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[Generalized lymphangiomatosis with chylopericardium]
A Manetti1, L De Simone, I Pollini
1Unità Operativa di Cardiologia, Ospedale A. Meyer, Firenze, Italia.
Summary
This case report details a child with lymphangiomatosis and chylopericardium, highlighting diagnostic methods and treatment challenges. The condition ultimately proved fatal due to widespread disease progression.
Area of Science:
- Pediatric Cardiology
- Medical Genetics
- Thoracic Surgery
Background:
- Lymphangiomatosis is a rare congenital disorder characterized by abnormal proliferation of lymphatic vessels.
- Chylopericardium, the accumulation of lymphatic fluid in the pericardial space, can be a life-threatening complication.
Observation:
- A case of a child diagnosed with lymphangiomatosis and chylopericardium at 11 months is presented.
- Ascendant lymphography revealed thoracic duct agenesis, a significant finding in lymphatic malformations.
- The patient underwent surgical intervention (pleuropericardial window) and dietary management (low-fat medium-chain triglyceride diet).
Findings:
- Initial treatment provided temporary clinical improvement, but the underlying lymphangiomatosis continued to progress.
- Extensive lymphangiomatosis affecting the abdomen, lungs, and bones was observed.
- The patient succumbed to the disease at 13 years of age.
Implications:
- This case underscores the challenges in managing pediatric lymphangiomatosis with chylopericardium.
- Early diagnosis and multidisciplinary management are crucial for improving outcomes in lymphatic anomalies.
- Further research into novel therapeutic strategies for extensive lymphangiomatosis is warranted.