Nisha Gadgil, Sandi Lam, Monika Pyarali, Michael Paldino, I-Wen Pan and Robert C. Dauser
Numerous surgical procedures facilitate revascularization of the ischemic brain in patients with moyamoya disease. Dural inversion is a technique in which flaps of dura mater centered around the middle meningeal artery are inverted, encouraging the formation of a rich collateral blood supply. This procedure has been used in combination with encephaloduroarteriosynangiosis for more than 20 years at the authors’ institution for the treatment of pediatric moyamoya disease. The objective of this study was to describe the clinical and radiographic outcomes for a cohort of consecutive pediatric moyamoya patients undergoing dural inversion.
Clinical and radiographic data on patients who had undergone dural inversion in the period from 1997 to 2016 were reviewed. Univariate and multivariate logistic regression and Kaplan-Meier analyses were performed to assess the risk of postoperative stroke, functional outcome, and the angiographic degree of revascularization.
Dural inversion was performed on 169 hemispheres in 102 patients. Median follow-up was 4.3 years. Six patients (3.6% of hemispheres) suffered postoperative ischemic or hemorrhagic stroke. Overall mortality was 1.0%. Good postoperative neurological status (modified Rankin Scale [mRS] score ≤ 2) was observed in 90 patients (88%); preoperative and postoperative mRS scores showed significant improvement (p < 0.001). Eighty-six percent of hemispheres had Matsushima grade A or B revascularization. Younger age was associated with postoperative stroke and poor functional outcome. Patients with secondary moyamoya syndrome had a significantly worse radiographic outcome. The cumulative 5-year Kaplan-Meier risk for stroke was 6.4%.
Dural inversion is a useful technique of cerebral revascularization in pediatric moyamoya disease. A 20-year experience demonstrates the safety and efficacy of this technique with a relatively low rate of postoperative stroke, good functional outcomes, and favorable angiographic results.
Aditya Vedantam, Katie M. Stormes, Nisha Gadgil, Stephen F. Kralik, Guillermo Aldave and Sandi K. Lam
Resection of posterior fossa tumors in children may be associated with persistent neurological deficits. It is unclear if these neurological deficits are associated with persistent structural damage to the cerebellar pathways. The purpose of this research was to define longitudinal changes in diffusion tensor imaging (DTI) metrics in white matter cerebellar tracts and the clinical correlates of these metrics in children undergoing resection of posterior fossa tumors.
Longitudinal brain DTI was performed in a cohort of pediatric patients who underwent resection of posterior fossa tumors. Fractional anisotropy (FA) of the superior cerebellar peduncles (SCPs) and middle cerebellar peduncles (MCPs) was measured on preoperative, postoperative, and follow-up DTI. Early postoperative (< 48 hours) and longer-term follow-up neurological deficits (mutism, ataxia, and extraocular movement dysfunction) were documented. Statistical analysis was performed to determine differences in FA values based on presence or absence of neurological deficits. Statistical significance was set at p < 0.05.
Twenty children (mean age 6.1 ± 4.1 years [SD], 12 males and 8 females) were included in this study. Follow-up DTI was performed at a median duration of 14.3 months after surgery, and the median duration of follow-up was 19.7 months. FA of the left SCP was significantly reduced on postoperative DTI in comparison with preoperative DTI (0.44 ± 0.07 vs 0.53 ± 0.1, p = 0.003). Presence of ataxia at follow-up was associated with a persistent reduction in the left SCP FA on follow-up DTI (0.43 ± 0.1 vs 0.55 ± 0.1, p = 0.016). Patients with early postoperative mutism who did not recover at follow-up had significantly decreased FA of the left SCP on early postoperative DTI in comparison with those who recovered (0.38 ± 0.05 vs 0.48 ± 0.06, p = 0.04).
DTI after resection of posterior fossa tumors in children shows that persistent reduction of SCP FA is associated with ataxia at follow-up.
Sudhakar Vadivelu, Pablo Harker and Mario Zuccarello
Melissa A. LoPresti, Vijay M. Ravindra, Monika Pyarali, Eric Goethe, Nisha Gadgil, Kathyrn Wagner, Peter Kan and Sandi Lam
Intracranial arteriovenous malformations (AVMs) are a common cause of intracranial hemorrhage in children. Exploring outcomes of treatment and factors that predict recurrence may help guide the management of pediatric AVMs. In this study, the authors review a single-center experience with pediatric AVMs, aiming to identify factors contributing to recurrence and areas for improvement in treatment.
A single-center retrospective review of patients ages 0–18 years with AVMs in the period from 2005 to 2018 was conducted. Demographic, clinical, and radiographic data were collected and examined to compare the clinical and functional outcomes of patients managed with and those managed without open microsurgical resection.
One hundred five patients were diagnosed and treated during the study period. Fifty (47.6%) had been treated surgically and 35 (33.3%) nonsurgically, and 20 (19%) had been treated primarily at an outside institution and thus were excluded from further analysis. Of the 50 treated surgically, 92% had complete obliteration on postoperative imaging, with the remaining patients having residual AVM and undergoing re-resection or SRS, ultimately resulting in a 96% radiographic cure rate. The surgically treated patients were followed up for an average of 3.34 years. Eighty-two percent had a modified Rankin Scale (mRS) score of 0–2. There was a recurrence rate of 12%, with the time to recurrence averaging 327.86 days (range 85–596 days). Of the 35 patients managed without open surgery, 77% underwent serial imaging surveillance, 20% underwent endovascular embolization, and 3% underwent stereotactic radiosurgery. Radiographic cure was seen in 6% of those treated nonsurgically, and all had undergone endovascular treatment alone. The nonsurgically treated patients were followed up for an average of 2.14 years. Eighty percent had an mRS score of 0–2.
The data revealed that patients with lower-grade AVMs treated surgically and those with higher-grade AVMs managed without surgery have good functional and clinical outcomes. A high rate of recurrence was noted, and the possible contributing factors were explored. The authors encourage further study into mechanisms to reduce AVM recurrence in pediatric patients and look forward to advances in the medical management of lesions that are considered unresectable.