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[Generalized lymphangiomatosis with chylopericardium]
A Manetti1, L De Simone, I Pollini
1Unità Operativa di Cardiologia, Ospedale A. Meyer, Firenze, Italia.
Insights
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.
Abstract:
A case of a child with lymphangiomatosis and chylopericardium is reported. Diagnosis of chylopericardium was performed at 11 months with ascendant lymphography which evidenced thoracic duct agenesia. At the age of 3 years he underwent operation of pleuro pericardial window. He was also treated with low fat medium chain triglyceride diet, and temporary clinical improvement occurred. The patient died at 13 years of age because of extensive lymphangiomatosis to abdomen, lungs and bones.