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Chylothorax associated with massive osteolysis (Gorham's syndrome)
Koichi Fujiu1, Ryuzo Kanno, Hiroyuki Suzuki
1First Department of Surgery, Fukushima Medical University, Japan. kfujiu@fmu.ac.jp
The Annals of Thoracic Surgery
|June 25, 2002
Summary
Gorham's syndrome with bilateral chylothorax is rare and challenging to treat. Vascular proliferation in the pleura and ribs can cause persistent chylothorax, leading to poor prognosis and death from lymphocytopenia.
Area of Science:
- Medicine
- Cardiology
- Oncology
Background:
- Gorham's syndrome is a rare disorder characterized by bone and soft tissue proliferation.
- Chylothorax, the accumulation of lymphatic fluid in the pleural space, can be a complication of Gorham's syndrome.
Observation:
- A 15-year-old male patient presented with bilateral chylothorax secondary to Gorham's syndrome.
- Despite thoracic duct ligation, fluid reaccumulation persisted, leading to the patient's death from lymphocytopenia.
Findings:
- Autopsy revealed significant vascular proliferation in the pleura and ribs, identified as the cause of persistent chylothorax.
- A review of 22 literature cases of Gorham's syndrome with chylothorax was conducted to analyze treatment outcomes.
Implications:
- This case highlights the critical role of vascular proliferation in the pathogenesis of chylothorax in Gorham's syndrome.
- Effective treatment strategies for Gorham's syndrome-associated chylothorax remain limited, underscoring the need for further research.