Congenital corneal anesthesia in children with the vacterl association

• Published in: American journal of ophthalmology Vol. 125; no. 1; pp. 96 - 98
• Main Authors: Cruysberg, J.R.M., Draaijer, R.W., Pinckers, A., Brunner, H.G.
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 1998; Elsevier; Elsevier Limited
• Subjects: Abnormalities, Multiple; Anus, Imperforate - complications; Biological and medical sciences; Child; Child, Preschool; Cornea - abnormalities; Corneal Diseases - etiology; Corneal Injuries; Diseases of cornea, anterior segment and sclera; Esophageal Atresia - complications; Female; Hand Deformities, Congenital - complications; Humans; Kidney - abnormalities; Male; Medical sciences; Ophthalmology; Pain Insensitivity, Congenital - complications; Self Mutilation - complications; Spine - abnormalities; Tetralogy of Fallot - complications; Tracheoesophageal Fistula - complications; Vision Disorders - complications; Esophageal disease; Diseases of the osteoarticular system; Cardiovascular disease; Vertebra; Keratopathy; Spine disease; Kidney; Association; Limb; Defect; Child; Trachea; Anorectal disease; Human; Kidney disease; Diseases of the trachea; Urinary system disease; Respiratory disease; Congenital disease; Esophagus; Symptomatology; Eye disease; Anus; Digestive diseases; Circulatory system; Anterior segment disease
• ISSN: 0002-9394; 1879-1891
• DOI: 10.1016/S0002-9394(99)80241-4

To describe ocular manifestations in patients with the VACTERL (vertebral, anal, cardiovascular, tracheoesophageal, renal, and limb defects) association. We reviewed the medical records of patients with the VACTERL association who had undergone treatment for amblyopia at the orthoptic department. A striking similarity was found in three unrelated children, who, in addition to clinical manifestations of VACTERL association, showed total corneal anesthesia of one or both eyes that had resulted in recurrent corneal erosions, keratitis, and permanent corneal opacities. Other ocular manifestations included nonparalytic strabismus, anisometropia, and amblyopia. Congenital corneal anesthesia may be part of the VACTERL association.