Von Willebrand disease (vwd) is the most common

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1 Breast Augmentation in the Patient With von Willebrand Disease The authors describe breast augmentation in a patient with von Willebrand disease (vwd), providing a template for treating such patients. Because there is no single ideal laboratory test predictive of surgical bleeding, preoperative history remains the most important diagnostic tool. A vigilant pursuit of signs of undiagnosed vwd or other coagulopathy in the preoperative patient may prevent unexpected intraoperative or postoperative hemorrhage. (Aesthetic Surg J 2006;26: ) Von Willebrand disease (vwd) is the most common inherited coagulopathy in the general population. Therefore, it is likely that the plastic surgeon will encounter diagnosed or undiagnosed vwd in patients seeking cosmetic procedures. (For the pathophysiology of this disease, see Background. ) Here, we present an operative approach to breast augmentation in a 22-year-old woman with type I vwd, outlining a system for perioperative management of such patients when they undergo breast augmentation. Case Report A 22-year-old woman who had been diagnosed with type I vwd 4 years earlier sought breast augmentation at the plastic surgery clinic at University of Illinois Medical Center (Figure A,B,C). This was her fourth consultation after having been rejected for surgery by physicians who were uncomfortable performing breast augmentation in a patient with this coagulopathy. This patient reported prolonged bleeding after wisdom tooth extraction, tonsillectomy, and adenoidectomy 2 years before she was diagnosed with vwd. In an evaluation of bleeding after traumatic fracture of the left olecranon 4 years earlier, prolonged bleeding time (13.5 min), low factor VIII activity ([FVIII] antihemophilic factor 50.3%), low FVIII-related antigen Sai S. Ramasastry, MD, Chicago, IL, is a board-certified plastic surgeon and an ASAPS member. Coauthors: Yi-Hsiang Chen, MD; PhD; David E. Morris, MD, Chicago, IL. (54%), low ristocetin cofactor activity (54%), normal von Willebrand Factor (vwf) multimer pattern and, subsequently, a normal vwf-fviii binding established a diagnosis of type I vwd. She underwent open reduction and internal fixation without complication, using perioperative desmopressin acetate (DDAVP, Aventis, Bridgewater, NJ) for the correction of hemostatic defects. In subsequent years, using desmopressin support, she successfully underwent operative procedures for the removal of tension wiring for the olecranon fracture and a talonavicular bone fusion. To ensure the best outcome in her breast augmentation surgery, our plastic surgery and hematology services collaborated closely during preoperative planning. Because of the nature of the subpectoral dissection used to create pockets for the implants, we performed the procedure on Background Von Willebrand factor (vwf) mediates platelet-platelet and platelet-subendothelial adhesion and is also the carrier protein for factor VIII (FVIII). Von Willebrand disease is a deficiency of functionally normal vwf, and a variable reduction in plasma FVIII level. It is the most common hereditary hemorrhagic diathesis and includes a heterogeneous group of disorders with many subtypes classified into 3 groups: Types I, II, and III. 1-3 Type I vwd, the most common type, representing more than 70% of cases, comprises the genetic mutations that result in a quantitative deficiency of qualitatively normal vwf. Type II vwd (2A, 2B, 2M, and 2N) represents mutations that result in functional defects of the mutated proteins. A deficiency in large multimeric vwf is seen in types 2A and 2B. In Type III vwd, the rarest type, and an autosomal recessive condition, vwf is essentially absent. 612 A ESTHETIC S URGERY J OURNAL ~ SEPTEMBER/OCTOBER 2006

2 an inpatient basis so that FVIII activity could be monitored closely and optimized. The patient was admitted to the hospital one day before the procedure to undergo baseline laboratory work. Factor VIII activity was 38%, FVIII-related antigen was 80%, and ristocetin cofactor activity was 98%. (The previous 2 levels of FVIII-related antigen and Ristocetin co-factor activity had been at 70% to 80% over the past 2 years after the patient had been started on an oral contraceptive.) Other parameters (prothrombin time, partial thromboplastin time, platelet count, hematocrit) were normal. To achieve a mean factor level at a minimum of 75%, we administered an intravenous loading dose of Humate- P, 30 μ/kg, one and one-half hours before surgery. Postoperatively, we administered maintenance doses, 15 μ/kg, at 12, 24, and 36 hours. We started her on intravenous desmopressin initially, followed by nasal spray 48 hours postoperatively, which we administered for 2 more days. Serial factor assays showed that FVIII procoagulant activity increased to 91% after the loading dose and was maintained between 51% and 107%. FVIII-related antigen was raised to 173%, while ristocetin cofactor activity increased to 114% after loading. Both levels were maintained at 82% to 122%. Intraoperatively, we created submuscular pockets, using blunt and cautery dissection, meticulously checking for hemostasis. We dusted the pockets with Avitene powder (Davol Inc. Cranston RI) before bilaterally inserting 380-cc smooth round saline implants. Nonautologous blood was available for surgery, but it was not used. We discharged the patient on postoperative day 2 after an uneventful course. She had no bleeding complications and her hematocrit remained stable. Early in her postoperative course she showed no evidence of bruising or hematoma (Figure D,E). We administered 1 dose of nasal desmopressin 1 hour before removing her sutures. Eleven months postoperatively, she had excellent results with no capsular contracture (Figure F,G,H,I,J,K). Evaluation The diagnosis of vwd requires detailed studies of hemostatic defects, since various types of vwd require different management approaches. Mild type I vwd is at times difficult to recognize because of varying degrees of hemostatic defects as well as clinical bleeding manifestations. Furthermore, plasma levels of vwf fluctuate, and this fluctuation may be exaggerated in various medical conditions, such as acute inflammation, pregnancy, and the use of oral contraceptives. The patient we present here provides an example of difficult to recognize vwd: she had prolonged bleeding after tonsillectomy and tooth extraction, yet the diagnosis was unrecognized until further work up. Plastic surgeons will likely encounter similar situations of undiagnosed, yet clinically apparent vwd. Guyuron et al 4 retrospectively studied the bleeding history of a group of 1000 patients who had undergone elective plastic surgery procedures. Of these, 9 patients (0.9%) were found to have vwd. There is no ideal single screening laboratory test predictive of surgical bleeding; preoperative history remains the most important predictive tool. Key points to elicit from patients include: A history of easy bruising or bleeding, mild mucocutaneous bleeding, menorrhagia, and bleeding that is greater than expected after cuts and prior surgery. A history of previous transfusions and use of medications that may affect coagulation, such as aspirin, nonsteroidal anti-inflammatory agents, and various over-the-counter herbal extracts. A family history of bleeding tendency. Clinical signs, such as ecchymosis and petechiae, may suggest a coagulopathy. In patients with suggestive personal and or family history of bleeding, screening coagulation studies, including platelet function analysis, should be carried out. The bleeding time alone in the absence of such history is not a useful predictor of surgical bleeding. If medication is not the cause and the screening coagulation tests are abnormal, perform additional studies, including work up for vwd. In the preoperative patient, a vigilant pursuit of signs of yet undiagnosed vwd or other coagulopathy might well prevent unexpected intraoperative or postoperative hemorrhage. Treatment The therapeutic principle for vwd is to restore an effective level of functionally normal vwf and Factor VIII activity. 5 Currently, the main therapeutic agents are desmopressin (1-deamino-8-D-arginine vasopressin [DDAVP]) and plasma products. Desmopressin is a synthetic vasopressin analog that functions by causing the release of preformed vwf and increases the numbers of multimers in plasma. Intermediate FVIII concentrate contains a significant amount of high-molecular-weight vwf multimers. At present, human antihemophilic factor/von Willebrand factor complex (Humate-P, Aventis Behring, Kankakee, Illinois) is the only Food and Drug Administration (FDA)-approved factor concentrate Augmentation in the Patient With A ESTHETIC S URGERY J OURNAL ~ September/october Von Willebrand Disease

3 A B C Figure. A-C, Preoperative views of a 22-year-old woman diagnosed with Type I vwd. D E D, E, Postoperative views 1 month after bilateral submuscular placement of 380-cc saline implants. F G H I J K F-K, Postoperative photographs 11 months after breast augmentation show good results with no capsular contracture. 614 Aesthetic Surgery Journal ~ September/October 2006 Volume 26, Number 5

4 approved for the treatment of vwd. Alternatively, cryoprecipitate has been used but carries a higher risk of viral transmission. Types 1 and 2A vwd typically respond to treatment with DDAVP, while Types 2B, 2M, 2N, and 3 are not responsive to desmopressin. Use Humate-P in vwd patients with active bleeding or when there is concern that desmopressin alone may be insufficient for hemostasis. In addition to the type of vwd, consider factors in treatment planning such as disease severity, anticipated degree of bleeding, and challenge in achieving hemostasis for a given surgical procedure. For minor bleeding or surgery, we recommend desmopressin or replacement therapy to maintain FVIII activity 30% for 3 to 4 days. For major bleeding or surgery, we recommend maintaining FVIII >50% for 5 to 10 days. Monitor FVIII coagulant activity and ristocetin cofactor activity to avoid undertreatment. Overtreatment, particularly when it results in sustained high FVIII activity, may potentially predispose patients to thromboembolic events. 6,7 The optimal management of vwd in aesthetic surgery has not been extensively discussed. One of the earliest discussions of vwd in the plastic surgical literature is that of Ship, 8 who reported on 6 patients he believed had vwd. In 2 of these patients, surgery was not undertaken. Another patient had significant bleeding during breast augmentation and was later found to have a depressed FVIII level. Shah et al 9 presented a series of 21 otolaryngologic procedures in 12 known vwd patients without hemorrhagic complication; procedures included myringotomies, tonsillectomies, adenoidectomies, and one patient who underwent blepharoplasty, submental lipectomy, and septorhinoplasty. In their protocol, types 1 and 2A patients received desmopressin preoperatively and postoperatively. Types 2B and 3 patients received factor VIII/vWF concentrate and aminocaproic acid (Amicar, Wyeth, Madison, NJ). Intraoperative laser use was advocated for hemostasis. Recently, Pacik and Hammond 10 described breast augmentation in a patient thought to have a mild form of type I vwd; the patient was treated with 3 doses of intravenous desmopressin perioperatively. 10 Although the patient we discuss underwent previous orthopedic procedures managed successfully with desmopressin alone, we approached her breast augmentation differently for 2 reasons: (1) the significant submuscular dissection necessary to place the implants, and (2) the resulting potential spaces into which development of hematoma is a risk. We used replacement therapy for the first 48 hours to ensure an adequate hemostatic mechanism and to monitor coagulation parameters closely throughout the perioperative course, as outlined above. Our experience is not unlike the experiences of surgeons performing noncosmetic surgery. Franchini et al 11 reported the use of Humate-P in 43 surgical procedures in 26 patients with vwd. The outcome was judged excellent/ very good in all except one procedure. In 11 minor operations, the average preoperative dose was 49.8 μ/kg (range, 42.9 to 61.5); the daily maintenance dose was 28.7 μ/kg (range, 21.4 to 34.8); and the duration of replacement was 4.2 days (range, 2 to 7). This replacement schedule is comparable to our loading dose of 30 μ/kg, maintenance dose of 30 μ/kg/d (15μ/kg q12h), and replacement duration of 2 days with an additional 3 days of desmopressin, taking into account the mild degree of presurgical factor deficiency in our patient. Intraoperatively, we dusted the submuscular pockets with microfibrillar collagen hemostat (Avitene, Davol Inc. Cranston, RI). Avitene flour is a microfibrillar collagen preparation that, since the 1970s, has been described as an adjunctive hemostatic agent in virtually all surgical subspecialties. 12 Its proposed mechanism is that it increases the surface area over which the coagulation pathways take place and stimulates platelet adherence and clot stabilization. Conclusion Although the vwd in our patient was quite mild, the reluctance of the initially consulted plastic surgeons to perform breast augmentation may be attributed to a low tolerance for a less than perfect outcome in an aesthetic procedure. We attribute her successful outcome to a concerted effort between the patient and the surgical and hematology teams. Hemorrhagic complications were prevented through a careful perioperative medical plan, meticulous operative hemostasis, and the use of Humate-P, desmopressin, and microfibrillar collagen. With collaborative effort, patients with vwd can generally undergo successful surgery and should be offered the benefits of aesthetic procedures. References 1. Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand disease. Blood 1987;69: Keeney S, Cumming AM. The molecular biology of von Willebrand disease. Clin Lab Haematol 2001;23: Sadler JE. A revised classification of von Willebrand disease. Thromb Haemost 1994;71: Guyuron B, Zarandy S, Tirgan A. Von Willebrand s disease and plastic surgery. Ann Plast Surg 1994;32: Mannucci PM. Treatment of von Willebrand s disease. New Engl J Med 2004;351: Augmentation in the Patient With A ESTHETIC S URGERY J OURNAL ~ September/october Von Willebrand Disease

5 6. Koster T, Blann AD, Briet E, Vandenbroucke JP, Rosendaal FR. Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet 1995;345: Mannucci PM, Chediak J, Hanna W, Byrnes J, Ledford M, Ewenstein BM, et al. Treatment of von Willebrand disease with a high-purity factor VIII/von Willebrand factor concentrate: a prospective, multicenter study. Blood 2002;99: Ship AG. Von Willebrand s disease a potential bleeding problem in plastic surgery. Plast Reconstr Surg 1976;58: Shah SB, Lalwani AK, Koerper MA. Perioperative management of von Willebrand s disease in otolaryngologic surgery. Laryngoscope 1998;108: Pacik PT, Hammond D. Augmentation mammaplasty and von Willebrand s Disease. Plast Reconstr Surg 2005;115: Franchini M, Rossetti G, Tagliaferri A, Pattacini C, Pozzoli D, Lippi G, et al. Efficacy and safety of factor VIII/von Willebrand factor concentrate (Haemate-P) in preventing bleeding during surgery or invasive procedures in patients with von Willebrand s disease. Harematologica 2003;88: Rybock JD, Long DM. Use of microfibrillar collagen as a topical hemostatic agent in brain tissue. J Neurosurg 1977;46: Reprint requests: Sai S. Ramasastry, MD, University of Illinois Department of Surgery M/C 958, 820 S. Wood St. 515 CSN, Chicago, IL Copyright 2006 by The American Society for Aesthetic Plastic Surgery, Inc X/$32.00 doi: /j.asj Aesthetic Surgery Journal ~ September/October 2006 Volume 26, Number 5