Hypercoagulopathy and dento-alveolar surgery: a case of exodontia in a patient with hughes' syndrome

Treatment

The UR7 was delivered whole with forceps; no sutures were required. The LR7 required a transalveolar approach for removal. A buccal flap was raised with a distal relieving incision, buccal bone was removed and the tooth sectioned horizontally and vertically prior to its delivery. The surgical site was closed with 4/0 braided polyglactin (Vicryl Rapide™). The patient was discharged home with routine aftercare instructions.

Post-operative complications

Within 24 hours of the surgery the patient attended the Emergency Department (ED) at Frenchay Hospital complaining of bleeding from the UR7 socket which was not controllable by pressure alone. Routine haematological investigations initiated in the ED were unremarkable, with a platelet count of 192 x 10⁹/L and nothing untoward from the clotting screen. The patient was then transferred to the OMFS department where the UR7 socket was packed with resorbable oxidized cellulose (Gelitacel®) and sutured with 3/0 braided polyglactin (Vicryl Rapide™). Haemostasis was achieved and the patient discharged.

Seven days later the patient re-attended complaining of pain and swelling in the right mandible. Clinical examination revealed a haematoma arising from the extraction socket of the LR7. Radiographic examination did not reveal any abnormality within the mandibular bone in this area excepting findings commensurate with a recent extraction (Figure 1). A routine antimicrobial was prescribed (Co-Amoxiclav 625 mg three times daily for 5 days) to prevent secondary infection of the haematoma. The patient was discharged on the understanding that he should re-attend if the symptoms did not resolve. In the meantime, the patient's mother contacted the OMFS department to inform them that he had ‘sticky blood syndrome’, which had not been mentioned by the patient and was thus not noted in his file.

Four days later the patient attended again complaining that the haematoma had not resolved and was now preventing mouth closure. On examination, there was a 30 x 30 mm haematoma arising from the LR7 socket and terminating above the level of the occlusal plane. The original mucoperiosteal flap was raised, the haematoma debrided, the area dressed with resorbable oxidized cellulose (Gelitacel®) and re-sutured as above. A further course of antibiotics was prescribed (Co-Amoxiclav 625 mg three times daily for 5 days).

Aftercare

The patient was subsequently followed up over two review appointments and, as healing was seen to be progressing, was discharged.

The patient was informed of the nature of his condition more formally, this being known as ‘Antiphospholipid Syndrome’ and a letter was sent to his general medical practitioner advising referral to haematology for appropriate investigation and management.

Antiphospholipid syndrome

‘Sticky blood syndrome’, more formally known as Antiphospholipid Antibody Sydrome (APLS) or eponymously Hughes' Syndrome, was first described in 1983 by GRV Hughes. It is a thrombophilic disorder in which venous and/or arterial thromobosis may occur.² This relates to the presence of auto-antibodies to phospholipid within the patient's serum. Deposition of such auto-antibodies in small blood vessels can lead to intimal hyperplasia and acute thromboses affecting cerebral, renal, pulmonary, cutaneous and cardiac arteries.³ Thrombocytopaenia might also feature.⁴

APLS can be associated with Systemic Lupus Erythematosus (SLE), though the majority of patients with the syndrome do not possess the criteria for the diagnosis of SLE and are therefore classified as having ‘Primary APLS’.⁵

In individuals with APLS, Anti-Apolipoprotein and Anti-Cardiolipin antibodies bind to Apolipoprotein H, leading to inhibition of Protein C. Antibodies against Protein S might also be present and these will act to decrease the efficiency of protein C and its role in the control of thrombosis.

The prevalence of various complications of APLS, estimated from a cohort study of 1,000 patients carried out in 2002, are listed in Table 1.⁶

Importantly, the condition can lead to catastrophic antiphospholipid syndrome; a potentially fatal state where multiple organ failure results from the occlusion of small blood vessels in various organs by the resultant thrombi.

In terms of oral surgery, the tendency towards thrombus formation can complicate procedures where bleeding is an issue, as excessive haematoma can result. Furthermore, as APLS is often treated with anticoagulants, increased bleeding must be considered in those on such treatments.³ Patients with APLS may also exhibit pulmonary and systemic hypertension.

Diagnosis

APLS is usually diagnosed after a clinically significant complication, prompting investigation. The ‘Sapporo Criteria’⁷ are used for the diagnosis of APLS. The presence of at least one of the clinical criteria and one of the laboratory criteria is diagnostic. The clinical criteria are defined as vascular thrombosis and pregnancy morbidity. Laboratory criteria include the presence, in plasma, of:

The above laboratory markers should be present on two or more occasions at least 12 weeks apart.⁸

Management of patients with APLS

Many sufferers are anticoagulated with Warfarin aiming for an international normalized ratio of 2.5.⁹ Treatment is often instituted following an acute event such as a venous thrombo-embolism (VTE). The duration of anticoagulation is controversial and must be determined on an individual basis. It is, however, suggested that short-term anticoagulation is insufficient for patients with APLS due to high risk of recurrent VTE.^10,11 Aspirin has not been shown to deliver any significant additional benefits. Prolonged anticoagulation has been shown to reduce the risk of recurrent VTE and, despite an unclear risk-benefit ratio for long term anticoagulation, this is recommended in most patients.

In addition to various obstetric morbidities, a well recognized complication of APLS is spontaneous abortion, which is used as a defining criterion for the diagnosis of the syndrome in such cases. Prevention requires a multidisciplinary approach and can typically involve corticosteroids, low dose aspirin, heparin and immunoglobulins, either on their own or in combination.¹²

Following the case described, the authors aimed to find out if there were any reports of complications in APLS patients undergoing oral surgery procedures. Utilizing Medline, searches were carried out with the terms ‘Antiphospholipid’, ‘Sticky blood’, ‘Hughes’ Syndrome', ‘Dental’, ‘Extraction’, ‘Exodontia’ and ‘Oral Surgery’ in all possible combinations. Thirty-eight articles and 19 case reports were found in all languages (Table 2) but only one case report detailed a case involving exodontia. The report described thrombotic and haemorrhagic complications in children with the lupus anticoagulant referring to prolonged bleeding after a dental extraction for four days.¹³ The authors were unable to find any articles or case reports discussing the presentation or management of patients with APLS requiring exodontia procedures.

In terms of exodontia, hypercoagulation is not in itself problematic. Complications arise when haemostasis is not adequately controlled, leading to an unmoderated haemostatic process, which in APLS patients can lead to the formation of significant haematomas.

The most important aspect of pre-operative management is recognition of the condition in the first place. A thorough medical history is thus crucial as patients with knowledge of their condition may not realize its implications for exodontia. Suspicion may be aroused by a history of VTE or pregnancy morbidity. Clinically, ‘Levido Reticulartis’, a purple discoloration of the skin, may be present. In APLS this is caused by the dilation of capillaries and venules in response to obstruction by small thrombi.

Where exodontia is indicated the patient will require appropriate haematological investigations and optimization prior to surgical treatment. Those patients on Warfarin should have their INR investigated in line with current guidelines.¹⁴ A full blood count is a useful investigation as thrombocytopaenia is a recognized complication and may require pre-surgical optimization.

The authors would advise that all measures to promote the formation of an adequate fibrin clot (not breaching the boundaries of the surgical site) are taken during the surgical procedure; such as packing with resorbable oxidized cellulose and closure, where possible, by primary intention with sutures. Adequate application of pressure with gauze post-operatively should also be used. The patient should not be discharged home until a fully stabilized clot is visible and routine follow-up would be advised.