Clinico-Radiological Outcome of CHIARI I Malformation with Syringomyelia after Surgery

Abstract

Clinico-Radiological Outcome of CHIARI I Malformation with Syringomyelia after Surgery

BACKGROUND Chari I malformation Type I represents a complex anatomical and / or a distended syrinx cavity. It is characterized by caudal descent of cerebellar tonsil. We wanted to analyse age and sex distribution of patients having CHAIRI I malformation with syringomyelia. We also wanted to analyse the clinical and radiological outcome of surgery. METHODS All patients who are surgically treated at the Department of Neurosurgery, SCB Medical College and Hospital, Cuttack, with a diagnosis of Chari-1 malformation with syringomyelia, reviewed prospectively from October 2015 - February 2018 were included in the study and this included minimum 6 weeks of follow up. Data regarding demographics, clinical symptoms and signs at the time of follow up was collected. Age groups of 2 - 65 years with CIM and syringomyelia were included. RESULTS 32 cases were included in the study. Out of which 19 were males (59.37%) and 13 were females (40.62%). Male to female ratio is 1:1.46. The mean age of the patients was 29.5 years. Clinical characteristics showed four common presentations. Sensory loss 62.5%, headache and neck pain 53.1% and lower limb weakness with hyperreflexia in 50%. In this study, all the patients underwent MRI. Patients with syringomyelia were taken in account. The surgical treatment is always individualized for each patient. Sub-occipital craniotomy along with removal of posterior arch of atlas and augmentation of duraplasty is the most commonly performed procedure. The risk for complications for duraplasty was minimum in our series. A favourable clinical outcome resulted from relief of spinal cord distention as the syrinx becomes smaller in size. CONCLUSIONS The average age of the patient in the study was 29.5 years with a range of 2 - 65 years. Female to male ratio was 1:1.46. Hence, Chari 1 malformation was traditionally viewed as the problem of young adults. Surgery gave a good result and on long term follow up no problems related to cervical spine instability was noted.

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