✓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.
Report of six cases
A. Leland Albright and Elizabeth C. Tyler-Kabara
Elizabeth C. Tyler-Kabara and Douglas Kondziolka
Elizabeth C. Tyler-Kabara
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.
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.
Brent R. O'Neill, Alexander K. Yu and Elizabeth C. Tyler-Kabara
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.
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.
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.
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.
Jeffrey P. Blount, R. Shane Tubbs, Mamehri Okor, Elizabeth C. Tyler-Kabara, John C. Wellons III, Paul A. Grabb and W. Jerry Oakes
The authors describe the technique of transecting the spinal cord in children born with myelomeningocele who have undergone multiple detherings and are functionally paraplegic.
The authors' technique involves identifying the neural placode and sectioning the normal spinal cord just superior to this site. No postoperative complications have been identified in 14 patients undergoing this procedure over an 11-year period. No patient at last follow up was found to have symptoms referable to a tethered spinal cord. The advantage of this procedure is to excise the normally pia-coated cord, which is unlikely to retether compared with the neural placode, which is often covered with scar tissue and does not have a well-formed pial surface—hence, predisposing it to frequent dorsal adhesions.
The authors believe that this technique is of benefit in a small, carefully selected group of myelodys-plastic patients with repetitive tethering of the spinal cord.
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.
Maria Koutourousiou, Paul A. Gardner, Juan C. Fernandez-Miranda, Elizabeth C. Tyler-Kabara, Eric W. Wang and Carl H. Snyderman
The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging and controversial management dilemmas in neurosurgery. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for both pediatric and adult craniopharyngiomas. The object of the present study was to present the results of EES and analyze outcome in both the pediatric and the adult age groups.
The authors retrospectively reviewed the records of patients with craniopharyngioma who had undergone EES in the period from June 1999 to April 2011.
Sixty-four patients, 47 adults and 17 children, were eligible for this study. Forty-seven patients had presented with primary craniopharyngiomas and 17 with recurrent tumors. The mean age in the adult group was 51 years (range 28–82 years); in the pediatric group, 9 years (range 4–18 years). Overall, the gross-total resection rate was 37.5% (24 patients); near-total resection (> 95% of tumor removed) was 34.4% (22 patients); subtotal resection (≥ 80% of tumor removed) 21.9% (14 patients); and partial resection (< 80% of tumor removed) 6.2% (4 patients). In 9 patients, EES had been combined with radiation therapy (with radiosurgery in 6 cases) as the initial treatment. Among the 40 patients (62.5%) who had presented with pituitary insufficiency, pituitary function remained unchanged in 19 (47.5%), improved or normalized in 8 (20%), and worsened in 13 (32.5%). In the 24 patients who had presented with normal pituitary function, new pituitary deficit occurred in 14 (58.3%). Nineteen patients (29.7%) suffered from diabetes insipidus at presentation, and the condition developed in 21 patients (46.7%) after treatment. Forty-four patients (68.8%) had presented with impaired vision. In 38 (86.4%) of them, vision improved or even normalized after surgery; in 5, it remained unchanged; and in 1, it temporarily worsened. One patient without preoperative visual problems showed temporary visual deterioration after treatment. Permanent visual deterioration occurred in no one after surgery. The mean follow-up was 38 months (range 1–135 months). Tumor recurrence after EES was discovered in 22 patients (34.4%) and was treated with repeat surgery (6 patients), radiosurgery (1 patient), combined repeat surgery and radiation therapy (8 patients), interferon (1 patient), or observation (6 patients). Surgical complications included 15 cases (23.4%) with CSF leakage that was treated with surgical reexploration (13 patients) and/or lumbar drain placement (9 patients). This leak rate was decreased to 10.6% in recent years after the introduction of the vascularized nasoseptal flap. Five cases (7.8%) of meningitis were found and treated with antibiotics without further complications. Postoperative hydrocephalus occurred in 7 patients (12.7%) and was treated with ventriculoperitoneal shunt placement. Five patients experienced transient cranial nerve palsies. There was no operative mortality.
With the goal of gross-total or maximum possible safe resection, EES can be used for the treatment of every craniopharyngioma, regardless of its location, size, and extension (excluding purely intraventricular tumors), and can provide acceptable results comparable to those for traditional craniotomies. Endoscopic endonasal surgery is not limited to adults and actually shows higher resection rates in the pediatric population.
Elizabeth C. Tyler-Kabara, Amin B. Kassam, Michael H. Horowitz, Louise Urgo, Constantinos Hadjipanayis, Elad I. Levy and Yue-Fang Chang
Object. Microvascular decompression (MVD) has become one of the primary treatments for typical trigeminal neuralgia (TN). Not all patients with facial pain, however, suffer from the typical form of this disease; many patients who present for surgical intervention actually have atypical TN. The authors compare the results of MVD performed for typical and atypical TN at their institution.
Methods. The results of 2675 MVDs in 2264 patients were reviewed using information obtained from the department database. The authors examined immediate postoperative relief in 2003 patients with typical and 672 with atypical TN, and long-term follow-up results in patients for whom more than 5 years of follow-up data were available (969 with typical and 219 with atypical TN). Outcomes were divided into three categories: excellent, pain relief without medication; good, mild or intermittent pain controlled with low-dose medication; and poor, no or poor pain relief with large amounts of medication. The results for typical and atypical TN were compared and patient history and pain characteristics were evaluated for possible predictive factors.
Conclusions. In this study, MVD for typical TN resulted in complete postoperative pain relief in 80% of patients, compared with 47% with complete relief in those with atypical TN. Significant pain relief was achieved after 97% of MVDs in patients with typical TN and after 87% of these procedures for atypical TN. When patients were followed for more than 5 years, the long-term pain relief after MVD for those with typical TN was excellent in 73% and good in an additional 7%, for an overall significant pain relief in 80% of patients. In contrast, following MVD for atypical TN, the long-term results were excellent in only 35% of cases and good in an additional 16%, for overall significant pain relief in only 51%. Memorable onset and trigger points were predictive of better postoperative pain relief in both atypical and typical TN. Preoperative sensory loss was a negative predictor for good long-term results following MVD for atypical TN.