Table 3.

Important consideration in the management of oral and dental manifestation in patients with Gaucher Disease

The gold standard for establishing the diagnosis of Gaucher disease is by detecting low levels of enzyme activity in peripheral blood cells compared to (same-day) normal controls. Biopsy and bone grafting or dental extractions should not be performed exclusively because of concern about the abnormal appearance of the mandibular bony matrix on x-ray in the context of overwhelming Gaucher cell infiltration. In cases where extractions have been performed, consider implants in patients with Gaucher disease using the same criteria as in other patients. There is delayed eruption of the teeth and permanent dentition in virtually all children with Gaucher disease, but both catch-up growth (height as well as bone age) is to be expected even in the absence of ERT. The severity of thrombocytopenia does not predict the risk of bleeding even among patients receiving ERT; therefore, evaluation of coagulation deficiencies and impaired platelet function tests is prudent before commencing any dental procedures. To prepare adequately for possible hemorrhaging, appropriate hematological replacement therapy prior to the procedure including the administration of anti-fibrinolytics (e.g., hexacapron) and/or platelet transfusion after the procedure in high risk patients should be considered. Suturing the area of the incision is prudent. Provide comprehensive follow-up after procedures to monitor possible complications; stress good oral hygiene and appropriate dental and periodontal follow-up for all patients.