Matthew J. McGirt, Vivek Mehta, Giannina Garces-Ambrossi, Oren Gottfried, Can Solakoglu, Ziya L. Gokaslan, Amer Samdani and George I. Jallo
Tethered cord syndrome (TCS) is frequently associated with scoliosis in the pediatric population. Following spinal cord untethering, many patients continue to experience progression of spinal deformity. However, the incidence rate, time course, and risk factors for scoliosis progression following tethered cord release remain unclear. The aim of this study was to determine factors associated with scoliosis progression and whether tethered cord release alone would halt curve progression in pediatric TCS.
The authors retrospectively reviewed 27 consecutive pediatric cases of spinal cord untethering associated with scoliosis. The incidence rate and factors associated with scoliosis progression (> 10° increased Cobb angle) after untethering were evaluated using the Kaplan-Meier method.
The mean age of the patients was 8.9 years. All patients underwent cord untethering for lower-extremity weakness, back and leg pain, or bowel and bladder changes. Mean ± SD of the Cobb angle at presentation was 41 ± 16°. The cause of the spinal cord tethering included previous myelomeningocele repair in 14 patients (52%), fatty filum in 5 (18.5%), lipomeningocele in 3 (11%), diastematomyelia in 2 (7.4%), arthrogryposis in 1 (3.7%), imperforate anus with an S-2 hemivertebra in 1 (3.7%), and lipomyelomeningocele with occult dysraphism in 1 (3.7%). Mean follow-up was 6 ± 2 years. Twelve patients (44%) experienced scoliosis progression occurring a median of 2.4 years postoperatively and 8 (30%) required subsequent fusion for progression. At the time of untethering, scoliosis < 40° was associated with a 32% incidence of progression, whereas scoliosis > 40° was associated with a 75% incidence of progression (p < 0.01). Patients with Risser Grades 0–2 were also more likely to experience scoliosis progression compared with Risser Grades 3–5 (p < 0.05). Whereas nearly all patients with Risser Grades 0–2 with curves > 40° showed scoliosis progression (83%), 54% of patients with Risser Grades 0–2 with curves < 40° progressed, and no patients with Risser Grades 3–5 with curves < 40° progressed following spinal cord untethering.
In this experience with pediatric TCS-associated scoliosis, patients with Risser Grades 3–5 and Cobb angles < 40° did not experience curve progression after tethered cord release. Patients with Risser Grades 0–2 and Cobb angles > 40° were at greatest risk of curve progression after cord untethering. Pediatric patients with TCSassociated scoliosis should be monitored closely for curve progression using standing radiographs after spinal cord untethering, particularly those with curves > 40° or who have Risser Grades 0–2.
Case report with histological and molecular differentiation of the tumors and review of the literature
Peter Kan, Oren Gottfried, Deborah T. Blumenthal, Jeannette J. Townsend, Ela Drozd-Borysiuk, Arthur R. Brothman and Randy L. Jensen
✓ Multiple metastatic brain tumors and multifocal primary brain tumors of a single histological type are well described in the literature. The concurrent presence of multiple primary brain tumors with different histological characteristics, however, is very rare. The authors describe the first known case in which an oligodendroglioma and a juvenile pilocytic astrocytoma (JPA) presented as synchronous primary brain tumors in the same patient.
This 43-year-old man presented with a 2-month history of progressive headaches, nausea, and vomiting. Magnetic resonance imaging demonstrated an enhancing heterogeneous right medial cerebellar lesion and a larger calcified, nonenhancing, heterogeneous right frontal lesion with surrounding edema and a mass effect. The results of a metastatic workup were unremarkable.
The patient underwent an initial right frontotemporal craniotomy and a subsequent suboccipital craniectomy 2 years later for resection of the posterior fossa lesion. Histological examination revealed the frontal and cerebellar lesions to be an oligodendroglioma and JPA, respectively. A molecular analysis detected a deletion of chromosome 1p36 in the oligodendroglioma, but not in the JPA. After the initial operation, the patient received follow-up care for his oligodendroglioma, but eventually required temozolomide for tumor progression. His condition remains stable both neurologically and according to imaging studies.
The authors describe the first known case in which a low-grade oligodendroglioma and a JPA presented as synchronous primary brain tumors. They review the literature on multiple primary brain tumors with different histological characteristics and discuss potential mechanisms for the development of these lesions.