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Erin N. Kiehna and Robert J. Bollo

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Erin N. Kiehna, Catherine F. McClung-Smith and Robert J. Bollo

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Robert J. Bollo, Judith L. Gooch and Marion L. Walker

Continuous infusion of baclofen is a treatment option for severe generalized dystonia. Catheter insertion within the third ventricle has been described as an alternative to standard intrathecal placement to maximize intracranial concentrations of baclofen. The authors describe their experience with a novel technique for stereotactic endoscopic insertion of baclofen infusion catheters in the third ventricle in 3 patients with severe secondary generalized dystonia. Insertion was successful in all 3 patients, and all of them experienced significant improvement in dystonia scores on the Barry-Albright Dystonia Scale. Follow-up ranged from 5.5 to 7 months (mean 6 months), and no mechanical complications or CSF leaks were observed. The stereotactic endoscopic insertion of a baclofen infusion catheter into the third ventricle appears to be a safe method for continuous intraventricular baclofen infusion in patients with generalized secondary dystonia.

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Sarah T. Garber, Robert J. Bollo and Jay K. Riva-Cambrin

Pediatric spinal pilomyxoid astrocytoma (PMA) is an extremely rare tumor that merits recognition as a specific, unique entity. The authors present the case of an intramedullary PMA in the thoracic spinal cord of an 11-year-old boy who presented with back pain, scoliosis, and multiple lung nodules. The patient underwent T5–11 laminoplasty and near-total resection of the spinal tumor. The final pathological diagnosis was WHO Grade II PMA. The patient did well for 14 months until the tumor progressed both clinically and radiographically. A literature review focusing on the clinical characteristics, histology, and treatment of PMAs provides a better understanding of these rare lesions. Because of the small number of cases optimal treatment guidelines have not been established, but gross-total resection and adjuvant chemotherapy with alkylating agents appear to confer a better long-term prognosis. Pediatric patients with PMAs can remain recurrence free at least 5 years after surgery, although these tumors may disseminate or dedifferentiate into more malignant gliomas. Recognition of intramedullary PMA as a unique entity in children is vital to the development of specific surgical and adjuvant treatment regimens.

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Michael Karsy, Daxa M. Patel and Robert J. Bollo

Magnetic resonance imaging–guided stereotactic laser ablation of intracranial targets, including brain tumors, has expanded dramatically over the past decade, but there have been few reports of complications, especially those occurring in a delayed fashion. Laser ablation of subependymal giant cell astrocytomas (SEGAs) is an attractive alternative to maintenance immunotherapy in some children with tuberous sclerosis complex (TSC); however, the effect of treatment on disease progression and the nature and frequency of potential complications remains largely unknown. The authors report the case of a 5-year-old boy with TSC who underwent stereotactic laser ablation of a SEGA at the right foramen of Monro on 2 separate occasions. After the second ablation, immediate posttreatment MRI revealed gadolinium extravasation from the tumor into the lateral ventricle. Nine months later, the patient presented with papilledema and delayed obstructive hydrocephalus secondary to intraventricular adhesions causing a trapped right lateral ventricle. This was successfully treated with endoscopic septostomy. The authors discuss the potential cause and clinical management of a delayed complication not previously reported after a relatively novel surgical therapy.

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Loyola V. Gressot, Akash J. Patel, Robert J. Bollo, Carrie A. Mohila and Andrew Jea

Juvenile xanthogranuloma (JXG) is a rare disease that is part of a spectrum of histiocytic dendritic cell disorders. The authors report an unusual case of a 6-week-old male who presented with seizures. Neuroimaging revealed disseminated intracranial disease involving the optic apparatus, basal ganglia, lateral ventricles, and brainstem. The patient did not have any cutaneous lesions or evidence of extracranial disease. The patient underwent open biopsy of a large right midbrain lesion; pathology was consistent with JXG. He underwent postoperative chemotherapy and is doing well 7 months after surgery with regression of the intracranial lesions. To the best of the authors' knowledge, this is the first report of a neonate with disseminated intracranial JXG without cutaneous stigmata.

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Matthew J. Kole, Jared S. Fridley, Andrew Jea and Robert J. Bollo

Currarino syndrome is a rare constellation of congenital anomalies characterized by the triad of sacral dysgenesis, presacral mass, and anorectal malformation. It is frequently associated with other congenital anomalies, often including occult spinal dysraphism. Mutations in the MNX1 gene are identified in the majority of cases. The authors report a rare case of Currarino syndrome in an infant with tethered cord syndrome and a dorsal lipomyelomeningocele continuous with a presacral intradural spinal lipoma, in addition to an imperforate anus and a scimitar sacrum. They review the literature to highlight patterns of occult spinal dysraphism in patients with Currarino syndrome and their relationship to tethered cord syndrome. Approximately 60% of the patients with Currarino syndrome reported in the literature have an occult spinal dysraphism. Published studies suggest that the risk of tethered cord syndrome may be higher among patients with a lipoma and lower among those with a teratoma or anterior meningocele.

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Akash J. Patel, Ahilan Sivaganesan, Robert J. Bollo, Alison Brayton, Thomas G. Luerssen and Andrew Jea

Object

Recent attempts to control health care costs focus on reducing or eliminating payments for complications, hospital-acquired conditions, and provider preventable conditions, with payment restrictions applied uniformly. A patient's preexisting comorbidities likely influence the perioperative complication incidence. This relationship has not previously been examined in pediatric neurosurgery.

Methods

The authors conducted a retrospective assessment of prospectively collected relevant patient comorbidities and morbidity and mortality events at a large pediatric neurosurgical unit over a 5-year period. The authors examined the impact of specific comorbidities and the cumulative effect of multiple comorbidities on complication incidence.

Results

A total of 1990 patients underwent 3195 procedures at the authors' institution during the 5-year study period. Overall, 396 complications were analyzed; 298 patients (15.0%) experienced at least one complication. One or more comorbidities were present in 45.9% of patients. Renal comorbidities were clearly associated with the increased incidence of complications (p = 0.02), and they were specifically associated with infection (p = 0.006). Neurological comorbidities had a borderline association with complications (p = 0.05), and they were specifically associated with death (p = 0.037). A patient's having more comorbidities did not correlate with an increased risk of a perioperative complication (p = 0.8275).

Conclusions

The complication incidence in pediatric neurosurgery is variable and may be influenced by the type of neurosurgical procedure and patient-related factors. While patient-related factors beyond the control of the provider can significantly impact complications and hospital-acquired conditions in pediatric neurosurgery, an increasing number of comorbidities do not correlate with an increased risk of complications per patient.

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Heather S. Spader, Robert J. Bollo, Christian A. Bowers and Jay Riva-Cambrin

OBJECTIVE

Intrathecal baclofen infusion systems to manage severe spasticity and dystonia are associated with higher infection rates in children than in adults. Factors unique to this population, such as poor nutrition and physical limitations for pump placement, have been hypothesized as the reasons for this disparity. The authors assessed potential risk factors for infection in a multivariate analysis.

METHODS

Patients who underwent implantation of a programmable pump and intrathecal catheter for baclofen infusion at a single center between January 1, 2000, and March 1, 2012, were identified in this retrospective cohort study. The primary end point was infection. Potential risk factors investigated included preoperative (i.e., demographics, body mass index [BMI], gastrostomy tube, tracheostomy, previous spinal fusion), intraoperative (i.e., surgeon, antibiotics, pump size, catheter location), and postoperative (i.e., wound dehiscence, CSF leak, and number of revisions) factors. Univariate analysis was performed, and a multivariate logistic regression model was created to identify independent risk factors for infection.

RESULTS

A total of 254 patients were evaluated. The overall infection rate was 9.8%. Univariate analysis identified young age, shorter height, lower weight, dehiscence, CSF leak, and number of revisions within 6 months of pump placement as significantly associated with infection. Multivariate analysis identified young age, dehiscence, and number of revisions as independent risk factors for infection.

CONCLUSIONS

Young age, wound dehiscence, and number of revisions were independent risk factors for infection in this pediatric cohort. A low BMI and the presence of either a gastrostomy or tracheostomy were not associated with infection and may not be contraindications for this procedure.

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Vijay M. Ravindra, Michael Karsy, Richard H. Schmidt, Philipp Taussky, Min S. Park and Robert J. Bollo

The authors report the case of a previously healthy 6-month-old girl who presented with right arm and leg stiffening consistent with seizure activity. An initial CT scan of the head demonstrated acute subarachnoid hemorrhage in the basal cisterns extending into the left sylvian fissure. Computed tomography angiography demonstrated a 7 × 6 × 5–mm saccular aneurysm of the inferior M2 division of the left middle cerebral artery. The patient underwent left craniotomy and microsurgical clip ligation with wrapping of the aneurysm neck because the vessel appeared circumferentially dysplastic in the region of the aneurysm. Postoperative angiography demonstrated a small remnant, sluggish distal flow, but no significant cerebral vasospasm. Fifty-five days after the initial aneurysm rupture, the patient presented again with an acute intraparenchymal hemorrhage of the left anterior temporal lobe. Angiogram revealed a circumferentially dysplastic superior division of the M2 branch, with a new 5 × 4–mm saccular aneurysm distinct from the first, with 2 smaller aneurysms distal to the new ruptured aneurysm. Endovascular parent vessel occlusion with Onyx was performed. Genetic testing revealed a mutation of the MYH11. To the authors' knowledge, this is the first report of rapid de novo aneurysm formation in an infant with an MYH11 mutation. The authors review the patient's clinical presentation and management and comprehensively review the literature on this topic.