EC Paediatrics

Hemophilia is an X-linked congenital bleeding disorder caused by a deficiency of coagulation factor VIII (FVIII) (in hemophilia A) or factor IX (FIX) (in hemophilia B). The deficiency is the result of mutations of the respective clotting factor genes. It affects males on the maternal side. The incidence is estimated to be 1 in 5,000 males for hemophilia A and 1 in 30,000 males for hemophilia B. The clinical manifestations are easy bruising, joint and muscle hemorrhage. Children with haemophilia rubber cup prophylaxis and supragingival scaling may be safely performed without replacement therapy. Factor replacement before frenectomy and other periodontal surgeries. Electrosurgery is done because of the possibility of continued bleeding. Most restorative procedures on primary teeth can be successfully completed. Thin rubber dam is preferred. Wedges and matrices can be used conventionally. A pulpotomy or pulpectomy is preferable to extraction but instrumentation in periapical area should be avoided. Nonvital teeth should be obturated 2 to 3 mm short of apex. Use retraction cords during crown preparation and antibiotic prophylaxis before extraction. Infiltration anesthesia can generally be administered without replacement therapy. A minimum of a 40% factor correction is mandatory before block anesthesia. In the absence of factor replacement, periodontal ligament (PDL) injections may be used. Use of topical hemostatic agents such as bovine thrombin, microfibrillar collagen hemostat gel foam, absorbable oxidized cellulose etc. Arch wires should be secured with elastic bands. Removable appliances are used with Adams or similar clasps without free ends and preformed bands and brackets are preferred. When general anesthesia is considered, oral intubation is preferred over nasal intubation, Intramuscular injections should be avoided. This article discuss about etiology, clinical features and management of children with hemophilia.

Keywords: Bleeding Disorder; Children; Dental Management; Hemophilia; Oral Health

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