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BACKGROUND: Chiari malformation type 1 (CM1) is a congenital hindbrain malformation characterized by herniation of the cerebellar tonsils below the foramen magnum. The term Chiari type 1.5 is used when herniation of the brainstem under the McRae line and anomalies of the craniovertebral junction are also present. These conditions are associated with several symptoms and signs, including headache, neck pain, and spinal cord syndrome. For symptomatic patients, surgical decompression is recommended. When radiographic indicators of craniovertebral junction (CVJ) instability or symptoms related to ventral brainstem compression are present, CVJ fixation should also be considered. CASE DESCRIPTION: We report the case of a 13-year-old girl who presented with severe tetraparesis after posterior decompression for Chiari malformation type 1.5, followed 5 days later by partial C2 laminectomy. Several months after the initial surgery, she underwent two fixations, first without and then with intraoperative cervical traction, leading to significant neurological improvement. DISCUSSION AND CONCLUSION: This case report underscores the importance of meticulous radiological analysis before CM surgery. For CM 1.5 patients with basilar invagination, CVJ fixation is recommended, and C2 laminectomy should be avoided. In the event of significant clinical deterioration due to nonadherence to these guidelines, our findings highlight the importance of traction with increased extension before fixation, even years after initial destabilizing surgery.
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(1) Background: Hemispherotomy is the generally accepted treatment for hemispheric drug-resistant epilepsy (DRE). Lateral or vertical approaches are performed according to the surgeon's preference. Multiple technical variations have been proposed since Delalande first described his vertical technique. We propose a sub-insular variation of the vertical parasagittal hemispherotomy (VPH) and describe our case series of patients operated on using this procedure. (2) Methods: Data from a continuous series of patients with hemispheric DRE who were operated on by the senior author (CR) using the modified sub-insular VPH technique were analyzed retrospectively. Pre-operative demographic and epilepsy characteristics, functional outcome, and surgical complications were extracted from medical charts. (3) Results: Twenty-five patients were operated on between August 2008 and August 2023; 23 have at least 3 months of follow-up. Of this group, 20 (86.9%) patients are seizure-free. Only two patients developed postoperative hydrocephalus (8.7%). All patients who were able to walk autonomously preoperatively and 20 (86.9%) of those with follow-up were able to walk without assistance. A total of 17 (74%) patients were able to perform adapted social activities at the latest follow-up. (4) Conclusions: Modified sub-insular VPH is a successful surgical technique for hemispheric DRE with seizure freedom rates similar to the largest series reported in the literature. Compared to other series, patients who were operated on with our modified technique had a lower rate of postoperative hydrocephalus and excellent long-term motor and cognitive outcomes.
RESUMEN
OBJECTIVE: Chiari malformation type I is typified by the downward herniation of the cerebellar tonsils through the foramen magnum, which can impede cerebrospinal fluid circulation and may lead to syringomyelia. The usual symptoms of this condition are neck pain and posterior headaches on Valsalva maneuver. Different surgical procedures have been described for cranio-cervical decompression (CCD), without a consensus being reached about the best suited technique. The primary end point of this study was to compare efficacy and complications rate of CCD using dural peeling (DPe) versus duraplasty (DP). The secondary end point was to find predictive factors of success of DPe. METHODS: Twenty-eight consecutive patients with Chiari malformation type I (12 women and 16 men) requiring CCD were enrolled at our institution between August 2011 and November 2015. Ten patients (35.7%) underwent DP, and 18 (64.3%) DPe. A standardized magnetic resonance imaging protocol was performed before and at least 3 months after surgery. Symptomatic outcome was evaluated at the last follow-up visit. RESULTS: Overall complications were more frequent in the DP (4 patients, 70%) group than in the DPe (none) group (P <0.05). All patients in the DP group improved clinically but only 12 patients (66.7%) in the DPe group (P = 0.1). Morphologic evolution at magnetic resonance imaging was similar in both groups. A moderate trend for changes in cerebellar tonsil conformation was shown in patients with clinical improvement (P = 0.07). Predictive factors of clinical improvement after DPe cannot be identified. CONCLUSIONS: CCD with DPe was less risky than with DP but had a lower responsive rate (66.7% vs. 100). Larger studies are therefore warranted to assess predictive factors of success of CCD with DPe.
