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Hemopneumothorax associated with Marfan syndrome and congenital afibrinogenemia

Ryuzo Kanno1, Hiroyuki Suzuki, Koichi Fujiu

  • 1First Department of Surgery, Fukushima Medical University, Hikarigaoka, Fukushima, Japan. ryuzo@fmu.ac.jp

The Annals of Thoracic Surgery
|April 10, 2003
PubMed

Insights

Congenital afibrinogenemia patients needing surgery require bleeding prophylaxis. This case highlights successful video-assisted thoracoscopic surgery for hemopneumothorax in a Marfan syndrome patient with afibrinogenemia, using fibrinogen therapy.

Area of Science:

  • Cardiology
  • Thoracic Surgery
  • Hematology

Background:

  • Congenital afibrinogenemia is a rare bleeding disorder.
  • Marfan syndrome is a genetic connective tissue disorder.
  • Afibrinogenemia necessitates careful management during surgical procedures to prevent excessive bleeding.

Observation:

  • A 14-year-old male with Marfan syndrome and congenital afibrinogenemia presented with hemopneumothorax.
  • Hemopneumothorax is a serious condition involving air and blood in the chest cavity.

Findings:

  • Video-assisted thoracoscopic surgery (VATS) was successfully performed.
  • Intravenous fibrinogen administration was used for perioperative bleeding prophylaxis.
  • Close monitoring of plasma fibrinogen levels was crucial for surgical success.

Implications:

  • This case demonstrates the feasibility of VATS in patients with congenital afibrinogenemia and Marfan syndrome.
  • Effective bleeding prophylaxis with fibrinogen is vital for managing complex surgical cases in patients with rare bleeding disorders.
  • This approach may improve outcomes for similar patients requiring thoracic surgery.

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