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Robert T. Buckley, Tiffany Morgan, Russell P. Saneto, Jason Barber, Richard G. Ellenbogen and Jeffrey G. Ojemann

Object

Functional hemispherectomy is a well-recognized surgical option for the treatment of unihemispheric medically intractable epilepsy. While the resultant motor deficits are a well-known and expected consequence of the procedure, the impact on other cortical functions has been less well defined. As the cortical control of swallowing would appear to be threatened after hemispherectomy, the authors retrospectively studied a pediatric population that underwent functional hemispherectomy for medically intractable epilepsy to characterize the incidence and severity of dysphagia after surgery.

Methods

A retrospective cohort (n = 39) of pediatric patients who underwent hemispherectomy at a single institution was identified, and available clinical records were reviewed. Additionally, the authors examined available MR images for integrity of the thalamus and basal ganglia before and after hemispherectomy. Clinical and video fluoroscopic assessments of speech pathology were reviewed, and the presence, type, and duration of pre- and postoperative dysphagia were recorded.

Results

New-onset, transient dysphagia occurred in 26% of patients after hemispherectomy along with worsening of preexisting dysphagia noted in an additional 15%. Clinical symptoms lasted a median of 19 days. Increased duration of symptoms was seen with late (> 14 days postoperative) pharyngeal swallow dysfunction when compared with oral dysphagia alone. Neonatal stroke as a cause for seizures decreased the likelihood of postoperative dysphagia. There was no association with seizure freedom or postoperative hydrocephalus.

Conclusions

New-onset dysphagia is a frequent and clinically significant consequence of hemispherectomy for intractable epilepsy in pediatric patients. This dysphagia was always self-limited except in those patients in whom preexisting dysphagia was noted.

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Isaac Josh Abecassis, John D. Nerva, Jason Barber, Jason Rockhill, Richard G. Ellenbogen, Louis J. Kim and Laligam N. Sekhar

OBJECTIVE

Brain arteriovenous malformations (bAVMs) are rare in pediatric patients but represent the most common cause of hemorrhagic stroke in this population. Pediatric patients demonstrate superior outcomes in comparison with adult patients with similar lesions and presentations. Most studies of clinical outcomes of pediatric bAVMs use the modified Rankin Scale (mRS), despite a lack of validation in pediatric patients.

METHODS

The authors interviewed the parents of 26 pediatric patients who underwent multimodality bAVM treatment and administered the Pediatric Quality of Life Inventory (PedsQL)—a well-validated tool for pediatric outcomes that quantifies performance in a physical, emotional, social, and school domains. They also reviewed clinical information from the patients' medical charts. Statistical analysis was performed using a log-transformed t-test, the Mann-Whitney exact test, the Kruskal-Wallis test, and Spearman correlation. In addition, the literature was reviewed for prior reports of clinical outcome of pediatric cases of bAVM.

RESULTS

The average PedsQL health-related quality of life score was 71 ± 24, with an average age at diagnosis of 12.5 years and an average follow-up period of 6.8 years. Seventeen patients (65%) presented with hemorrhage and 4 (15%) with seizures. PedsQL scores correlated strongly and at a statistically significant level (p < 0.001) with mRS, Pediatric Overall Performance Category (POPC), Pediatric Cerebral Performance Category (PCPC), and Glasgow Outcome Scale scores. Multivariate modeling validated special education, corrective devices, and cure status as significant predictors of PedsQL scores. Statistically significant risk factors for undergoing placement of a ventriculoperitoneal shunt included lower Glasgow Coma Scale motor scores on admission (p = 0.042), cerebellar location (p = 0.046), and nidus volume (p = 0.017). Neither treatment modality nor location statistically affected clinical outcomes at follow-up.

CONCLUSIONS

There have been few studies of long-term clinical outcomes of bAVM in pediatric patients, and previously published studies have used conventional metrics that have been validated in the adult population, such as the mRS. Although these metrics can serve as reasonable surrogates, an accurate understanding of overall health-related quality of life is contingent on utilizing validated toolsets, such as the PedsQL.