Lara L. Cohen, Brian W. Yang, Nora P. O’Neill, Mark R. Proctor, Michael P. Glotzbecker and Daniel J. Hedequist
Patients with trisomy 21 (Down syndrome; DS) often have atlantoaxial instability (AAI), which, if severe, causes myelopathy and neurological deterioration. Children with DS and AAI who undergo cervical spine fusion have a high rate of nonunion requiring revision surgery. Recombinant human bone morphogenetic protein–2 (rhBMP-2) is a TGF-β growth factor that is used to induce bone formation in spine fusion. Although previous studies in the adult population have reported no reduction in pseudarthrosis rates with the use of rhBMP-2, there is a lack of literature in the pediatric DS population. This study describes the use of rhBMP-2 in children with DS and AAI during revision to treat nonunion.
A retrospective review of a cervical spine fusion database (n = 175) was conducted. This database included all cervical spine fusions using modern instrumentation at the authors’ institution from 2002 to 2019. Patients with DS who underwent a revision utilizing rhBMP-2 were included in the study. The number of prior fusions, use of rhBMP-2 in fusions, length of stay, halo use, and surgical data were collected. Postoperative complications and length of follow-up were also recorded.
Eight patients (75% female) met the inclusion criteria. The average age at revision with rhBMP-2 was 11 years (range 3–19 years). All patients were diagnosed with nonunion after an initial cervical fusion. All revisions were posterior fusions of C1–2 (n = 2) or occiput to cervical (n = 6). All revisions included implant revisions, iliac crest bone grafting, and rhBMP-2 use. One patient required irrigation and debridement of an rhBMP-induced seroma. Another patient required return to the operating room to repair a dural tear. There were no neurological, infectious, airway, or implant-related complications. Revision utilizing rhBMP-2 achieved fusion in 100% (n = 8) of patients. The average length of follow-up was 42.6 months. All patients demonstrated solid fusion mass on the last radiograph.
This is the first case series reporting the successful use of rhBMP-2 to facilitate cervical spine fusion in patients with DS after previous nonunion. In addition, few rhBMP-2–related postoperative complications occurred.
Seunggu J. Han, Isaac Yang, Jose J. Otero, Brian J. Ahn, Tarik Tihan, Michael W. McDermott, Mitchel S. Berger, Susan M. Chang and Andrew T. Parsa
Gliosarcoma can arise secondarily, after conventional adjuvant treatment of high-grade glioma. The current literature on the occurrence of secondary gliosarcoma (SGS) after glioblastoma multiforme (GBM) is limited, with only 12 reported cases. The authors present a large series of histologically confirmed SGSs, with follow-up to describe the clinical and radiological presentation, pathological diagnosis, and treatment outcomes.
Gliosarcoma cases were identified using the University of California, San Francisco's Departments of Neurological Surgery and Neuropathology databases. Through a retrospective chart review, cases of gliosarcoma were considered SGS if the following inclusion criteria were met: 1) the patient had a previously diagnosed intracranial malignant glioma that did not have gliosarcoma components; and 2) the histopathological tissue diagnosis of the recurrence confirmed gliosarcoma according to the most current WHO criteria. Extensive review of clinical, surgical, and pathology notes was performed to gather clinical and pathological data on these cases.
Thirty consecutive patients in whom SGS had been diagnosed between 1996 and 2008 were included in the analysis. All patients had previously received a diagnosis of malignant glioma. For the initial malignant glioma, all patients underwent resection, and 25 patients received both external-beam radiation and chemotherapy. Three patients received radiotherapy alone, 1 patient was treated with chemotherapy alone, and 1 patient's tumor rapidly recurred as gliosarcoma, requiring surgical intervention prior to initiation of adjuvant therapy. The median time from diagnosis of the initial tumor to diagnosis of gliosarcoma was 8.5 months (range 0.5–25 months). All but 1 patient (who only had a biopsy) underwent a second operation for gliosarcoma; 8 patients went on to receive radiotherapy (4 had brachytherapy, 3 had external-beam radiation, and 1 had Gamma Knife surgery); and 14 patients received additional chemotherapy. The median length of survival from the time of gliosarcoma diagnosis was 4.4 months (range 0.7–46 months). The median survival from the time of the original GBM diagnosis was 12.6 months (range 5.7–47.4 months). Patients who had received concurrent and adjuvant temozolomide for GBM had worse outcomes than those who had not (4.3 and 10.5 months, respectively; p = 0.045). There was no difference in time to diagnosis of gliosarcoma in these 2 groups (8 and 8.5 months; p = 0.387). Two patients who had not received radiation therapy for GBM had an anecdotally very prolonged survival (20.9 and 46.4 months).
The data underscore the difficulty associated with management of this disease. The strikingly poor survival of patients with SGS who had previously received combined radiation and temozolomide chemotherapy for GBM may reflect a unique molecular profile of GBM that eventually recurs as SGS. Further work will be required, controlling for multiple prognostic factors with larger numbers of patients.
Hillary Shurtleff, Molly Warner, Andrew Poliakov, Brian Bournival, Dennis W. Shaw, Gisele Ishak, Tong Yang, Mahesh Karandikar, Russell P. Saneto, Samuel R. Browd and Jeffrey G. Ojemann
The authors describe their experience with functional MR (fMR) imaging in children as young as 5 years of age, or even younger in developmental age equivalent. Functional MR imaging can be useful for identifying eloquent cortex prior to surgical intervention. Most fMR imaging clinical work has been done in adults, and although children as young as 8 years of age have been included in larger clinical series, cases in younger children are rarely reported.
The authors reviewed presurgical fMR images in eight patients who were 8 years of age or younger, six of whom were 5 or 6 years of age. Each patient had undergone neuropsychological testing. Three patients functioned at a below-average level, with adaptive functioning age scores of 3 to 4 years. Self-paced finger tapping (with passive movement in one patient) and silent language tasks were used as activation tasks. The language task was modified for younger children, for whom the same (not novel) stimuli were used for extensive practice ahead of time and in the MR imaging unit. Patient preparation involved techniques such as having experienced staff present to work with patients and providing external management during imaging. Six of eight patients had extensive training and practice prior to the procedure. In the two youngest patients, this training included use of a mock MR unit.
All cases yielded successful imaging. Finger tapping in all seven of the patients who could perform it demonstrated focal motor activation in the frontal-parietal region, with expected activation elsewhere, including in the cerebellum. Three of four patients had the expected verb generation task activations, with left-hemisphere dominance, including a 6-year-old child who functioned at the 3-year, 9-month level. The only child (an 8-year-old) who was not prepared prior to the imaging session for the verb generation task failed this task due to movement artifact.
Despite the challenges of successfully using fMR imaging in very young and clinically involved patients, these studies can be performed successfully in children with a chronological age of 5 or 6 years and a developmental age as young as 3 or 4 years.
2010 AANS Annual Meeting Philadelphia, Pennsylvania May 1–5, 2010
Phoenix, Arizona • March 6–9, 2013