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Brandon G. Rocque, Samir Lapsiwala and Bermans J. Iskandar

Object

The clinical diagnosis of cerebrospinal fluid (CSF) shunt malfunction can be challenging. In this prospective study, the authors evaluated a common method of interrogating shunts: the shunt tap; specifically, its ability to predict proximal malfunction.

Methods

The authors performed standardized shunt taps in a consecutive series of cases involving children with suspected or proven shunt malfunction, assessing flow and, when possible, opening pressure. Data were collected prospectively, and results analyzed in light of surgical findings.

Results

A shunt tap was performed prior to 68 operative explorations in 51 patients. Of the 68 taps, 28 yielded poor or no CSF flow on aspiration. After 26 of these 28 procedures, proximal catheter obstruction was identified. After 28 taps with good CSF return and normal or low opening pressure, 18 shunts were found to have a proximal obstruction, 8 had no obstruction, and 2 had a distal obstruction. Another 12 taps with good CSF flow had high opening pressure; subsequent surgery showed distal obstruction in 11 of the shunts, and proximal obstruction in 1. The positive predictive value of poor flow was 93%, while good flow on shunt tap predicted adequate proximal catheter function in only 55% of cases.

Conclusions

Poor flow of CSF on shunt tap is highly predictive of obstruction of the proximal catheter. Because not all patients with good flow on shunt tap underwent surgical shunt exploration, the specificity of this test cannot be determined. Nonetheless, a shunt tap that reveals good flow with a normal opening pressure can be misleading, and management of such cases should be based on clinical judgment.

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W. Jerry Oakes

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Brandon G. Rocque, Timothy M. George, John Kestle and Bermans J. Iskandar

Object

The purpose of this study was to report the results of a survey of the American Society of Pediatric Neurosurgeons (ASPN) on treatment of Chiari malformation Type I (CM-I) with syringomyelia.

Methods

A questionnaire was circulated during the 2006 meeting of the ASPN, in which surgeons were surveyed on their management of patients with CM-I and syringomyelia. The survey consisted of questions about 4 clinical scenarios, common causes of surgical failures, and complications.

Results

There were 72 respondents, representing more than 90% of attendees at the 2006 ASPN meeting and approximately half of the society's members. The majority of respondents (85%) reported that they perform posterior fossa decompression as first-line treatment for CM-I with syringomyelia. Seven percent perform bony decompression alone, 36% open the dura, and 27% shrink the tonsils. Very few respondents indicated that they offer syrinx drainage as first-line therapy (< 3%). Although all respondents reported that they treat symptomatic CM-I/syringomyelia patients surgically, 15% of respondents indicated that they do not operate on asymptomatic patients. Finally, respondents stated that their most common complications are pseudomeningocele and chemical meningitis.

Conclusions

This survey, given to a representative group of experienced North American pediatric neurosurgeons, confirms that posterior fossa decompression is still the preferred treatment modality in children with CM-I and syringomyelia, regardless of symptoms. Although most surgeons open the dura, preferred techniques for decompression vary. In contrast to the results of past surveys, conservative follow-up is now only used by a minority of respondents and only in the asymptomatic patient, and primary syrinx drainage seems to have lost popularity. A multicenter trial of surgical outcomes has been designed based on the information from this survey.

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Brandon G. Rocque, Kaushik Amancherla, Sean M. Lew and Sandi Lam

Cranioplasty is routinely performed following decompressive craniectomy in both adult and pediatric populations. In adults, this procedure is associated with higher rates of complications than is elective cranial surgery. This study is a review of the literature describing risk factors for complications after cranioplasty surgery in pediatric patients. A systematic search of PubMed, Cochrane, and SCOPUS databases was undertaken. Articles were selected based on their titles and abstracts. Only studies that focused on a pediatric population were included; case reports were excluded. Studies in which the authors assessed bone flap storage method, timing of cranioplasty, material used (synthetic vs autogenous), skull defect size, and/or complication rates (bone resorption and surgical site infection) were selected for further analysis. Eleven studies that included a total of 441 cranioplasties performed in the pediatric population are included in this review.

The findings are as follows: 1) Based on analysis of pooled data, using cryopreserved bone flaps during cranioplasty may lead to a higher rate of bone resorption and lower rate of infection than using bone flaps stored at room temperature. 2) In 3 of 4 articles describing the effect of time between craniectomy and cranioplasty on complication rate, the authors found no significant effect, while in 1 the authors found that the incidence of bone resorption was significantly lower in children who had undergone early cranioplasty. Pooling of data was not possible for this analysis. 3) There are insufficient data to assess the effect of cranioplasty material on complication rate when considering only cranioplasties performed to repair decompressive craniectomy defects. However, when considering cranioplasties performed for any indication, those in which freshly harvested autograft is used may have a lower rate of resorption than those in which stored autograft is used. 4) There is no appreciable effect of craniectomy defect size or patient age on complication rate.

There is a paucity of articles describing outcomes and complications following cranioplasty in children and adolescents. However, based on the studies examined in this systematic review, there are reasons to suspect that method of flap preservation, timing of surgery, and material used may be significant. Larger prospective and retrospective studies are needed to shed more light on this important issue.

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Sandi Lam, Dominic Harris, Brandon G. Rocque and Sandra A. Ham

Object

Endoscopic third ventriculostomy (ETV) is an alternative to ventriculoperitoneal shunting for hydrocephalus treatment. Choice of treatment options raises questions about which patients are likely to benefit from ETV. The authors performed a population-based analysis using an administrative claims database, examining current practice and outcomes for pediatric patients in the US.

Methods

The authors queried the MarketScan (Truven Health Analytics) database for Current Procedural Terminology codes corresponding to ETV and ventriculoperitoneal shunting from 2003 to 2011; they included patients 19 years or younger and extracted data from initial and subsequent hospitalizations. Hydrocephalus etiology was classified with ICD-9-CM coding. ETV failure was defined as any subsequent ETV or shunt procedure.

Results

Five hundred one patients underwent ETV. Of these, 46% were female. The mean age was 8.7 ± 6.4 years (± SD). The mean follow-up was 1.9 ± 1.8 years. Etiology of hydrocephalus was primarily tumor (41.7%) and congenital/aqueductal stenosis (24.4%). ETV was successful in 354 patients (71%). The mean time to failure was 109.9 ± 233 days. Of the 147 patients with ETV failure, 35 (24%) underwent repeat ETV and 112 (76%) had shunt placement. Patients in age groups 0 to < 6 months and 6 months to < 1 year had a significantly higher rate of ETV failure than those 10–19 years (HR 2.9, p = 0.05; and HR 2.3, p = 0.001, respectively). History of prior shunt was associated with higher risk of failure (HR 2.5, p < 0.001). There were no significant associations between hydrocephalus etiology and risk of failure. A second wave of failures occurred at 2.5–3.5 years postoperative in tumor and congenital/aqueductal stenosis patients; this was not observed in other etiology groups.

Conclusions

This study represents a cross-section of nationwide ETV practice over 9 years. ETV success was more likely among children 1 year and older and those with no history of prior shunt.

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Brandon G. Rocque, Mick P. Kelly, Joseph H. Miller, Yiping Li and Paul A. Anderson

Object

Use of recombinant human bone morphogenetic protein–2 has risen steadily since its approval by the FDA for use in anterior lumbar interbody fusion in 2002. The FDA has not approved the use of bone morphogenetic protein (BMP) in children. Age less than 18 years or lack of evidence of epiphyseal closure are considered by the manufacturer to be contraindications to BMP use. In light of this, the authors performed a query of the database of one of the nation's largest health insurance companies to determine the rate of BMP use and complications in pediatric patients undergoing spinal fusion.

Methods

The authors used the PearlDiver Technologies private payer database containing all records from United Health-Care from 2005 to 2011 to query all cases of pediatric spinal fusion with or without BMP use. A review of the literature was also performed to examine the complications associated with BMP use in pediatric spinal fusion.

Results

A total of 4658 patients underwent spinal fusion. The majority was female (65.4%), and the vast majority was age 10–19 years (94.98%) and underwent thoracolumbar fusion (93.13%). Bone morphogenetic protein was used in 1752 spinal fusions (37.61%). There was no difference in the rate of BMP use when comparing male and female patients or age 10 years or older versus less than 10 years. Anterior cervical fusions were significantly less likely to use BMP (7.3%). Complications occurred in 9.82% of patients treated with versus 9.88% of patients treated without BMP. The complication rate was nearly identical in male versus female patients and in patients older versus younger than 10 years. Comparison of systemic, wound-related, CNS, and other complications showed no difference between groups treated with and without BMP. The reoperation rate was also nearly identical.

Conclusions

Bone morphogenetic protein is used in a higher than expected percentage of pediatric spinal fusions. The rate of acute complications in these operations does not appear to be different in patients treated with versus those treated without BMP. Caution must be exercised in interpreting these data due to the many limitations of the administrative database as a data source, including the short length of follow-up.

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Brandon G. Rocque, E. Ralee' Bishop, Mallory A. Scogin, Betsy D. Hopson, Anastasia A. Arynchyna, Christina J. Boddiford, Chevis N. Shannon and Jeffrey P. Blount

OBJECT

The purpose of this study is to explore various aspects of health-related quality of life (HRQOL) in children with spinal dysraphism.

METHODS

The authors enrolled a prospective cohort of 159 patients from the multidisciplinary spina bifida clinic. Surveys were distributed to caregivers of patients with spina bifida who were 5 years old and older. Data were collected using the Health Utilities Index Mark 3 focusing on vision, speech, hearing, dexterity, ambulation, cognition, emotions, and pain. Each participant received an overall HRQOL utility score and individual domain subscores. These were correlated with demographic and treatment variables. Analysis was done using SPSS statistics (version 21).

RESULTS

There were 125 patients with myelomeningocele, 25 with lipomyelomeningocele, and 9 with other dysraphisms. Among patients with myelomeningocele, 107 (86%) had CSF shunts in place, 14 (11%) had undergone Chiari malformation Type II decompression, 59 (47%) were community ambulators, and 45 (36%) were nonambulatory. Patients with myelomeningocele had significantly lower overall HRQOL scores than patients with closed spinal dysraphism. Among patients with myelomeningocele, younger patients had higher HRQOL scores. Patients with impaired bowel continence had lower overall HRQOL scores. History of a ventriculoperitoneal shunt was associated with worse HRQOL (overall score, ambulation, and cognition subscores). History of Chiari malformation Type II decompression was associated with worse overall, speech, and cognition scores. Patients who could ambulate in the community had higher overall and ambulation scores. A history of tethered cord release was correlated with lower pain subscore. No association was found between sex, race, insurance type, or bladder continence and HRQOL.

CONCLUSIONS

Patients with myelomeningocele have significantly lower HRQOL scores than those with other spinal dysraphisms. History of shunt treatment and Chiari decompression correlate with lower HRQOL scores.