New presenting symptoms of Chiari I malformation: report of two cases.

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
Benedetta Ludovica PettoriniShailendra Magdum

Abstract

Chiari Malformation type I nowadays represents one of the most debated and treated entities of neurosurgery. Several different symptoms of cerebellar, brainstem and spinal cord pathology have been described and attributed to this malformation. In this paper, we reported a very uncommon association of Chiari I malformation with isolated hemihypertrophy in one case and clawing hands in the second case. We report the first case of a 14-year-old girl who presented with a 6-month history of left claw hand, with no evidence of radiculopathy and myopathy; MRI scan revealed a symmetrical herniation of cerebellar tonsils to the level of C1 (0.7 mm), downward displacement of the obex and holocord syrinx. We assisted to the complete resolution of the left clawing hand immediately after decompressive craniectomy, C1 laminectomy, opening of the dura and collagen matrix duraplasty. The second case is a 9-year-old boy who presented with hemihypertrophy affecting the left arm and chest wall, without involvement of the face. These case reports can suggest the association of hemihypertrophy and claw hand with Chiari I malformation and syringomyelia. Further studies are necessary to assess the real incidence of focal motor deficits and mesodermic...Continue Reading

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Citations

Oct 11, 2011·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·M-D BenjaminP Ebrad
May 13, 2011·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Shavonne L MasseyIgnacio Valencia
Feb 18, 2015·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Suhas Udayakumaran, Chiazor U Onyia
May 9, 2012·Pediatric Emergency Care·Muhammad WaseemKonstantin Dobrenkov
Feb 15, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Giuseppe TalamontiGiuseppe D'Aliberti
Jun 5, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Palma CiaramitaroDiego Garbossa

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