TY - JOUR
AU - PhD, Henrik Menke, MD,
AB - Abstract Patients suffering from moderate or severe hemophilia A are particularly vulnerable to trauma injury, being on high risk of immediate exsanguination. Due to a rareness of this disease, there are very few reports about the management of severe injuries of the affected patients. No guidelines for the management of burn trauma of hemophiliac patients have been yet established. Since, to our knowledge, this is the first case report about a successful treatment of a severely burned hemophiliac, requiring intensive care, long-time intubation, and multiple epifascial necrosectomies of third grade wounds, the authors are proposing the approach to this condition. The factor VIII has been substituted, enhancing its activity to over 70% during the planned interventions and keeping it above 30% in the intervals between the operations. To minimize the risk of surgical bleeding, our therapeutic strategy consisted of multiple short radical operations. The patient was healed and was dismissed after 68 days. The surgical blood loss was comparable with noncoagulopathic patients with similar burn extension. In case of a vast burn injury of the moderate or severe hemophiliacs, where the trauma-related consumption coagulopathy is expected and deep necrosectomies are required, the authors suggest starting a substitution of the factor VIII immediately after the admission, followed by a structured operative planning. Severely burned patients tend to develop a consumption coagulopathy, accentuated by a hemodilution during hydrotherapy in a shock phase and exacerbated by the extended surgeries on the vast body surface. When the clinically relevant consumption coagulopathy cannot be compensated with the administration of plasma, vitamin K, prothrombin complex concentrates and thrombocytes, advanced diagnostics often reveal a pre-existing, silent, mild coagulation disorder. The presence of more severe coagulopathies such as hemophilia A is particularly challenging in these conditions. Today, hemophilia A can be successfully treated with desmopressin and factor VIII supplementation. These therapies can also be administered preoperatively should the affected patient require elective or urgent surgery. Nevertheless, pronounced bleeding must still be expected. Due to a low incidence of hemophilia no guidelines exist for medical emergencies of severe burn injury of moderate to severe grade hemophiliacs. In this case report, we describe our experience with a life threatening burn injury of a multimorbid, moderate-hemophilic patient. METHODS A 62-year-old hemophilic male was recovered in our burn center with second and third degree burns of 28% TBSA. The injury occurred when his polyester shirt caught fire from a candle. The burned areas were located on the face, neck, thorax, abdomen, shoulders, arms, and hips (Figure 1). The Abbreviated Burn Severity Index score was counted 8. Figure 1. View largeDownload slide Admission of the haemophiliac; deeply burned areas are visible on the face, neck, ventral thorax, and both shoulders and arms. Figure 1. View largeDownload slide Admission of the haemophiliac; deeply burned areas are visible on the face, neck, ventral thorax, and both shoulders and arms. Patient's medical history included hepatitis C, chronic kidney failure, hypertension, hypertensive heart disease, cachexia, and opiate dependence related to chronic pain after multiple vertebral fractures. The most relevant to the patient's care at this phase was however the moderate grade hemophilia A with factor VIII activity 1 to 5%. The large wounds and pre-existing opiate dependency made an analgesia request so high, that intubation was necessary at day 1. The arterial and venous central catheters were initially placed in the right groin region due to the extensive burn areas on the upper body and also to avoid a risk of hemothorax. Under the infusion therapy of 8700 ml Ringer's solution in the first 24 hours, no catecholamines were needed to maintain the circulation and urination at physiological levels. Most of the wounds were clearly third grade deep and therefore required radical necrosectomies. A substitution therapy with a recombinant antihemophilic factor (Kogenate Bayer) was administered daily between first and 46th day (Table 1) to avoid spontaneous bleeding or severe hemorrhage during the epifascial excisions. The factor VIII levels were enhanced to over 70% of normal prior each surgery and maintained over 30% in the intervals between operations (Table 3, Figure 2). In addition, vitamin K and fresh frozen plasma were given when the levels of other clothing factors were dropping. Starting from the fourth day, seven surgical steps were performed in the following month (Table 2). For a better control, by an expected bleeding diathesis, a therapeutic strategy consisted of multiple short, small surface, radical surgeries. Third degree wounds on chicks and ventral surface of neck were debrided and covered with full-thickness skin grafts from the groins. Necrotic areas on chin, dorsal neck, thorax, upper dorsum, shoulders, arms, and axillas were epifascial excised and covered with split-thickness skin grafts from the thighs, right forearm, lower dorsum, and abdomen. Furthermore, an uneventful tracheotomy was performed during the second surgery. Table 1. Factor VIII substitution plan View Large Table 1. Factor VIII substitution plan View Large Table 3. Coagulation profile: selected days View Large Table 3. Coagulation profile: selected days View Large Figure 2. View largeDownload slide Factor VIII activity in course of recovery; the arrows indicate the days of surgical interventions. Figure 2. View largeDownload slide Factor VIII activity in course of recovery; the arrows indicate the days of surgical interventions. Table 2. Surgical therapy View Large Table 2. Surgical therapy View Large The blood lost required transfusion of 28 units of blood and 12 units of fresh frozen plasma in course of the treatment. The factor VIII, quick's time/international normalized ratio and the activated partial thromboplastin time were controlled daily (Table 3, Figure 2). Twelve days after the last surgery the patient was extubated. Physiotherapy has been started immediately after the admission. Under strictly sterile conditions, only mild wound infections were noticed, requiring 10 days of intravenous antibiotic therapy. No pneumonia was observed. After 68 days of hospital stay, the patient was transferred to rehabilitation clinic. The substitution therapy alone for this patient costed 280,000 &OV0556; and was covered separately by a procedural rate. DISCUSSION A therapy of hemophilia A with the factor VIII is known for more than 50 years.1 It allows the affected patients to live longer; however, the hemophiliacs still tend to develop secondary disorders, like hemarthrosis and remain more vulnerable to trauma. The recombined factor VIII is being successfully administered to the hemophiliacs before elective operations, even with high bleeding risk, making, eg, heart surgeries possible2 and allowing child birth.3 A real challenge are unexpected life-threatening events, where pronounced bleeding is expected, eg, in the polytraumas, aneurysm ruptures, and severe injuries. In these conditions also, the otherwise healthy patients tend to develop the clothing disorders4,5 which increases the risk of mortality.6 A pathophysiological mechanism of the postinjury coagulopathy in burned patients can be explained with a consumption of coagulation factors II, VII, and X due to large wounds and hemodilution during the infusion therapy and long surgeries. In the previously noncoagulopathic patients, a reactive elevation of coagulation factor VIII and fibrinogen often is being observed in these conditions.7,8 The latter mechanism cannot be expected in a hemophiliac. That is why we have been administering the factor VIII from the first day preventively, before any bleeding or clotting-values changes emerged. Simultaneously, the vitamin K injections and fresh frozen plasma infusions were given, as it is usually required by this extension of burned surface. This protocol allowed us to perform a tracheotomy, insert central catheters and excise the necrotic material in the epifascial manner. In relevant literature, we found only three compelling case reports9,–11 about burned hemophilic patients. The presented patients were much younger, healthier, and suffering from less severe burn trauma (Table 4)11,9,10. This allowed the attending physicians to wait and let the wounds heal, before performing surgery to correct the remaining defects. Table 4. Previous case reports View Large Table 4. Previous case reports View Large The previous authors9,–11 postulated that a substitution of the factor VIII was not needed while performing sharp excisions, since the bleeding from the injured small cutaneous arterioles could stop due to their retraction and the platelet plugging. They have indicated the factor VIII responsible for the coagulation of the vessels larger than small arterioles and therefore important only by the excision in deeper layers. None of the previous authors performed in fact any epifascial necrosectomy, but only debridements or the sharp excisions of the skin layers on smaller surfaces. Two of them9,10 reported, however, a substitution of the factor VIII, one preventively, and the other prior the escarotomy. They also both recommended the substitution in case of deeper excision. Third grade burn injuries of our patient required the excision not only of the superficial skin layers but also of the subcutaneous fat and sometimes of the fascia, where the larger vessels are normally found. Also unlike the previous authors, we had to perform many more surgeries, a tracheotomy and insert or replace the central catheters. All those procedures would have been too risky without the factor VIII substitution. A tracheostomy is generally contraindicated in the presence of hemophilia, since it may easily cause a blood aspiration. We were, however, expecting a longer period of mechanical ventilation, and under the substitution therapy no major bleeding at the first surgery and while placing central venous and arterial catheters was observed; we found it, therefore, legitimate to perform the tracheostomy at the time of the second surgery. No complications occurred. Maintaining the coagulation on the normal levels our patient experienced no complications and a surgical blood loss was comparable with noncoagulopathic patient with similar burn extension. The higher than usual amount of transfusions12,–14 was caused by patient's anemia at the admission, the critical illness and exceptionally high number of blood sampling. Against the current restrictive transfusion protocols,15 we kept the patient's hemoglobin levels over 10 g/dl to provide him with some reserves in case of unexpected hemorrhagic events and to avoid aggravating his cardiac decompensation. After carefully reviewing relevant literature, we were unable to find any other work confirming or denying the previous author's statement regarding the redundancy of the factor VIII substitution while performing tangential necrosectomies. Based on our experience, we also believe that using a knife on a coagulopathic patient can always be risky. The wounds preoperatively evaluated as deep second grade, might turn even deeper once started a sharp excision, and making necessary the epifascial necrosectomy. Also the sharp excision, especially in the elderly patients with thinner skin, can accidentally end up in the deeper layer then planned. CONCLUSIONS A treatment of a burn injury of a hemophiliac patient can be successful. In case of life-threatening burn injury where tracheotomy, large vessel catheters to hydrotherapy and more radical necrosectomies are required and also where a consumption coagulopathy is expected, we recommend the substitution of the factor VIII. It is a powerful tool that properly administered, together with other necessary medicines, allows a survival and surgical therapy of the most threatening trauma like a burn injury of a hemophiliac. ACKNOWLEDGMENTS We thank Prof. Inge Scharrer, MD and Charis von Auer, MD from the University Clinic of Mainz for their precious help in adjusting the substitution therapy. REFERENCES 1. B Nascimento, LT Goodnough, JH Levy Cryoprecipitate therapy. Br J Anaesth  2014; 113: 922– 34. Google Scholar CrossRef Search ADS PubMed  2. P Bhave, D McGiffin, J Shaw Guide to performing cardiac surgery in patients with hereditary bleeding disorders. J Card Surg  2015; 30: 61– 9. Google Scholar CrossRef Search ADS PubMed  3. V Sharma, A Khalid, AJ Cohen Management of pregnancy in a patient with severe hemophilia type a. AJP Rep  2013; 3: 29– 32. Google Scholar CrossRef Search ADS PubMed  4. MJ Cohen, M Kutcher, N Matijevic Clinical and mechanistic drivers of acute traumatic coagulopathy. 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TI - A Severely Burned Hemophiliac
JF - Journal of Burn Care & Research
DO - 10.1097/BCR.0000000000000339
DA - 2017-01-01
UR - https://www.deepdyve.com/lp/oxford-university-press/a-severely-burned-hemophiliac-gflC1wXkC4
SP - e456
EP - e459
VL - 38
IS - 1
DP - DeepDyve
ER -