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Combined ventral and dorsal rhizotomies for dystonic and spastic extremities

Report of six cases

A. Leland Albright and Elizabeth C. Tyler-Kabara

✓Six children with secondary dystonia as the primary movement disorder in their extremities but with coexisting spasticity were treated with combined ventral and dorsal rhizotomies, resulting in long-term improvement in their dystonia and no adverse side effects. Combined rhizotomies can be considered in the treatment of children who are not candidates for intrathecal baclofen, particularly severely disabled children who have dystonia and spasticity in their extremities, but hypotonia in their neck and trunk.

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Erratum: Bilateral subthalamic nucleus deep brain stimulation for dopa-responsive dystonia in a 6-year-old child 

Case report 

Elizabeth C. Tyler-Kabara and Douglas Kondziolka

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Editorial: Sphenoidal encephaloceles

Elizabeth C. Tyler-Kabara

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Prevalence of tethered spinal cord in infants with VACTERL

Clinical article

Brent R. O'Neill, Alexander K. Yu, and Elizabeth C. Tyler-Kabara

Object

The term VACTERL represents a nonrandom association of birth defects including vertebral malformations, anal atresia, cardiac anomalies, tracheoesophageal fistulas (TEFs), renal anomalies, and limb malformations. Clinical experience and a few published case series suggest that a tethered spinal cord (TSC) occurs commonly in children with VACTERL, but to date, no study has defined the prevalence of TSC in patients with VACTERL. Such information would guide decisions about the appropriateness of screening spinal imaging.

Methods

The authors reviewed the charts of all patients discharged from the neonatal intensive care unit at Children's Hospital Pittsburgh in the past 14 years with the diagnosis of VACTERL, TEF, or anal atresia. During that period, the authors' protocol has been to use spinal ultrasound to screen this population for TSC. The charts were reviewed for the presence of a TSC requiring surgery and for the features of VACTERL.

Results

Thirty-three patients with VACTERL and adequate spinal imaging studies were identified. In 13 (39%) of these, a TSC requiring surgery was identified. Among patients without VACTERL, the incidence of TSC was 7.9% in those with anal atresia and 2.4% in those with TEF. False-negative ultrasounds were identified in 21.4% of patients with TSC.

Conclusions

Children with VACTERL should undergo MR imaging screening for TSC. In infants with anal atresia without VACTERL, the incidence of TSC is much lower than in those with VACTERL.

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The role of cell therapy for stroke

Douglas Kondziolka, Lawrence Wechsler, Elizabeth Tyler-Kabara, and Cristian Achim

Cellular therapy has been evaluated in small animals, subhuman primates, and now humans for the potential repair of brain injury due to stroke. Experimental striate stroke models have proven useful for the purpose of evaluating different treatment paradigms. Early clinical trials involving neuronal transplantation in patients suffering motor-related stroke in the basal ganglia region have begun.

This research will be described in this report.

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Unusual findings during abdominal placement of a ventriculoperitoneal shunt

Report of three cases

R. Shane Tubbs, Elizabeth C. Tyler-Kabara, John C. Wellons III, Jeffrey P. Blount, and W. Jerry Oakes

✓The authors present three cases of infants born with myelodysplasia. Each infant underwent closure of a myelomeningocele and within 2 to 4 days placement of a ventriculoperitoneal (VP) shunt. In each case, on opening the peritoneal cavity, the authors observed egress of a dark or creamy dark fluid. None of the patients had a history of abdominal birth trauma. The decision was made to continue the procedures and send samples of the unusual fluids to the laboratory for culture and analysis. The cultures proved to be nondiagnostic and the characteristics of the fluid samples were most consistent with those of blood-tinged chyle. The authors hypothesize that, occasionally, the mechanical tautness that is created with repair of myelomeningoceles is sufficient to rupture small lymphatic vessels and accompanying blood vessels of the abdomen. An alternative hypothesis is that abdominal compression due to closure of the myelomeningocele may temporarily compress the liver, leading to raised intraportal pressures and resulting in weeping of chyle from the gastrointestinal tract. This abnormal fluid accumulation did not lead to chronic ascites, VP shunt infection, or dysfunction at long-term follow-up examination and abdominal visceral function has not been an issue.

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Endoscopic endonasal skull base surgery in the pediatric population

Clinical article

Srinivas Chivukula, Maria Koutourousiou, Carl H. Snyderman, Juan C. Fernandez-Miranda, Paul A. Gardner, and Elizabeth C. Tyler-Kabara

Object

The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies.

Methods

A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed.

Results

A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eighty-five patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up.

Conclusions

Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population.

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Stereotactic radiosurgery for residual neurocytoma

Report of four cases

Elizabeth Tyler-Kabara, Douglas Kondziolka, John C. Flickinger, and L. Dade Lunsford

✓ The purpose of this report was to review the results of stereotactic radiosurgery in the management of patients with residual neurocytomas after initial resection or biopsy procedures. Four patients underwent stereotactic radiosurgery for histologically proven neurocytoma. Clinical and imaging studies were performed to evaluate the response to treatment.

Radiosurgery was performed to deliver doses to the tumor margin of 14, 15, 16, and 20 Gy, depending on tumor volume and proximity to critical adjacent structures. More than 3 years later, imaging studies revealed significant reductions in tumor size. No new neurological deficits were identified at 53, 50, 42, and 38 months of follow up. The authors' initial experience shows that stereotactic radiosurgery appears to be an effective treatment for neurocytoma.

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Bilateral subthalamic nucleus deep brain stimulation for dopa-responsive dystonia in a 6-year-old child

Case report

Matthew J. Tormenti, Nestor D. Tomycz, Keith A. Coffman, Douglas Kondziolka, Donald J. Crammond, and Elizabeth C. Tyler-Kabara

Tyrosine hydroxylase (TH) deficiency is a rare autosomal recessive metabolic disease that results in the decreased production of catecholamines. Standard treatment relies on combinations of levodopa and carbidopa, anticholinergic agents, serotonergic agonists, and monamine oxidase B inhibitors. Unfortunately, severely affected children often require escalating doses of medication and suffer from dyskinesias as well as significant on/off symptomatology.

The authors present a case of medically intractable dopa-responsive dystonia in a 6-year-old boy whose condition significantly improved with bilateral subthalamic nucleus deep brain stimulation. This case is unique in its novel approach to tyrosine hydroxylase deficiency and the young age of the patient.

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MRI-guided laser interstitial thermal therapy using the Visualase system and Navigus frameless stereotaxy in an infant: technical case report

Jonathan J. Lee, Dave Clarke, Eric Hoverson, Elizabeth C. Tyler-Kabara, and Winson S. Ho

Laser interstitial thermal therapy (LITT) is increasingly used as a surgical option for the treatment of epilepsy. Placement of the laser fibers relies on stereotactic navigation with cranial fixation pins. In addition, the laser fiber is stabilized in the cranium during the ablation using a cranial bolt that assumes maturity of the skull. Therefore, younger infants (< 2 years of age) have traditionally not been considered as candidates for LITT. However, LITT is an appealing option for patients with familial epilepsy syndromes, such as tuberous sclerosis complex (TSC), due to the multiplicity of lesions and the likely need for multiple procedures. A 4-month-old infant with TSC presented with refractory focal seizures despite receiving escalating doses of 5 antiepileptic medications. Electrographic and clinical seizures occurred numerous times daily. Noninvasive evaluations, including MRI, magnetoencephalography, scalp EEG, and SPECT, localized the ictal onset to a left frontal cortical tuber in the premotor area. In this paper, the authors report a novel technique to overcome the challenges of performing LITT in an infant with an immature skull by repurposing the Navigus biopsy skull mount for stereotactic placement of a laser fiber using electromagnetic-based navigation. The patient underwent successful ablation of the tuber and remained seizure free 4 months postoperatively. To the authors’ knowledge, this is the youngest reported patient to undergo LITT. A safe method is described to perform LITT in an infant using commonly available tools without dedicated instrumentation beyond standard stereotactic navigation, a biopsy platform, and the Visualase system.