Ruth E. Bristol

Spinal cord ependymoma: Radical surgical resection and outcome

OBJECTIVE Several authors have noted increased neurological deficits and worsening dysesthesia in the postoperative period in patients with spinal cord ependymoma. We describe the neurological progression and pain evolution of these patients over the 1-year period after surgery. In addition, our favored method of en bloc tumor resection is illustrated, and the rate of complications, recurrence, and survival in this group of patients is addressed. METHODS We operated on 26 patients (12 male and 14 female) with low-grade spinal cord ependymomas between 1975 and 2001. The median age at diagnosis was 42 years. Tumors extended into the cervical cord in 13 patients, the thoracic cord in 7 patients, and the conus medullaris in 6 patients. Eleven patients had previous surgery and/or radiation therapy. RESULTS We achieved a gross total resection in 88% of patients, whereas 8% had a subtotal resection and 4% had a biopsy. Only 1 patient developed a recurrence over a mean follow-up period of 31 months. CONCLUSION We conclude that radical surgical resection of spinal cord ependymomas can be safely achieved in the majority of patients. A trend toward neurological improvement from a postoperative deficit can be expected between 1 and 3 months after surgery and continues up to 1 year. Postoperative dysesthesias begin to improve within 1 month of surgery and are significantly better by 1 year after surgery. The best predictor of outcome is the preoperative neurological status.

TREATMENT OPTIONS FOR THIRD VENTRICULAR COLLOID CYSTS: COMPARISON OF OPEN MICROSURGICAL VERSUS ENDOSCOPIC RESECTION

OBJECTIVEWe retrospectively reviewed our experience treating third ventricular colloid cysts to compare the efficacy of endoscopic and transcallosal approaches. METHODSBetween September 1994 and March 2004, 55 patients underwent third ventricular colloid cyst resection. The transcallosal approach was used in 27 patients; the endoscopic approach was used in 28 patients. Age, sex, cyst diameter, and presence of hydrocephalus were similar between the two groups. RESULTSThe operating time and hospital stay were significantly longer in the transcallosal craniotomy group compared with the endoscopic group. Both approaches led to reoperations in three patients. The endoscopic group had two subsequent craniotomies for residual cysts and one repeat endoscopic procedure because of equipment malfunction. The transcallosal craniotomy group had two reoperations for fractured drainage catheters and one operation for epidural hematoma evacuation. The transcallosal craniotomy group had a higher rate of patients requiring a ventriculoperitoneal shunt (five versus two) and a higher infection rate (five versus none). Intermediate follow-up demonstrated more small residual cysts in the endoscopic group than in the transcallosal craniotomy group (seven versus one). Overall neurological outcomes, however, were similar in the two groups. CONCLUSIONCompared with transcallosal craniotomy, neuroendoscopy is a safe and effective approach for removal of colloid cysts in the third ventricle. The endoscope can be considered a first-line treatment for these lesions, with the understanding that a small number of these patients may need an open craniotomy to remove residual cysts.

Management of Cranial Base Chondrosarcomas

OBJECTIVE: Chondrosarcomas are rare, infiltrative, progressive lesions that occur at the cranial base. Their intimate association with cranial nerves and major vessels of the head and neck often precludes complete surgical resection. METHODS: Between 1983 and 2003, 23 patients (14 females, 9 males) were treated at our institution with the diagnosis of chondrosarcoma of the cranial base (mean age at presentation, 43 yr). A retrospective chart review was performed to evaluate presentation, management, and adjunctive treatment. All living patients were contacted for a current examination and disease status. RESULTS: The 23 patients underwent 43 surgical resections. Follow-up ranged from 8 months to 25 years (mean, 97 mo). Ten patients underwent various adjuvant radiation therapies. Five patients have died. Four patients have no evidence of disease, and 13 have residual tumor. One was lost to follow-up. Of 14 patients with 5 years of follow-up, 13 are living. Therefore, the absolute 5 year survival rate is 93%. The 10 year survival rate is 71%. CONCLUSION: Because of the intricate nature of the cranial base, a team approach is preferable for managing these challenging lesions. Maximum cytoreductive surgery should be pursued as an initial strategy to minimize neurological injury. Adjuvant stereotactic radiosurgery can be used to treat residual disease or small recurrences. This cohort also illustrates that patients with chondrosarcomas have better long-term survival rates than patients with chordomas of the cranial base.

Cavernous malformations of the brainstem presenting in childhood: surgical experience in 40 patients.

BACKGROUND: Brainstem cavernous malformations (BSCMs) are believed to compose 9% to 35% of all cerebral cavernous malformations, but these lesions have been reported in children in very limited numbers. OBJECTIVE: To review surgical outcomes of pediatric patients with BSCMs treated at 1 institution. METHODS: We retrospectively analyzed the course of 40 pediatric patients (19 males, 21 females; age range, 10 months to 18.9 years; mean, 12.3 years) who underwent surgery between 1984 and 2009. Age, sex, presenting symptoms, location of lesion, surgical approach, new postoperative deficits, Glasgow Outcome Scale score, recurrences, and resolution of baseline symptoms were recorded. RESULTS: Thirty-nine patients experienced hemorrhage before surgery. Lesion locations included the pons (n = 22), midbrain (n = 4), midbrain and thalamus (n = 4), pontomesencephalic junction (n = 3), medulla (n = 3), pontomedullary junction (n = 3), and cervicomedullary junction (n = 1). Mean lesion size was 2.3 cm. Mean length of hospital stay was 10.7 days. The average clinical follow-up was 31.9 months in 36 patients with follow-up after discharge. At last follow-up, 5 patients had experienced symptoms and/or imaging consistent with rehemorrhage, either from a residual that enlarged or true recurrence (5.25% annual rebleed risk per patient after surgery); 2 required reoperation for further resection of cavernoma. Mean Glasgow Outcome Scale score was 4.2 on admission, 4.05 at discharge, and 4.5 at latest follow-up. Preoperative symptoms and deficits improved in 16 patients (40%). New neurological deficits developed in 19 patients (48%) and resolved in 9, leaving 10 patients (25%) with new permanent deficit. CONCLUSION: Compared with adults, pediatric patients with BCSMs tend to have larger lesions and higher rates of recurrence (regrowth of residual lesion). Given the greater life expectancy of children, surgical treatment seems warranted in those with surgically accessible lesions that have bled. Outcomes were similar to those in our adult series of patients with BSCMs.

Pars screw fixation of a hangman's fracture: technical case report.

OBJECTIVE AND IMPORTANCE: Traumatic spondylolisthesis of the axis may be treated by external immobilization or surgical fixation. CLINICAL PRESENTATION: We report the case of a 23-year-old man who sustained an Effendi Type II fracture of the axis, for which halo immobilization did not provide adequate stability. INTERVENTION: The unstable fracture was treated by placing lag screws in the pars interarticularis of C2, which reduced the fracture directly but sacrificed no normal spinal motion. The patient developed a solid fusion, and cervical alignment was normal at his 6-month follow-up examination. CONCLUSION: Although this technique has been reported previously, it is more commonly used in multilevel cervical fusions than for stand-alone repair of C2. Management options, anatomy, and technical considerations for the treatment of traumatic spondylolisthesis are reviewed.

Evaluation of operative procedures for symptomatic outcome after decompression surgery for Chiari type i malformation

The wide spectrum of symptoms and radiographic findings in patients with Chiari I malformation makes the decision to proceed with intervention controversial. We evaluated symptomatic outcomes using diverse surgical techniques in 104 patients who underwent decompression surgery. The symptoms of most patients improved. Patients with syringomyelia showed less symptomatic improvement; however, syringomyelia was not associated with postoperative symptomatic worsening. Durotomy was performed in 97.1% and arachnoid opening was performed in 60.6% with visualization of the fourth ventricle in 51.9% of patients. Neither arachnoid opening nor fourth ventricle visualization was associated with the clinical outcome. Duraplasty was performed in 94.2% of patients. A Chiari plate was used in 13.4% of patients and was associated with favorable outcomes. Use of postoperative steroids or muscle relaxants was not associated with outcome. Syringomyelia showed a 62.5% improvement rate on postoperative MRI. In conclusion, bony decompression and dural opening are important aspects of Chiari I surgery, with symptomatic improvement observed in most patients.

Transvenous to arterial Onyx embolization

Background/objective Transarterial infusion of Onyx is now widely used in the treatment of arteriovenous malformations (AVMs) and dural arteriovenous fistulas (DAVFs). Transvenous Onyx embolization is rarely performed, and usually in cases of carotid cavernous fistulas. We describe a novel technique of transvenous Onyx embolization in the treatment of three high risk DAVFs and one vein of Galen (VOG) AVM. Methods Cases were evaluated with specific attention to technical caveats, clinical indications, and complications. Patients were treated during 2011–2012 by two operators (FCA and CGM). Standard transfemoral venous approaches were employed in order to obtain access into the draining venous system in all cases. Results Four female patients, aged 5 months to 68 years, were treated. Two patients, one presenting with headache and the other with a bruit, demonstrated high risk DAVFs involving the transverse–sigmoid sinuses. One patient, who was asymptomatic, manifested a high risk ethmoidal DAVF that was discovered incidentally during treatment of a basilar aneurysm. The final patient presented with symptoms of a pineal region mass and was found to have a VOG AVM. In all cases, a microcatheter was navigated through the draining venous pouch and into the ostium of an arterial feeder. A single Onyx infusion from this position filled multiple arterial feeders and resulted in cures in the first three patients and a significant reduction in AVM nidus in the fourth patient. No complications occurred. Conclusions Transvenous Onyx infusion into a single arterial feeder can produce retrograde embolization of numerous other arterial feeders supplying high risk DAVFs and complex cerebral AVMs. This technique appears to be safe and potentially curative in select cases.

Supracerebellar infratentorial endoscopically controlled resection of pineal lesions: case series and operative technique

OBJECT The heterogeneous clinical manifestations and operative characteristics of pathological entities in the pineal region represent a significant challenge in terms of patient selection and surgical approach. Traditional surgical options have included endoscopic transventricular resection; open supratentorial microsurgical approaches through the midline, choroidal fissure, lateral ventricle, and tentorium; and supracerebellar infratentorial (SCIT) approaches through the posterior fossa. The object of the current study was to review the preoperative characteristics and outcomes for a cohort of patients treated purely via the novel endoscopically controlled SCIT approach. METHODS A single-institution series of 9 consecutive patients (4 male and 5 female patients [10 total cases]; mean age 21 years, range 6-37 years) treated via the endoscopically controlled SCIT approach for a pathological entity in the pineal region was retrospectively reviewed. The mean follow-up time was 13.2 months. RESULTS The endoscopically controlled SCIT approach was successfully used to approach a variety of pineal lesions, including pineal cysts (6 patients), epidermoid tumor, WHO Grade II astrocytoma (initial biopsy and recurrence), and malignant mixed germ cell tumor (1 patient each). Gross-total resection and/or adequate cyst fenestration was achieved in 8 cases. Biopsy with conservative debulking was performed for the single case of low-grade astrocytoma and again at the time of recurrence. The mean preoperative tumor and cyst volumes were 9.9 ± 4.4 and 3.7 ± 3.2 cm(3), respectively. The mean operating times were 212 ± 71 minutes for tumor cases and 177 ± 72 minutes for cysts. Estimated blood loss was less than 150 ml for all cases. A single case (pineal cyst) was converted to an open microsurgical approach to enhance visualization. There were no operative complications, as well as no documented CSF leaks, additional CSF diversion procedures, or air emboli. Seven patients underwent concomitant third ventriculostomy into the quadrigeminal cistern. At the time of the last follow-up evaluation, all patients had a stable or improved modified Rankin Scale score. CONCLUSIONS The endoscopically controlled SCIT approach may be used for the biopsy and resection of appropriately selected solid tumors of the pineal region, in addition to the fenestration and/or resection of pineal cysts. Preoperative considerations include patient presentation, anticipated disease and vascularity, degree of local venous anatomical distortion, and selection of optimal paramedian trajectory.

Surgical and Endovascular Treatment of Pediatric Spinal Arteriovenous Malformations

OBJECTIVE Pediatric spinal arteriovenous malformations (AVMs) are rare and complex lesions to treat. There are few reports of the endovascular and microsurgical treatment of these lesions in the pediatric population, and the treatment outcomes of these patients are not well described. The aim of this study was the clinical and radiographic outcomes of spinal AVMs in pediatric patients treated via endovascular and microsurgical modalities. METHODS We identified nine children (5 boys, 4 girls; average age 11 years, range 3-17 years) treated for spinal AVMs between 1998 and 2010. Their charts were reviewed. RESULTS Spinal AVMs most frequently involved the thoracic spinal cord. Four patients had associated Klippel-Trènaunay-Weber syndrome and one had hereditary hemorrhagic telangiectasia. There were two intramedullary, four conus medullaris, and three mixed extradural-intradural lesions. The most common presenting signs and symptoms were subarachnoid hemorrhage (n = 3) and paraparesis (n = 5). Endovascular intervention was used exclusively in two patients, and combined endovascular and microsurgical intervention was used in four patients. Surgery was the sole treatment in three patients with excellent results. There were two treatment-related complications: one case of subarachnoid hemorrhage and one case of scrotal swelling. The mean follow-up was 28.5 months and the median was 8 months (range, 1-65 months). The mean pretreatment World Health Organization (WHO)/Zubrod score was 2.4 (range, 1-4), and the mean post-treatment score was 1.4 (range, 0-4). One patient (11%) had a recurrence. CONCLUSIONS Pediatric spinal AVMs require complex combined microsurgical and endovascular techniques to achieve favorable outcomes.

Complex dural arteriovenous fistula in Bannayan-Riley-Ruvalcaba syndrome

In this paper the authors report the case of a complex dural arteriovenous fistula (dAVF) with high-risk features in a 14-year-old girl with Bannayan-Riley-Ruvalcaba syndrome (BRRS), a phosphatase and tensin homolog-associated syndrome, presenting with signs and symptoms of increased intracranial pressure (ICP) that had previously been attributed to pseudotumor cerebri. This fistula was obliterated following 2 stages of embolization, and the patient experienced immediate symptomatic improvement. At the 2-month follow-up evaluation, the fistula remained angiographically occluded, and her symptoms continue to improve. This is the third reported case of an intracranial dAVF in a patient with BRRS. Because high-risk dAVFs can result in devastating morbidity, early detection with vascular imaging is crucial for patients with BRRS presenting with signs of increased ICP. Goals of treatment should include complete fistula obliteration whenever possible.