442 - Hereditary sensory autonomic neuropathy presenting as recurrent traumatic injury to the tongue

Hereditary sensory autonomic neuropathy (HSAN) is a group of rare genetic disorders in which affected patients have a diminished capacity to feel pain. Patients with HSAN may present with a wide range of factitial injuries, where injury to the oral cavity may be an early presenting sign. While existing literature on HSAN is scant, many reports highlight the long-term outcomes that may include enucleation of eyes, amputation of fingers and limbs, and premature death. This case describes a five-month-old female who initially presented with extensive trauma to her tongue. The repetitive injury to the tongue caused it to heal with a bifid deformity and the patient was later diagnosed with HSAN type 4. This case highlights the importance of recognition of extensive oral trauma as one of the early signs of HSAN that should provoke a timely referral for neurological assessment.