Overview
Chylous ascites is a rare condition characterized by the accumulation of chyle (a milky, lipid-rich lymphatic fluid) within the peritoneal cavity. It results from disruption or obstruction of the lymphatic system, leading to leakage of chyle from lymphatic vessels into the abdominal cavity. The condition can occur as a congenital or acquired disorder. Congenital chylous ascites, also known as congenital chyloperitoneum, typically presents in neonates or infants and may be associated with lymphatic malformations, intestinal lymphangiectasia, or primary lymphatic dysplasia. Acquired forms can result from abdominal surgery, trauma, malignancy (particularly lymphomas), cirrhosis, infections such as tuberculosis, or radiation therapy. The primary clinical features include progressive abdominal distension, abdominal discomfort or pain, and weight gain due to fluid accumulation. Patients may also experience malnutrition, failure to thrive (in children), dyspnea from increased abdominal pressure, and peripheral edema. Diagnosis is confirmed by paracentesis revealing a milky peritoneal fluid with elevated triglyceride levels (typically greater than 200 mg/dL) and a predominance of lymphocytes. Treatment depends on the underlying cause and severity. Conservative management includes dietary modification with a high-protein, low-fat diet supplemented with medium-chain triglycerides (MCTs), which are absorbed directly into the portal venous system rather than through lymphatics. Total parenteral nutrition may be required in severe cases. Octreotide, a somatostatin analogue, has been used to reduce lymphatic flow. Repeated therapeutic paracentesis may be necessary to relieve symptoms. In refractory cases, surgical intervention such as peritoneovenous shunting, ligation of leaking lymphatic vessels, or lymphovenous anastomosis may be considered. The prognosis varies widely depending on the etiology, with congenital forms sometimes resolving spontaneously in infants.
Clinical phenotype terms— hover any for plain English:
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
1 eventEunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Chylous ascites.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Chylous ascites at this time.
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Specialists
View all specialists →No specialists are currently listed for Chylous ascites.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Chylous ascites.
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Start the conversation →Latest news about Chylous ascites
Disease timeline:
New recruiting trial: A Prospective Natural History Study of Lymphatic Anomalies
A new clinical trial is recruiting patients for Chylous ascites
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Common questions about Chylous ascites
What is Chylous ascites?
Chylous ascites is a rare condition characterized by the accumulation of chyle (a milky, lipid-rich lymphatic fluid) within the peritoneal cavity. It results from disruption or obstruction of the lymphatic system, leading to leakage of chyle from lymphatic vessels into the abdominal cavity. The condition can occur as a congenital or acquired disorder. Congenital chylous ascites, also known as congenital chyloperitoneum, typically presents in neonates or infants and may be associated with lymphatic malformations, intestinal lymphangiectasia, or primary lymphatic dysplasia. Acquired forms can re