein. ascites is rare in pediatrics, and differs in etiology from that of
TIPS: Transjugular intrahepatic portosystemic shunt.
adults [39–41,43,44]. In adults, the leading etiology of chylous

1286 Expert Rev. Gastroenterol. Hepatol. 9(10), (2015)

 Management of ascites in children Review

ascites is lymphatic obstruction secondary to abdominal malig- that results in hypoxic damage to hepatocytes and subsequent
nancy, however, neoplasia as a cause of chylous ascites is rare centrilobular fibrosis [48]. The development of venous collater-
in infants and children [41,44]. In the pediatric population, the als is an important compensatory mechanism. Complications
most common causes of chylous ascites are congenital, includ- of Budd–Chiari syndrome include intractable ascites, portal
ing lymphatic hypoplasia or dysplasia such as lymphangiomato- hypertension, cirrhosis, liver failure and gastrointestinal bleed-
sis and intestinal lymphangiectasia [40,44,45]. Chylous ascites in ing. Diagnosis is made non-invasively by abdominal ultraso-
children has been described secondary to surgical injury or nography or MRI. Anticoagulation is the mainstay of
blunt trauma, either accidental or from child abuse [41]. In treatment to prevent recurrence or extension of thrombosis.
addition, chronic liver disease, heart failure and miscellaneous In some patients, porto-caval shunts may be required to
inflammatory processes, including tuberculosis, filariasis and restore patency of hepatic venous outflow.
sarcoidosis may contribute to the formation of intraperitoneal
chyle [40,41,46]. Heart failure
Chylous ascites in children generally presents with abdominal Chronic heart failure may lead to the development of
distension, vomiting, edema and malnutrition secondary to ste- congestive hepatopathy. On biopsy, congestive hepatopathy is
atorrhea or protein-losing enteropathy [40,41]. Diagnostic para- characterized by sinusoidal dilation and congestion progress-
centesis is essential in identifying chyloperitoneum. Chyle is ing to pericellular fibrosis, bridging fibrosis and ultimately to
Downloaded by [University of Canberra] at 21:53 22 October 2017

characteristically milky and turbid, in contrast to the straw- cirrhosis [49]. Children with chronic heart failure in the set-
colored and transparent appearance of ascitic fluid caused by ting of single-ventricle physiology, particularly following Fon-
cirrhosis. An elevated peritoneal fluid to plasma triglyceride tan surgical palliation, have markedly elevated systemic
ratio (two- to eightfold; or total triglyceride, >110 ng/dl) sug- venous pressure, making them especially susceptible to the
gests a diagnosis of chylous ascites [40]. Alternate diagnostic development of chronic liver disease and ascites [49]. Liver
strategies may include lymphoscintigraphy, lymphangiogram or synthetic function is generally preserved in early congestive
surgical exploratory laparotomy. hepatopathy, and the development of depressed serum albu-
The initial management of chylous ascites is nutritional. min levels is generally the result of protein-losing enteropa-
Dietary modification with a high-protein, low-fat diet with thy rather than hepatic dysfunction [49]. Congestive
supplemental medium chain triglycerides has been demon- hepatopathy is evaluated non-invasively with routine labora-
strated to be effective in reducing chylous ascites [41,46]. tory tests and imaging such as ultrasound, CT or MRI. The
Medium chain triglycerides bypass the enteric lymphatics as evolution of liver fibrosis in congestive hepatopathy may be
they are not re-esterified within the intestinal epithelium [46]. evaluated non-invasively by transient elastography or contrast-
Instead, they directly enter the portal system, thus reducing enhanced ultrasound [49]. Transient elastography employs
the formation and flow of chyle. If chylous ascites proves ultrasound to measure the relative stiffness of a cylindrical
refractory to enteral therapy, fasting with total parenteral hepatic sample [49]. Contrast-enhanced ultrasound measures
nutrition (TPN) is often implemented. TPN is an effective transhepatic movement of microbubble contrast agents to
strategy as it bypasses the gut, thereby reducing the produc- detect the presence of cirrhosis [49]. Results may be con-
tion of chyle. Octreotide, a somatostatin analogue, has been firmed by liver biopsy.
utilized as an adjunctive therapy to TPN in the treatment
chylous ascites in children and adults [46]. The mechanism by Biliary ascites
which somatostatin is effective in reducing the formation of Biliary ascites results from iatrogenic or traumatic injury to
chyle is incompletely understood. It has been proposed that extrahepatic bile ducts. In neonates and children, biliary asci-
somatostatin attenuates intestinal absorption of fat, moderates tes may also manifest from spontaneous rupture of biliary
lymph flow in major lymphatic channels and reduces triglyc- malformations such as choledocal cysts. In neonates, the
eride concentrations in thoracic duct lymph [47]. Surgical cor- most common location of rupture is the junction of com-
rection of lymphatic obstruction from malrotation, mesenteric mon bile duct and cystic duct [51]. The mechanism leading
cysts, hernias and lymphatic-peritoneal fistulas is also effective to the spontaneous rupture of extrahepatic bile ducts leading
in promoting resolution of chylous ascites [41]. Lastly, thera- to the development of biliary ascites is not understood. Pro-
peutic paracentesis or surgical peritoneovenous shunts may be posed mechanisms include: decreased blood flow to ductal
effective in managing chylous ascites. walls with resultant necrosis and perforation, increased pres-
sure within the bile duct due to stricture and congenital
Budd–Chiari syndrome weakness of the ductal wall [51]. Clinical consequences of
Budd–Chiari syndrome describes the clinical sequelae follow- injury or rupture of a bile duct include biliary peritonitis
ing thrombosis or obstruction of hepatic venous outflow at and formation of psuedocysts. Clinically, biliary ascites may
the level of the hepatic veins or the inferior vena cava. present with mild direct hyperbilirubinemia, feeding intoler-
A variety of hypercoagulable states, myeloproliferative diseases ance and abdominal distension [51]. Diagnosis is made non-
being the most common, are generally the primary cause. invasively with ultrasound, magnetic resonance cholangiopan-
Budd–Chiari syndrome results in a congestive hepatopathy creatography or hepatobiliary scintigraphy, or invasively by

informahealthcare.com 1287
 Review Lane, Hsu & Murray

endoscopic retrograde cholangiopancreatography. Treatment is complications Diagnosis is made by paracentesis and measure-
generally surgical. ment of elevated lipase or amylase in ascitic fluid [56]. Gener-
ally, treatment involves bowel rest, TPN, octreotide,
Sinusoidal-obstruction syndrome endoscopic retrograde cholangiopancreatography, or
Hepatic veno-occlusive disease/sinusoidal-obstruction syndrome surgery [57].
(SOS) is an obliterative venulitis of the terminal hepatic ven-
ules, and occurs as a result of cytoreductive therapy prior to Systemic inflammatory disorders
hematopoietic stem cell transplantation [52]. SOS occurs from Systemic inflammatory disorders such as systemic lupus erythe-
toxic destruction of hepatic sinusoidal endothelial cells and sub- matosus or rheumatoid arthritis may promote the development
sequent occlusion of terminal hepatic venules. Clinically, SOS of ascites. The mechanism directing the development of lupus
presents as jaundice, tender hepatomegaly, ascites and unex- peritonitis is unclear. Some have proposed that elevated inflam-
plained weight gain. Diagnosis is clinical and can be confirmed matory cytokines such as TNF-a and IL-6, as well as deposi-
by transjugular liver biopsy. Treatment includes meticulous tion of immune complexes in the mesenteric vasculature may
fluid management to avoid fluid overload and paracentesis. play a role in the pathogenesis of serositis and subsequent for-
Pharmacologic strategies include: defibrotide, thrombolytic mation of ascites. Diagnosis of sterile inflammatory peritonitis
agents or methylprednisolone. While surgical portocaval shunts is made based on clinical suspicion, and examination of ascitic
Downloaded by [University of Canberra] at 21:53 22 October 2017

and TIPS have been utilized in the adult population, they are fluid revealing leukocytosis with no evidence of malignancy or
not used in the pediatric patients with SOS. Their use in adults infection. One study demonstrated that lupus peritonitis was
has not been shown to significantly affect survival. Liver trans- associated with low complement levels, elevated anti-DNA anti-
plantation may ultimately be required. body and IL-6 in the ascitic fluid [58]. Abdominal ultrasonogra-
phy and computed tomography may reveal bowel wall
Peritoneal infection thickening, intraluminal fluid collection and ascites.
Infection of the peritoneal cavity may result in the formation
of ascites. A variety of bacterial, parasitic and viral agents may Nephrotic syndrome
lead to the development of peritonitis and ascites. In endemic Hypervolemia and hypoalbuminemia are likely the main factors
areas, tuberculosis is a cause of congenital or acquired ascites in driving the pathogenesis of the formation of ascites in the set-
infants and children [51,53]. Clinical suspicion is raised in the ting of nephrotic syndrome. The decreased effective arterial
setting of fever, abdominal pain and progressive abdominal dis- blood volume activates the sympathetic nervous system, renin–
tension. Diagnosis is made by paracentesis with ascitic fluid angiotensin–aldosterone system and secretion of ADH, leading
analysis that is positive for adenosine deaminase activity, acid to renal sodium and water retention and development of
fast staining or PCR positive for Mycobacterium tuberculosis hypervolemia. Additionally, the hypoalbuminemia characteristic
[53,54]. Laparoscopy and peritoneal biopsy may be for the diag- of nephrotic syndrome may result in low oncotic gradients in
nosis of tuberculous peritonitis [54]. hepatic sinusoids and in splanchnic capillaries, favoring a net
movement of fluid into the peritoneal cavity. In comparing
Intestinal inflammation adults and children with nephrotic syndrome, one study found
Ascites is a rare extraintestinal manifestation of inflammatory that ascites tended to be more prevalent among pediatric
bowel disease. While secondary malignancy or portal hyperten- patients [59]. They also identified that, in general, pediatric
sion can occur in the setting of inflammatory bowel disease patients with nephrotic syndrome and ascites tended to have
and may contribute to the development of ascites, transudative lower serum albumin levels than adults [59]. Interestingly,
ascites may also occur [55]. The mechanism promoting the within pediatric patients with nephrotic syndrome with and
development of ascites in inflammatory bowel disease without without ascites, there was not a significant difference in serum
liver involvement is unknown. Proposed mechanisms include albumin levels, indicating that other factors in addition to
transmural bowel inflammation and lymphatic stasis [55]. Diag- hypoalbuminemia are important in promoting the formation of
nosis is generally by exclusion of alternate causes of ascites, ascites.
including portal hypertension and infection.
Ascites as a rare presentation of eosinophilic enteropathy Urinary ascites
with serosal involvement must also be considered in children Accumulation of urine in the peritoneal cavity may occur sec-
presenting with non-portal hypertension associated ascites and ondary to obstructive uropathy, complex urinary anomalies and
intestinal symptoms. from iatrogenic or traumatic perforation of the ureter, renal pel-
vis or bladder [51]. Urinary ascites in the setting of urinary mal-
Pancreatic ascites formations generally presents in the fetal or neonatal period. In
Pancreatic ascites is caused by leakage of pancreatic fluid into infants and children, posterior urethral valves are most common
the peritoneal cavity. In infants and children, pancreatic ascites etiology of urinary ascites [51]. Clinically, neonates may present
most commonly results from infection or trauma. Fluid shifts with respiratory distress due to lung hypoplasia. Azotemia,
and systemic inflammatory response are common clinical hyperkalemia, hyponatremia and metabolic acidosis can also be

1288 Expert Rev. Gastroenterol. Hepatol. 9(10), (2015)

 Management of ascites in children Review

seen. Diagnosis is made by ultrasound, cystourethrograms or respiratory reserve and compromised immunity, these infants
cystoscopy. The identification of an elevated creatinine level in and children are particularly vulnerable to complications from
the ascitic fluid is diagnostic. Management is generally surgical. ascites.
Elucidation of the underlying diagnosis can often be made
Malignancy through examination of the liver (particularly with ultrasound
Malignant ascites describes the presence of malignant cells in or histologic studies). The advent of gene identification has
the peritoneal cavity and is a poor prognostic sign [60]. Perito- obviated the requirement for liver biopsy only in rare cases of
neal carcinomatosis is the result of peritoneal surface malignan- inherited liver disease, although this may change in the future
cies generally from ovarian, colorectal, pancreatic and uterine as non-invasive methods are increasingly used to characterize
origin or extra-abdominal tumors originating from lymphoma, diagnoses.
lung and breast. The pathophysiology of malignant ascites is This review outlines the general approach to ascites in chil-
complex and multifactorial, but is generally due to altered vas- dren. Meticulous management of fluids and diuretics can aid
cular permeability and lymphatic obstruction with a net effect in elimination and prevention of ascites. When ascites is refrac-
that favors the accumulation of intraperitoneal fluid. Diagnosis tory to medical therapy, surgical peritoneovenous shunt options
is made by paracentesis and examination of ascitic fluid. The have largely fallen out of favor in lieu of the TIPS shunt/
ascites fluid in peritoneal carcinomatosis has positive cytology, concurrent liver transplantation listing. TIPS are being used in
Downloaded by [University of Canberra] at 21:53 22 October 2017

elevated protein concentrations and a low serum-ascites albumin larger numbers in pediatrics but remain a relatively new treat-
gradient. Ascitic cytology is highly sensitive and is the gold stan- ment option, often limited by patient size or vascular anatomic
dard in diagnosing peritoneal carcinomatosis [48]. The presence deviations. It is unknown whether the outcomes of TIPS as a
of tumor markers such as CEA, CA-125 and a-fetoprotein treatment in children mirrors that of adults, where there is no
ascitic fluid may be helpful in determining the presence of long-term survival benefit, but can serve as a bridge to liver
malignant ascites and origin of the primary malignancy [48]. transplantation.
Laparoscopic biopsy is often necessary. Therapeutic options are
limited and management is frequently palliative. Depending on Five-year view
primary tumor biology and stage, surgical debulking and intra- With increasing numbers of adults on the liver transplant
peritoneal chemotherapy may be utilized [48]. waitlist, we expect to see sicker pediatric patients as they
wait for deceased donor organs to become available. In
Pseudo-ascites light of increased wait times, it is of paramount importance
Intrabdominal cystic masses often masquerade as ascites. that we improve ascites management to minimize both pre-
Mesenteric or omental cysts as well as other cystic tumors of and post-transplant morbidity. The future is likely to lie in
fluid collections which originate from abdominal organs such diagnosis and development of expedited multidisciplinary
as ovarian tumors or hematocolpos may be mistaken as ascites. care for management of ascites. Some centers are adopting
Abdominal lymphangioma is a rare tumor associated with the the early institution of parenteral nutrition in younger
development of mesenteric, omental or retroperitoneal cysts patients with biliary atresia [50], with the goal of improving
that is frequently difficult to distinguish from ascites from alter- nutritional status and tightly controlling fluid balance in
nate causes. Diagnosis is made by abdominal ultrasound but these particularly nutritionally vulnerable patients. In
cannot be differentiated from ascites without laparoscopy. patients who are continuing to decline despite fortified,
Treatment is surgical excision of the primary tumor [52]. high-calorie formula enteral feeds, parenteral nutrition can
be the key to restoring them to a healthier state with the
Other hope of ultimately prolonging meaningful survival and
Iatrogenic causes of intraperitoneal fluid collections are of par- improving long-term outcomes.
ticular concern in the neonatal population. Intraperitoneal fluid
collections may be due to gastric perforation from feeding Financial & competing interests disclosure
tubes and subsequent intraperitoneal formula accumulation, or The authors have no relevant affiliations or financial involvement with
from umbilical catheter perforation with leakage of parenteral any organization or entity with a financial interest in or financial con-
nutrition into the peritoneal cavity [51]. flict with the subject matter or materials discussed in the manuscript.
This includes employment, consultancies, honoraria, stock ownership or
Expert commentary options, expert testimony, grants or patents received or pending or
Ascites contributes to a significant degree of morbidity in chil- royalties.
dren with chronic liver disease. Due to their decreased No writing assistance was utilized in the production of this manuscript.

informahealthcare.com 1289
 Review Lane, Hsu & Murray

Key issues
. Disruption of the balance in hydrostatic and oncotic forces regulating splanchnic, portal and hepatic blood, and lymphatic flow, results
in the accumulation of ascites.
. Restriction of sodium intake to 1–2 mEq sodium/kg body weight is critical to the medical management of ascites.
. Combination treatment with spironolactone and furosemide results in more rapid mobilization of ascites, and is usually needed in cases
of ascites from significant portal hypertension.
. Development of hepatorenal syndrome in children is often marked by progressive oliguria and a rise in serum creatinine. Caution must
be exercised when using diuretics in the setting of cirrhotic ascites and renal dysfunction due to the risk of pre-renal azotemia and renal
failure with excessive intravascular volume depletion.
. Fluid restriction is recommended only in setting of hyponatremia with serum sodium values of <125 mEq/l.
. Albumin is integral in maintaining intravascular oncotic pressure. Supplemental albumin (0.5–1 g/kg of dry weight of 5 or 25% albumin
intravenously) may be utilized to support intravascular volume when serum concentrations fall below 2.5 g/dl.
. Diagnostic paracentesis should be utilized when ascites accumulates quickly, and in the setting of clinical deterioration suggesting
infection, as evidenced by the development of encephalopathy, fever or increasing abdominal pain.
Downloaded by [University of Canberra] at 21:53 22 October 2017

. The difference in simultaneously measured concentrations of albumin in the serum and ascitic fluid, or the serum-ascites albumin
gradient (SAAG), aids in the differentiation of cirrhotic from non-cirrhotic ascites. A SAAG ratio of >1.1 g/dl is diagnostic of ascites sec-
ondary to portal hypertension, where as a SAAG <1.1 g/dl is seen in the absence of portal hypertension.
. In adults, transjugular intrahepatic portosystemic shunting for the treatment of refractory ascites has been found to be superior to
paracentesis and prolongs transplant-free survival, however, does not result in a long-term survival benefit.

References 5. Runyon B; Practice Guidelines Committee, 12. Ginès P, Berl T, Bernardi M, et al.
American Association for the Study of Liver Hyponatremia in cirrhosis: from
Papers of special note have been highlighted as:
. of interest
Diseases (AASLD). Management of adult pathogenesis to treatment. Hepatology
.. of considerable interest patients with ascites due to cirrhosis. 1998;28:851-64
Hepatology 2004;39:841-56 13. Alves de Mattos A. Current indications for
1. Pinto R, Schneider A, da Silveira T.
6. Runyon B. Management of adult patients the use of albumin in the treatment of
Cirrhosis in children and adolescents: An
with ascites caused by cirrhosis. Hepatology cirrhosis. Ann Hepatol 2011;10(Suppl 1):
overview. World J Hepatol 2015;7(3):
1998;27(1):264-72 S15-20
392-405
7. Koo WW, Gupta JM. Breast milk sodium. . Reviews the major indications for the use
. Describes clinical manifestations
Arch Dis Child 1982;57:500-2 of intravenous albumin in the treatment
and management of end-stage
8. Santos J, Planas R, Pardo A, et al. of cirrhosis.
liver disease and cirrhosis in pediatric
patients. Spironolactone alone or in combination 14. Ginès P, Tito L, Arroyo V, et al.
with furosemide in treatment of moderate Randomized comparative study of
2. Giefer M, Murray K, Colletti R. ascites in nonazotemic cirrhosis. therapeutic paracentesis with and without
Pathophysiology, Diagnosis, and A randomized comparative study of efficacy intravenous albumin in cirrhosis.
Management of Pediatric Ascites. and safety. J Hepatol 2003;39:187-92 Gastroenterology 1988;94:1493-502
J Pediatr Gastroenterol Nutr 2011;52(5):
. Provides evidence to of support safety .. Demonstrated that intravenous albumin
503-13
and efficacy of spironolactone as is important in preventing renal and
. This review summarizes the physiologic
monotherapy as compared with electrolyte complications and pathologic
mechanisms for cirrhotic and
spironolactone with furosemide as activation of endogenous vasoactive
noncirrhotic ascites with
treatment for moderate ascites. systems in cirrhotics with ascites treated
particular attention to unique aspects in
9. El-Bokl MA, Senousy BE, El-Karmouty KZ, with large-volume paracentesis.
children.
et al. Spot urinary sodium for assessing 15. Denzer B. New method of diagnosis of
3. European Association for Study of Liver. dietary sodium restriction in cirrhotic ascites. peritonitis in infancy and childhood:
EASL Clinical Practice Guidelines on the World J Gastroenterol 2009;15(29):3631-5 preliminary report. Am J Dis Child 1920;
Management of Ascites, SBP, and
10. John S, Thuluvath PJ. Hyponatremia in 113-14
hepatorenal syndrome in cirrhosis.
J Hepatol 2010;53(3):397-417 cirrhosis: pathophysiology and management. 16. Kramer R, Sokol R, Yerushalmi B, et al.
World J Gastroenterol 2015;21(11): Large-Volume Paracentesis in Management
4. Ginès P, Cárdenas A, Arroyo V, Rodes J. 3197-205 of Ascites in Children. J Pediatr
Management of Cirrhosis and Ascites. N
11. Gianotti RJ, Cardenas A. Hyponatraemia Gastroenterol Nutr 2001;33(3):245-9
Engl J Med 2004;350:1646-54
and cirrhosis. Gastroenterol Rep (Oxf) .. First publication to demonstrate that
. Provides comprehensive review of 2014;2(1):21-6 large-volume paracentesis is a safe and
pathophysiology and management of
ascites.

1290 Expert Rev. Gastroenterol. Hepatol. 9(10), (2015)

 Management of ascites in children Review

effective method for managing ascites in Spontaneous bacterial Peritonitis: 39. Lesser GT, Bruno MS, Enselberg K.
children. a consensus document. J Hepatol 2000; Chylous ascites: newer insights and many
17. Runyon B, Montano A, Akriviadis E, et al. 32(1):142-53 remaining enigmas. Arch Intern Med
The serum–ascites albumin gradient is 29. Rimola A, Garcia-Tsao G, Navasa M, et al. 1970;125:1073-7
superior to the exudate–transudate concept Diagnosis, treatment and prophylaxis of 40. Aalami OO, Allen DB, Organ CH.
in the differential diagnosis of ascites. Ann spontaneous bacterial peritonitis: Chylous ascites: A collective review. Surgery
Intern Med 1992;117:215-20 a consensus document. J Hepatol 2000;32: 2000;128(5):761-78
.. Demonstrated that the serum-ascites 142-53 41. Cochran WJ, Klish WJ, Broan MR, et al.
albumin gradient is a marker for portal 30. Haghighat M, Dehghani SM, Alborzi A, Chylous ascites in Infants and Children:
hypertension, and that the et al. Organisms causing spontaneous A case report and literature review. J Pediatr
exudate-transudate concept should be bacterial peritonitis in children with liver Gastroenterol Nutr 1985;4:668-73
discarded in the classification of ascites. disease and ascites in southern Iran. World J . Reviews literature of cases of pediatric
18. Tito M, Ginès P, Arroyo V, et al. Total Gastroenterol 2006;12:5890-2 chylous ascites. Discusses use of total
paracentesis associated with intravenous 31. Caruntu FA, Benea L. Spontaneous bacterial parenteral nutrition as safe and effective
albumin in the management of patients peritonitis: pathogenesis, diagnosis, therapeutic modality for chylous ascites.
with cirrhosis and ascites. Gastroenterology treatment. J Gastrointestin Liver Dis
42. Cárdenas A, Chopra S. Chylous ascites. Am
1990;98(1):146-51 2006;15:51-6
Downloaded by [University of Canberra] at 21:53 22 October 2017

J Gastroenterol 2002;97(8):1896-900
19. Angueira C, Kadakia S. Effects of 32. Gines P, Arroyo V, Vargas V, et al.
43. Unger SW, Chandler JG. Chylous ascites in
large-volume paracentesis on pulmonary Paracentesis with intravenous infusion of
infants and children. Surgery 1983;93(3):
function in patients with tense cirrhotic albumin as compared with peritoneovenous
455-61
ascites. Hepatology 1994;20(4 Pt 1):825-8 shunting in cirrhosis with refractory ascites.
. Reviews existing literature describing
20. Runyon BA. Paracentesis of ascitic fluid. N Engl J Med 1991;325:829-35
clinical presentation, diagnosis and
A safe procedure. Arch Intern Med 1986; 33. Fulenwider JT, Smith RB 3rd, Redd SC,
et al. Peritoneovenous shunts. Lessons management of chylous ascites in infants
146(11):2259-61
learned from an eight-year experience with and children.
21. Grabau C, Crago S, Hoff L, et al.
70 patients. Arch Surg 1984;119:1133-7 44. Browse NL, Wilson NM, Russo F, et al.
Performance standards for therapeutic
Ochs A, Rossle M, Haag K, et al. The Aetiology and treatment of chylous ascites.
abdominal paracentesis. Hepatology 2004; 34.
transjugular intrahepatic portosystemic Br J Surg 1992;79(11):1145-50
40(2):484-8
stent-shunt procedure for refractory ascites. 45. Ingle SB, Hinge Ingle CR. Primary
22. Runyon BA. Management of adult patients
N Engl J Med 1995;332:1192-7 intestinal lymphangiectasia: Minireview.
with ascites due to cirrhosis: an update.
Heyman MB, LaBerge JM, Somberg KA, World J Clin Cases 2014;2(10):528-33
Hepatology 2009;49(6):2087-107 35.
et al. Transjugular intrahepatic 46. Karagol BS, Zenciroglu A, Gokce S, et al.
23. Panos M, Moore K, Vlavianos P, et al.
portosystemic shunts (TIPS) in children. Therapeutic management of neonatal
Single, total paracentesis for tense ascites:
J Pediatr 1997;131(6):914-19 chylous ascites: report of a case and review
sequential hemodynamic changes and right
. Retrospective case review of pediatric of the literature. Acta Paediatr 2010;99(9):
atrial size. Hepatology 1990;11:662-7
1307-10
24. Pozzi M, Osculati G, Boari G, et al. Time TIPS placements.
. Reviews the therapeutic management
course of circulatory and humoral effects of 36. Johnson SP, Leyendecker JR, Joseph FB,
rapid total paracentesis in cirrhotic patients et al. Transjugular portosystemic shunts in procedures on chylous ascites in
with tense, refractory ascites. pediatric patients awaiting liver childhood, including the use of medium-
Gastroenterology 1994;106:709-19 transplantation. Transplantation 1996;62(8): chain triglyceride-based diets,
1178-81 somatostatin and TPN.
25. Arnold C, Haag K, Blum HE, Rössle M.
Acute hemoperitoneum after large-volume . Case series suggesting that TIPS 47. Collard JM, Laterre PF, Boemer F, et al.
paracentesis. Gastroenterology 1997;113(3): placement in children is technically Conservative treatment of postsurgical
978-82 feasible, does not complicate subsequent lymphatic leaks with somatostatin-14. Chest
surgery, and is useful in treatment of 2000;117:902-5
26. Koulaouzidis A, Bhat S, Saeed AA.
Spontaneous bacterial peritonitis. World J acute variceal hemorrhage in pediatric 48. Aydinli M, Bayraktar Y. Budd-Chiari
Gastroenterol 2009;15(9):1042-9 patients awaiting liver transplantation. syndrome: etiology, pathogenesis and
diagnosis. World J Gastroenterol 2007;
27. Vieira S, Matte U, Kieling C, et al. Infected 37. Guerrini GP, Pleguezuelo M, Maimone S,
13(19):2693-6
and Noninfected Ascites in Pediatric et al. Impact of tips preliver transplantation
Patients. J Pediatr Gastroenterol Nutr 2005; for the outcome posttransplantation. Am J 49. Rychik J, Veldtman G, Rand E. The
40(3):289-94 Transplant 2009;9(1):192-200 precarious state of the liver after a Fontan
operation: summary of a multidisciplinary
. Describes prevalence of spontaneous 38. Runyon BA. Ascites and bacterial
symposium. Pediatr Cardiol 2012;33(7):
bacterial peritonitis in children and peritonitis. In: Sleisenger MH, Fordtran JS,
1001-12
reviews clinical characteristics of children editors. Gastrointestinal disease:
pathophysiology, diagnosis, management. . Review summarizes a consensus view
with ascites who are more likely to have
infected vs noninfected ascites. 9th edition. Saunders Elsevier: Philadelphia; achieved at a multidisciplinary
2010 symposium. The discussion describes the
28. Rimola A, Garcı́a-Tsao G, Navasa M, et al.
pathophysiology of liver disease,
Diagnosis, treatment and prophylaxis of

informahealthcare.com 1291
 Review Lane, Hsu & Murray

diagnostic strategies, and unique 52. Egozi EI, Ricketts RR. Mesenteric and 56. Runyon BA. Amylase levels in ascitic fluid.
histopathology of the liver after the omental cysts in children. Am Surg 1997; J Clin Gastroenterol 1987;9(2):172-4
Fontan operation for congenital heart 63(3):287-90 57. D’Cruz AJ, Kamath PS, Ramachandra C,
disease. . Case series of children with mesenteric Jalihal A. Pancreatic ascites in children. Acta
50. Sullivan JS, Sundaram SS, Pan Z, Sokol RJ. and omental cysts is reported. Paediatr Jpn 1995;37(5):630-3
Parenteral nutrition supplementation in Watanabe R, Fujii H, Kamogawa Y, et al.
53. Riquelme A, Calvo M, Salech F, et al. 58.
biliary atresia patients listed for liver Chronic lupus peritonitis is characterized by
Value of adenosine deaminase (ADA) in
transplantation. Liver Transpl 2012;18(1): the ascites with a large content of
ascitic fluid for the diagnosis of tuberculous
120-8 interleukin-6. Tohoku J Exp Med 2015;
peritonitis: a meta-analysis. J Clin
51. Aslam M, DeGrazia M, Gregory ML. Gastroenterol 2006;40(8):705-10 235(4):289-94
Diagnostic evaluation of neonatal ascites. Ackerman Z. Ascites in Nephrotic
54. Dinler G, Sensoy G, Helek D, Kalayci AG. 59.
Am J Perinatol 2007;24(10):603-9 syndrome. Incidence, patients’
Tuberculous peritonitis in children: report
. Discusses case of congenital ascites of nine patients and review of the literature. characteristics, and complications. J Clin
secondary to posterior urethral valves and World J Gastroenterol 2008;14(47):7235-9 Gastroenterol 1996;22(1):31-4
reviews literature regarding 55. Paspatis GA, Kissamitaki V, Kyriakakis E, 60. Sangisetty SL, Miner TJ. Malignant ascites:
pathophysiology, diagnosis, and et al. Ascites associated with the initial A review of prognostic factors,
management of fetal and neonatal ascites.
Downloaded by [University of Canberra] at 21:53 22 October 2017

presentation of Crohn’s disease. Am J pathophysiology and therapeutic measures.

Gastroenterol 1999;94(7):1974-6 World J Gastrointest Surg 2012;4(4):87-95

1292 Expert Rev. Gastroenterol. Hepatol. 9(10), (2015)