Dental Management of a Child With Moyamoya Disease and Down Syndrome: A Case Report and Review.

Abstract

Moyamoya disease (MMD) is a rare cerebrovascular occlusive disorder involving Internal carotid arteries, which may present in association with Down syndrome (Trisomy 21). These patients are at increased risk of intraoperative complications such as hyperventilation, transient ischemia, stroke and cerebral haemorrhage. This case report describes the dental management of an 8-year-old female patient with MMD and associated Down syndrome (DS) receiving antiplatelet therapy, along with a comprehensive literature review. The patient presented with dental pain in the upper right quadrant and multiple carious teeth. The child was cognitively impaired but potentially cooperative, and behavior management was achieved through desensitization and audiovisual distraction techniques. Dental treatment was performed in staged, short appointments and included selective caries excavation and restoration of teeth (#11, #21, #53, #55, #63, #65, #83, #85) using glass ionomer cement. Extraction of decayed teeth (#54, #64, #75 root stumps) was carried out under local anaesthesia (LA) without discontinuation of aspirin therapy. Patient was monitored intraoperatively for SpO₂ and postoperatively for extraction-site bleeding, which was effectively controlled using cold application and local pressure. Acetaminophen was prescribed for analgesia. Over a one-year follow-up period, the patient demonstrated significant improvement in oral health, pain reduction, and overall quality of life. This case highlights that the choice of anaesthesia and management protocol should be individualized based on the patient's level of cooperation, extent of dental treatment needs, and associated comorbidities, including bleeding risk and airway considerations.