Bodner Lipa, Woldenberg Yitzhak, Pinsk Vered, Levy Jacov
Department of Oral and Maxillofacial Surgery, Soroka Medical Center, Ben Gurion University of the Negev, Beer-Sheva, Israel.
ASDC J Dent Child. 2002 Sep-Dec;69(3):293-6, 235.
To report the incidence and severity of the oral and dental manifestations associated with congenital insensitivity to pain with anhidrosis (CIPA).
Young children with CIPA underwent orofacial examination. The tongue, lips, and buccal mucosa were examined for soft tissue disorder. Missing and luxated teeth were recorded.
Twenty four patients (14 males and 10 females, mean age 60 months, range 9-144 months) with CIPA showed moderate to severe self-mutilation. Oral self-mutilation, such as biting injuries and scarring of soft tissues (tongue, lip, and buccal mucosa) were found in all patients. Fingertip biting was also found in most patients. Among infant patients, the mutilation was typically characterized by decubital ulcers of the tongue. Many edentulous areas due to previously extracted teeth were also found.
Early diagnosis and specific dental care for patients with CIPA can be helpful in prevention of the fingertip biting and orofacial manifestations seen in this disorder.
报告与先天性无痛觉伴无汗症（CIPA）相关的口腔和牙齿表现的发生率及严重程度。
对患有CIPA的幼儿进行口腔颌面检查。检查舌头、嘴唇和颊黏膜是否存在软组织病变。记录缺失和脱位的牙齿情况。
24例（14例男性和10例女性，平均年龄60个月，范围9 - 144个月）患有CIPA的患者表现出中度至重度自残行为。所有患者均存在口腔自残行为，如咬伤和软组织（舌头、嘴唇和颊黏膜）瘢痕形成。大多数患者还存在咬指尖的情况。在婴儿患者中，自残行为的典型特征是舌头出现褥疮性溃疡。还发现许多因先前拔牙导致的无牙区。
对CIPA患者进行早期诊断和特定的牙科护理有助于预防该疾病中出现的咬指尖和口腔颌面表现。
