Full Mouth Rehabilitation of a Child with Aicardia-Goutières: A Rare Syndrome

Abstract

Aicardi-Goutières Syndrome (AGS) is a rare genetic disorder with autosomal recessive inheritance. AGS is characterised by an earlyonset encephalopathy that usually, but not always, results in severe intellectual and physical disability. Involuntary muscular spasms between the ages of four months and four years are the typical starting point for Aicardi syndrome. Hepatosplenomegaly, increased liver enzymes, thrombocytopenia, and abnormal neurologic signs in a subgroup of AGS children at birth strongly imply congenital infection. Agenesis of the corpus callosum, chorioretinal lacunae, and seizures are all symptoms of Aicardi syndrome. They frequently exhibit the subacute onset of a severe encephalopathy that is characterised by intense irritability, sporadic sterile pyrexias, loss of abilities, and slowed head growth. In 40% of cases, skin lesions like chilblains can appear on the fingers, toes, and ears. This disease can be diagnosed with Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) scans with the appearance of calcification of the basal ganglia. The associated behavioral challenges with syndromic patients demand pharmacological management of oral rehabilitation. The literature is scarce regarding the oral manifestations of this syndrome. Hence, authors present the successful fullmouth rehabilitation of severe Early Childhood Caries (ECC) in a 3-year-old child with AGS under General Anaesthesia (GA).