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.

先天性疼痛不敏感在兒童早期是一種很罕见的疾病。迄今為止，根據感覺和自主神經功能障礙、外周神經病變、臨床特徵及遺傳異常的不同模式，已經確定了五種不同類型的遺傳性感覺障礙和自主神經病變。疼痛缺失和自殘是該綜合症的特徵性表現。口腔內的牙齒可以造成口內組織和舌頭的損傷。一旦診斷了該病，牙醫應該和神經科醫生合作。使用口內保護罩防止咬傷，由此可避免口內的組織創傷。這裏，我們提出了一個患有先天性疼痛不敏感（遺傳性感覺和自主神經病變；HSAN，V型）的6個月大的男童的案例，他通過啃咬對舌頭和手指造成自殘，隨後我們對其治療方案進行了討論。該報導的結果顯示，對先天性疼痛不敏感的患者進行早期診斷和特異的口腔科治療，在預防該病症伴隨的特徵性口腔和牙齒的問題方面是非常重要的。