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Virendra R. Desai, Aditya Vedantam, Sandi K. Lam, Lucia Mirea, Stephen T. Foldes, Daniel J. Curry, P. David Adelson, Angus A. Wilfong, and Varina L. Boerwinkle

OBJECTIVE

Determining language laterality in patients with intractable epilepsy is important in operative planning. Wada testing is the gold standard, but it has a risk of stroke. Both Wada and task-based functional MRI (tb-fMRI) require patient cooperation. Recently, resting-state fMRI (rs-fMRI) has been explored for language lateralization. In the present study, the correlation between rs-fMRI and tb-fMRI in language lateralization is estimated in a pediatric population with intractable epilepsy.

METHODS

rs-fMRI and tb-fMRI language lateralization testing performed as part of epilepsy surgery evaluation was retrospectively reviewed.

RESULTS

Twenty-nine patients underwent rs-fMRI and tb-fMRI; a total of 38 rs-fMRI studies and 30 tb-fMRI studies were obtained. tb-fMRI suggested left dominance in 25 of 30 cases (83%), right in 3 (10%), and in 2 (7%) the studies were nondiagnostic. In rs-fMRI, 26 of 38 studies (68%) suggested left dominance, 3 (8%) right dominance, 6 (16%) bilateral, and 3 (8%) were nondiagnostic. When tb-fMRI lateralized to the left hemisphere (25 cases), rs-fMRI was lateralized to the left in 23 patients (92%) and it was bilateral/equal in 2 (8%). When tb-fMRI lateralized to the right (3 cases), rs-fMRI lateralized to the right in all cases (100%). The overall concordance rate was 0.93 (95% CI 0.76–0.99) when considering cases with tb-fMRI and rs-fMRI performed within 6 months of each other, and tb-fMRI results were not nondiagnostic.

CONCLUSIONS

rs-fMRI significantly correlated with tb-fMRI in lateralizing language and suggests the potential role for identifying hemispheric dominance via rs-fMRI. Further investigation and validation studies are warranted.

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Virendra R. Desai, Jeffrey S. Raskin, Arvind Mohan, JoWinsyl Montojo, Valentina Briceño, Daniel J. Curry, and Sandi Lam

OBJECTIVE

Intrathecal baclofen pumps are generally placed in children for the treatment of spasticity and dystonia. Use of implants in this pediatric population with comorbidities is reported to have a high risk of complications and infections. With the aim of reducing baclofen pump–related infections, a quality improvement project was instituted at the authors’ institution.

METHODS

A workflow paradigm unique to baclofen pump implantation aimed at decreasing implant-related infections was implemented. All baclofen pump operations performed at the authors’ institution between August 2012 and June 2016 were reviewed. An infection prevention protocol was created and implemented in August 2014 based on a literature review and the consensus opinion of the pediatric neurosurgeons in the group. Compliance with the prevention bundle was tracked. Case outcomes before and after implementation of the protocol with a minimum of 3 months of follow-up were retrospectively reviewed. Univariate and multivariate analyses were performed to assess the association of the steps in the prevention bundle with infection and complication outcomes.

RESULTS

A total of 128 baclofen pump surgeries were performed (64 preprotocol and 64 postprotocol). The patient age range was 3 to 27 years. The overall compliance rate with the infection prevention bundle was 82%. The pre- and postimplementation infection rates were 12.5% and 6.3%, respectively (p = 0.225). The total pre- and postimplementation complication rates were 23.4% and 9.4%, respectively (p = 0.032). The absolute and relative risk reductions for infections were 6.3% (95% CI 3.8%–16.3%) and 50%, respectively; for complications, the absolute and relative risk reductions were 14.1% (95% CI 1.5%–26.7%) and 60%, respectively.

CONCLUSIONS

The total complication rate following intrathecal baclofen pump surgery was significantly lower after implementation of the quality improvement protocol. This study is an example of using checklist standardization to diminish special cause variability.

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Rory R. Mayer, Steven W. Hwang, Gaddum D. Reddy, David L. Morales, William E. Whitehead, Daniel J. Curry, Robert J. Bollo, Thomas G. Luerssen, and Andrew Jea

Object

Left ventricular assist devices (LVADs) are continuous or pulsatile flow devices that could potentially be life-saving measures for patients with end-stage heart failure. These devices have clear advantages over extracorporeal membrane oxygenation (ECMO) and are often used in adults. They are only recently being commonly used in the pediatric age group. As the use of LVADs becomes more mainstream in children, it is important to determine the complication profile associated with these devices. Furthermore, with the increasing application of LVADs in children, pediatric neurosurgeons are seeing a correlative increase in associated neurological complications. In this study, the authors reviewed the incidence of neurological complications due to LVAD use in the pediatric age group and the role of neurosurgery in treatment.

Methods

The authors examined data regarding patients with LVADs from the Texas Children's Hospital Heart Center database (July 01, 2007, to June 30, 2011) and recorded neurological complications requiring neurosurgical consultation. They identified 2 children who underwent craniotomies during LVAD treatment.

Results

Intracranial hemorrhage occurred in 3 (6.5%) of the 46 patients treated with an LVAD at the authors' institution. Of these patients, 2 were treated with craniotomies for life-threatening intracranial hemorrhages. The 3 patients in the neurosurgical cohort presented with cerebral infarction, decreased level of consciousness, and/or seizure. At the last follow-up (286, 503, and 550 days), 1 patient (Case 2) had no decline in neurological status, underwent a successful heart transplant, and was discharged home; 1 patient (Case 1) died of refractory cardiac failure; and 1 patient (Case 3) was on an LVAD for destination therapy (that is, the LVAD is not a bridge to transplantation but rather the final treatment). This patient was discharged from the hospital, but he died of overwhelming fungemia at 286 days while on VAD support.

Conclusions

Intracranial hemorrhage is a serious and feared complication of LVAD treatment. While the surgical risk is substantial due to systemic anticoagulation and significant medical comorbidities, neurosurgical evacuation of hemorrhage plays an important life-saving role that can yield successful and acceptable outcomes.

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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.

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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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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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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, 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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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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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.