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Matthew D. Smyth, David D. Limbrick Jr., Jeffrey G. Ojemann, John Zempel, Shenandoah Robinson, Donncha F. O'Brien, Russell P. Saneto, Monisha Goyal, Richard E. Appleton, Francesco T. Mangano, and Tae Sung Park

Object

The authors conducted a multiinstitutional, retrospective analysis to better define outcome and prognostic indicators for temporal lobe epilepsy surgery for suspected mesial temporal sclerosis (MTS) in young children.

Methods

Data were collected for all children undergoing temporal resections at four epilepsy centers over approximately 10 years. Children with a histopathological diagnosis of neoplasm were excluded.

Forty-nine patients (28 boys and 21 girls) were included in the study. Their mean age at surgery was 9.1 years (range 1.25–13.9 years). The mean age at seizure onset was 3.2 years (range birth–10 years). Histopathological examination demonstrated MTS in 26 cases, gliosis in nine, dysplasia in five, gliosis with dysplasia in four, and nonspecific or normal findings in five. Forty-one anterior temporal lobectomies (nine tailored) and eight selective amygdalohippocampectomies were performed (28 left side, 21 right side). Twenty-nine children (59.2%) underwent invasive monitoring. Operative complications included extraaxial hematomas (two cases), cerebrospinal fluid leaks (two cases), and hydrocephalus (one case), each in children undergoing invasive monitoring. The mean duration of follow up was 26.4 months (range 5–74 months) overall and 23.9 months (range 6–74 months) for the Engel Class I subgroup. Outcomes at the most recent follow-up examination were categorized as Engel Class I–II in 31 (63.3%) of 49 children overall, 20 (76.9%) of 26 children with confirmed MTS, four (36.4%) of 11 children with gliosis, and four (57.1%) of seven children with dysplasia. All patients who underwent selective amygdalohippocampectomies had confirmed MTS and Engel Class I outcomes. Patients with more than one seizure type (p = 0.048) or moderate to severe developmental delay (p = 0.03) had significantly worse outcomes (Engel Class III or IV). Age at seizure onset, age at surgery, and duration of seizure disorder were not significantly related to outcome. There was a trend for bilateral or extratemporal findings on electroencephalography (EEG) (p = 0.157), high preoperative seizure frequency (p = 0.097), and magnetic resonance (MR) imaging findings inconsistent with MTS (p = 0.142) to be associated with worse outcome, although it did not reach statistical significance. In only 12 (46.1%) of the 26 patients with confirmed MTS was the condition prospectively diagnosed on preoperative MR imaging.

Conclusions

Younger children with temporal lobe epilepsy have satisfying surgical outcomes, particularly when MTS is present. Magnetic resonance imaging may not be as sensitive in detecting MTS in children as in older patients. Negative predictors identified include multiple seizure types and preoperative developmental delay. Multifocal or bilateral EEG findings, high preoperative seizure frequency, and MR imaging findings inconsistent with MTS also independently suggested worse outcome.

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James M. Johnston, David D. Limbrick Jr., Wilson Z. Ray, Stephanie Brown, Joshua Shimony, and Tae Sung Park

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.

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David D. Limbrick Jr., Prithvi Narayan, Alexander K. Powers, Jeffrey G. Ojemann, Tae Sung Park, Mary Bertrand, and Matthew D. Smyth

Object

Hemispherotomy generally is performed in hemiparetic patients with severe, intractable epilepsy arising from one cerebral hemisphere. In this study, the authors evaluate the efficacy of hemispherotomy and present an analysis of the factors influencing seizure recurrence following the operation.

Methods

The authors performed a retrospective review of 49 patients (ages 0.2–20.5 years) who underwent functional hemispherotomy at their institution. The first 14 cases were traditional functional hemispherotomies, and included temporal lobectomy, while the latter 35 were performed using a modified periinsular technique that the authors adopted in 2003.

Results

Thirty-eight of the 49 patients (77.6%) were seizure free at the termination of the study (mean follow-up 28.6 months). Of the 11 patients who were not seizure free, all had significant improvement in seizure frequency, with 6 patients (12.2%) achieving Engel Class II outcome and 5 patients (10.2%) achieving Engel Class III. There were no cases of Engel Class IV outcome. The effect of hemispherotomy was durable over time with no significant change in Engel class over the postoperative follow-up period. There was no statistical difference in outcome between surgery types. Analysis of factors contributing to seizure recurrence after hemispherotomy revealed no statistically significant predictors of treatment failure, although bilateral electrographic abnormalities on the preoperative electroencephalogram demonstrated a trend toward a worse outcome.

Conclusions

In the present study, hemispherotomy resulted in freedom from seizures in nearly 78% of patients; worthwhile improvement was demonstrated in all patients. The seizure reduction observed after hemispherotomy was durable over time, with only rare late failure. Bilateral electrographic abnormalities may be predictive of posthemispherotomy recurrent seizures.

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Editorial

Posthemorrhagic ventricular dilation

Joseph H. Piatt

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David D. Limbrick Jr., Amit Mathur, James M. Johnston, Rebecca Munro, James Sagar, Terrie Inder, Tae Sung Park, Jeffrey L. Leonard, and Matthew D. Smyth

Object

Intraventricular hemorrhage (IVH) and progressive posthemorrhagic ventricular dilation (PPHVD) may result in significant neurological morbidity in preterm infants. At present, there is no consensus regarding the optimal timing or type of neurosurgical procedure to best treat PPHVD. Conflicting data exist regarding the relative risks and benefits of two commonly used temporizing neurosurgical procedures (TNPs), ventricular access devices ([VADs] or ventricular reservoirs) versus ventriculosubgaleal (VSG) shunts. This study was designed to address this issue.

Methods

This is a single-center, 10-year retrospective review of all preterm infants admitted to the St. Louis Children's Hospital neonatal intensive care unit (NICU) with Papile Grade III–IV IVH. The development of PPHVD and the requirement for and type of TNP were recorded. Rates of TNP complication, ventriculoperitoneal (VP) shunt implantation, shunt infection, and mortality rates were used to compare the efficacy and limitations of each TNP type.

Results

Over this 10-year interval, 325 preterm infants with Grade III–IV IVH were identified, with trends showing an increasing number of affected infants annually, and an increasing number of TNPs were required annually. Ninety-five (29.2%) of the 325 infants underwent a TNP for PPHVD (65 VADs, 30 VSG shunts). The rate of permanent VP shunt implantation for all TNPs was 72.6% (69 of 95 infants). Forty-nine (75.4%) of the 65 infants treated with VADs and 20 (66.7%) of the 30 treated with VSG shunts required VP shunts (p = 0.38). There was no statistical difference between VAD or VSG shunt with regard to TNP-related infection (p = 0.57), need for TNP revision (p = 0.16), subsequent shunt infection (p = 0.77), shunt revision rate (p = 0.58), or mortality rate (p = 0.24).

Conclusions

Rates of IVH and PPHVD observed at the authors' center have increased over time. In contrast to recent literature, the results from the current study did not demonstrate a difference in complication rate or requirement for permanent VP shunt placement between VADs and VSG shunts. Definitive conclusions will require a larger, prospective trial.

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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.

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Jakub Godzik, Michael P. Kelly, Alireza Radmanesh, David Kim, Terrence F. Holekamp, Matthew D. Smyth, Lawrence G. Lenke, Joshua S. Shimony, Tae Sung Park, Jeffrey Leonard, and David D. Limbrick

Object

Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I–associated syringomyelia. A secondary objective was to explore the role of craniovertebral junction (CVJ) characteristics as additional risk factors for scoliosis.

Methods

The authors conducted a retrospective review of pediatric patients evaluated for CM-I with syringomyelia at a single institution in the period from 2000 to 2012. Syrinx morphology and CVJ parameters were evaluated with MRI, whereas the presence of scoliosis was determined using standard radiographic criteria. Multiple logistic regression was used to analyze radiological features that were independently associated with scoliosis.

Results

Ninety-two patients with CM-I and syringomyelia were identified. The mean age was 10.5 ± 5 years. Thirty-five (38%) of 92 patients had spine deformity; 23 (66%) of these 35 patients were referred primarily for deformity, and 12 (34%) were diagnosed with deformity during workup for other symptoms. Multiple regression analysis revealed maximum syrinx diameter > 6 mm (OR 12.1, 95% CI 3.63–40.57, p < 0.001) and moderate (5–12 mm) rather than severe (> 12 mm) tonsillar herniation (OR 7.64, 95% CI 2.3–25.31, p = 0.001) as significant predictors of spine deformity when controlling for age, sex, and syrinx location.

Conclusions

The current study further elucidates the association between CM-I and spinal deformity by defining specific radiographic characteristics associated with the presence of scoliosis. Specifically, patients presenting with larger maximum syrinx diameters (> 6 mm) have an increased risk of scoliosis.

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Chester K. Yarbrough, Jacob K. Greenberg, Matthew D. Smyth, Jeffrey R. Leonard, Tae Sung Park, and David D. Limbrick Jr.

Object

Historically, assessment of clinical outcomes following surgical management of Chiari malformation Type I (CM-I) has been challenging due to the lack of a validated instrument for widespread use. The Chicago Chiari Outcome Scale (CCOS) is a novel system intended to provide a less subjective evaluation of outcomes for patients with CM-I. The goal of this study was to externally validate the performance of the CCOS.

Methods

Patients undergoing surgery for CM-I between 2001 and 2012 were reviewed (n = 292). Inclusion criteria for this study were as follows: 1) patients receiving primary posterior fossa decompression; 2) at least 5.5 months of postoperative clinical follow-up; and 3) patients ≤ 18 years of age at the time of surgery. Outcomes were evaluated using the CCOS, along with a “gestalt” impression of whether patients experienced significant improvement after surgery. A subgroup of 118 consecutive patients undergoing operations between 2008 and 2010 was selected for analysis of interrater reliability (n = 73 meeting inclusion/exclusion criteria). In this subgroup, gestalt and CCOS scores were independently determined by 2 reviewers, and interrater reliability was assessed using the intraclass correlation coefficient (ICC) and kappa (κ) statistic.

Results

The median CCOS score was 14, and 67% of patients had improved gestalt scores after surgery. Overall, the CCOS was effective at identifying patients with improved outcome after surgery (area under curve = 0.951). The interrater reliability of the CCOS (ICC = 0.71) was high, although the reliability of the component scores ranged from poor to good (ICC 0.23–0.89). The functionality subscore demonstrated a low ICC and did not add to the predictive ability of the logistic regression model (likelihood ratio = 1.8, p = 0.18). When analyzing gestalt outcome, there was moderate agreement between raters (κ = 0.56).

Conclusions

In this external validation study, the CCOS was effective at identifying patients with improved outcomes and proved more reliable than the authors' gestalt impression of outcome. However, certain component subscores (functionality and nonpain symptoms) were found to be less reliable, and may benefit from further definition in score assignment. In particular, the functionality subscore does not add to the predictive ability of the CCOS, and may be unnecessary. Overall, the authors found the CCOS to be an improvement over the previously used assessment of outcome at their institution.

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Jacob K. Greenberg, Eric Milner, Chester K. Yarbrough, Kim Lipsey, Jay F. Piccirillo, Matthew D. Smyth, Tae Sung Park, and David D. Limbrick Jr.

OBJECT

Chiari malformation Type I (CM-I) is a common and often debilitating neurological disease. Efforts to improve treatment of CM-I are impeded by inconsistent and limited methods of evaluating clinical outcomes. To understand current approaches and lay a foundation for future research, the authors conducted a systematic review of the methods used in original published research articles to evaluate clinical outcomes in patients treated for CM-I.

METHODS

The authors searched PubMed, Embase, the Cumulative Index to Nursing and Allied Health Literature, ClinicalTrials.gov, and Cochrane databases to identify publications between January 2003 and August 2013 that met the following criteria: 1) reported clinical outcomes in patients treated for CM-I; 2) were original research articles; 3) included at least 10 patients or, if a comparative study, at least 5 patients per group; and 4) were restricted to patients with CM-I.

RESULTS

Among the 74 papers meeting inclusion criteria, there was wide variation in the outcome methods used. However, all approaches were broadly grouped into 3 categories: 1) “gestalt” impression of overall symptomatic improvement (n = 45 papers); 2) postoperative change in specific signs or symptoms (n = 20); or 3) results of various standardized assessment scales (n = 22). Among standardized scales, 11 general function measures were used, compared with 6 disease-specific tools. Only 3 papers used scales validated in patients with CM-I. To facilitate a uniform comparison of these heterogeneous approaches, the authors appraised articles in multiple domains defined a priori as integral to reporting clinical outcomes in CM-I. Notably, only 7 articles incorporated patient-response instruments when reporting outcome, and only 22 articles explicitly assessed quality of life.

CONCLUSIONS

The methods used to evaluate clinical outcomes in CM-I are inconsistent and frequently not comparable, complicating efforts to analyze results across studies. Development, validation, and incorporation of a small number of disease-specific patient-based instruments will improve the quality of research and care of CM-I patients.

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Travis R. Ladner, Jacob K. Greenberg, Nicole Guerrero, Margaret A. Olsen, Chevis N. Shannon, Chester K. Yarbrough, Jay F. Piccirillo, Richard C. E. Anderson, Neil A. Feldstein, John C. Wellons III, Matthew D. Smyth, Tae Sung Park, and David D. Limbrick Jr.

OBJECTIVE

Administrative billing data may facilitate large-scale assessments of treatment outcomes for pediatric Chiari malformation Type I (CM-I). Validated International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code algorithms for identifying CM-I surgery are critical prerequisites for such studies but are currently only available for adults. The objective of this study was to validate two ICD-9-CM code algorithms using hospital billing data to identify pediatric patients undergoing CM-I decompression surgery.

METHODS

The authors retrospectively analyzed the validity of two ICD-9-CM code algorithms for identifying pediatric CM-I decompression surgery performed at 3 academic medical centers between 2001 and 2013. Algorithm 1 included any discharge diagnosis code of 348.4 (CM-I), as well as a procedure code of 01.24 (cranial decompression) or 03.09 (spinal decompression or laminectomy). Algorithm 2 restricted this group to the subset of patients with a primary discharge diagnosis of 348.4. The positive predictive value (PPV) and sensitivity of each algorithm were calculated.

RESULTS

Among 625 first-time admissions identified by Algorithm 1, the overall PPV for CM-I decompression was 92%. Among the 581 admissions identified by Algorithm 2, the PPV was 97%. The PPV for Algorithm 1 was lower in one center (84%) compared with the other centers (93%–94%), whereas the PPV of Algorithm 2 remained high (96%–98%) across all subgroups. The sensitivity of Algorithms 1 (91%) and 2 (89%) was very good and remained so across subgroups (82%–97%).

CONCLUSIONS

An ICD-9-CM algorithm requiring a primary diagnosis of CM-I has excellent PPV and very good sensitivity for identifying CM-I decompression surgery in pediatric patients. These results establish a basis for utilizing administrative billing data to assess pediatric CM-I treatment outcomes.