Abstract
Congenital insensitivity to pain with anhidrosis (CIPA) is a type IV hereditary sensory and autonomic neuropathy associated with an autosomal recessive genetic disease with mental retardation.CIPA individuals lack pain sensation and perspiration function due to the absence of unmyelinated Cand thin myelinated Aδ fibers. However, since thick myelinated Aβ fibers are present, tactile sensation is considered to be functioning. Patients with this disease show bite wounds to the tongue, lips,and fingers due to self-mutilation, and conduct self tooth extraction that occasionally causes mandibular osteomyelitis ; therefore, it is a disease for which dental treatment and care are important.In this study, an 11-year and 5-month-old girl, diagnosed with CIPA, exhibited mandibular osteomyelitis due to self tooth extraction. Six teeth were lost in 2 months, and sequestrectomy was performed in the area.Before surgery, dental pulp vitality was assessed in our department to determine the removal range of mandibular sequesters and indicate which teeth required extraction. Since all of the teeth measured were non-responsive to a conventional electric pulp test (EPT) this dental pulp vitality diagnostic test was considered useless, and the presence of pulp pulse waves was examined using transmitted-light plethysmography (TLP), in which the presence of pulpal blood flow could be objectively measured.As a result, the presence of pulp pulse waves in the mandibular right second premolar, located in the border region of sequesters, was confirmed. The tooth was diagnosed as vital, and was preserved without performing tooth extraction. TLP appears to be very useful for the diagnosis of teeth in patients with congenital insensitivity to pain with anhidrosis. At 10 months after surgery, a favorable clinical course has been shown based on TLP and X-ray examinations of the preserved tooth and mandibular bone.
