Congenital inenitivity to pain with anhidroi: ocular and ytemic manifetation Original Reearch Article

PURPOE: To report the incidence and everity of the ophthalmologic manifetation in patient with congenital inenitivity to pain with anhidroi. METHOD: Fifteen Bedouin children with congenital inenitivity to pain with anhidroi underwent complete ocular examination, including refraction and aement of corneal enation, and a detailed neurologic examination, including meaurement of median nerve motor and enory conduction. Patient with corneal ulcer were treated appropriately. REULT: In the 15 children (eight girl and even boy, with a mean age of 3.75 ± 2.67 year; range, 9 month to 9 year), corneal enation wa abent in both eye. Corneal opacitie were preent in 10 children, five of whom had bilateral corneal opacitie. Corneal ulcer were found in even children, two of whom had bilateral ulcer, and in three children the ulcer recurred. The corneal ulcer were characterized by very poor healing. The urgical procedure included four lateral tarorrhaphie, two corneal patch graft, and one penetrating keratoplaty. All the patient had elf-inflicted injurie varying from kin ulcer, burn, and bone fracture to autoamputation of fingertip and tongue. Many patient howed delayed healing and repair of bone and kin injurie. All patient had attack of hyperpyrexia, moderate mental retardation, and hypotonicity with abent uperficial enation to light touch. Reult of median nerve motor and enory conduction tudie were within normal limit. CONCLUION: The patient with congenital inenitivity to pain and anhidroi and abent corneal enation howed a marked tendency to develop corneal ulcer that healed poorly. Congenital inenitivity to pain and anhidroi, although rare, hould be conidered in the differential diagnoi of neurotrophic keratiti.