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Aditya Vedantam, Daniel Hansen, Bradley Daniels and Sandi Lam

The authors report an unusual case of acute, rapidly progressive, unilateral neck swelling following extubation after elective left anterior temporal lobectomy with amygdalohippocampectomy. Due to severe neck swelling, the patient developed critical airway compromise, brachial plexopathy, and Horner's syndrome. After critical airway management and appropriate rehabilitation, the patient recovered completely and remains seizure free at 1.5 years of follow-up.

This case highlights the importance of early recognition of acute postoperative sialadenitis and the steps needed to prevent serious morbidity and possible mortality from this rare complication.

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Guillermo Aldave, Daniel Hansen, Steven W. Hwang, Amee Moreno, Valentina Briceño and Andrew Jea

OBJECTIVE

Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique.

METHODS

The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome.

RESULTS

Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8–30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26–37 months). Computed tomography–based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and –13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and –12.8%, respectively.

CONCLUSIONS

Spinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.

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Guillermo Aldave, Daniel Hansen, Valentina Briceño, Thomas G. Luerssen and Andrew Jea

OBJECTIVE

The authors previously demonstrated the use of a validated Objective Structured Assessment of Technical Skills (OSATS) tool for evaluating residents' operative skills in pediatric neurosurgery. However, no benchmarks have been established for specific pediatric procedures despite an increased need for meaningful assessments that can either allow for early intervention for underperforming trainees or allow for proficient residents to progress to conducting operations independently with more passive supervision. This validated methodology and tool for assessment of operative skills for common pediatric neurosurgical procedures—external ventricular drain (EVD) placement and shunt surgery— was applied to establish its procedure-based feasibility and reliability, and to document the effect of repetition on achieving surgical skill proficiency in pediatric EVD placement and shunt surgery.

METHODS

A procedure-based technical skills assessment for EVD placements and shunt surgeries in pediatric neurosurgery was established through the use of task analysis. The authors enrolled all residents from 3 training programs (Baylor College of Medicine, Houston Methodist Hospital, and University of Texas–Medical Branch) who rotated through pediatric neurosurgery at Texas Children's Hospital over a 26-month period. For each EVD placement or shunt procedure performed with a resident, the faculty and resident (for self-assessment) completed an evaluation form (OSATS) based on a 5-point Likert scale with 7 categories. Data forms were then grouped according to faculty versus resident (self) assessment, length of pediatric neurosurgery rotation, postgraduate year level, and date of evaluation (“beginning of rotation,” within 1 month of start date; “end of rotation,” within 1 month of completion date; or “middle of rotation”). Descriptive statistical analyses were performed with the commercially available SPSS statistical software package. A p value < 0.05 was considered statistically significant.

RESULTS

Five attending evaluators (including 2 fellows who acted as attending surgeons) completed 260 evaluations. Twenty house staff completed 269 evaluations for self-assessment. Evaluations were completed in 562 EVD and shunt procedures before the surgeons left the operating room. There were statistically significant differences (p < 0.05) between overall attending (mean 4.3) and junior resident (self; mean 3.6) assessments, and between overall attending (mean 4.8) and senior resident (self; mean 4.6) assessment scores on general performance and technical skills. The learning curves produced for the residents demonstrate a stereotypical U- or V-shaped curve for acquiring skills, with a significant improvement in overall scores at the end of the rotation compared with the beginning. The improvement for junior residents (Δ score = 0.5; p = 0.002) was larger than for senior residents (Δ score = 0.2; p = 0.018).

CONCLUSIONS

The OSATS is an effective assessment tool as part of a comprehensive evaluation of neurosurgery residents' performance for specific pediatric procedures. The authors observed a U-shaped learning curve, contradicting the idea that developing one's surgical technique and learning a procedure represents a monotonic, cumulative process of repetitions and improvement.

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Arnold H. Menezes, Jeremy D. W. Greenlee, Reid A. Longmuir, Daniel R. Hansen and Kingsley Abode-Iyamah

The authors present the case of a 14-year-old boy with holocord syringohydromyelia extending into the brainstem, cerebral peduncle, internal capsule, and cerebral cortex. At the posterior fossa exploration, an opaque thickened arachnoid with occlusion of the foramen of Magendie was encountered. Careful documentation of postoperative regression of the syringocephaly, syringobulbia, and syringohydromyelia was made. The pathophysiology is discussed.

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Aditya Vedantam, Daniel Hansen, Valentina Briceño, Alison Brayton and Andrew Jea

OBJECTIVE

There is limited literature on patient-reported outcomes (PROs) and health-related quality of life (HRQOL) outcomes in pediatric patients undergoing surgery for craniovertebral junction pathology. The aim of the present study was to assess surgical and quality of life outcomes in children who had undergone occipitocervical or atlantoaxial fusion.

METHODS

The authors retrospectively reviewed the demographics, procedural data, and outcomes of 77 consecutive pediatric patients who underwent posterior occipitocervical or atlantoaxial fusion between 2008 and 2015 at Texas Children's Hospital. Outcome measures (collected at last follow-up) included mortality, neurological improvement, complications, Scoliosis Research Society Outcomes Measure–22 (SRS-22) score, SF-36 score, Neck Disability Index (NDI), and Pediatric Quality of Life Inventory (PedsQL). Multivariate linear regression analysis was performed to identify factors affecting PROs and HRQOL scores at follow-up.

RESULTS

The average age in this series was 10.6 ± 4.5 years. The median follow-up was 13.9 months (range 0.5–121.5 months). Sixty-three patients (81.8%) were treated with occipitocervical fusion, and 14 patients (18.1%) were treated with atlantoaxial fusion. The American Spinal Injury Association (ASIA) grade at discharge was unchanged in 73 patients (94.8%). The average PRO metrics at the time of last follow-up were as follows: SRS-22 score, 4.2 ± 0.8; NDI, 3.0 ± 2.6; the parent's PedsQL (ParentPedsQL) score, 69.6 ± 22.7, and child's PedsQL score, 75.5 ± 18.7. Multivariate linear regression analysis revealed that older age at surgery was significantly associated with lower SRS-22 scores at follow-up (B = −0.06, p = 0.03), and the presence of comorbidities was associated with poorer ParentPedsQL scores at follow-up (B = −19.68, p = 0.03).

CONCLUSIONS

This study indicates that occipitocervical and atlantoaxial fusions in children preserve neurological function and are associated with acceptable PROs and ParentPedsQL scores, considering the serious nature and potential for morbidity in this patient population. However, longer follow-up and disease-specific scales are necessary to fully elucidate the impact of occipitocervical and atlantoaxial fusions on children.

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Aditya Vedantam, Daniel Hansen, Valentina Briceño, Amee Moreno, Sheila L. Ryan and Andrew Jea

OBJECTIVE

The purpose of this study was to describe patterns of transfer, resource utilization, and clinical outcomes associated with the interhospital transfer of pediatric neurosurgical patients.

METHODS

All consecutive, prospectively collected requests for interhospital patient transfer to the pediatric neurosurgical service at Texas Children's Hospital were retrospectively analyzed from October 2013 to September 2014. Demographic patient information, resource utilization, and outcomes were recorded and compared across predefined strata (low [< 5%], moderate [5%–30%], and high [> 30%]) of predicted probability of mortality using the Pediatric Risk of Mortality score.

RESULTS

Requests for pediatric neurosurgical care comprised 400 (3.7%) of a total of 10,833 calls. Of 400 transfer admissions, 96.5%, 2.8%, and 0.8% were in the low, moderate, and high mortality risk groups, respectively. The median age was 54 months, and 45% were female. The median transit time was 125 minutes. The majority of transfers were after-hours (69.8%); nearly a third occurred during the weekend (32.3%). The median intensive care unit stay for 103 patients was 3 days (range 1–269 days). Median length of hospital stay was 2 days (range 1–269 days). Ninety patients (22.5%) were discharged from the emergency room after transfer. Seventy-seven patients (19.3%) required neurosurgical intervention after transfer, with the majority requiring a cranial procedure (66.2%); 87.3% of patients were discharged home.

CONCLUSIONS

This study highlights patient characteristics, resource utilization, and outcomes among pediatric neurosurgical patients. Opportunities for quality improvement were identified in diagnosing and managing isolated skull fractures and neck pain after trauma.

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Daniel Hansen, Aditya Vedantam, Valentina Briceño, Sandi K. Lam, Thomas G. Luerssen and Andrew Jea

OBJECTIVE

The emphasis on health-related quality of life (HRQOL) outcomes is increasing, along with an emphasis on evidence-based medicine. However, there is a notable paucity of validated HRQOL instruments for the pediatric population. Furthermore, no standardization or consensus currently exists concerning which HRQOL outcome measures ought to be used in pediatric neurosurgery. The authors wished to identify HRQOL outcomes used in pediatric neurosurgery research over the past 10 years, their frequency, and usage trends.

METHODS

Three top pediatric neurosurgical journals were reviewed for the decade from 2005 to 2014 for clinical studies of pediatric neurosurgical procedures that report HRQOL outcomes. Similar studies in the peer-reviewed journal Pediatrics were also used as a benchmark. Publication year, level of evidence, and HRQOL outcomes were collected for each article.

RESULTS

A total of 31 HRQOL studies were published in the pediatric neurosurgical literature over the study period. By comparison, there were 55 such articles in Pediatrics. The number of publications using HRQOL instruments showed a significant positive trend over time for Pediatrics (B = 0.62, p = 0.02) but did not increase significantly over time for the 3 neurosurgical journals (B = 0.12, p = 0.5). The authors identified a total of 46 different HRQOL instruments used across all journals. Within the neurosurgical journals, the Hydrocephalus Outcome Questionnaire (HOQ) (24%) was the most frequently used, followed by the Health Utilities Index (HUI) (16%), the Pediatric Quality of Life Inventory (PedsQL) (12%), and the 36-Item Short Form Health Survey (SF-36) (12%). Of the 55 articles identified in Pediatrics, 22 (40%) used a version of the PedsQL. No neurosurgical study reached above Level 4 on the Oxford Centre for Evidence-Based Medicine (OCEBM) system. However, multiple studies from Pediatrics achieved OCEBM Level 3, several were categorized as Level 2, and one reached Level 1.

CONCLUSIONS

The frequency of studies using HRQOL outcomes in pediatric neurosurgical research has not increased over the past 10 years. Within pediatric neurosurgery, high-quality studies and standardization are lacking, as compared with contemporary studies in Pediatrics. In general, although the HOQ, HUI, PedsQL, and SF-36 instruments are emerging as standards in pediatric neurosurgery, even greater standardization across the specialty is needed, along with the design and implementation of more rigorous studies.

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Gregory W. Albert, Arnold H. Menezes, Daniel R. Hansen, Jeremy D. W. Greenlee and Stuart L. Weinstein

Object

The authors conducted a study to evaluate the unique presenting signs and symptoms of Chiari malformation Type I (CM-I) in children younger than 6 years of age and highlight the benefits of early surgical treatment in this patient population.

Methods

The authors reviewed the medical records of patients who presented to the neurosurgery department before their 6th birthday and subsequently underwent surgery for CM-I. They identified 39 patients who had been evaluated between 1984 and 2007 and examined the medical records for presentation, surgical intervention, and outcome.

Results

Children aged 0–2 years commonly presented with oropharyngeal dysfunction (77.8%). Children aged 3–5 years more frequently presented with syringomyelia (85.7%), scoliosis (38.1%), and/or headache (57.1%). All patients underwent posterior fossa craniectomy. Additionally, in many patients cervical laminectomy and/or duraplasty was performed. A few patients required transoral decompression and occipitocervical fusion. In most cases, surgery led to resolution or dramatic improvement of initial symptoms.

Conclusions

Early recognition and surgical treatment of CM-I in young children leads to good outcomes in the majority of patients. Additional therapies for oropharyngeal dysfunction, syringomyelia, and scoliosis can frequently be avoided.

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Gregory W. Albert, Nader S. Dahdaleh, Chandan Reddy, Daniel R. Hansen, Timothy W. Vogel, Hiroto Kawasaki and Matthew A. Howard III

Object

In this study the authors sought to determine whether any correlations existed between postimplantation head CT findings and the need to perform decompression surgery in patients with grid electrodes.

Methods

The authors identified 74 patients who underwent intracranial electrode monitoring for medically refractory epilepsy from January 2000 through June 2008. Only the 46 patients who had head CT scans available for review were included in the study. The authors were able to determine the number and types of electrodes placed as well as complications experienced. They reviewed the CT scans for abnormal findings including extraaxial fluid collections, intracranial hemorrhages, and signs of mass effect.

Results

All patients developed some degree of extraaxial fluid collection following the placement of intracranial electrodes. The maximum width of the extraaxial fluid collection and the degree of midline shift were not predictive of the need for decompressive surgery. The presence, but not degree, of midline shift was associated with the need for decompressive surgery. Likewise, the presence of ventricular asymmetry was correlated with the need for removal of the electrodes and bone flap. Patients without midline shift or ventricular asymmetry on CT did not require decompressive surgery.

Conclusions

After undergoing placement of intracranial electrodes all patients develop extraaxial fluid collections. In addition, many patients develop signs of mass effect including midline shift and ventricular asymmetry. When these findings are absent it is highly unlikely that surgical decompression is required.

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Chandan G. Reddy, Nader S. Dahdaleh, Gregory Albert, Fangxiang Chen, Daniel Hansen, Kirill Nourski, Hiroto Kawasaki, Hiroyuki Oya and Matthew A. Howard III

A wide range of devices is used to obtain intracranial electrocorticography recordings in patients with medically refractory epilepsy, including subdural strip and grid electrodes and depth electrodes. Penetrating depth electrodes are required to access some brain regions, and 1 target site that presents a particular technical challenge is the first transverse temporal gyrus, or Heschl gyrus (HG). The HG is located within the supratemporal plane and has an oblique orientation relative to the sagittal and coronal planes. Large and small branches of the middle cerebral artery abut the pial surface of the HG and must be avoided when planning the electrode trajectory.

Auditory cortex is located within the HG, and there are functional connections between this dorsal temporal lobe region and medial sites commonly implicated in the pathophysiology of temporal lobe epilepsy. At some surgical centers, depth electrodes are routinely placed within the supratemporal plane, and the HG, in patients who require intracranial electrocorticography monitoring for presumed temporal lobe epilepsy. Information from these recordings is reported to facilitate the identification of seizure patterns in patients with or without auditory auras.

To date, only one implantation method has been reported to be safe and effective for placing HG electrodes in a large series of patients undergoing epilepsy surgery. This well-established approach involves inserting the electrodes from a lateral trajectory while using stereoscopic stereotactic angiography to avoid vascular injury. In this report, the authors describe an alternative method for implantation. They use frameless stereotaxy and an oblique insertion trajectory that does not require angiography and allows for the simultaneous placement of subdural grid arrays. Results in 19 patients demonstrate the safety and efficacy of the method.