Extreme microglossia

Abstract

The congenital anomaly of extreme microglossia is uncommon and fewer than 50 cases have been described. The microglossia has often occurred in association with limb abnormalities and, therefore, these cases have been grouped together as the hypoglossia-hypodactylia syndrome within the oromandibular-limb hypogenesis syndromes. We present five cases seen at our referral centre. Surprisingly for this number none had limb anomalies but all had marked micrognathia-Gorlin-Hall classification type 5-two requiring tracheostomy for upper airway obstruction. All required tube feeding for between 4 and 17 months. Long term follow-up is not yet available.