Flap cover in a patient with severe haemophilia type A

ABSTRACT

Haemophilia A is a rare haematological disorder due to deficiency of Factor VIII, causing an abnormal coagulation response to injury. In severe haemophilia A, Factor VIII level is <1%, often manifesting with spontaneous bleeding into joints. Judicious use of recombinant Factor VIII therapy to maintain adequate levels in the intraoperative, immediate and late post-operative periods, together with adjuvant pro-coagulants, can ensure a safe outcome following surgery. We describe the successful management of one such patient suffering from Marjolin's ulcer of the right gluteal region, who needed wide local excision followed by flap cover. A protocol for management of such patients is also suggested. This is the first such case report from the Indian subcontinent, with only a few such published reports from the West.

KEY WORDS

Factor VIII - flap cover - haemophilia

• REFERENCES

• 1 ICMR Task Force. Collaborative Study on Hemophilia. New Delhi: Indian Council of Medical Research; 1990
• 2 White 2^nd GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Factor VIII and Factor IX Subcommittee. Definitions in hemophilia. Recommendation of the Scientific Subcommittee on factor VIII and factor IX of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001; 85: 560
• 3 Knott PD, Khariwala SS, Minarchek J. Hemophilia B and free tissue transfer: Medical and surgical management. Ann Plast Surg 2005; 54: 336-8
• 4 Ozkan O, Chen HC, Mardini S, Cigna E, Hao SP, Hung KF. et al. Microvascular free tissue transfer in patients with hematological disorders. Plast Reconstr Surg 2006; 118: 936-44
• 5 Lee BK, Shim JS. A case of heel reconstruction with a reverse sural artery flap in a hemophilia B patient. Arch Plast Surg 2012; 39: 150-3
• 6 Drimmer MA, Demas C, Saidi P, Kim HC. Reconstructive plastic surgery in hemophiliacs. Plast Reconstr Surg 1988; 81: 91-3
• 7 Ahuja RB. Mechanics of movement for rotation flaps and a local flap template. Plast Reconstr Surg 1989; 83: 733-7
• 8 Johnson KA, Zhou ZY. Costs of care in hemophilia and possible implications of health care reform. Hematology Am Soc Hematol Educ Program 2011; 2011: 413-8
• 9 Chehab N. A review of coagulation products used in the treatment of hemophilia. Cleve Clin.. 2002: V
• 10 Factor VIII Brands in India, 2011. Available from: http://www.Drugsupdate.com [Last accessed on 2017 May 02].
• 11 Roberts I, Prieto-Merino D, Manno D. Mechanism of action of tranexamic acid in bleeding trauma patients: An exploratory analysis of data from the CRASH-2 trial. Crit Care 2014; 18: 685
• 12 Kempton CL, Meeks SL. Toward optimal therapy for inhibitors in hemophilia. Hematology Am Soc Hematol Educ Program 2014; 2014: 364-71
• 13 Powell JS. Recombinant factor VIII in the management of hemophilia A: Current use and future promise. Ther Clin Risk Manag 2009; 5: 391-402