381 - Severe Mandibular Crowding in a Child with Infantile Pompe Disease: A Case Report

Introduction:

Pompe disease is a rare, multisystemic, hereditary disease which is caused by a mutation in the GAA gene. Pompe disease belongs to both the lysosomal storage disorders (LSDs) group and the glycogen storage disorders (GSDs) groups. The symptoms of the disease include rapid progressive muscle weakness, hypotonia, myopathy, respiratory failure, hepatosplenomegaly, difficulty in feeding, aphonia, weakness in tongue, macroglossia, and inability to control oral secretions.

Case report:
This is a case of a 4 year 2 month-old male patient with Pompe disease who was referred to the Hospital Dentistry Clinic at Mott’s Children hospital with the chief complaint of: “Crowded lower front teeth that are pointing straight back and in three different rolls.”  Patient presents with severe crowding in mandibular dentition which may be related to constant ventilator use. The case report includes clinical findings and possible treatment course for our patient and literature review of dental, oral facial manifestation related to Pompe disease.