Iorio Matthew L, Barbour John R
Department of Plastic Surgery, Georgetown University Hospital, Washington, DC, USA.
Department of Plastic Surgery, Georgetown University Hospital, Washington, DC, USA
J Child Neurol. 2014 Jul;29(7):962-4. doi: 10.1177/0883073813482973. Epub 2013 Apr 2.
Congenital insensitivity to pain is a rare sensory neuropathy that manifests with multiple and recurrent injuries secondary to a lack of negative pain stimuli. When present with compulsive onychophagia, prompt recognition and behavioral management to prevent chronic digital infection or amputation is imperative. We present the case of two 7 year-old monozygotic twins that presented with congenital insensitivity to pain and compulsive onychophagia without directed parental counseling or behavioral modification strategies. The presenting child was noted to have an acute digital felon and osteomyelitis of several distal phalanges, and the sister had a similar history with distal phalangeal amputations. The occurrence of these overlapping disorders in monozygotic twins has not been previously reported, however, patient and parental education with behavioral modifications of injurious and compulsive behavior is the cornerstone of management. In addition, we have provided a review of the diagnosis for clinical differentiation.
先天性无痛觉是一种罕见的感觉神经病变,表现为由于缺乏疼痛负性刺激而导致的多处反复损伤。当伴有强迫性咬甲癖时,必须及时识别并进行行为管理,以防止慢性手指感染或截肢。我们报告了一例7岁同卵双胞胎病例,他们患有先天性无痛觉和强迫性咬甲癖,但未接受针对性的家长咨询或行为矫正策略。就诊患儿被发现患有急性手指脓性指头炎和多个远端指骨骨髓炎,其姐妹有类似病史并接受了远端指骨截肢手术。此前尚未报道同卵双胞胎中出现这些重叠病症的情况,然而,对患者和家长进行教育并对伤害性和强迫性行为进行行为矫正,是治疗的基石。此外,我们还提供了临床鉴别诊断的综述。