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Akash J. Patel, Jacob Cherian, Daniel H. Fulkerson, Benjamin D. Fox, Joshua J. Chern, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Andrew Jea

Object

Translaminar screw (TLS) fixation can be used safely and efficaciously for upper cervical fusion in children. No published studies have evaluated this technique in the thoracic spine of the pediatric population, and thus the authors undertook such an analysis.

Methods

The upper thoracic spines (T1–4) of 130 patients, consisting of 70 boys and 60 girls, were studied using CT scans. Laminar height and thickness, screw length, and screw angle were measured. Exclusion criteria included the following: patients older than 18 years of age, trauma or congenital abnormalities of the thoracic spine, or absent demographic information or imaging studies through T-4. Statistical analysis was performed using paired or unpaired Student t-tests (p < 0.05) and linear regression analysis.

Results

The mean laminar heights for T-1, T-2, T-3, and T-4 were as follows: 12.3 ± 3.4, 13.0 ± 3.5, 13.4 ± 3.8, and 14.7 ± 4.1 mm, respectively. The mean laminar widths were 6.5 ± 1.3, 6.6 ± 1.3, 6.6 ± 1.3, and 6.6 ± 1.4 mm, respectively. The mean screw lengths were 29.9 ± 4.1, 25.2 ± 3.5, 22.7 ± 3.2, and 21.6 ± 3.1 mm, respectively. The mean screw angles were 47° ± 4°, 48° ± 4°, 51° ± 4°, and 53° ± 5°, respectively. There were no significant differences between the right and left sides. However, significant differences were found when comparing patients younger than 8 years with those who were 8 years or older, and when comparing boys and girls.

Conclusions

Careful preoperative thin-cut CT with sagittal reconstruction is mandatory to determine if the placement of TLSs is feasible in the pediatric population. Based on CT analysis, the insertion of TLSs in the pediatric thoracic spine is possible in all patients older than 8 years and in many patients younger than 8 years. Boys could accept longer screws in the upper thoracic spine compared with girls.

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Daniel H. Fulkerson, Krishna B. Satyan, Lillian M. Wilder, James J. Riviello, Stephen A. Stayer, William E. Whitehead, Daniel J. Curry, Robert C. Dauser, Thomas G. Luerssen and Andrew Jea

Object

Neurophysiological monitoring of motor evoked potentials (MEPs) during complex spine procedures may reduce the risk of injury by providing feedback to the operating surgeon. While this tool is a well-established surgical adjunct in adults, clinical data in children are sparse. The purpose of this study was to determine the reliability and safety of MEP monitoring in a group of children younger than 3 years of age undergoing neurosurgical spine procedures.

Methods

A total of 10 consecutive spinal procedures in 10 children younger than 3 years of age (range 5–31 months, mean 16.8 months) were analyzed between January 1, 2008, and May 1, 2010. Motor evoked potentials were elicited by transcranial electric stimulation. A standardized anesthesia protocol for monitoring consisted of a titrated propofol drip combined with bolus dosing of fentanyl or sufentanil.

Results

Motor evoked potentials were documented at the beginning and end of the procedure in all 10 patients. A mean baseline stimulation threshold of 533 ± 124 V (range 321–746 V) was used. Six patients maintained MEP signals ≥ 50% of baseline amplitude throughout the surgery. There was a greater than 50% decrease in intraoperative MEP amplitude in at least 1 extremity in 4 patients. Two of these patients returned to baseline status by the end of the case. Two patients had a persistent decrement or variability in MEP signals at the end of the procedure; this correlated with postoperative weakness. There were no complications related to the technique of monitoring MEPs.

Conclusions

A transcranial electric stimulation protocol monitoring corticospinal motor pathways during neurosurgical procedures in children younger than 3 years of age was reliably and safely implemented. A persistent intraoperative decrease of greater than 50% in this small series of 10 pediatric patients younger than 3 years of age predicted a postoperative neurological deficit. The authors advocate routine monitoring of MEPs in this pediatric age group undergoing neurosurgical spine procedures.

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Jonathan G. Thomas, Steven W. Hwang, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Andrew Jea

Object

Lumbar disc herniation is rare in the pediatric age group, but may still cause a significant amount of pain and disability. Whereas minimally invasive surgery (MIS) for lumbar disc herniation is routinely performed in adults, it has not yet been described in the pediatric population. The purpose of this study was to describe the surgical results of pediatric MIS-treated lumbar disc disease.

Methods

The authors retrospectively reviewed a series of 6 consecutive cases of lumbar microdiscectomy performed using MIS techniques between April 2008 and July 2010. Presenting symptoms, physical examination findings, and preoperative MR imaging results were obtained from medical records. Perioperative results, including blood loss, length of hospital stay, and complications were assessed. Findings at latest follow-up evaluation were also recorded.

Results

This report represents the first surgical series regarding pediatric lumbar microdiscectomies performed using MIS. The mean patient age was 16 years (range 14–17 years); there were 4 girls and 2 boys. Preoperative signs and symptoms of radiculopathic pain were notable in 100% of patients, and myotomal weakness was noted in 33% of patients. The first line of treatment in all patients was a period of conservative management lasting an average of 11.5 months (range 6–12 months). The mean intraoperative blood loss was 10.8 ml, and the mean postoperative length of stay was 1.3 days. There were no complications in this small series.

Conclusions

The treatment of pediatric lumbar disc herniation by using MIS techniques can be safe and efficacious. However, further study with a larger number of patients and longer follow-up is needed to compare outcomes between MIS and open microdiscectomies.

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Steven W. Hwang, Jonathan G. Thomas, Todd J. Blumberg, William E. Whitehead, Daniel J. Curry, Robert C. Dauser, Thomas G. Luerssen and Andrew Jea

Object

Significant lumbar kyphosis is frequently observed in patients with myelomeningocele and has been associated with increasing functional impairment, decreased abdominal volume, respiratory impairment, discomfort, and skin ulcerations overlying the prominent gibbus. Treatment of severe kyphotic deformities can include kyphectomy, with or without ligation of the thecal sac, with posterior spinal fixation. However, most series have reported a high rate of morbidity and complications associated with surgical intervention for correction of kyphosis in patients with myelomeningocele. The authors describe a technique in which pedicle screw (PS)–only constructs are used without transection of the thecal sac to treat severe kyphosis successfully, with minimal morbidity.

Methods

The authors retrospectively reviewed medical records and radiographic images in 2 patients with myelomeningoceles in whom kyphectomies had been performed at the authors' institution between January 2007 and July 2010. They also reviewed the existing literature for case reports or published series of patients with myelomeningocele treated with kyphectomies, to evaluate the outcomes.

Results

Both patients were male and had thoracic-level myelomeningoceles that had been repaired at birth, with associated paraplegia. Neither patient had any significant scoliotic deformity associated with the kyphosis, and both had fixation from T-9 to the ilium, which was performed using PS constructs, along with L1–2 kyphectomies. The patient in Case 1 was 20 years old and was treated for progressive kyphosis and an ulcerated nonhealing wound over the gibbus. The patient in Case 2 was 10 years old and was treated for progressive pain and functional impairment. The 2 patients had a mean correction of 63%, with a mean correction of kyphotic deformity from 136° to 51°. Neither patient developed any complication in the short term postoperatively, whereas published series have reported high complication rates, including wound infection, poor wound healing, CSF leakage, pseudarthrosis, and shunt malfunction.

Conclusions

Severe kyphotic deformities in patients with myelomeningocele can be safely treated using PS-only constructs without ligation of the thecal sac. Further evaluation with a larger sample and longer follow-up are needed to detect any associated complications, such as proximal junctional kyphosis. Further evaluation may also validate whether PS-only constructs permit successful outcomes with a shorter construct and fewer instrumented levels.

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John R. W. Kestle, Jay Riva-Cambrin, John C. Wellons III, Abhaya V. Kulkarni, William E. Whitehead, Marion L. Walker, W. Jerry Oakes, James M. Drake, Thomas G. Luerssen, Tamara D. Simon and Richard Holubkov

Object

Quality improvement techniques are being implemented in many areas of medicine. In an effort to reduce the ventriculoperitoneal shunt infection rate, a standardized protocol was developed and implemented at 4 centers of the Hydrocephalus Clinical Research Network (HCRN).

Methods

The protocol was developed sequentially by HCRN members using the current literature and prior institutional experience until consensus was obtained. The protocol was prospectively applied at each HCRN center to all children undergoing a shunt insertion or revision procedure. Infections were defined on the basis of CSF, wound, or pseudocyst cultures; wound breakdown; abdominal pseudocyst; or positive blood cultures in the presence of a ventriculoatrial shunt. Procedures and infections were measured before and after protocol implementation.

Results

Twenty-one surgeons at 4 centers performed 1571 procedures between June 1, 2007, and February 28, 2009. The minimum follow-up was 6 months. The Network infection rate decreased from 8.8% prior to the protocol to 5.7% while using the protocol (p = 0.0028, absolute risk reduction 3.15%, relative risk reduction 36%). Three of 4 centers lowered their infection rate. Shunt surgery after external ventricular drainage (with or without prior infection) had the highest infection rate. Overall protocol compliance was 74.5% and improved over the course of the observation period. Based on logistic regression analysis, the use of BioGlide catheters (odds ratio [OR] 1.91, 95% CI 1.19–3.05; p = 0.007) and the use of antiseptic cream by any members of the surgical team (instead of a formal surgical scrub by all members of the surgical team; OR 4.53, 95% CI 1.43–14.41; p = 0.01) were associated with an increased risk of infection.

Conclusions

The standardized protocol for shunt surgery significantly reduced shunt infection across the HCRN. Overall protocol compliance was good. The protocol has established a common baseline within the Network, which will facilitate assessment of new treatments. Identification of factors associated with infection will allow further protocol refinement in the future.

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Steven W. Hwang, George Al-Shamy, William E. Whitehead, Daniel J. Curry, Robert Dauser, Thomas G. Luerssen and Andrew Jea

Endoscopic third ventriculostomy (ETV) is an accepted option in the treatment of obstructive hydrocephalus in children and is considered by many pediatric neurosurgeons to be the treatment of choice in this population. The procedure involves perforation of the floor of the third ventricle, specifically, the tuber cinereum, which is part of the hypothalamic-pituitary axis of cerebral endocrine regulation. Endocrine dysfunction, such as amenorrhea, weight gain, and precocious puberty, which are recognized only months to years after the procedure, may be underreported because patients and physicians may not relate the endocrine sequelae to the ETV. Few detailed reports of endocrinerelated complications following ETV exist to better understand these issues. In this study, the authors add to the literature with case descriptions of and correlative laboratory findings in 2 adolescent girls who underwent ETV for obstructive hydrocephalus and in whom amenorrhea subsequently developed.

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Steven W. Hwang, Jonathan G. Thomas, William E. Whitehead, Daniel J. Curry, Robert C. Dauser, Eugene S. Kim, Thomas G. Luerssen and Andrew Jea

Congenital long QT syndrome (LQTS) is a rare genetic ion transmembrane disorder that has been associated with multiple various genetic mutations including life-threatening cardiac arrhythmias and sudden death. Left thorascopic sympathectomy is an effective treatment for patients who are refractory to medical therapy or who need frequent epicardial internal cardiodefibrillator intervention. Although there is substantial literature about this therapy in adults, few reports detail the outcomes in children who undergo left thorascopic sympathectomies to treat LQTS.

The authors report the successful use of a left thoracic sympathectomy for the treatment of an 11-year-old girl who had persistently symptomatic LQTS, even after implantation of an automatic cardioverter-defibrillator. The patient remained clinically stable without arrhythmias through 6 months' of follow-up.

The authors also reviewed the relevant literature and found that it suggests that 77% of patients will have immediate resolution of their symptoms/arrhythmias after the procedure. When the outcome definition was broadened to include patients who had only 1 or 2 cardiac episodes in the follow-up period, 88% of cases were considered successful. The results of this case study and literature review suggest that left thorascopic sympathectomy is a safe and effective approach for treating pediatric patients with LQTS.

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Akash J. Patel, Jacob Cherian, Benjamin D. Fox, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Andrew Jea

Object

National and international meetings, such as the Congress of Neurological Surgeons (CNS) and the American Association of Neurological Surgeons (AANS) meetings, provide a central location for the gathering and dissemination of research. The purpose of this study was to determine the publication rates of both oral and poster presentations at CNS and AANS meetings in peer-reviewed journals.

Methods

The authors reviewed all accepted abstracts, presented as either oral or poster presentations, at the CNS and AANS meetings from 2003 to 2005. This information was then used to search PubMed to determine the rate of publication of the abstracts presented at the meetings. Abstracts were considered published if the data presented at the meeting was identical to that in the publication.

Results

The overall publication rate was 32.48% (1243 of 3827 abstracts). On average, 41.28% of oral presentations and 29.03% of poster presentations were eventually published. Of those studies eventually published, 98.71% were published within 5 years of presentation at the meeting. Published abstracts were published most frequently in the Journal of Neurosurgery and Neurosurgery.

Conclusions

Approximately one-third of all presentations at the annual CNS and AANS meetings will be published in peer-reviewed, MEDLINE-indexed journals. These meetings are excellent forums for neurosurgical practitioners to be exposed to current research. Oral presentations have a significantly higher rate of eventual publication compared with poster presentations, reflecting their higher quality. The Journal of Neurosurgery and Neurosurgery have been the main outlets of neurosurgical research from these meetings.

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Leonardo Rangel-Castilla, Steven W. Hwang, Andrew Jea, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Robert C. Dauser

Multiple-suture synostosis is typically associated with syndromic craniosynostosis but has been occasionally reported in large series of nonsyndromic children. The diagnosis of multiple fused sutures usually occurs at the same time, but rarely has the chronological development of a secondary suture synostosis been noted. The development of secondary bicoronal suture synostosis requiring surgical intervention has only been reported, to date, after surgical intervention and is hypothesized to arise from a disruption of inhibitory factors from the dura. The disinhibition of these factors permits the sutures to then fuse at an early stage. The authors report on a patient who developed secondary unilateral coronal synostosis after the diagnosis of an isolated sagittal synostosis. The secondary synostosis was identified at the time of the initial surgical intervention and ultimately required a second procedure of a frontoorbital advancement.

The clinical appearance of this phenomenon may be subtle, and surgeons should monitor for the presence of secondary synostosis during surgery as it may require intervention. Failure to identify the secondary synostosis may necessitate another surgery or result in a poor cosmetic outcome. The authors recommend close clinical follow-up for the short term in patients with isolated sagittal synostosis.

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Leonardo Rangel-Castilla, Steven W. Hwang, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Andrew Jea

Tuberculosis (TB) is a common disease worldwide that is caused by Mycobacterium tuberculosis. Tuberculosis of the spine, also called Pott disease, is the most common site of bony dissemination. Although children are disproportionately affected, spinal TB is nonetheless rare in very young children. Cases involving infants requiring surgical intervention have been previously reported, and they are often associated with greater management challenges given the technical difficulty with instrumentation in very young children.

This case involved a 3-year-old girl with TB centered at T-6, who presented with myelopathy from spinal cord compression and a severe kyphotic deformity (> 60°). She underwent a single-stage costotransversectomy for vertebral column resection, followed by reconstruction with an anterior expandable titanium cage and posterior pedicle screw instrumentation. At last follow-up, the patient was clinically and radiographically stable.

The authors report on the youngest patient with spinal TB treated surgically with this strategy and review the literature regarding prior cases involving young children. Although limited by the paucity of cases in the literature, surgical debridement and spinal fusion appear to provide a safe alternative to prolonged bed rest or casting and may offer additional benefits of a faster recovery and ambulation.