KERATOENDOTHELIITIS FUGAX HEREDITARIA

Course overview

KERATOENDOTHELIITIS FUGAX HEREDITARIA IS A RARE INFLAMMATORY GENETIC CONDITION CHARACTERIZED BY RECURRENT EPISODES OF DEBILITATING UNILATERAL CORNEAL AND CONJUNCTIVAL HYPEREMIA, CORNEAL EDEMA, VISUAL IMPAIRMENT, CORNEAL OPACIFICATION, AND PHOTOPHOBIA THAT LAST FOR TWO TO FIVE DAYS. LACRIMATION, PAIN, COLORED HALOS, DIPLOPIA, A MILD ANTERIOR CHAMBER REACTION, GUTTATA-LIKE CHANGES (PSEUDOGUTTATA), AND A ‘GRITTY’ FEELING MAY ALSO OCCUR. ITS INHERITANCE PATTERN IS AUTOSOMAL DOMINANT. THE PATHOLOGY GENERALLY BEGINS TEMPORALLY BEFORE QUICKLY PROGRESSING TO SURROUND THE ENTIRETY OF THE CORNEA. IN THE ACUTE PHASE, THE PAIN OR DISCOMFORT IS OFTEN SEVERE ENOUGH TO DISRUPT SLEEP. THIS ARTICLE COVERS THE GENETICS, PATHOPHYSIOLOGY, AND EVALUATION OF THE PATIENT WITH KERATOENDOTHELIITIS FUGAX HEREDITARIA. IT ALSO EXPLORES WHAT IS KNOWN ABOUT MANAGEMENT, POTENTIAL COMPLICATIONS, AND PROGNOSIS FOR PATIENTS.