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  • Journal of Neurosurgery: Pediatrics x
  • By Author: Luerssen, Thomas G. x
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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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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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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.

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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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Joshua J. Chern, Charles G. Macias, Andrew Jea, Daniel J. Curry, Thomas G. Luerssen and William E. Whitehead

Object

Patients with CSF shunts often present to the emergency department (ED) with suspected shunt malfunction. Timely assessment and treatment are important factors affecting patient outcomes. A protocol was implemented at a tertiary children's hospital ED to expedite the care of these patients. This study evaluated the effectiveness of this protocol.

Methods

The protocol assigned all patients with CSF shunts into 1 of 3 pathways. If a patient presented with altered mental status, the Cushing triad, acute focal neurological deficit, ongoing seizure activity, or severe dehydration due to emesis, an ED physician was immediately notified (emergency pathway). If a patient presented with emesis, headache, increasing frequency of seizure, or parental concern for shunt malfunction, the patient entered the expedited pathway, and imaging studies were ordered prior to physician evaluation. All other patients entered the default pathway, in which a physician would evaluate the patient before deciding on further workup. Outcomes of interest included measures of timeliness in the ED and clinical outcomes. Comparisons were made between preprotocol and protocol periods and among the 3 pathways.

Results

The total time to complete both ED physician evaluation and to initiate imaging studies was significantly shorter in the protocol period than in the preprotocol period (104 vs 147 minutes). Similar time saving over the 2 processes was demonstrated comparing expedited and default pathways during the protocol period (95 vs 134 minutes, a 29% difference). Clinically, more patients underwent surgery in the expedited pathway than the default pathway (36% vs 17%), and patients in the expedited pathway had a shorter hospital stay (3.4 ± 0.9 days vs 5.7 ± 4.0 days; p = 0.02).

Conclusions

An ED-based protocol helped identify patients at risk for shunt failure early in the triage process and shortened the assessment process prior to neurosurgical intervention. Improving the timeliness of care for patients with shunt failure is important because morbidity and mortality associated with shunt failure are time dependent.

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Akash J. Patel, Benjamin D. Fox, Daniel H. Fulkerson, Sasidhar Yallampalli, Anna Illner, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen and Andrew Jea

Posterior reversible encephalopathy syndrome (PRES) has been described in the setting of malignant hypertension, renal disease, eclampsia, and immunosuppression. In addition, a single case of intraoperative (posterior fossa craniotomy) PRES has been reported; however, this case occurred in an adult.

The authors present a clinically and radiographically documented case of intraoperative PRES complicating the resection of a posterior fossa tumor in a 6-year-old child. During tumor resection, untoward force was used to circumferentially dissect the tumor, and excessive manipulation of the brainstem led to severe hypertension for a 10-minute period. An immediate postoperative MR image was obtained to rule out residual tumor, but instead the image showed findings consistent with PRES. Moreover, the patient's postoperative clinical findings were consistent with PRES.

Aggressive postoperative management of blood pressure and the institution of anticonvulsant therapy were undertaken. The patient made a good recovery; however, he required a temporary tracheostomy and tube feedings for prolonged lower cranial nerve dysfunction.

Posterior reversible encephalopathy syndrome can occur as a result of severe hypertension during surgery, even among young children. With prompt treatment, the patient in the featured case experienced significant clinical and radiographic recovery.

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Andrew Jea, Ernesto Coscarella, Murali Chintagumpala, Meena Bhattacharjee, William E. Whitehead, Daniel J. Curry and Thomas G. Luerssen

Multiple metastatic brain tumors and multifocal primary brain tumors of a single histological type have been published in the adult and pediatric literature. However, the simultaneous occurrence of multiple primary brain tumors with different cell types is rare. Even more rare is the pediatric presentation of multiple primary brain tumors with different cell types.

The authors describe the case of an 8-year-old boy who presented with a 2-week history of progressive headache, nausea and vomiting, and imbalance. Brain MR imaging demonstrated a heterogeneously enhancing mixed solid/cystic mass of the left cerebellar hemisphere and a larger, midline, more homogeneously enhancing lesion of the superior vermis. Spinal MR imaging was unremarkable.

The patient underwent a suboccipital craniotomy and subsequent gross-total resection of both mass lesions. Pathological examination revealed the left cerebellar and superior vermian lesions to be a juvenile pilocytic astrocytoma and a medulloblastoma, respectively. The patient did well in the immediate postoperative period, was discharged home, and underwent neurooncological follow-up.

To the best of the authors' knowledge, they describe the first known pediatric case in which a medulloblastoma and a juvenile pilocytic astrocytoma presented as synchronous primary brain tumors. They review the literature on multiple primary brain tumors with different histological characteristics and rehash potential mechanisms for their development.