Emilia Rizzo1,2, Ailsa Elizabeth Ritchie3, Vinay Shivamurthy4, Ata Siddiqui5, Ming Lim6,7. 1. Faculty of Medicine and Surgery, University of Catania, Piazza dell'Università 2, 95124 Catania, Italy. 2. Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK. 3. Paediatric Ophthalmology, Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, London SE1 7EH, UK. 4. Paediatric Rheumatology, Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, London SE1 7EH, UK. 5. Department of Neuroradiology, King's College Hospital NHS Foundation Trust, London SE5 9RS, UK. 6. Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London SE1 7RS, UK. 7. Faculty of Life Sciences and Medicine, Kings College London, London SE1 7RS, UK.
Abstract
Background/goal: Hypertrophic pachymeningitis is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. This condition has largely been reported in adults, but there are very few reports in children. METHODS: We describe a 14-year-old boy with idiopathic hypertrophic pachymeningitis, who presented with deteriorating vision on a background of severe headache. We evaluated pediatric cases of hypertrophic pachymeningitis and compared treatments and their relation to outcomes. RESULTS: There are only eleven pediatric cases of hypertrophic pachymeningitis reported in the literature. In the patients treated with steroids either at presentation or subsequent relapses, a good response was reported. In the cases with delayed initiation of steroid treatment, this was often related to an incomplete recovery. In our patient, this delay may have contributed to his poor visual outcome. CONCLUSIONS: Early initiation of steroid treatment in children with idiopathic hypertrophic pachymeningitis may improve outcomes.
Background/goal: Hypertrophic pachymeningitis is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. This condition has largely been reported in adults, but there are very few reports in children. METHODS: We describe a 14-year-old boy with idiopathic hypertrophic pachymeningitis, who presented with deteriorating vision on a background of severe headache. We evaluated pediatric cases of hypertrophic pachymeningitis and compared treatments and their relation to outcomes. RESULTS: There are only eleven pediatric cases of hypertrophic pachymeningitis reported in the literature. In the patients treated with steroids either at presentation or subsequent relapses, a good response was reported. In the cases with delayed initiation of steroid treatment, this was often related to an incomplete recovery. In our patient, this delay may have contributed to his poor visual outcome. CONCLUSIONS: Early initiation of steroid treatment in children with idiopathic hypertrophic pachymeningitis may improve outcomes.
Authors: Samah K Aburahma; Abdel Ghani M Anabtawi; Hala S Al Rimawi; Mwaffaq A Elheis; Aliaa H Mohtaseb Journal: Pediatr Neurol Date: 2009-06 Impact factor: 3.372