Gingivectomy for gingival enlargement in a child with I-cell disease: a report of case

I-cell disease is a rare autorecessive metabolic disorder that is classified as one of the lysosomal storage diseases. Gingival enlargement is a representative oral manifestation of patients with I-cell disease. This report describes a case with a satisfactory prognosis after gingivectomy for gingival enlargement accompanying I-cell disease in a 2-year-old boy. The chief complaints were both eating disorders and night terrors accompanied by gingival enlargement of both the maxillary and mandibular alveolar gingivae, especially in the region of the first deciduous molars. Although it was supposed that there were various hazards associated with gingivectomy caused by aortic incompetence, sigmoidal spinal curvature and hypertrophy of the laryngopharynx, gingivectomy with an internal bevel incision was performed under general anesthesia to improve the chief complaints. A piece of the enlarged gingivae dissected during the operation was examined histopathologically, and the histopathological diagnosis was gingival hyperplasia. The patient's signs and symptoms of the eating disorders and night terrors have improved since the gingivectomy operation. Recurrence of the gingival enlargement has not been identified up to 18 months after the surgery. It is therefore concluded that gingivectomy for gingival enlargement accompanying I-cell disease was effective for this 2-year-old boy.