Marfan syndrome coagulopathy in a patient undergoing bilateral video assisted thoracic surgery (VATS): A case report

Abstract

Introduction

Marfan syndrome is an inherited connective tissue disease that contributes to a wide range of health challenges, many of which can cause potential harm to several organs due to underlying coagulopathy.

Case presentation

A 20-year-old male with Marfan syndrome underwent an elective bilateral video-assisted thoracic surgery (VATS) for the management of recurrent pneumothorax after three prior episodes that required hospitalization. The procedure included mechanical and chemical pleurodesis, stapling of the apex of the right lung due to the presence of subpleural blebs, and placement of bilateral chest tubes (one per side). The patient was electively admitted to the pediatric intensive care unit (PICU) after the operation. The postoperative chest x-ray done for hours after the operation showed a large amount of fluid in the left pleural cavity, suspicious for a left hemothorax. A unit of fresh frozen plasma (FFP) was promptly administered, and the patient was taken back to the operating room for a VATS evacuation of the hemothorax and to upsize the chest tube. All clots were removed from the left chest, which was copiously irrigated with 8 L of fluid. No arterial bleeding was identified. A thromboelastography (TEG) test was performed and the resuscitation continued with two additional units of packed red blood cells intra-operatively plus postoperative 1:1:1 administration of FFP, platelets and cryoprecipitate. The patient remained hemodynamically stable overnight in the PICU. Four days later, he was medically cleared for a safe discharge.

Conclusion

Patients with Marfan syndrome have an intrinsic coagulopathy and need closer surveillance, as well as prompt intervention if needed, during and after surgical procedures.