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

OBJECTIVE

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.

CASE REPORTS: 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.

CONCLUSIONS

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 disorders in Chiari I malformation in order to clarify their pathogenetical mechanisms.