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Published on: February 28, 2025
[Spontaneous idiopathic chylopericardium in childhood]
1Cardiologie pédiatrique, Université Paris V, Necker-Enfants malades, Paris.
Insights
A rare case of primary idiopathic chylopericardium in a child was successfully treated. Thoracic duct ligation and pericardectomy resolved the pericardial effusion, preventing recurrence.
Area of Science:
- Cardiology
- Pediatric Surgery
- Thoracic Surgery
Background:
- Primary idiopathic chylopericardium is a rare condition characterized by the accumulation of chylous fluid in the pericardial space.
- Diagnosis in pediatric patients often involves identifying chest pain and fatigue as key symptoms.
Observation:
- A 13-year-old child presented with symptoms suggestive of pericardial effusion.
- Initial management included pericardiocentesis, draining 800mL of chylous fluid, but the cause remained elusive despite extensive investigations.
- Recurrent effusions occurred despite a medium-chain triglyceride diet.
Findings:
- Advanced imaging, including CT scans, lymphoscintigraphy, and MRI, failed to reveal a direct connection between the thoracic duct and the pericardium.
- Surgical intervention involving thoracic duct ligation and partial pericardectomy was performed due to recurrent effusions.
- The surgical approach resulted in complete resolution of the chylopericardium.
Implications:
- This case highlights thoracic duct ligation and partial pericardectomy as an effective treatment for refractory primary idiopathic chylopericardium in children.
- The findings suggest that surgical management can be considered when conservative measures fail.
- Successful long-term resolution without recurrence underscores the viability of this surgical strategy.
Abstract:
Here we report a case of a primary idiopathic chylopericardium in a 13 years old child. Pericardial effusion was diagnosed because the child suffered chest pain and fatigue. Pericardial drainage was performed and 800mL of chylous fluid was evacuated. Extensive investigations were performed but no cause could be found. Thoracic CT scan, lymphoscintigraphy and MRI did not evidence any communication between the thoracic duct and pericardium. After 2 recurrences of pericardial effusion while the child was on a medium chain triglycerides regimen, it was decided to ligate the thoracic duct and to do a partial pericardectomy. The result was excellent with complete resolution of the pericardial effusion and no recurrence since 3 years.
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