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Spiros l. Blackburn and Matthew D. Smyth

✓The use of an absorbable hydrogel dural sealant has been approved for neurosurgical applications with no published reports of complications to date. The authors present the case of a 13-year-old girl with syringomyelia and quadriparesis who underwent posterior fossa decompression and dural augmentation for Chiari malformation Type I. Dural closure was performed with a dural substitute patch, hydrogel dural sealant, and gelatin sponge. Magnetic resonance imaging, performed after initial postoperative improvement in the patient's quadriparesis deteriorated, demonstrated an expanding epidural mass collection causing cervicomedullary compression. Exploration on postoperative Day 15 revealed an expanded layer of hydrogel underlying a layer of gelatin sponge. The authors conclude that hydrogel dural sealants should be used cautiously in spaces that cannot tolerate significant mass effect.

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Matthew R. Reynolds, Spiros L. Blackburn and Matthew D. Smyth

The authors present the case of a 3-year-old child with Kleeblattschädel, or cloverleaf skull deformity, and a Chiari malformation Type I who developed an ossified pseudomeningocele after posterior fossa decompression. To their knowledge, this is the first report of a postoperative ossified pseudomeningocele in a patient with Kleeblattschädel and the only case of an ossified pseudomeningocele located outside the lumbosacral region. A genetic basis for the ossification process seems likely given the child's history of premature cranial suture closure. The authors draw attention to this rare complication and review the available body of literature on this topic.

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Thomas L. Beaumont, Jakub Godzik, Sonika Dahiya and Matthew D. Smyth

The authors report the case of a 14-year-old male with a subependymal giant cell astrocytoma (SEGA) that occurred in the absence of tuberous sclerosis complex (TSC). The patient presented with progressive headache and the sudden onset of nausea and vomiting. Neuroimaging revealed an enhancing left ventricular mass located in the region of the foramen of Monro with significant mass effect and midline shift. The lesion had radiographic characteristics of SEGA; however, the diagnosis remained unclear given the absence of clinical features of TSC. The patient underwent gross-total resection of the tumor with resolution of his symptoms. Although tumor histology was consistent with SEGA, genetic analysis of both germline and tumor DNA revealed no TSC1/2 mutations. Similarly, a comprehensive clinical evaluation failed to reveal any clinical features characteristic of TSC. Few cases of SEGA without clinical or genetic evidence of TSC have been reported. The histogenesis, genetics, and clinical approach to this rare lesion are briefly reviewed.

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M. Mohsin Shah, Matthew D. Smyth and Albert S. Woo

✓The authors present a case of scalp and facial edema following craniofacial reconstruction for metopic craniosynostosis in which recombinant human bone morphogenetic protein–2 (rhBMP-2) was used to treat cranial defects related to the frontoorbital reconstruction. The extent of swelling, the onset, and duration were unusual for such cases and suggested a possible role of rhBMP-2 in inducing a local inflammatory response. The edema rapidly resolved after the patient underwent surgery to remove the rhBMP-2 implants.

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Daniel Refai, Richard J. Perrin and Matthew D. Smyth

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Jeffrey H. Miller, David D. Limbrick Jr., Matthew Callen and Matthew D. Smyth

The spontaneous resolution of isolated tonsillar ectopia in Chiari malformation Type I (CM-I) is a known and reported entity in 2 previous single study case reports. However, it has not been previously described in monozygotic twins. Two children, ~ 1 year of age with CM-I and presumed episodes of pallid syncope or breath-holding spells presented for neurosurgical evaluation. Although Chiari decompression was considered, the authors decided to proceed with conservative management with close follow-up due to the uncertain nature of these episodes. Approximately 4 years later, both children's symptoms had resolved. Repeated MR imaging examinations also showed spontaneous resolution of the malformation in both girls. These cases emphasize that when patients with CM-I present with atypical symptoms, spontaneous resolution or improvement is possible, which may influence the decision to pursue a trial of nonsurgical management. The possible pathophysiological mechanisms and genetic influences of CM-I are also briefly discussed.

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Rory K. J. Murphy, Matthew R. Reynolds, David B. Mansur and Matthew D. Smyth

Cavernous sinus (CS) hemangiomas are rare vascular abnormalities that constitute 0.4%–2% of all lesions within the CS. Cavernous sinus hemangiomas are high-flow vascular tumors that tend to hemorrhage profusely during resection, leading to incomplete resection and high morbidity and mortality. While Gamma Knife surgery (GKS) has proven to be an effective treatment of CS hemangiomas in the adult population, few reports of GKS for treatment of CS hemangiomas exist in the pediatric literature. Here, the authors present the first case of a 15-year-old girl with a biopsy-proven CS hemangioma who achieved complete resolution of her symptoms and a complete imaging-defined response following GKS. If suspicion for a CS hemangioma is high in a pediatric patient, GKS may be considered as an effective treatment modality, thus avoiding the morbidities of open resection.

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Anna Jeong, Jennifer Strahle, Ananth K. Vellimana, David D. Limbrick Jr., Matthew D. Smyth and Mary Bertrand


Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment.


During the period between 2003 and 2015, 80 patients underwent hemispherotomy at the authors' institution. Video electroencephalography (EEG) reports were reviewed for ESES or continuous spikes and waves during sleep (CSWS). Patients with preoperative ESES (> 85% slow-wave sleep occupied by spike waves), a unilateral structural lesion amenable to surgery, and more than 6 months of follow-up data were included in the analysis. Clinical data, EEG recordings, neuropsychological testing, and parental and clinician reports were retrospectively reviewed.


Nine patients were eligible for study inclusion. Age at seizure onset ranged from birth to 4.2 years (mean 1.9 years), age at ESES diagnosis ranged from 3.5 to 8.8 years (mean 6.0 years), and age at hemispherotomy ranged from 3.7 to 11.5 years (mean 6.8 years). All patients had drug-resistant epilepsy. The duration of epilepsy prior to hemispherotomy ranged from 2.7 to 8.9 years (mean ± SD, 5.0 ± 2.2 years). Engel Class I seizure outcome was observed in all 9 children, with a mean follow-up of 3.0 years (range 0.5–6.1 years). Hemispherotomy terminated ESES in 6 of 6 patients with available postoperative sleep EEG. All children had preoperative neuropsychological impairments. Developmental regression was halted postoperatively, but none of the children returned to their original pre-ESES baseline. Four children demonstrated academic gains, 2 of whom transitioned to mainstream classes.


Children with drug-resistant ESES and a unilateral structural lesion should be evaluated for hemispherotomy as they may experience the cessation of seizures, termination of ESES, and improvement in neuropsychological status.