Results: The key features of KTWS are: Venous varicosities and cutaneous haemangiomata in association with asymmetrical overgrowth of the limbs and the extremities (Klippel, Trenauney 1900, Weber 1907, Gorlin 2001). Arterio-venous fistulas are an additional inconsistent component (Weber 1907). The asymmetrical overgrowth of the craniofacial skeleton may lead to the malformation of the jaws as well as malocclusion. A unilateral increase in facial dimensions in both the hard and soft tissues, particularly the lips, teeth and periodontal tissues has been noted (Defraia et al 2004). These anomalies may need to be corrected surgically.Patients develop thrombocytopaenia consequent upon platelet sequestration in the larger cavernous haemangiomata (Viljoen 1988). They run an increased risk of chronic disseminated intravascular coagulopathy (DIC). A moderate reduction in platelet count, reduced production of fibrinogen and other plasma coagulation factors as well as an increased rate of fibrinolysis is evident in these patients. Pulmonary arterial hypertension (Ulrich et al 2006) and pulmonary thromboembolism as well as deep vein thrombosis (DVT) are life threatening risks at anaesthesia and in the post-operative period (Huiras et al 2005).
Conclusion: The authors of this paper recommend that treatment is best done in hospital with a multidisciplinary team of specialists. A full clotting profile including INR, PTT and aPTT. Additionally, MRI or CT imaging to exclude undetected haemangiomas is necessary. A full medical assessment is required to exclude any surgical postoperative risks during the dental management.