Management of a burn patient with haemophilia B and literature review
Michael Cheung1, Aruna Wijewardana1, Alice Chang1, 1Royal North Shore Hospital
Abstract
Haemostasis in a haemophilia B patient with severe burn injury requires multimodal approach in excess of standard burn surgical management. We present a case demonstrating judicious use of Factor IX product and tranexamic acid (TXA) in addition to the standard use of adrenaline tumescence and cauterisation achieved haemostasis in a surgical burn patient with severe haemophilia B.
A 28 year-old man with a history of poorly controlled epilepsy and severe haemophilia B sustained 6% total body surface area (TBSA) deep dermal to full thickness burn to his left neck, shoulder and forearm from boiling water sustained during a tonic clonic seizure. He underwent 2.5% TBSA debridement and split thickness skin graft to left shoulder and forearm with additional factor IX given preoperatively and postoperatively under the guidance of a haematologist. Intravenous TXA was administered intraoperatively to minimise blood loss. Postoperatively, he recovered well with stable haemoglobin. Preoperative haemoglobin level was 129g/L and postoperative was 119g/L. No blood transfusion was required and his graft took well.
There is limited literature on haemostasis for patients with haemophilia B in burn surgery due to the rarity of the condition. To date there is only one published paper on burn surgery in one haemophilia patient and it commented on the benefit of additional Factor IX infusion to stem bleeding. (Yaish and Rodgers, 2008) TXA was found to be effective in controlling blood loss in tangential burn debridement and reduced the need for blood transfusion in the literature (Randomised control study, Jennes et al. 2003 and a cohort study, Dominguez et al. 2017). In this patient with an extremely high bleeding risk we were able to avoid a blood transfusion through the use of Factor IX and TXA as well as meticulous surgical cauterisation. This may be a useful treatment approach for future haemophilia patients requiring burn surgery.
Reference
DOMINGUEZ, A., ALSINA, E., LANDIN, L., GARCIA-MIGUEL, J. F., CASADO, C. & GILSANZ, F. 2017. Transfusion requirements in burn patients undergoing primary wound excision: effect of tranexamic acid. Minerva Anestesiol, 83, 353-360.
JENNES, S., DEGRAVE, E., DESPIEGELEER, X. & GRENEZ, O. 2003. Effect of Tranexamic Acid on Blood Loss in Burn Surgery: A Preliminary Study. The Journal of Burn Care & Rehabilitation, 24, S59-S59.
YAISH, H. & RODGERS, G. M. 2008. A haemophilia B patient with severe burn injury: increased requirement for factor IX replacement therapy associated with venous thromboembolism. Haemophilia, 14, 607-9.
Biography
Plastic Surgery and Burns Registrar (unaccredited) at Royal North Shore Hospital