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Vijay M. Ravindra, Rajiv R. Iyer, Al-Wala Awad, Robert J. Bollo, Huirong Zhu and Douglas L. Brockmeyer

OBJECTIVE

The authors’ objective was to better understand the anatomical load-bearing relationship between the atlantooccipital joint and the upper cervical spine and its influence on the clinical behavior of patients with Chiari malformation type I (CM-I) and craniocervical pathology.

METHODS

In a single-center prospective study of patients younger than 18 years with CM-I from 2015 through 2017 (mean age 9.91 years), the authors measured the occipital condyle–C2 sagittal vertebral alignment (C–C2SVA; defined as the position of a plumb line from the midpoint of the occiput (C0)–C1 joint relative to the posterior aspect of the C2–3 disc space), the pB–C2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body on sagittal MRI), and the CXA (clivoaxial angle). Control data from 30 patients without CM-I (mean age 8.97 years) were used for comparison. The primary outcome was the need for anterior odontoid resection and/or occipitocervical fusion with or without odontoid reduction. The secondary outcome was the need for two or more Chiari-related operations.

RESULTS

Of the 60 consecutive patients with CM-I identified, 7 underwent anterior odontoid resection or occipitocervical fusion and 10 underwent ≥ 2 decompressive procedures. The mean C–C2SVA was greater in the overall CM-I group versus controls (3.68 vs 0.13 mm, p < 0.0001), as was the pB–C2 (7.7 vs 6.4 mm, p = 0.0092); the CXA was smaller (136° vs 148°, p < 0.0001). A C–C2SVA ≥ 5 mm was found in 35% of CM-I children and 3.3% of controls (p = 0.0006). The sensitivities and specificities for requiring ventral decompression/occipitocervical fusion were 100% and 74%, respectively, for C–C2SVA ≥ 5 mm; 71% and 94%, respectively, for CXA < 125°; and 71% and 75%, respectively, for pB–C2 ≥ 9 mm. The sensitivities and specificities for the need for ≥ 2 decompressive procedures were 60% and 70%, respectively, for C–C2SVA ≥ 5 mm; 50% and 94%, respectively, for CXA < 125°; and 60% and 76%, respectively, for pB–C2 ≥ 9 mm. The log-rank test demonstrated significant differences between C–C2SVA groups (p = 0.0007) for the primary outcome. A kappa value of 0.73 for C–C2SVA between raters indicated substantial agreement.

CONCLUSIONS

A novel screening measurement for craniocervical bony relationships, the C–C2SVA, is described. A significant difference in C–C2SVA between CM-I patients and controls was found. A C–C2SVA ≥ 5 mm is highly predictive of the need for occipitocervical fusion/ventral decompression in patients with CM-I. Further validation of this screening measurement is needed.

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Vijay M. Ravindra, Robert J. Bollo, Ilyas M. Eli, Julius Griauzde, Arianna Lanpher, Jennifer Klein, Huirong Zhu, Douglas L. Brockmeyer, John R. W. Kestle, William T. Couldwell, R. Michael Scott and Edward Smith

OBJECTIVE

Large experiences with the treatment of pediatric arteriovenous malformations (AVMs) remain relatively rare, with limited data on presentation, treatment, and long-term functional outcomes. Because of the expected long lifespan of children, caregivers are especially interested in outcome measures that assess quality of life. The authors’ intention was to describe the long-term functional outcomes of pediatric patients who undergo AVM surgery and to identify predictors of sustained neurological deficits.

METHODS

The authors analyzed a 21-year retrospective cohort of pediatric patients with intracranial AVMs treated with microsurgery at two institutions. The primary outcome was a persistent neurological deficit at last follow-up. Secondary outcome measures included modified Rankin Scale (mRS) score and independent living.

RESULTS

Overall, 97 patients (mean age 11.1 ± 4.5 years; 56% female) were treated surgically for intracranial AVMs (mean follow-up 77.5 months). Sixty-four patients (66%) presented with hemorrhage, and 45 patients (46%) had neurological deficits at presentation. Radiologically, 39% of lesions were Spetzler-Martin grade II. Thirty-seven patients (38%) with persistent neurological deficits at last follow-up were compared with those without deficits; there were no differences in patient age, presenting Glasgow Coma Scale score, AVM size, surgical blood loss, or duration of follow-up. Multivariate analysis demonstrated that a focal neurological deficit on presentation, AVM size > 3 cm, and lesions in eloquent cortex were independent predictors of persistent neurological deficits at long-term follow-up. Overall, 92% of the children had an mRS score ≤ 2 on long-term follow-up.

CONCLUSIONS

Pediatric patients with AVMs treated with microsurgical resection have good functional and radiological outcomes. There is a high rate (38%) of persistent neurological deficits, which were independently predicted by preoperative deficits, AVMs > 3 cm, and lesions located in eloquent cortex. This information can be useful in counseling families on the likelihood of long-term neurological deficits after cerebral AVM surgery.