Primary idiopathic chylopericardium
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Updated: Sep 26, 2026

An Intact Pericardium Ischemic Rodent Model
Published on: September 2, 2021
Mohamad H Ossiani1, Roy G K McCauley, Hitendra T Patel
1Tufts-New England Medical Center, 750 Washington Street, Boston, MA 021110, USA.
Insights
Primary idiopathic chylopericardium, a rare pediatric pericardial effusion of unknown cause, requires clinical awareness. Early identification is crucial for managing this condition, even in infants.
Area of Science:
- Pediatric Cardiology
- Thoracic Surgery
- Rare Diseases
Background:
- Primary idiopathic chylopericardium is a rare cause of pericardial effusion in children.
- It is distinct from secondary causes like surgery, trauma, infections, or tumors.
Observation:
- This report details two cases of primary idiopathic chylopericardium in pediatric patients.
- One case involved an infant as young as 6 weeks old, representing the youngest reported instance.
Findings:
- The etiology of primary idiopathic chylopericardium remains unknown.
- The condition presents as an enlarged cardiac silhouette in affected children.
Implications:
- Clinicians should consider primary idiopathic chylopericardium in pediatric patients with enlarged cardiac silhouettes.
- Awareness of this rare entity is vital for timely diagnosis and management in children.
Abstract:
Primary idiopathic chylopericardium is rare and represents a fraction of all pericardial effusions in the pediatric population. Most cases of chylopericardium occur as a complication of cardiac surgery or after trauma. They may also be caused by tuberculosis, neoplasm, or congenital lymphangiomatosis. Primary idiopathic chylopericardium is a rare disease with unknown etiology. In assessing pediatric patients with an enlarged cardiac silhouette one should be aware of this entity and its presentation. We report two cases of primary idiopathic chylopericardium, one of which at 6 weeks of age is the youngest case to our knowledge so far reported.
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