B Sezgin Bolgül, N Hamamci, E Ağaçkiran, S Çelenk, B Ayna

Abstract

Congenital insensitivity to pain is a rare disorder seen in early childhood. Five different types of hereditary sensory and autonomic neuropathy have been identified, to date, with different patterns of sensory and autonomic dysfunction, peripheral neuropathy, clinical features, and genetic abnormalities. Absence of pain and self-mutilation are characteristic findings of this syndrome. Teeth in the oral cavity can cause damage to the oral tissues and tongue. When diagnosed, there should be cooperation between the dentist and neurologist. Using an oral shield prevents biting, and thus tissue trauma can be prevented. Here, we present the case of a 6-month-old boy with congenital insensitivity to pain (hereditary sensory and autonomic neuropathies; HSAN type V) with self-mutilation injuries to his tongue and fingers caused by biting, along with a discussion of treatment strategies. The results of this report suggest that early diagnosis and specific dental management for patients with congenital insensitivity to pain are important for prevention of the characteristic oral and dental problems accompanying this disorder.