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Abstracts for the 2014 Annual Meeting of The American Society of Pediatric Neurosurgeons

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A comparison of costs associated with endoscope-assisted craniectomy versus open cranial vault repair for infants with sagittal synostosis

Clinical article

Timothy W. Vogel, Albert S. Woo, Alex A. Kane, Kamlesh B. Patel, Sybill D. Naidoo, and Matthew D. Smyth

Object

The surgical management of infants with sagittal synostosis has traditionally relied on open cranial vault remodeling (CVR) techniques; however, minimally invasive technologies, including endoscope-assisted craniectomy (EAC) repair followed by helmet therapy (HT, EAC+HT), is increasingly used to treat various forms of craniosynostosis during the 1st year of life. In this study the authors determined the costs associated with EAC+HT in comparison with those for CVR.

Methods

The authors performed a retrospective case-control analysis of 21 children who had undergone CVR and 21 who had undergone EAC+HT. Eligibility criteria included an age less than 1 year and at least 1 year of clinical follow-up data. Financial and clinical records were reviewed for data related to length of hospital stay and transfusion rates as well as costs associated with physician, hospital, and outpatient clinic visits.

Results

The average age of patients who underwent CVR was 6.8 months compared with 3.1 months for those who underwent EAC+HT. Patients who underwent EAC+HT most often required the use of 2 helmets (76.5%), infrequently required a third helmet (13.3%), and averaged 1.8 clinic visits in the first 90 days after surgery. Endoscope-assisted craniectomy plus HT was associated with shorter hospital stays (mean 1.10 vs 4.67 days for CVR, p < 0.0001), a decreased rate of blood transfusions (9.5% vs 100% for CVR, p < 0.0001), and a decreased operative time (81.1 vs 165.8 minutes for CVR, p < 0.0001). The overall cost of EAC+HT, accounting for hospital charges, professional and helmet fees, and clinic visits, was also lower than that of CVR ($37,255.99 vs $56,990.46, respectively, p < 0.0001).

Conclusions

Endoscope-assisted craniectomy plus HT is a less costly surgical option for patients than CVR. In addition, EAC+HT was associated with a lower utilization of perioperative resources. Theses findings suggest that EAC+HT for infants with sagittal synostosis may be a cost-effective first-line surgical option.

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Gamma Knife surgery for a hemangioma of the cavernous sinus in a child

Case report

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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Association of magnetic resonance imaging identification of mesial temporal sclerosis with pathological diagnosis and surgical outcomes in children following epilepsy surgery

Clinical article

Aimen Kasasbeh, Edward C. Hwang, Karen Steger-May, S. Kathleen Bandt, Amy Oberhelman, David Limbrick, Michelle M. Miller-Thomas, Joshua S. Shimony, and Matthew D. Smyth

Object

Mesial temporal sclerosis (MTS) is widely recognized as a significant underlying cause of temporal lobe epilepsy. Magnetic resonance imaging is routinely used in the preoperative evaluation of children with epilepsy. The purpose of this study was to evaluate the prevalence, reliability, and prognostic value of MRI identification of MTS and MRI findings indicative of MTS in a series of patients who underwent resection of the medial temporal lobe for medically refractory epilepsy.

Methods

The authors reviewed the medical records and preoperative MRI reports of 25 patients who had undergone medial temporal resections (anterior temporal lobectomy or functional hemispherotomy) for medically intractable epilepsy. The preoperative MRI studies were presented for blinded review by 2 neuroradiologists who independently evaluated the radiographs for selected MTS features and provided a final interpretation. To quantify interrater agreement and accuracy, the findings of the 2 blinded neuroradiologists, the nonblinded clinical preoperative radiology report, and the final pathology interpretation were compared.

Results

The preoperative MRI studies revealed MTS in 6 patients (24%), and histopathological analysis verified MTS in 8 (32%) of 25 specimens. Six MRI features of MTS were specifically evaluated: 1) increased hippocampal signal intensity, 2) reduced hippocampal size, 3) atrophy of the ipsilateral hippocampal collateral white matter, 4) enlarged ipsilateral temporal horn, 5) reduced gray-white matter demarcation in the temporal lobe, and 6) decreased temporal lobe size. The most prevalent feature of MTS identified on MRI was a reduced hippocampal size, found in 11 of the MRI studies (44%). Analysis revealed moderate interrater agreement for MRI identification of MTS between the 2 blinded neuroradiologists and the nonblinded preoperative report (Cohen κ 0.40–0.59). Interrater agreement was highly variable for different MTS features indicative of MTS, ranging from poor to near perfect. Agreement was highest for increased hippocampal signal and decreased temporal lobe size and was consistently poor for reduced gray-white matter demarcation. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and proportion perfect agreement were highest for increased hippocampal signal and reduced hippocampal size. An MRI finding of MTS was not predictive of seizure outcome in this small series.

Conclusions

Mesial temporal sclerosis identification on brain MRI in children evaluated for medial temporal resections has a PPV of 55%–67% and an NPV of 79%–87%. Increased hippocampal signal and reduced hippocampal size were associated with high predictive values, while gray-white differentiation and an enlarged temporal horn were not predictive of MTS. Seizure outcome following medial temporal resections was not associated with MRI findings of MTS or MRI abnormalities indicative of MTS in this small sample size.

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Editorial: Hemispherotomy

James T. Rutka

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Palliative hemispherotomy in children with bilateral seizure onset

Clinical article

Michael Anthony Ciliberto, David Limbrick, Alexander Powers, Jeffrey B. Titus, Rebecca Munro, and Matthew D. Smyth

Object

Intractable epilepsy is a significant burden on families and on the cognitive development and quality of life (QOL) of patients. Periinsular hemispherotomy (PIH) for medically intractable epilepsy can benefit patients who qualify for this procedure. The ideal hemispherotomy candidate has ipsilateral ictal and interictal epileptiform activity, unilateral MR imaging abnormalities, contralateral hemiplegia, and a normal contralateral hemisphere. However, certain patients present with a mixed picture of bilateral electroencephalography (EEG) findings and severe intractable epilepsy, prompting consideration of a more aggressive treatment approach. This report introduces the possibility of surgery for patients who normally would not meet criteria for this treatment modality.

Methods

In this retrospective chart review, the authors report on 7 patients with bilateral seizure onset noted on routine or video-EEG monitoring. A QOL phone questionnaire, based on the Quality of Life in Childhood Epilepsy tool, was administered to a parent. The authors reviewed each patient's chart for surgical complications, changes in examination, QOL, limited neuropsychological outcomes, and seizure outcomes. They also investigated each chart for MR imaging and EEG findings as well as the patient's epilepsy clinic notes for seizure semiology and frequency.

Results

All patients enjoyed a decrease in seizure frequency and a subjective increase in QOL after PIH. Five patients (71%) achieved Engel Class I or II seizure control. The mean follow-up was 3.64 years (2–5.3 years). One patient is now off all antiseizure medication. No patient had a decrement in Full Scale IQ on postsurgical testing, and 2 (28.5%) of 7 individuals had increased adaptive and social functioning. Postsurgical examination changes included hemiplegia and homonymous hemianopia.

Conclusions

Hemispherotomy in patients with intractable epilepsy is generally reserved for individuals with unilateral epileptiform abnormalities or lesions on MR imaging. Seven patients in this study benefited from surgery despite bilateral seizure onset with improvement in seizure control and overall QOL. Thus, bilateral ictal onset does not necessarily preclude consideration for hemispherotomy in selected patients with severe medically refractory epilepsy.

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Intraoperative magnetic resonance imaging to reduce the rate of early reoperation for lesion resection in pediatric neurosurgery

Clinical article

Manish N. Shah, Jeffrey R. Leonard, Gabrielle Inder, Feng Gao, Michael Geske, Devon H. Haydon, Melvin E. Omodon, John Evans, Diego Morales, Ralph G. Dacey, Matthew D. Smyth, Michael R. Chicoine, and David D. Limbrick

Object

This study describes the pediatric experience with a dual-multifunction-room IMRIS 1.5-T intraoperative magnetic resonance imaging (iMRI) suite and analyzes its impact on clinical variables associated with neurosurgical resection of intracranial lesions, including safety and efficacy.

Methods

Since the inception of the iMRI–guided resection program in April 2008 at both Barnes-Jewish and St. Louis Children's Hospital, a prospective database recorded the clinical variables associated with demographics and outcome with institutional review board approval. A similarly approved retrospective database was constructed from February 2006 to March 2010 for non–iMRI resections. These databases were retrospectively reviewed for clinical variables associated with resection of pediatric (age 20 months–21 years) intracranial lesions including brain tumors and focal cortical dysplasia. Patient demographics, operative time, estimated blood loss, additional resection, length of stay, pathology, and complications were analyzed.

Results

The authors found that 42 iMRI–guided resections were performed, whereas 103 conventional resections had been performed without the iMRI. The mean patient age was 10.5 years (range 20 months–20 years) in the iMRI group and 9.8 years (range 2–21 years) in the conventional group (p = 0.41). The mean duration of surgery was 350 minutes in the iMRI group and 243 minutes in the conventional group (p < 0.0001). The mean hospital stay was 8.2 days in the iMRI group, and 6.6 days in the conventional group, and this trended toward significance (p = 0.05). In the first 2 weeks postoperatively, there were 8 reoperations (7.77%) in the conventional group compared with none in the iMRI group, which was not significant in a 2-tailed test (p = 0.11) but trended toward significance in a 1-tailed test (p = 0.06). The significant complications included reoperation for hydrocephalus or infection: 6.8% (conventional) versus 4.8% (iMRI).

Conclusions

Intraoperative MR imaging–guided resections resulted in a trend toward reduction in the need for repeat surgery in the immediate 2-week postoperative period compared with conventional pediatric neurosurgical resections for tumor or focal cortical dysplasia. Although there is an increased operative time, the iMRI suite offers a comparable safety and efficacy profile while potentially reducing the per-case cost by diminishing the need for early reoperation.

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Endoscopically assisted versus open repair of sagittal craniosynostosis: the St. Louis Children's Hospital experience

Clinical article

Manish N. Shah, Alex A. Kane, J. Dayne Petersen, Albert S. Woo, Sybill D. Naidoo, and Matthew D. Smyth

Object

This study investigated the differences in effectiveness and morbidity between endoscopically assisted wide-vertex strip craniectomy with barrel-stave osteotomies and postoperative helmet therapy versus open calvarial vault reconstruction without helmet therapy for sagittal craniosynostosis.

Methods

Between 2003 and 2010, the authors prospectively observed 89 children less than 12 months old who were surgically treated for a diagnosis of isolated sagittal synostosis. The endoscopic procedure was offered starting in 2006. The data associated with length of stay, blood loss, transfusion rates, operating times, and cephalic indices were reviewed.

Results

There were 47 endoscopically treated patients with a mean age at surgery of 3.6 months and 42 patients with open-vault reconstruction whose mean age at surgery was 6.8 months. The mean follow-up time was 13 months for endoscopic versus 25 months for open procedures. The mean operating time for the endoscopic procedure was 88 minutes, versus 179 minutes for the open surgery. The mean blood loss was 29 ml for endoscopic versus 218 ml for open procedures. Three endoscopically treated cases (6.4%) underwent transfusion, whereas all patients with open procedures underwent transfusion, with a mean of 1.6 transfusions per patient. The mean length of stay was 1.2 days for endoscopic and 3.9 days for open procedures. Of endoscopically treated patients completing helmet therapy, the mean duration for helmet therapy was 8.7 months. The mean pre- and postoperative cephalic indices for endoscopic procedures were 68% and 76% at 13 months postoperatively, versus 68% and 77% at 25 months postoperatively for open surgery.

Conclusions

Endoscopically assisted strip craniectomy offers a safe and effective treatment for sagittal craniosynostosis that is comparable in outcome to calvarial vault reconstruction, with no increase in morbidity and a shorter length of stay.

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Preventing cerebrospinal fluid leak following transection of a tight filum terminale

Clinical article

Joshua J. Chern, R. Shane Tubbs, Akash J. Patel, Amber S. Gordon, S. Kathleen Bandt, Matthew D. Smyth, Andrew Jea, and W. Jerry Oakes

Object

Tethered cord release for a tight filum terminale is a common pediatric operation associated with low morbidity and mortality rates. While almost all would agree that keeping patients lying flat after the operation will prevent a CSF leak, the optimal period of doing so has not been determined. In this study, the authors examined whether a longer length of stay in the hospital for the sole purpose of maintaining patients flat correlates with a decreased rate of CSF leakage.

Methods

Intraoperative and postoperative data were retrospectively collected in 222 cases of simple tethered cord release at 3 large children's hospitals. Risk factors for postoperative CSF leakage were identified.

Results

Thirty-eight patients were maintained lying flat for 24 hours, 86 for 48 hours, and 98 for 72 hours at the individual surgeon's discretion. A CSF leak occurred in 13 patients (5.9%) and pseudomeningocele developed in 9 patients (4.1%). In the univariate analysis, operating time, use of the microscope, use of dural sealant, and duration of remaining flat after surgery failed to correlate with the occurrence of complications.

Conclusions

A longer hospital stay for maintaining patients flat after a simple tethered cord release appears not to prevent CSF leakage. However, a larger patient cohort will be needed to detect small differences in complication rates.

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Patients with Chiari malformation Type I presenting with acute neurological deficits: case series

Clinical article

Chester K. Yarbrough, Alexander K. Powers, Tae Sung Park, Jeffrey R. Leonard, David D. Limbrick, and Matthew D. Smyth

Object

A subset of patients with Chiari malformation Type I (CM-I) presented with acute onset of a neurological deficit. In this study the authors summarize their experience with these patients' clinical presentation, imaging results, timing of surgery, and outcome following decompression.

Methods

The authors reviewed clinical records, imaging studies, and operative notes from all patients undergoing posterior fossa decompression for CM-I at St. Louis Children's Hospital from 1990 to 2008. Of the 189 patients who underwent surgery, 6 were identified with the acute onset of a neurological deficit at presentation.

Results

All 6 children (age range 3–14 years, 3 boys and 3 girls) had either syringomyelia (5 patients) or T2 signal changes in the spinal cord (1 patient) and CM-I on initial MR imaging. Three patients presented after minor trauma (1 with paraparesis, 2 with sensory deficits). Three patients presented without a clear history of trauma (1 with abrupt onset of spontaneous dysphagia and ataxia, 2 with sensory deficits). Decompression was performed at a mean 7.7 ± 4.9 days after symptom onset (7.0 ± 1.6 days after neurosurgical evaluation). In 1 patient, symptoms had resolved by the time of surgery; in the remainder of the patients, clear improvements were noted within 2 weeks of surgery, with complete resolution of symptoms by 12 months postoperatively. Follow-up MR images were obtained in 4 patients, demonstrating improvement in the extent of the syrinx in each patient.

Conclusions

Children with CM-I and syringomyelia can develop acute spinal cord or bulbar deficits with relatively minor head or neck injuries. The prognosis for symptomatic improvement in the observed deficit is good, with each patient in our series showing resolution of deficits over time. However, based on this relatively limited experience, the authors suggest that patients who present with an acute neurological deficit and are found to have CM-I be managed with early posterior fossa decompression. Patients with CM-I and syringomyelia may be at higher risk of acute neurological deficit than those without a syrinx.