Sophie M Jones1, Kate A Smith2, Minakshi Jain2, Jemima E Mellerio3, Anna Martinez3, Ken K Nischal4. 1. Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom; Developmental Biology Unit, Institute of Child Health, University College London, London, United Kingdom. 2. Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom. 3. Department of Dermatology, Great Ormond Street Hospital, London, United Kingdom. 4. Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom; Developmental Biology Unit, Institute of Child Health, University College London, London, United Kingdom; UPMC Childrens Eye Center, Childrens Hospital of Pittsburgh, Pittsburgh, Pennsylvania. Electronic address: nischalkk@upmc.edu.
Abstract
PURPOSE: To determine the frequency of meibomian gland dysfunction (MGD) in children with epidermolysis bullosa (EB). DESIGN: Hospital-based cross-sectional study. PARTICIPANTS: One hundred five children with different forms of EB. METHODS: Prospective ophthalmic examination of children with EB presenting over seventeen months including meibomian gland assessment using a recognized classification. MAIN OUTCOME MEASURES: Frequency of MGD. RESULTS: One hundred five children were recruited, 8.6% with junctional EB, 34.3% with simplex EB, 34.3% with autosomal recessive dystrophic EB, and 22.9% autosomal dominant dystrophic EB. Mean age was 7.42 years (range, 0.08-17.75 years). Ninety-two children (87.62%) demonstrated 1 or more features of MGD. CONCLUSIONS: Most children with EB exhibit signs of MGD. To the best of our knowledge, this is the first prospective ocular surface evaluation in children with EB to include lid margin evaluation using a recognized classification system. Our findings help explain some of the ocular surface anomalies seen in children with EB.
PURPOSE: To determine the frequency of meibomian gland dysfunction (MGD) in children with epidermolysis bullosa (EB). DESIGN: Hospital-based cross-sectional study. PARTICIPANTS: One hundred five children with different forms of EB. METHODS: Prospective ophthalmic examination of children with EB presenting over seventeen months including meibomian gland assessment using a recognized classification. MAIN OUTCOME MEASURES: Frequency of MGD. RESULTS: One hundred five children were recruited, 8.6% with junctional EB, 34.3% with simplex EB, 34.3% with autosomal recessive dystrophic EB, and 22.9% autosomal dominant dystrophic EB. Mean age was 7.42 years (range, 0.08-17.75 years). Ninety-two children (87.62%) demonstrated 1 or more features of MGD. CONCLUSIONS: Most children with EB exhibit signs of MGD. To the best of our knowledge, this is the first prospective ocular surface evaluation in children with EB to include lid margin evaluation using a recognized classification system. Our findings help explain some of the ocular surface anomalies seen in children with EB.
Authors: Brendon W H Lee; Jeremy C K Tan; Melissa Radjenovic; Minas T Coroneo; Dedee F Murrell Journal: Orphanet J Rare Dis Date: 2018-05-22 Impact factor: 4.123