title: Congenital corneal anesthesia.
abstract: OBJECTIVE
  To report the various clinical presentations of congenital corneal anesthesia.
  
  
  METHODS
  The medical records of children diagnosed with congenital corneal anesthesia between January 2006 and June 2012 at the L.V. Prasad Eye Institute, Hyderabad, India, were retrospectively reviewed for demographics and clinical characteristics.
  
  
  RESULTS
  A total of 40 patients (29 males) were included. Median age at presentation was 12 months (interquartile range, 6-60 months). Bilateral involvement was noted in 28 cases (70%). Generalized pain insensitivity with anhidrosis was documented in 10 cases (25%). The spectrum of corneal lesions included geographic epithelial defects (32 eyes), sterile ulcers (15 eyes), sterile ulcers with hypopyon (11 eyes), secondary bacterial keratitis (2 eyes), superficial/ deep corneal vascularisation (5 eyes). Generalized anesthesia with self-mutilating behavior was recognized in 10 cases. In all cases, sterile corneal ulcers resolved on average 30 days after permanent 2/3-width paramedian tarsorrhapy. One eye progressed to the stage of anterior staphyloma and subsequently underwent auto evisceration. Four eyes that underwent keratoplasty for visual rehabilitation had poor outcomes.
  
  
  CONCLUSIONS
  Accurate diagnosis, recognition of risk factors, and thorough neurological assessment is important for lessening long-term sequelae of congenital corneal anesthesia. Judicious use of tear substitutes and expedited tarsorrhaphy is crucial to restoration of useful vision in these children.
authors.affiliation: Cornea and Anterior Segment Services, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India. Electronic address: muralidhar@lvpei.org.
authors.forename: Muralidhar
authors.initials: M
authors.lastname: Ramappa
authors.affiliation: Cornea and Anterior Segment Services, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India.
authors.forename: Sunita
authors.initials: S
authors.lastname: Chaurasia
authors.affiliation: Kallam Anji Reddy Molecular Genetics Laboratory, Brien Holden Eye Research Centre, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India.
authors.forename: Subhabrata
authors.initials: S
authors.lastname: Chakrabarti
authors.affiliation: Kallam Anji Reddy Molecular Genetics Laboratory, Brien Holden Eye Research Centre, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India.
authors.forename: Inderjeet
authors.initials: I
authors.lastname: Kaur
mesh_terms: D002675:Child, Preschool; D003315:Cornea / Q000503:physiopathology; D003316:Corneal Diseases / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology; D003941:Diagnostic Techniques, Ophthalmological; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000175:diagnosis; D006801:Humans; D006987:Hypesthesia / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012677:Sensation / Q000502:physiology