Dental Management of a Young Child Affected by Galactosialidosis and a Gigantic Abdominal Growth

Abstract EN

Galactosialidosis (GS) is a rare form of lysosomal storage disease that involves a broad spectrum of skeletal and soft tissue abnormalities.

We report here on a 4-year 7-month-old boy with mild mental retardation, exhibiting multiple caries cavities and associated infectious foci and macroglossia.

A huge abdominal enlargement due to peritoneal ascites was evident.

Behavioral management and patient positioning on the dental chair represented a true challenge.

The patient was treated under general anesthesia.

However, life-threatening postoperative complications occurred because of the impossibility of extubating the patient.

A very careful preanesthetic assessment is crucial in children affected by general conditions associated with airway anomalies, such as GS.