✓Depressed skull fractures overlying the major venous sinus are often managed nonoperatively because of the high associated risks of surgery in these locations. In the presence of clinical and radiographic evidence of sinus occlusion, however, surgical therapy may be necessary. The authors present the case of a 9-year-old boy with a depressed skull fracture overlying the posterior third of the superior sagittal sinus. After initial conservative treatment, delayed signs of intracranial hypertension and a symptomatic tonsillar herniation with tonsillar necrosis developed. Possible causes as well as diagnostic and treatment options are reviewed.
John R. Vender and Karin Bierbrauer
Ben J. Bixenmann, Beth M. Kline-Fath, Karin S. Bierbrauer and Danesh Bansal
Syringomyelia can be diagnosed in isolation but is more commonly found in the presence of craniocervical junction anomalies or spinal dysraphism. The origin of syringomyelia has been hypothesized to be either congenital or acquired. The purpose of this study was to determine the incidence of syringomyelia within the fetal and postnatal population with neural tube defects (NTDs).
A review was performed of the authors' fetal MRI database of pregnancies with imaging between March 2004 and November 2011 for evaluation of an intrauterine anomaly detected via prenatal ultrasonography. Those cases with an NTD were then selected and a chart review was performed of all prenatal and postnatal imaging as well as available clinical history.
A total of 2362 fetal MRI examinations were performed, and 109 of these were patients with an NTD. Of the 2362 studies reviewed, 2 cases of fetal syringomyelia were identified. Both fetal syrinxes were identified in fetuses with CSF flow disturbances (1 case each of encephalocele and myelomeningocele). Both fetal MRI examinations were performed late in gestation, at 31 and 38 weeks, respectively. The patient with an encephalocele was excluded from the spinal NTD population; therefore a syrinx was identified in 0.08% (2/2362) of the entire population of fetuses who underwent MRI, or 0.9% (1/109) of fetuses with a spinal NTD. Sixty-three of the 109 patients with an NTD had postnatal clinical data available for review. Twenty-nine (46%) of 63 had a syrinx identified during the follow-up period. Of this group, 50 patients had an open NTD and 27 (54%) of 50 developed a syrinx. Among the patients with an open NTD who developed a syrinx, only 7% did not have or develop hydrocephalus, compared with 35% of the patients who did not develop a syrinx (p < 0.05). There were nonsignificantly more frequent shunt revisions among those patients who developed a syrinx, and a syrinx developed in all patients who required surgical Chiari malformation decompression or tethered cord release. The initial identification of a spinal cord syrinx varied greatly between patients, ranging from 38 weeks gestation to greater than 4 years of age.
These data suggest that syringomyelia is not a congenital embryonic condition. A syrinx was not identified in fetuses who underwent imaging for other intrauterine anomalies. In the population of patients with NTDs who are known to be at high risk for developing syringomyelia, the pathology was only identified in 2 third-trimester fetuses or postnatally, typically in the presence of hydrocephalus, shunt placement, Chiari malformation decompression, or tethered cord release. The study supports the authors' hypothesis that a syrinx is an acquired lesion, most likely due to the effects of abnormal CSF flow.
Michelle C. Caruso, Margot C. Daugherty, Suzanne M. Moody, Richard A. Falcone Jr., Karin S. Bierbrauer and Gary L. Geis
Methylprednisolone sodium succinate (MPSS) has been studied as a pharmacological adjunct that may be given to patients with acute spinal cord injury (ASCI) to improve neurological recovery. MPSS treatment became the standard of care in adults despite a lack of evidence supporting clinical benefit. More recently, new guidelines from neurological surgeon groups recommended no longer using MPSS for ASCI, due to questionable clinical benefit and known complications. However, little information exists in the pediatric population regarding MPSS use in the setting of ASCI. The aim of this paper was to describe steroid use and side effects in patients with ASCI at the authors’ Level 1 pediatric trauma center in order to inform other hospitals that may still use this therapy.
A retrospective chart review was conducted to determine adherence in ordering and delivery according to the guideline of the authors’ institution and to determine types and frequency of complications. Inclusion criteria included age < 17 years, blunt trauma, physician concern for ASCI, and admission for ≥ 24 hours or treatment with high-dose intravenous MPSS. Exclusion criteria included penetrating trauma, no documentation of ASCI, and incomplete medical records. Charts were reviewed for a predetermined list of complications.
A total of 602 patient charts were reviewed; 354 patients were included in the study. MPSS was administered in 59 cases. In 34 (57.5%) the order was placed correctly. In 13 (38.2%) of these 34 cases, MPSS was administered according to the recommended timeline protocol. Overall, only 13 (22%) of 59 patients received the therapy according to protocol with regard to accurate ordering and administration.
Among the patients with ASCI, 20 (55.6%) of the 36 who received steroids had complications, which was a significantly higher rate than in those who did not receive steroids (8 [24.2%] of 33, p = 0.008). Among the patients without ASCI, 10 (43.5%) of the 23 who received steroids also experienced significantly more complications than patients who did not receive steroids (50 [19.1%] of 262, p = 0.006).
High-dose MPSS for ASCI was not delivered to pediatric patients according to protocol with a high degree of reliability. Patients receiving steroids for pediatric ASCI were significantly more likely to experience complications than patients not receiving steroids. The findings presented, including complications of steroid use, support removal of high-dose MPSS as a treatment option for pediatric ASCI.
Ellen L. Air, Weihong Yuan, Scott K. Holland, Blaise V. Jones, Karin Bierbrauer, Mekibib Altaye and Francesco T. Mangano
The goal in this study was to compare the integrity of white matter before and after ventriculoperitoneal (VP) shunt insertion by evaluating the anisotropic diffusion properties with the aid of diffusion tensor (DT) imaging in young children with hydrocephalus.
The authors retrospectively identified 10 children with hydrocephalus who underwent both pre- and postoperative DT imaging studies. The DT imaging parameters (fractional anisotropy [FA], mean diffusivity, axial diffusivity, and radial diffusivity) were computed and compared longitudinally in the splenium and genu of the corpus callosum (gCC) and in the anterior and posterior limbs of the internal capsule (PLIC). The patients' values on DT imaging at the pre- and postshunt stages were compared with the corresponding age-matched controls as well as with a large cohort of healthy children in the database.
In the gCC, 7 of 10 children had abnormally low preoperative FA values, 6 of which normalized postoperatively. All 3 of the 10 children who had normal preoperative FA values had normal FA values postoperatively as well. In the PLIC, 7 of 10 children had abnormally high FA values, 6 of which normalized postoperatively, whereas the other one had abnormally low postoperative FA. Of the remaining 3 children, 2 had abnormally low preoperative FA values in the PLIC; this normalized in 1 patient after surgery. The other child had a normal preoperative FA value that became abnormally low postoperatively. When comparing the presurgery frequency of abnormally low, normal, and abnormally high FA values to those postsurgery, there was a statistically significant longitudinal difference in both gCC (p = 0.02) and PLIC (p = 0.002).
In this first longitudinal DT imaging study of young children with hydrocephalus, DT imaging anisotropy yielded abnormal results in several white matter regions of the brain, and trended toward normalization following VP shunt placement.
Patrick C. Hsieh, Stephen L. Ondra, Andrew W. Grande, Brian A. O'Shaughnessy, Karin Bierbrauer, Kerry R. Crone, Ryan J. Halpin, Ian Suk, Tyler R. Koski, Ziya L. Gokaslan and Charles Kuntz IV
Recurrent tethered cord syndrome (TCS) has been reported to develop in 5–50% of patients following initial spinal cord detethering operations. Surgery for multiple recurrences of TCS can be difficult and is associated with significant complications. Using a cadaveric tethered spinal cord model, Grande and colleagues demonstrated that shortening of the vertebral column by performing a 15–25-mm thoracolumbar osteotomy significantly reduced spinal cord, lumbosacral nerve root, and terminal filum tension. Based on this cadaveric study, spinal column shortening by a thoracolumbar subtraction osteotomy may be a viable alternative treatment to traditional surgical detethering for multiple recurrences of TCS.
In this article, the authors describe the use of posterior vertebral column subtraction osteotomy (PVCSO) for the treatment of 2 patients with multiple recurrences of TCS. Vertebral column resection osteotomy has been widely used in the surgical correction of fixed spinal deformity. The PVCSO is a novel surgical treatment for multiple recurrences of TCS. In such cases, PVCSO may allow surgeons to avoid neural injury by obviating the need for dissection through previously operated sites and may reduce complications related to CSF leakage. The novel use of PVCSO for recurrent TCS is discussed in this report, including surgical considerations and techniques in performing PVCSO.
Jennifer M. Strahle, Rukayat Taiwo, Christine Averill, James Torner, Chevis N. Shannon, Christopher M. Bonfield, Gerald F. Tuite, Tammy Bethel-Anderson, Jerrel Rutlin, Douglas L. Brockmeyer, John C. Wellons III, Jeffrey R. Leonard, Francesco T. Mangano, James M. Johnston, Manish N. Shah, Bermans J. Iskandar, Elizabeth C. Tyler-Kabara, David J. Daniels, Eric M. Jackson, Gerald A. Grant, Daniel E. Couture, P. David Adelson, Tord D. Alden, Philipp R. Aldana, Richard C. E. Anderson, Nathan R. Selden, Lissa C. Baird, Karin Bierbrauer, Joshua J. Chern, William E. Whitehead, Richard G. Ellenbogen, Herbert E. Fuchs, Daniel J. Guillaume, Todd C. Hankinson, Mark R. Iantosca, W. Jerry Oakes, Robert F. Keating, Nickalus R. Khan, Michael S. Muhlbauer, J. Gordon McComb, Arnold H. Menezes, John Ragheb, Jodi L. Smith, Cormac O. Maher, Stephanie Greene, Michael Kelly, Brent R. O’Neill, Mark D. Krieger, Mandeep Tamber, Susan R. Durham, Greg Olavarria, Scellig S. D. Stone, Bruce A. Kaufman, Gregory G. Heuer, David F. Bauer, Gregory Albert, Jeffrey P. Greenfield, Scott D. Wait, Mark D. Van Poppel, Ramin Eskandari, Timothy Mapstone, Joshua S. Shimony, Ralph G. Dacey Jr., Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.
Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors’ goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.
A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).
Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB–C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.
Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.