- Email: [email protected]
Hemimandibulectomy and vascularized fibula flap in bisphosphonate-induced mandibular osteonecrosis with polycythaemia rubra vera
Int. J. Oral Maxillofac. Surg. 2013; 42: 120–123 doi:10.1016/j.ijom.2012.03.019, available online at http://www.sciencedirect.com
Case Report Reconstructive Surgery
Hemimandibulectomy and vascularized fibula flap in bisphosphonate-induced mandibular osteonecrosis with polycythaemia rubra vera
N. Ghazali, J. C. Collyer, J. V. Tighe Maxillofacial Unit, Queen Victoria Hospital, East Grinstead, United Kingdom
N. Ghazali, J. C. Collyer, J. V. Tighe: Hemimandibulectomy and vascularized fibula flap in bisphosphonate-induced mandibular osteonecrosis with polycythaemia rubra vera. Int. J. Oral Maxillofac. Surg. 2013; 42: 120–123. # 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. This report presents the successful management of an advanced and refractory bisphosphonate-related osteonecrosis of the jaws (BRONJ) by hemimandibulectomy and an osteocutaneous fibula flap reconstruction in a patient with polycythaemia rubra vera, a rare haematological condition in which there is increased risk of thrombosis and haemorrhage. Union of the vascularized bone with the mandible depends on obtaining a BRONJ-free margin and rigid fixation of the bony ends. Magnetic resonance imaging can provide accurate delineation of necrotic bone and area of osteomyelitis. Placement of a 1 cm margin beyond this can envisage a BRONJ-free margin. Aggressive medical management of polycythaemia rubra vera by venesection, asprin and cytoreduction therapy along with anticoagulant prophylaxis against thromboembolic events in the first 2 weeks following major surgery can provide the basis of a good surgical and flap outcome. Nevertheless, the possibility of unpredictable haemorrhage must be considered throughout.
Accepted for publication 16 March 2012 Available online 18 April 2012
The use of vascularized reconstruction in the management of bisphosphonaterelated osteonecrosis of the jaws (BRONJ) is controversial. It is suggested that segmental resection and vascularized reconstruction are reserved for advanced cases of BRONJ and in those that are refractory to multiple debridement and conservative measures.1,2 This option is perceived as carrying an increased risk of postoperative
in the presence of essential thrombocytosis, antithrombotic medication and von Willebrand’s disease.3 The increased risk of thrombosis and haemorrhage with PRV can make vascularized reconstruction challenging in this group of patients. The authors present the case of a patient with an advanced and refractory case of BRONJ with PRV, who was successfully
0901-5027/010120 + 04 $36.00/0
complications, such as recurrence of BRONJ at the resection margins and nonunion. This could outweigh the potential advantages. Polycythaemia rubra vera (PRV) is a rare, chronic myeloproliferative disorder characterized by increased red cell mass (erythrocytosis), leading to hyperviscosity with an increased risk of thrombosis.3 Haemorrhage can also occur with PRV
# 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
BRONJ treatment in polycythaemia rubra vera patient
121
managed by hemimandibulectomy and vascularized fibula flap reconstruction. Case report
An 82-year-old woman with long-standing PRV and significant osteoporosis, developed BRONJ after the extraction of a periodontally compromised lower right molar tooth. She had been treated with oral alendronate (70 mg weekly) following the diagnosis of osteoporosis-induced collapse of multiple spinal vertebral bodies 7 years earlier. With the diagnosis of BRONJ, alendronate was stopped and strontium ranelate (8 mg once per month) was prescribed as an alternative. The PRV was managed with hydroxycarbamide (500 mg 3 days/week and 1 g for the remaining 4 days/week), daily aspirin (75 mg) and the option of venesection to maintain a haematocrit level of less than 0.45. Other medical problems included multiple transient ischaemic attacks, gout, hypothyroidism and hypertension. There was no history of smoking. At the initial stage, the BRONJ was unresponsive to conservative measures, including a course of pentoxyfylline and tocopherol. The sequestrum rapidly extended beyond the socket of the extracted tooth, causing inferior dental nerve paraesthesia. There was intractable jaw pain, odynophagia and chewing difficulties. Local debridement was repeated twice. The symptoms persisted and the patient became at high-risk of pathological fracture from a pencil-thin mandible (Fig. 1). Resection of the affected bone was considered 11 months after the diagnosis of BRONJ due to the refractory response to repeated localized debridement and the persistence of BRONJrelated symptoms. Imaging of the mandible was obtained using magnetic resonance imaging (MRI). Assessment of the necrotic bone was made by correlation of T1, STIR and post-gadolinium enhancement sequences. Bony destruction was seen affecting the right body and ramus of the mandible, extending from just distal of the right lower canine to the antegonial notch. There was also loss of continuity of the inferior dental canal with only a thin rim of the inferior border of the mandible remaining. In the light of this finding, a right hemimandibulectomy and vascularized fibula reconstruction was planned. The presence of pathological fracture was found intra-operatively within the BRONJ-involved pencil-thin area of the mandible (Fig. 2). The resection margin was sited 1 cm beyond the area of osteo-
Fig. 1. Orthopantomogram showing advanced BRONJ of the right mandible, refractory to conservative management and two separate debridements (11 months after diagnosis of BRONJ).
necrosis as determined by MRI and this was placed at the mandibular angle posteriorly and in the socket of the lower right second incisor anteriorly. Harvesting of a fibula osteocutaneous flap with septocutaneous perforators was performed via the lateral approach under tourniquet control. The fibula bone was osteotomized at one site to reproduce normal facial contours and was internally fixed using a 2.0 Unilock rigid internal fixation system (Synthes Ltd -UK, Welwyn Garden City, Hertfordshire, UK) (Fig. 3). End-to-end arterial anastomosis was performed between the peroneal artery to the facial artery while venous anastomosis was performed between the peroneal venae commitantae to the common facial vein using 3 mm vein coupler system (Synovis, St Paul, USA). The duration of surgery was 450 min.
The preoperative haemoglobin level was 14.8 g/dl and the haematocrit was 0.376. The patient remained on her usual PRV regime of hydroxycarbamide and aspirin, and received preoperative subcutaneous low-molecular weight heparin (4500 unit Tinzaparin) on the evening before the operation. During surgery, fluid rehydration was preferred over intraoperative blood transfusion as the estimated overall blood loss was approximately 400 ml. Intraoperative anticoagulation was not used. Immediate postoperative haemoglobin was 9.7 g/dl and the haematocrit was 0.306, which are ideal values for microvascular success. Histological examination of the resected specimen was consistent with drug-induced osteonecrosis. The specimen was composed of chronic active osteomyelitis, with bony sequestration, suppuration and periosteal
Fig. 2. Intraoperative view of the right hemi-mandible showing the presence of a pathological fracture within the area of BRONJ.
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Ghazali et al.
Fig. 3. Immediate postoperative orthopanthomogram showing the extent of the resection and reconstruction with a vascularized fibula flap.
bone formation. The sequestra were covered by heavy bacterial aggregates. There were areas of mucosal ulceration. Normal bone was found at both resection margins. The patient developed transient sinus bradycardia secondary to vagal stimulation but apart from this, the postoperative recovery was unremarkable and her BRONJ symptoms had resolved completely. She received 7 days postoperative intravenous antibiotic prophylaxis (coamoxiclav 1.5 g three times/day). The PRV regime was continued and daily subcutaneous low-molecular weight heparin (4500 units Tinzaparin nocte) was administered prophylactically against deep vein thrombosis/pulmonary embolism while she received in-patient hospital care for 2 weeks. Nutritional requirement was provided through nasogastric tube feeding for 7 days and oral intake was commenced thereafter. There was partial take of the skin graft at the donor site, which healed with regular dressings by 4 weeks post operation. The osteocutaneous fibula flap survived in its entirety following the operation (follow up period 24 months). The authors are unable to provide radiographic evidence for this due to the unit policy of not subjecting patients to radiation in the absence of on going clinical problems. Discussion
This case demonstrates the successful management of two difficult coexisting clinical scenarios relating to microvascular reconstruction in one patient. While the reliability of vascularized fibula reconstruction has been described previously in BRONJ cases,1,2 this report also documents the additional management required in the presence of PRV, which carries the
potential risk of flap failure due to thrombosis and haemorrhage. Vascularized bone flaps can provide bony continuity for restoration of facial form and oral function following segmental resection, and reduce the risk of fistulation and metalwork exposure. Using vascularized bone flaps in BRONJ carries the risk of recurrence of BRONJ at the resection site, nonunion and transfer of malignancy to the jaw in cases of BRONJ related to bisphosphonate use in disseminated malignancy.2 Complete healing (union) at the fibula– mandible interface is subject to conventional factors related to fracture healing. In this case, the fibula bone was held rigidly in direct contact with BRONJ-free, normal mandibular bone, facilitating healing. Failure to obtain a BRONJ-free margin is cited as one of the causes of nonunion.1,2 The authors found the high sensitivity of MRI imaging for bone marrow pathology4 aided in locating the extent of bone necrosis. By adding 1 cm beyond the limit of the MRI-delineated osteonecrosis, a BRONJfree margin was reliably obtained. A reduction in the risk of postoperative BRONJ recurrence may have been facilitated by an 11 month alendronate-free period preoperatively, maintenance of good oral health, and avoidance of trauma, including the use of dentures. Postoperative hyperbaric oxygen has been advocated1 to encourage healing and prevent BRONJ recurrence following surgery, but the authors have not utilized this option due to logistic difficulties. The conventional medical management of PRV includes the use of venesection, aspirin (75 mg daily) unless contraindicated and cytoreductive therapy in certain situations, such as: when venesection is not tolerated; presence of symptomatic or
progressive splenomegaly; presence of other indicators of disease progression; and thrombocytosis.3 Although it is recognized that thrombosis and haemorrhage are major causes of morbidity and mortality in PRV, there are no guidelines regarding the management of patients with PRV who are undergoing surgery. A large, retrospective multicentre study of patients with PRV and essential thrombocytosis undergoing various types of surgery found that the surgical outcome was complicated by vascular occlusion and haemorrhagic episodes in 7.7% and 10.5% cases, respectively.5 The risk of thromboembolic events was highest in the first 2 weeks in both PV and essential thrombocytosis. Arterial thrombosis was more frequently seen in essential thrombocytosis and in those with other risk factors for arterial thrombosis (e.g. diabetes, smokers) while venous thrombosis was more frequently seen in PRV. Heparin was prescribed in those undergoing major surgery and in addition, anticoagulants were administered in PRV. Haematological intervention (i.e. venesection or cytoreduction therapy) was preferentially given to PRV patients with platelet counts of more than 1000 109/l, those over 60 years at the time of surgery, undergoing major surgery, who had developed a thrombotic event during follow-up and in those with white cell counts of more than 10 109/l5. Owing to the rarity of the condition, there is only one report in the literature of the successful outcome of vascularized reconstruction in a patient with PRV,6 in which the anterolateral thigh flap was used to reconstruct the anterior floor of the mouth following resection of squamous cell carcinoma. Their approach relied on preoperative venesection, minimal blood loss and good intraoperative hydration to prevent any thrombotic event. Intraoperative anticoagulation was not advocated but it was proposed that anticoagulants might be useful in PV even when essential thrombocytosis is absent, due to its effect in preventing thrombosis in the microcirculation. The management of the present case tended to mirror the findings of the large, retrospective multicentre study. Aggressive haematological control of erythrocytosis was achieved by cytoreduction therapy. Prevention of flap microcirculation thrombosis with aspirin was instituted preoperatively. Haemoglobin levels were closely monitored and the intraoperative blood loss was not replaced. The cytoreductive agent and anticoagulant regime were continued immediately in the postoperative period. Low-molecular weight heparin was administered regularly
BRONJ treatment in polycythaemia rubra vera patient for 2 weeks postoperatively while the patient remained in hospital care. This was crucial during the time when the risk of a thromboembolic event was at the highest, particularly as the donor site was the lower limb. Postoperative thromboembolic events were avoided completely. Despite taking both low-molecular weight heparin and aspirin during the postoperative period, there were no bleeding episodes. The entire flap survived, including the cutaneous component. The reliability of the fibula skin paddle based on septocutaneous perforators has been questioned7 but attention to the technical detail surrounding flap harvesting8 has improved its dependability in mandibular reconstruction. Apart from the fibular flap, other vascularized flaps have been used successfully in BRONJ-related reconstructions of the mandible.2 Competing interests
None declared. Funding
None. Ethical approval
None.
Acknowledgements. The authors would
like to acknowledge Dr AW Barrett, Consultant Oral Pathologist and Dr Lekha Chandrasekaran, Consultant Radiologist, both of the Queen Victoria Hospital, for their expert commentaries in the relevant areas during the preparation of this paper.
5.
6.
References 1. Nocini PF, Saia G, Bettini G, Ragazzo M, Blandamura S, Chiarini L, et al. Vascularized fibula flap reconstruction of the mandible in bisphosphonate-related osteonecrosis. Eur J Surg Oncol 2009;35:373–9. 2. Seth R, Futran ND, Alam DS, Knott PD. Outcomes of vascularized bone graft reconstruction of the mandible in bisphosphonaterelated osteonecrosis of the jaws. Laryngoscope 2010;120:2165–71. 3. McMullin MF, Bareford D, Campbell P, Green AR, Harrison C, Hunt B, et al. On behalf of the General Haematology Task Force of the British Committee for standards in Haematology. Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis. Br J Haematol 2005;130: 174–95. 4. Mirowitz SA, Apicella P, Reinus WR, Hammerman AM. MR imaging of bone marrow lesion: relative conspicuousness on T1-
7.
8.
123
weighted, fat suppressed T2-weighted and STIR images. AJR 1994;162:215–21. Ruggeri M, Rodeghiero F, Tosetto A, Castaman G, Scognamiglio F, Finazzi G, et al. for the Gruppo Italiano Malattie Ematologiche dell’Adulto (GIMEMA) Chronic Myeloproliferative Diseases Working Party. Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: a retrospective survey. Blood 2008;111: 666–71. Lin SJ, Yu P. Free tissue transfer in a head and neck cancer patient with polycythemia vera. Plast Reconstr Surg 2009;124:169e–70e. Schusterman MA, Reece GP, Miller MJ, Harris S. The osteocutaneous free fibula flap: is the skin flap reliable? Plast Reconstr Surg 1992;90:787–91. Jones NF, Monstrey S, Gambier BA. Reliability of the fibular osteocutaneous flap for mandibular reconstruction: anatomical and surgical confirmation. Plast Reconstr Surg 1996;97:707–16.
Address: Naseem Ghazali Maxillofacial Unit Queen Victoria Hospital Holtye Road East Grinstead RH19 3DZ United Kingdom Tel: +44 01432 414 313; Fax: +44 01342 414 114 E-mail: [email protected]
Case Report Reconstructive Surgery
Hemimandibulectomy and vascularized fibula flap in bisphosphonate-induced mandibular osteonecrosis with polycythaemia rubra vera
N. Ghazali, J. C. Collyer, J. V. Tighe Maxillofacial Unit, Queen Victoria Hospital, East Grinstead, United Kingdom
N. Ghazali, J. C. Collyer, J. V. Tighe: Hemimandibulectomy and vascularized fibula flap in bisphosphonate-induced mandibular osteonecrosis with polycythaemia rubra vera. Int. J. Oral Maxillofac. Surg. 2013; 42: 120–123. # 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. This report presents the successful management of an advanced and refractory bisphosphonate-related osteonecrosis of the jaws (BRONJ) by hemimandibulectomy and an osteocutaneous fibula flap reconstruction in a patient with polycythaemia rubra vera, a rare haematological condition in which there is increased risk of thrombosis and haemorrhage. Union of the vascularized bone with the mandible depends on obtaining a BRONJ-free margin and rigid fixation of the bony ends. Magnetic resonance imaging can provide accurate delineation of necrotic bone and area of osteomyelitis. Placement of a 1 cm margin beyond this can envisage a BRONJ-free margin. Aggressive medical management of polycythaemia rubra vera by venesection, asprin and cytoreduction therapy along with anticoagulant prophylaxis against thromboembolic events in the first 2 weeks following major surgery can provide the basis of a good surgical and flap outcome. Nevertheless, the possibility of unpredictable haemorrhage must be considered throughout.
Accepted for publication 16 March 2012 Available online 18 April 2012
The use of vascularized reconstruction in the management of bisphosphonaterelated osteonecrosis of the jaws (BRONJ) is controversial. It is suggested that segmental resection and vascularized reconstruction are reserved for advanced cases of BRONJ and in those that are refractory to multiple debridement and conservative measures.1,2 This option is perceived as carrying an increased risk of postoperative
in the presence of essential thrombocytosis, antithrombotic medication and von Willebrand’s disease.3 The increased risk of thrombosis and haemorrhage with PRV can make vascularized reconstruction challenging in this group of patients. The authors present the case of a patient with an advanced and refractory case of BRONJ with PRV, who was successfully
0901-5027/010120 + 04 $36.00/0
complications, such as recurrence of BRONJ at the resection margins and nonunion. This could outweigh the potential advantages. Polycythaemia rubra vera (PRV) is a rare, chronic myeloproliferative disorder characterized by increased red cell mass (erythrocytosis), leading to hyperviscosity with an increased risk of thrombosis.3 Haemorrhage can also occur with PRV
# 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
BRONJ treatment in polycythaemia rubra vera patient
121
managed by hemimandibulectomy and vascularized fibula flap reconstruction. Case report
An 82-year-old woman with long-standing PRV and significant osteoporosis, developed BRONJ after the extraction of a periodontally compromised lower right molar tooth. She had been treated with oral alendronate (70 mg weekly) following the diagnosis of osteoporosis-induced collapse of multiple spinal vertebral bodies 7 years earlier. With the diagnosis of BRONJ, alendronate was stopped and strontium ranelate (8 mg once per month) was prescribed as an alternative. The PRV was managed with hydroxycarbamide (500 mg 3 days/week and 1 g for the remaining 4 days/week), daily aspirin (75 mg) and the option of venesection to maintain a haematocrit level of less than 0.45. Other medical problems included multiple transient ischaemic attacks, gout, hypothyroidism and hypertension. There was no history of smoking. At the initial stage, the BRONJ was unresponsive to conservative measures, including a course of pentoxyfylline and tocopherol. The sequestrum rapidly extended beyond the socket of the extracted tooth, causing inferior dental nerve paraesthesia. There was intractable jaw pain, odynophagia and chewing difficulties. Local debridement was repeated twice. The symptoms persisted and the patient became at high-risk of pathological fracture from a pencil-thin mandible (Fig. 1). Resection of the affected bone was considered 11 months after the diagnosis of BRONJ due to the refractory response to repeated localized debridement and the persistence of BRONJrelated symptoms. Imaging of the mandible was obtained using magnetic resonance imaging (MRI). Assessment of the necrotic bone was made by correlation of T1, STIR and post-gadolinium enhancement sequences. Bony destruction was seen affecting the right body and ramus of the mandible, extending from just distal of the right lower canine to the antegonial notch. There was also loss of continuity of the inferior dental canal with only a thin rim of the inferior border of the mandible remaining. In the light of this finding, a right hemimandibulectomy and vascularized fibula reconstruction was planned. The presence of pathological fracture was found intra-operatively within the BRONJ-involved pencil-thin area of the mandible (Fig. 2). The resection margin was sited 1 cm beyond the area of osteo-
Fig. 1. Orthopantomogram showing advanced BRONJ of the right mandible, refractory to conservative management and two separate debridements (11 months after diagnosis of BRONJ).
necrosis as determined by MRI and this was placed at the mandibular angle posteriorly and in the socket of the lower right second incisor anteriorly. Harvesting of a fibula osteocutaneous flap with septocutaneous perforators was performed via the lateral approach under tourniquet control. The fibula bone was osteotomized at one site to reproduce normal facial contours and was internally fixed using a 2.0 Unilock rigid internal fixation system (Synthes Ltd -UK, Welwyn Garden City, Hertfordshire, UK) (Fig. 3). End-to-end arterial anastomosis was performed between the peroneal artery to the facial artery while venous anastomosis was performed between the peroneal venae commitantae to the common facial vein using 3 mm vein coupler system (Synovis, St Paul, USA). The duration of surgery was 450 min.
The preoperative haemoglobin level was 14.8 g/dl and the haematocrit was 0.376. The patient remained on her usual PRV regime of hydroxycarbamide and aspirin, and received preoperative subcutaneous low-molecular weight heparin (4500 unit Tinzaparin) on the evening before the operation. During surgery, fluid rehydration was preferred over intraoperative blood transfusion as the estimated overall blood loss was approximately 400 ml. Intraoperative anticoagulation was not used. Immediate postoperative haemoglobin was 9.7 g/dl and the haematocrit was 0.306, which are ideal values for microvascular success. Histological examination of the resected specimen was consistent with drug-induced osteonecrosis. The specimen was composed of chronic active osteomyelitis, with bony sequestration, suppuration and periosteal
Fig. 2. Intraoperative view of the right hemi-mandible showing the presence of a pathological fracture within the area of BRONJ.
122
Ghazali et al.
Fig. 3. Immediate postoperative orthopanthomogram showing the extent of the resection and reconstruction with a vascularized fibula flap.
bone formation. The sequestra were covered by heavy bacterial aggregates. There were areas of mucosal ulceration. Normal bone was found at both resection margins. The patient developed transient sinus bradycardia secondary to vagal stimulation but apart from this, the postoperative recovery was unremarkable and her BRONJ symptoms had resolved completely. She received 7 days postoperative intravenous antibiotic prophylaxis (coamoxiclav 1.5 g three times/day). The PRV regime was continued and daily subcutaneous low-molecular weight heparin (4500 units Tinzaparin nocte) was administered prophylactically against deep vein thrombosis/pulmonary embolism while she received in-patient hospital care for 2 weeks. Nutritional requirement was provided through nasogastric tube feeding for 7 days and oral intake was commenced thereafter. There was partial take of the skin graft at the donor site, which healed with regular dressings by 4 weeks post operation. The osteocutaneous fibula flap survived in its entirety following the operation (follow up period 24 months). The authors are unable to provide radiographic evidence for this due to the unit policy of not subjecting patients to radiation in the absence of on going clinical problems. Discussion
This case demonstrates the successful management of two difficult coexisting clinical scenarios relating to microvascular reconstruction in one patient. While the reliability of vascularized fibula reconstruction has been described previously in BRONJ cases,1,2 this report also documents the additional management required in the presence of PRV, which carries the
potential risk of flap failure due to thrombosis and haemorrhage. Vascularized bone flaps can provide bony continuity for restoration of facial form and oral function following segmental resection, and reduce the risk of fistulation and metalwork exposure. Using vascularized bone flaps in BRONJ carries the risk of recurrence of BRONJ at the resection site, nonunion and transfer of malignancy to the jaw in cases of BRONJ related to bisphosphonate use in disseminated malignancy.2 Complete healing (union) at the fibula– mandible interface is subject to conventional factors related to fracture healing. In this case, the fibula bone was held rigidly in direct contact with BRONJ-free, normal mandibular bone, facilitating healing. Failure to obtain a BRONJ-free margin is cited as one of the causes of nonunion.1,2 The authors found the high sensitivity of MRI imaging for bone marrow pathology4 aided in locating the extent of bone necrosis. By adding 1 cm beyond the limit of the MRI-delineated osteonecrosis, a BRONJfree margin was reliably obtained. A reduction in the risk of postoperative BRONJ recurrence may have been facilitated by an 11 month alendronate-free period preoperatively, maintenance of good oral health, and avoidance of trauma, including the use of dentures. Postoperative hyperbaric oxygen has been advocated1 to encourage healing and prevent BRONJ recurrence following surgery, but the authors have not utilized this option due to logistic difficulties. The conventional medical management of PRV includes the use of venesection, aspirin (75 mg daily) unless contraindicated and cytoreductive therapy in certain situations, such as: when venesection is not tolerated; presence of symptomatic or
progressive splenomegaly; presence of other indicators of disease progression; and thrombocytosis.3 Although it is recognized that thrombosis and haemorrhage are major causes of morbidity and mortality in PRV, there are no guidelines regarding the management of patients with PRV who are undergoing surgery. A large, retrospective multicentre study of patients with PRV and essential thrombocytosis undergoing various types of surgery found that the surgical outcome was complicated by vascular occlusion and haemorrhagic episodes in 7.7% and 10.5% cases, respectively.5 The risk of thromboembolic events was highest in the first 2 weeks in both PV and essential thrombocytosis. Arterial thrombosis was more frequently seen in essential thrombocytosis and in those with other risk factors for arterial thrombosis (e.g. diabetes, smokers) while venous thrombosis was more frequently seen in PRV. Heparin was prescribed in those undergoing major surgery and in addition, anticoagulants were administered in PRV. Haematological intervention (i.e. venesection or cytoreduction therapy) was preferentially given to PRV patients with platelet counts of more than 1000 109/l, those over 60 years at the time of surgery, undergoing major surgery, who had developed a thrombotic event during follow-up and in those with white cell counts of more than 10 109/l5. Owing to the rarity of the condition, there is only one report in the literature of the successful outcome of vascularized reconstruction in a patient with PRV,6 in which the anterolateral thigh flap was used to reconstruct the anterior floor of the mouth following resection of squamous cell carcinoma. Their approach relied on preoperative venesection, minimal blood loss and good intraoperative hydration to prevent any thrombotic event. Intraoperative anticoagulation was not advocated but it was proposed that anticoagulants might be useful in PV even when essential thrombocytosis is absent, due to its effect in preventing thrombosis in the microcirculation. The management of the present case tended to mirror the findings of the large, retrospective multicentre study. Aggressive haematological control of erythrocytosis was achieved by cytoreduction therapy. Prevention of flap microcirculation thrombosis with aspirin was instituted preoperatively. Haemoglobin levels were closely monitored and the intraoperative blood loss was not replaced. The cytoreductive agent and anticoagulant regime were continued immediately in the postoperative period. Low-molecular weight heparin was administered regularly
BRONJ treatment in polycythaemia rubra vera patient for 2 weeks postoperatively while the patient remained in hospital care. This was crucial during the time when the risk of a thromboembolic event was at the highest, particularly as the donor site was the lower limb. Postoperative thromboembolic events were avoided completely. Despite taking both low-molecular weight heparin and aspirin during the postoperative period, there were no bleeding episodes. The entire flap survived, including the cutaneous component. The reliability of the fibula skin paddle based on septocutaneous perforators has been questioned7 but attention to the technical detail surrounding flap harvesting8 has improved its dependability in mandibular reconstruction. Apart from the fibular flap, other vascularized flaps have been used successfully in BRONJ-related reconstructions of the mandible.2 Competing interests
None declared. Funding
None. Ethical approval
None.
Acknowledgements. The authors would
like to acknowledge Dr AW Barrett, Consultant Oral Pathologist and Dr Lekha Chandrasekaran, Consultant Radiologist, both of the Queen Victoria Hospital, for their expert commentaries in the relevant areas during the preparation of this paper.
5.
6.
References 1. Nocini PF, Saia G, Bettini G, Ragazzo M, Blandamura S, Chiarini L, et al. Vascularized fibula flap reconstruction of the mandible in bisphosphonate-related osteonecrosis. Eur J Surg Oncol 2009;35:373–9. 2. Seth R, Futran ND, Alam DS, Knott PD. Outcomes of vascularized bone graft reconstruction of the mandible in bisphosphonaterelated osteonecrosis of the jaws. Laryngoscope 2010;120:2165–71. 3. McMullin MF, Bareford D, Campbell P, Green AR, Harrison C, Hunt B, et al. On behalf of the General Haematology Task Force of the British Committee for standards in Haematology. Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis. Br J Haematol 2005;130: 174–95. 4. Mirowitz SA, Apicella P, Reinus WR, Hammerman AM. MR imaging of bone marrow lesion: relative conspicuousness on T1-
7.
8.
123
weighted, fat suppressed T2-weighted and STIR images. AJR 1994;162:215–21. Ruggeri M, Rodeghiero F, Tosetto A, Castaman G, Scognamiglio F, Finazzi G, et al. for the Gruppo Italiano Malattie Ematologiche dell’Adulto (GIMEMA) Chronic Myeloproliferative Diseases Working Party. Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: a retrospective survey. Blood 2008;111: 666–71. Lin SJ, Yu P. Free tissue transfer in a head and neck cancer patient with polycythemia vera. Plast Reconstr Surg 2009;124:169e–70e. Schusterman MA, Reece GP, Miller MJ, Harris S. The osteocutaneous free fibula flap: is the skin flap reliable? Plast Reconstr Surg 1992;90:787–91. Jones NF, Monstrey S, Gambier BA. Reliability of the fibular osteocutaneous flap for mandibular reconstruction: anatomical and surgical confirmation. Plast Reconstr Surg 1996;97:707–16.
Address: Naseem Ghazali Maxillofacial Unit Queen Victoria Hospital Holtye Road East Grinstead RH19 3DZ United Kingdom Tel: +44 01432 414 313; Fax: +44 01342 414 114 E-mail: [email protected]