Acute deterioration of adults with Chiari I malformation associated with extensive syrinx

Abstract

Background: Chiari I malformation (CMI) is usually a chronic disorder, although it may also present as a life-threatening condition with need for urgent decompression. Studies of acute deterioration of CMI are scarce. The identification of preoperative radiological factors that predict acute deterioration in adult patients with CMI facilitates planning of early decompression and decreases CMI-related morbidity. Patients and methods: This is a retrospective study of all adult patients with CMI who were referred to Sahlgrenska University Hospital, Sweden and underwent occipitocervical decompression in the period 2006–2016. In total, 65 consecutive patients were included and data regarding the preoperative degree of tonsillar herniation and syrinx (size, length and degree of rostral extension) were registered. Acute deterioration and the need for urgent surgical decompression within 24 h of hospitalisation were noted. Results: Three patients presented with acute deterioration of symptoms (4.6%) and showed an increased length and size of syrinx and more rostral extension of syrinx as compared to non-acute patients with CMI. Conclusion: Our study highlights the incidence of acute deterioration of CMI and shows the importance of assessing preoperative size, length and rostral extension of CMI-associated syrinx as indicators of acute deterioration so that CMI patients can be assigned to early surgical decompression.