Severe Polytrauma in a Man with von Willebrand Disease − Case Report

Introduction

Von Willebrand disease is a common bleeding disorder with a described prevalence of about 1%. People with vWD can suffer from various bleeding symptoms, such as epistaxis, bleeding from gums, prolonged bleeding from traumatic wounds or after surgeries. Severe bleeding primarily includes bleeding into the gastrointestinal tract (GIT) with anemia or bleeding into vital organs, especially the brain. In managing such situations, doctors face challenges like achieving and maintaining effective levels of vWF and FVIII. This is influenced by both individual patient characteristics and the severity of the bleeding, concomitant injuries, and the need or extent of any invasive procedure.

Case Description

A 22-year-old man sustained head and lower limb injuries during a traffic accident. He had previously received vWF/FVIII concentrate several times for dental treatment, but the possible presence of antibodies against vWF had not been tested. His baseline FVIII level was 22% and vWF activity was 6−7%. Upon examination after the accident, his Glasgow Coma Scale (GCS) score was 3, he had dilated pupils with no photoreaction to light. Physical examination revealed an occipital hematoma on the left, scars on the forehead, nasal and oral bleeding, and swelling and deformation of the right lower limb.

Examinations confirmed multiple fractures of the right tibia and fibula, and whole-body CT revealed a left-sided epidural hematoma, subdural hematoma, subarachnoid hemorrhage, skull fracture including the basal skull, and lung contusion. The findings on the right lower limb were consistent with developing compartment syndrome.

The patient was urgently managed with tranexamic acid, received 500 IU of vWF/FVIII concentrate and cryoprecipitate. A craniotomy was decided to remove the hematoma and allow for external decompression. Additionally, fasciotomy and external fixation of the injured left lower limb were necessary. During the surgery, blood loss reached nearly 2 liters, thus massive transfusions of blood products and vWF/FVIII concentrate at a dose of 4000 IU were administered.

Subsequently, administration of vWF/FVIII concentrates continued in the first 3 days post-surgery at a dose of 4000 IU/24 hours, then at the same dose every 12 hours on the 4th−5th day. On the 3rd day post-surgery, vWF and FVIII activity levels were 140% and 272%. In the following days, the doses of vWF/FVIII concentrate were reduced, and a tracheostomy and internal fixation of the lower limb fractures were performed under enhanced substitution therapy.

MRI examination after 3 weeks showed brain damage in the form of contusion and a picture of diffuse axonal injury. On the 35th postoperative day, cranioplasty with cryopreserved bone part of the skull was performed. The procedure was again secured with increased substitution of vWF/FVIII concentrates. The patient's condition gradually improved significantly, regained consciousness, and during long-term follow-up, no neurological deficit was observed.

Conclusion

The presented case report illustrates the management of severe trauma in a young man with vWD type 2A and the successful handling of the post-traumatic period, including the effective securing of all invasive procedures with early and repeated administration of vWF/FVIII concentrate. Although substitution therapy was accompanied by elevated measured levels of vWF and FVIII, no venous thrombosis was documented. However, the authors emphasize the need to test factor levels during substitution therapy.

(eza)

Source: Koroki T., Abe T., Kamimura S., Ochiai H. Severe traumatic brain injury in a patient with von Willebrand disease type 2A successfully treated with factor VIII/von Willebrand factor concentrates: a case report. Am J Case Rep 2022; 23: e936690, doi: 10.12659/AJCR.936690.