Microsurgical Reconstruction of the Mandible in a Patient with Evans Syndrome: A Case Report and Review of the Literature

Abstract

In this report, we describe the first successful case of microvascular free tissue transfer in a patient with Evans Syndrome (ES), a rare form of idiopathic thrombocytopenic purpura (ITP) and associated autoimmune hemolytic anemia (AIHA). Microvascular surgery in the setting of ES is likely to have higher complication rates because of the increased risk of postoperative bleeding and free flap thrombosis. The case presented here opens up to the feasibility of microvascular reconstruction of patients with coagulation disorders like ES. Every effort should be made to control for hemolytic, thrombocytopenic, and thrombophilic states associated with ES. In the absence of evidence-based treatment guidelines for ES, personalized treatment protocols with high-dose corticosteroids, immunoglobulin, and postoperative anticoagulation regimen are highly recommended.

Keywords

Evans Syndrome - idiopathic thrombocytopenic purpura - microsurgery - thrombosis - fibula flap

• References

• 1 Norton A, Roberts I. Management of Evans syndrome. Br J Haematol 2006; 132 (2) 125-137
• 2 Cines DB, Bussel JB. How I treat idiopathic thrombocytopenic purpura (ITP). Blood 2005; 106 (7) 2244-2251
• 3 Marõnas JM, Llamas P, Caffarena JM. Mitral valve replacement and splenectomy in a patient with chronic idiopathic thrombocytopenic purpura. Thorac Cardiovasc Surg 1982; 30 (6) 407-408
• 4 Nakajima T, Iyoda A, Iizasa T, Saitoh Y, Hiroshima K, Fujisawa T. Surgical treatment of a superior sulcus tumor in a patient with idiopathic thrombocytopenic purpura: report of a case. Surg Today 2005; 35 (12) 1078-1080
• 5 Mori Y, Hadama T, Takasaki H , et al. Aortic valve replacement and splenectomy in a patient with chronic idiopathic thrombocytopenic purpura—preoperative management with high-dose gamma-globulin. Heart Vessels 1991; 6 (2) 121-124
• 6 Diz-Küçükkaya R, Hacihanefioğlu A, Yenerel M , et al. Antiphospholipid antibodies and antiphospholipid syndrome in patients presenting with immune thrombocytopenic purpura: a prospective cohort study. Blood 2001; 98 (6) 1760-1764
• 7 de Lange J, van den Akker HP, van den Berg H. Central giant cell granuloma of the jaw: a review of the literature with emphasis on therapy options. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007; 104 (5) 603-615
• 8 Bataineh AB, Al-Khateeb T, Rawashdeh MA. The surgical treatment of central giant cell granuloma of the mandible. J Oral Maxillofac Surg 2002; 60 (7) 756-761
• 9 Cordeiro PG, Disa JJ, Hidalgo DA, Hu QY. Reconstruction of the mandible with osseous free flaps: a 10-year experience with 150 consecutive patients. Plast Reconstr Surg 1999; 104 (5) 1314-1320
• 10 Chen HC, Coskunfirat OK, Ozkan O , et al. Guidelines for the optimization of microsurgery in atherosclerotic patients. Microsurgery 2006; 26 (5) 356-362
• 11 Baumeister S, Follmar KE, Zenn MR, Erdmann D, Levin LS. Strategy for reoperative free flaps after failure of a first flap. Plast Reconstr Surg 2008; 122 (3) 962-971
• 12 Handschel J, Burghardt S, Naujoks C, Kübler NR, Giers G. Parameters predicting complications in flap surgery. Oral Surg Oral Med Oral Pathol Oral Radiol 2013; 115 (5) 589-594
• 13 Shaw RJ, Rogers SN. Venous microvascular anastomotic failure due to prothrombin gene mutation. Plast Reconstr Surg 2005; 115 (5) 1451-1452
• 14 Salgarello M, Cervelli D, Barone-Adesi L. A massive arterial thrombosis of a free anterolateral thigh flap in a patient with antiphospholipid syndrome. Microsurgery 2008; 28 (6) 447-451
• 15 Davison SP, Kessler CM, Al-Attar A. Microvascular free flap failure caused by unrecognized hypercoagulability. Plast Reconstr Surg 2009; 124 (2) 490-495
• 16 Asai E, Okouchi M, Momiyama M, Kajikawa A, Ueda K. Free flap failure in an anticardiolipin antibody-positive patient with neoplasm—a case report. Microsurgery 2010; 30 (3) 238-241
• 17 Loeffelbein DJ, Baumann CM, Mücke T, Wolff KD, Hölzle F, Kesting MR. Sticky platelet syndrome as a possible cause for free flap failure—a case report. Microsurgery 2010; 30 (6) 466-468
• 18 Herrera FA, Lee CK, Kryger G , et al. Microsurgery in the hypercoagulable patient: review of the literature. J Reconstr Microsurg 2012; 28 (5) 305-312
• 19 Wang TY, Serletti JM, Cuker A , et al. Free tissue transfer in the hypercoagulable patient: a review of 58 flaps. Plast Reconstr Surg 2012; 129 (2) 443-453
• 20 Bates SM, Greer IA, Pabinger I, Sofaer S, Hirsh J. American College of Chest Physicians. Venous thromboembolism, thrombophilia, antithrombotic therapy, and pregnancy: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest 2008; 133: 844S-846S
• 21 George JN. Idiopathic thrombocytopenic purpura in adults: current issues for pathogenesis, diagnosis and management. Hematol J 2004; 5 (Suppl. 03) S12-S14
• 22 Schmidt RE, Budde U, Schäfer G, Stroehmann I. High-dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura. Lancet 1981; 2 (8244) 475-476
• 23 British Committee for Standards in Haematology General Haematology Task Force. Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy. Br J Haematol 2003; 120 (4) 574-596