DOI: 10.36347/sjmcr.2017.v05i11.002

Pages: 694-696

Congenital insensitivity to pain and anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy (HSAN) type IV is a rare autosomal recessive disorder. It is one of the rare hereditary sensory autonomic neuropathies. The ophtalmological manifestations of this syndrome include the absence of corneal sensation, leads to corneal ulcers and opacities. Patients with hereditary sensory and autonomic neuropathies are rarely seen in pediatric ophthalmology practices. We describe the case of a boy with CIPA revealed by a self-inflected eye trauma. We suspect congenital corneal insensitivity as part of CIPA as the boy had an history compatible with pain insensitivity involving at least another trigeminal branch which is the mandibular nerve. Normal development of visual function is expected in patients with CIPA without corneal opacities. Care for dry eye, prevention of corneal infection, and daily observation of the ocular surface are crucial for maintaining good visual function in CIPA patients. The aim of reporting this case is to draw the attention of clinicians to this rare syndrome that may give vital and functional complications. The diagnosis can be easily evoking with a certain number of clinical signs without using expensive tests. Rapid diagnosis, evaluation and appropriate management can prevent the complications of corneal anesthesia and the loss of visual function in patients diagnosed with CIPA.