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Nikita G. Alexiades, Edward S. Ahn, Jeffrey P. Blount, Douglas L. Brockmeyer, Samuel R. Browd, Gerald A. Grant, Gregory G. Heuer, Todd C. Hankinson, Bermans J. Iskandar, Andrew Jea, Mark D. Krieger, Jeffrey R. Leonard, David D. Limbrick Jr., Cormac O. Maher, Mark R. Proctor, David I. Sandberg, John C. Wellons III, Belinda Shao, Neil A. Feldstein and Richard C. E. Anderson

OBJECTIVE

Complications after complex tethered spinal cord (cTSC) surgery include infections and cerebrospinal fluid (CSF) leaks. With little empirical evidence to guide management, there is variability in the interventions undertaken to limit complications. Expert-based best practices may improve the care of patients undergoing cTSC surgery. Here, authors conducted a study to identify consensus-driven best practices.

METHODS

The Delphi method was employed to identify consensual best practices. A literature review regarding cTSC surgery together with a survey of current practices was distributed to 17 board-certified pediatric neurosurgeons. Thirty statements were then formulated and distributed to the group. Results of the second survey were discussed during an in-person meeting leading to further consensus, which was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree).

RESULTS

Seventeen consensus-driven best practices were identified, with all participants willing to incorporate them into their practice. There were four preoperative interventions: (1, 2) asymptomatic AND symptomatic patients should be referred to urology preoperatively, (3, 4) routine preoperative urine cultures are not necessary for asymptomatic AND symptomatic patients. There were nine intraoperative interventions: (5) patients should receive perioperative cefazolin or an equivalent alternative in the event of allergy, (6) chlorhexidine-based skin preparation is the preferred regimen, (7) saline irrigation should be used intermittently throughout the case, (8) antibiotic-containing irrigation should be used following dural closure, (9) a nonlocking running suture technique should be used for dural closure, (10) dural graft overlay should be used when unable to obtain primary dural closure, (11) an expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF, (12) paraxial muscles should be closed as a layer separate from the fascia, (13) routine placement of postoperative drains is not necessary. There were three postoperative interventions: (14) postoperative antibiotics are an option and, if given, should be discontinued within 24 hours; (15) patients should remain flat for at least 24 hours postoperatively; (16) routine use of abdominal binders or other compressive devices postoperatively is not necessary. One intervention was prioritized for additional study: (17) further study of additional gram-negative perioperative coverage is needed.

CONCLUSIONS

A modified Delphi technique was used to develop consensus-driven best practices for decreasing wound complications after cTSC surgery. Further study is required to determine if implementation of these practices will lead to reduced complications. Discussion through the course of this study resulted in the initiation of a multicenter study of gram-negative surgical site infections in cTSC surgery.

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Robert P. Naftel, Nicole A. Safiano, Michael Falola, Jeffrey P. Blount, W. Jerry Oakes and John C. Wellons III

Object

Children experiencing frequent shunt failure consume medical resources and represent a disproportionate level of morbidity in hydrocephalus care. While biological causes of frequent shunt failure may exist, this study analyzed demographic and socioeconomic patient characteristics associated with frequent shunt failure.

Methods

A survey of 294 caregivers of children with shunt-treated hydrocephalus provided demographic and socioeconomic characteristics. Children experiencing at least 10 shunt failures were considered frequent shunt-failure patients. Multivariate regression models were used to control for variables.

Results

Frequent shunt failure was experienced by 9.5% of the patients (28 of 294). By univariate analysis, white race (p = 0.006), etiology of hydrocephalus (p = 0.022), years-with-shunt (p < 0.0001), and surgeon (p = 0.02) were associated with frequent shunt failure. Upon multivariate analysis, white race remained the key independent factor associated with frequent shunt failure (OR 5.8, 95% CI 1.2–27.8, p = 0.027). Race acted independently from socioeconomic factors, including income, level of education, and geographic location, and clinical factors, such as etiology of hydrocephalus, surgeon, and years-with-shunt. Additionally, after multivariate analysis surgeon and years-with-shunt remained associated with frequent shunt failure (p = 0.043 and p = 0.0098, respectively), although etiology of hydrocephalus was no longer associated (p = 0.1).

Conclusions

White race was the primary independent factor associated with frequent shunt failure. Because races use health care differently and the diagnosis of shunt failure is often subjective, a disparity in diagnosis and treatment has arisen. These findings call for objective criteria for the preoperative and intraoperative diagnosis of shunt failure.

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Joshua J. Chern, Mitchel Muhleman, R. Shane Tubbs, Joseph H. Miller, James M. Johnston, John C. Wellons III, Jeffrey P. Blount, W. Jerry Oakes and Curtis J. Rozzelle

Object

Most children with spina bifida aperta have implanted CSF shunts. However, the efficacy of adding surveillance imaging to clinical evaluation during routine follow-up as a means to minimize the hazard of shunt failure has not been thoroughly studied.

Methods

A total of 396 clinic visits were made by patients with spina bifida aperta and shunt-treated hydrocephalus in a spina bifida specialty clinic during the calendar years 2008 and 2009 (initial clinic visit). All visits were preceded by a 6-month period during which no shunt evaluation of any kind was performed and were followed by a subsequent visit in the same clinic. At the initial clinic visit, 230 patients were evaluated by a neurosurgeon (clinical evaluation group), and 166 patients underwent previously scheduled surveillance CT scans in addition to clinical evaluation (surveillance imaging group). Subsequent unexpected events, defined as emergency department (ED) visits and caregiver-requested clinic visits, were reviewed. The time to an unexpected event and the likelihood of event occurrence in each of the 2 groups were compared using Cox proportional hazards survival analysis. The outcome and complications of shunt surgeries were also reviewed.

Results

The clinical characteristics of the 2 groups were similar. In the clinical evaluation group, 2 patients underwent shunt revision based on clinical findings in the initial visit. In the subsequent follow-up period, there were 27 visits to the ED and 25 requested clinic visits that resulted in 12 shunt revisions. In the surveillance imaging group, 11 patients underwent shunt revision based on clinical and imaging findings in the initial visit. In the subsequent follow-up period, there were 15 visits to the ED and 9 requested clinic visits that resulted in 8 shunt revisions. Patients who underwent surveillance imaging on the day of initial clinic visit were less likely to have an unexpected event in the subsequent follow-up period (relative risk 0.579, p = 0.026). The likelihood of needing shunt revision and the morbidity of shunt malfunction was not significantly different between the 2 groups.

Conclusions

Surveillance imaging in children with spina bifida aperta and shunted hydrocephalus decreases the likelihood of ED visits and caregiver-requested clinic visits in the follow-up period, but based on this study, its effect on mortality and morbidity related to shunt malfunction was less clear.

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Robert P. Naftel, Emily Tubergen, Chevis N. Shannon, Kimberly A. Gran, E. Haley Vance, W. Jerry Oakes, Jeffrey P. Blount and John C. Wellons III

Object

Because there is no gold standard for preoperative diagnosis of shunt failure, understanding the sensitivity, specificity, and predictive values of symptoms, signs, and diagnostic tests enables practitioners to make logical clinical decisions. Parents of children with shunts undergo educational instruction to enable them to recognize shunt failure. The authors prospectively investigated parental ability to recognize shunt failure.

Methods

Data were prospectively collected on 205 consecutive encounters in 153 children with shunted hydrocephalus presenting to the emergency department or clinic, or as an inpatient consultation, to the Children's Hospital of Alabama between April and October 2010. Regardless of the complaint, all parents were asked if they believed the shunt was in failure. Six children were excluded from analysis because a parental response was lacking. Using the Shunt Design Trial definitions, shunt failure was diagnosed intraoperatively or ruled out if the child did not undergo shunt revision within 1 week of presentation. Sensitivity, specificity, predictive values, and accuracy were calculated using the parental response and shunt failure diagnosis. Secondarily, parents were compared based on their experience with shunt failure in their children; experienced parents were defined as having experienced at least 3 shunt failures. Post hoc analysis evaluated diagnostic test characteristics among hydrocephalus causes and compared parental recognition of shunt failure to head CT and shunt series diagnostic test characteristics. Parents also completed a standardized shunt failure survey regarding their shunt teaching education and symptom tracking.

Results

Children enrolled were a mean age of 6.9 years old, 92 (46%) of the encounters were with male patients, and most patients were Caucasian (69%) and had undergone an average of 2.8 previous shunt revisions. Seventy-one children (36%) were diagnosed with shunt failure. Parental response diagnostic test characteristics were: positive predictive value (PPV) of 41%, negative predictive value (NPV) of 79%, sensitivity of 83%, specificity of 34%, and accuracy of 52% for shunt failure. Sixty-three parents were considered experienced and responded with a PPV of 29%, NPV of 92%, sensitivity of 94%, specificity of 23%, and accuracy of 41%. One hundred thirty-six parents were considered inexperienced and responded with a PPV of 48%, NPV of 75%, sensitivity of 80%, specificity of 41%, and accuracy of 57%. When statistically compared, experienced parents had significantly lower PPV (29% vs 48%, respectively; p = 0.035) and accuracy (41% vs 57%, respectively; p = 0.049) than inexperienced parents. On post hoc analysis, parental recognition of shunt failure was inferior to head CT and shunt series diagnostic tests with a lower specificity (20% vs 88%, respectively; p < 0.0005), PPV (44% vs 84%, respectively; p < 0.0005), NPV (61% vs 85%, respectively; p = 0.006), and accuracy (47% vs. 85%, respectively; p < 0.0005).

Conclusions

The overall parental response had the greatest value in ruling out shunt failure, reflected in the high NPV, particularly in experienced parents. The head CT and shunt series provide more favorable diagnostic test characteristics than the parental response. Although educational interventions have decreased shunt-related deaths, parents have difficulty differentiating shunt failure from alternative diagnoses.

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Joshua J. Chern, Joseph H. Miller, R. Shane Tubbs, Thomas R. Whisenhunt, James M. Johnston, John C. Wellons III, Curtis J. Rozzelle, Jeffrey P. Blount and W. Jerry Oakes

Object

A large volume of patients presented to a Level I pediatric trauma center during and after a recent tornado disaster. Injuries of the central and peripheral nervous systems and the medical responses of a pediatric neurosurgical team are reviewed.

Methods

The clinical courses of patients who suffered cranial, spinal, and peripheral nerve injuries due to the tornado storm are reported. The clinical actions taken by the neurosurgical team during and after the event are reviewed and the lessons learned are discussed.

Results

The tornado storm system moved through the Tuscaloosa and Birmingham metropolitan areas on the early evening hours of April 27, 2011. Twenty-four patients received care from the neurosurgical team. A total of 11 cranial (including placement of an external ventricular drain), 2 spine, and 2 peripheral procedures were performed for the victims. Nine procedures were performed within the first 12 hours of the event, and an additional 6 surgeries were performed in the following 24 hours. Injuries of the peripheral nervous system often presented in a delayed fashion. Several key components were identified that enabled adequate neurosurgical care for a large influx of acute patients.

Conclusions

Massive casualties due to tornados are rare. A well-organized physician team working with the hospital administration may decrease the mortality and morbidity of such events.

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R. Shane Tubbs, Joshua Beckman, Robert P. Naftel, Joshua J. Chern, John C. Wellons III, Curtis J. Rozzelle, Jeffrey P. Blount and W. Jerry Oakes

Object

The diagnosis and treatment of Chiari malformation Type I (CM-I) has evolved over the last few decades. The authors present their surgical experience of over 2 decades of treating children with this form of hindbrain herniation.

Methods

The authors conducted a retrospective review of their institutional experience with the surgical treatment of the pediatric CM-I from 1989 to 2010.

Results

The 2 most common presentations were headache/neck pain (40%) and scoliosis (18%). Common associated diagnoses included neurofibromatosis Type 1 (5%) and idiopathic growth hormone deficiency (4.2%). Spine anomalies included scoliosis (18%), retroversion of the odontoid process (24%), Klippel-Feil anomaly (3%), and atlantooccipital fusion (8%). Approximately 3% of patients had a known family member with CM-I. Hydrocephalus was present in 48 patients (9.6%). Syringomyelia was present in 285 patients (57%), and at operation, 12% of patients with syringomyelia were found to have an arachnoid veil occluding the fourth ventricular outlet. Fifteen patients (3%) have undergone reoperation for continued symptoms or persistent large syringomyelia. The most likely symptoms and signs to resolve following surgery were Valsalva-induced headache and syringomyelia. The average hospital stay and “return to school” time were 3 and 12 days, respectively. The follow-up for this group ranged from 2 months to 15 years (mean 5 years). Complications occurred in 2.4% of cases; there was no mortality. No patient required acute return to the operating room, and no blood transfusions were performed.

Conclusions

The authors believe this to be the largest reported series of surgically treated pediatric CM-I patients and hope that their experience will be of use to others who treat this surgical entity.

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Robert P. Naftel, Chevis N. Shannon, Gavin T. Reed, Richard Martin, Jeffrey P. Blount, R. Shane Tubbs and John C. Wellons III

Object

The use of intraventricular endoscopy to achieve diagnosis or to resect accessible intraventricular or paraventricular tumors has been described in the literature in both adults and children. Traditionally, these techniques have not been used in patients with small ventricles due to the perceived risk of greater morbidity. The authors review their experience with the effectiveness and safety of endoscopic brain tumor management in children with small ventricles.

Methods

Between July 2002 and December 2009, 24 children with endoscopically managed brain tumors were identified. Radiological images were reviewed by a radiologist blinded to study goals and clinical setting. Patients were categorized into small-ventricle and ventriculomegaly groups based on frontal and occipital horn ratio. Surgical success was defined a priori and analyzed between groups. Trends were identified in selected subgroups, including complications related to pathological diagnosis and surgeon experience.

Results

Six children had small ventricles and 18 had ventriculomegaly. The ability to accomplish surgical goals was statistically equivalent in children with small ventricles and those with ventriculomegaly (83% vs 89%, respectively, p = 1.00). There were no complications in the small-ventricle cohort, but in the ventriculomegaly cohort there were 2 cases of postoperative hemorrhages and 1 case of infection. All hemorrhagic complications occurred in patients with high-grade tumor histopathological type and were early in the surgeon's endoscopic career.

Conclusions

Based on our experience, endoscopy should not be withheld in children with intraventricular tumors and small ventricles. Complications appear to be more dependent on tumor histopathological type and surgeon experience than ventricular size.

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R. Shane Tubbs, Marios Loukas, Mohammadali M. Shoja, Robert J. Spinner, Erik H. Middlebrooks, William R. Stetler Jr., Leslie Acakpo-Satchivi, John C. Wellons III, Jeffrey P. Blount and W. Jerry Oakes

Object

The suprascapular nerve may become entrapped as it travels deep to the suprascapular ligament, necessitating decompression. The present study was performed to verify the feasibility of a minimally invasive, endoscopically assisted technique for decompressing the suprascapular nerve in the supraspinous fossa.

Methods

The authors performed dissection and decompression of the suprascapular ligament using an endoscopically assisted technique via a 3-cm skin incision in 10 adult cadavers (20 sides). Measurements were also made of the depth from the skin to the suprascapular ligament.

Results

A mean depth of 4 cm was necessary to reach the suprascapular ligament from the skin surface. With the authors' approach, no obvious injury occurred to the suprascapular or other vicinal neurovascular structures (such as the spinal accessory nerve and suprascapular vessels).

Conclusions

The results of this cadaveric study demonstrate that access to the suprascapular nerve can be obtained endoscopically via a small suprascapular incision. This approach obviates a large incision, entry into the glenohumeral joint, and reduces the risk of spinal accessory nerve injury in the posterior cervical triangle, or atrophy of the trapezius or supraspinatus muscles from a standard larger dissection. To the authors' knowledge an endoscopically assisted approach to decompressing the suprascapular nerve as it courses deep to the suprascapular ligament has not been reported previously.

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Cuong J. Bui, R. Shane Tubbs, Gigi Pate, Traci Morgan, Douglas C. Barnhart, Leslie Acakpo-Satchivi, John C. Wellons III, W. Jerry Oakes and Jeffrey P. Blount

Object

There is conflicting information in the literature regarding the increased risk of ventriculoperitoneal (VP) shunt infection after abdominal surgical procedures such as gastrostomy tube (GT) placement and Nissen fundoplication (NF) in the young patient. To further elucidate this potential association, the authors reviewed their institutional experience with such cases.

Methods

The authors retrospectively reviewed the records of all patients with shunted hydrocephalus who also underwent NF procedures and/or GT placements over a 9-year period.

Results

During this 9-year period, 3065 cerebrospinal fluid (CSF) shunts were placed and 1630 NF procedures/GT placements were performed. Ninety-six patients were identified who received both a CSF shunt and NF procedures/GT placements. Seventy-nine patients had a functioning VP shunt (without recent [< 6 months] CSF infection or shunt revision) at the time of their abdominal procedure (NF procedure or GT placement). Of this latter cohort, there were 38 males and 41 females with a mean age of 6.4 months; 12.7% of these patients developed a shunt infection and 60% of these infections were due to Staphylococcus species.

Conclusions

Based on this study, an NF procedure/GT placement in a child with a VP shunt appears to carry approximately the same risk of shunt infection as a shunt placement operation. Moreover, the microbiology observed in these patients with infection did not differ significantly from the usual microorganisms responsible for VP shunt infections in infants/neonates. Finally, but not conclusively, an open NF procedure/GT placement may carry a higher infection risk than a laparoscopic-assisted NF procedure/GT placement.

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Jeffrey P. Blount, R. Shane Tubbs, John C. Wellons III, Leslie Acakpo-Satchivi, David Bauer and W. Jerry Oakes

✓ In certain highly selected circumstances, division of a distally nonfunctional or dysfunctional cord can be a means of definitive untethering that spares and protects more rostral neurological function and results in definitive untethering. The authors reviewed their institutional experience with such cases and evaluated the limited literature. Based on their experience, treatment can be effective in carefully selected patients who undergo spinal cord transection, and the rate of repetitive tethered spinal cord can be decreased. Although uncommon, spinal cord transection appears to be an effective therapy in carefully selected patents with symptoms of repetitive cord tethering.