The authors describe 2 cases of triventricular hydrocephalus initially presenting as aqueductal stenosis that subsequently developed tumors of the pineal and tectal region. The first case resembled late-onset idiopathic aqueductal stenosis on serial imaging. Subsequent imaging revealed a new tumor in the pineal region causing mass effect on the midbrain. The second case presented in a more typical pattern of aqueductal stenosis during infancy. On delayed follow-up imaging, an enlarging tectal mass was discovered. In both cases hydrocephalus was successfully treated by cerebrospinal fluid diversion prior to tumor presentation. The differential diagnoses, diagnostic testing, and treatment course for these unusual cases are discussed. The importance of follow-up MRI in cases of idiopathic aqueductal stenosis is emphasized by these exemplar cases.
Jarod L. Roland, Richard L. Price, Ashwin A. Kamath, S. Hassan Akbari, Eric C. Leuthardt, Brandon A. Miller and Matthew D. Smyth
Aimen Kasasbeh, Edward C. Hwang, Karen Steger-May, S. Kathleen Bandt, Amy Oberhelman, David Limbrick, Michelle M. Miller-Thomas, Joshua S. Shimony and Matthew D. Smyth
Mesial temporal sclerosis (MTS) is widely recognized as a significant underlying cause of temporal lobe epilepsy. Magnetic resonance imaging is routinely used in the preoperative evaluation of children with epilepsy. The purpose of this study was to evaluate the prevalence, reliability, and prognostic value of MRI identification of MTS and MRI findings indicative of MTS in a series of patients who underwent resection of the medial temporal lobe for medically refractory epilepsy.
The authors reviewed the medical records and preoperative MRI reports of 25 patients who had undergone medial temporal resections (anterior temporal lobectomy or functional hemispherotomy) for medically intractable epilepsy. The preoperative MRI studies were presented for blinded review by 2 neuroradiologists who independently evaluated the radiographs for selected MTS features and provided a final interpretation. To quantify interrater agreement and accuracy, the findings of the 2 blinded neuroradiologists, the nonblinded clinical preoperative radiology report, and the final pathology interpretation were compared.
The preoperative MRI studies revealed MTS in 6 patients (24%), and histopathological analysis verified MTS in 8 (32%) of 25 specimens. Six MRI features of MTS were specifically evaluated: 1) increased hippocampal signal intensity, 2) reduced hippocampal size, 3) atrophy of the ipsilateral hippocampal collateral white matter, 4) enlarged ipsilateral temporal horn, 5) reduced gray-white matter demarcation in the temporal lobe, and 6) decreased temporal lobe size. The most prevalent feature of MTS identified on MRI was a reduced hippocampal size, found in 11 of the MRI studies (44%). Analysis revealed moderate interrater agreement for MRI identification of MTS between the 2 blinded neuroradiologists and the nonblinded preoperative report (Cohen κ 0.40–0.59). Interrater agreement was highly variable for different MTS features indicative of MTS, ranging from poor to near perfect. Agreement was highest for increased hippocampal signal and decreased temporal lobe size and was consistently poor for reduced gray-white matter demarcation. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and proportion perfect agreement were highest for increased hippocampal signal and reduced hippocampal size. An MRI finding of MTS was not predictive of seizure outcome in this small series.
Mesial temporal sclerosis identification on brain MRI in children evaluated for medial temporal resections has a PPV of 55%–67% and an NPV of 79%–87%. Increased hippocampal signal and reduced hippocampal size were associated with high predictive values, while gray-white differentiation and an enlarged temporal horn were not predictive of MTS. Seizure outcome following medial temporal resections was not associated with MRI findings of MTS or MRI abnormalities indicative of MTS in this small sample size.
Suresh N. Magge, Matthew D. Smyth, Lance S. Governale, Liliana Goumnerova, Joseph Madsen, Becca Munro, Stephen V. Nalbach, Mark R. Proctor, R. Michael Scott and Edward R. Smith
Discovery of a syrinx in a child, without a readily identifiable proximate cause such as a Chiari malformation, tumor, or site of tethering, is often a cause of concern for families and a source of consternation for clinicians. There is a paucity of data describing the natural history of an idiopathic syrinx in the pediatric population. The authors present the combined data of 2 major pediatric neurosurgical centers to describe their experience with this condition.
Data were collected at Children's Hospital Boston and St. Louis Children's Hospital according to institutional review board–approved protocols and captured visits over a 2.5-year interval (October 2006–March 2009), with records reviewed if the patient had a preexisting diagnosis of syrinx. Patients were identified by ICD-9 codes derived from departmental databases. All pediatric patients (age < 19 years) in whom idiopathic syrinx had been diagnosed, as defined by MR imaging findings (dilated central canal in the spinal cord of ≥ 1 mm in axial dimension and extending over at least 2 vertebral levels), were included.
Forty-eight patients met the criteria for idiopathic syrinx during this period, and in 32 of them detailed follow-up imaging was available. Discovery of a syrinx was incidental in 6 patients, whereas the others were referred for imaging because of the presence of pain, neurological symptoms, scoliosis, or skin markings. The average age at the first MR imaging session was 9.7 years, with a mean syrinx size of 4 mm (range 1.2–9.4 mm). The majority (52%) of patients had a thoracic syrinx, with the average lesion spanning 7.1 vertebral levels. The average follow-up was 23.8 months (range 2–64 months), and subgroups of patients with < 3 years and ≥ 3 years of follow-up were independently reviewed. Overall, symptoms improved in 34% and worsened in 9%; 57% of the patients remained asymptomatic or stable. Radiographically (in the subgroup of 32 patients with detailed follow-up imaging), syrinx size decreased in 25% of patients, increased in 12.5%, and remained unchanged in 62.5%, with no apparent correlation between change in syrinx size and clinical symptoms.
Clinically, children with an idiopathic syrinx remained asymptomatic, stable, or improved in 91% of cases. The majority of syringes (87.5%) remained stable or shrank over time, with no apparent correlation between changes in size and changes in symptoms. Although longer follow-up is needed, these data suggest that the natural history of an idiopathic syrinx in children is benign, and that repeated imaging may not be necessary.
Amy Lee, Andrea E. Van Pelt, Alex A. Kane, Thomas K. Pilgram, Daniel P. Govier, Albert S. Woo and Matthew D. Smyth
Deformational plagiocephaly (DP) is the leading cause of head shape abnormalities in infants. Treatment options include conservative measures and cranial molding. Pediatric neurosurgeons and craniofacial plastic surgeons have yet to agree on an ideal therapy, and no definable standards exist for initiating treatment with helmets. Furthermore, there may be differences between specialties in their perceptions of DP severity and need for helmet therapy.
Requests to participate in a web-based questionnaire were sent to diplomates of the American Board of Pediatric Neurological Surgery and US and Canadian members of the Pediatric Joint Section of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons and the American Cleft Palate–Craniofacial Association. Questions focused on educational background; practice setting; volume of DP patients; preferences for evaluation, treatment, follow-up; and incentives or deterrents to treat with helmet therapy. Six examples of varying degrees of DP were presented to delineate treatment preferences.
Requests were sent to 302 neurosurgeons and 470 plastic surgeons, and responses were received from 71 neurosurgeons (24%) and 64 plastic surgeons (14%). The following responses represented the greatest variations between specialties: 1) 8% of neurosurgeons and 26% of plastic surgeons strongly agreed with the statement that helmet therapy is more beneficial than conservative therapy (p < 0.01); and 2) 25% of neurosurgeons and 58% of plastic surgeons would treat moderate to severe DP with helmets (p < 0.01).
Survey responses suggest that neurosurgeons are less likely to prescribe helmet therapy for DP than plastic surgeons. Parents of children with DP are faced with a costly treatment decision that may be influenced more strongly by referral and physician bias than medical evidence.
Matthew D. Smyth, Penny K. Sneed, Samuel F. Ciricillo, Michael S. Edwards, William M. Wara, David A. Larson, Michael T. Lawton, Philip H. Gutin and Michael W. Mcdermott
Object. Stereotactic radiosurgery for arteriovenous malformations (AVMs) is an accepted treatment option, but few reports have been published on the results of this treatment in children. In this study the authors describe a series of pediatric patients with a minimum follow-up duration of 36 months.
Methods. From 1991 to 1997, 40 children (26 boys and 14 girls) with AVMs were treated with radiosurgery at the University of California at San Francisco (UCSF). Follow-up information was available for 31 children (20 boys and 11 girls) in whom the median age at initial treatment was 11.2 years (range 3.4–17.5 years). The median follow-up duration was 60 months (range 6–99 months). Sixteen percent of the AVMs were Spetzler—Martin Grade II; 68%, Grade III; 10%, Grade IV; and 6%, Grade V. The mean volume of the AVMs was 5.37 cm3 and the median volume was 1.6 cm3. The mean marginal dose of radiation was 16.7 Gy and the median dose was 18 Gy (range 12–19 Gy).
Angiography performed in 26 children confirmed obliteration of the AVM nidus in nine patients (35%), partial response in 16 patients (62%), and no response in one patient (4%). In five patients who refused angiography, magnetic resonance (MR) imaging revealed obliteration in two patients and partial response in three patients, bringing the overall obliteration rate associated with initial radiosurgery to 35%. Logistic regression analysis confirmed a significant correlation between marginal dose prescription and response (p = 0.025); in AVMs that received at least 18 Gy there was a 10-fold increase in the obliteration rate (63%) over AVMs that received a lower dose. Lesions smaller than 3 cm3 were associated with a sixfold increased obliteration rate (53%) over lesions larger than 3 cm3 (8%), but AVM volume was not a statistically significant predictor of response (p = 0.09). Twelve patients have since undergone repeated radiosurgery and are currently being followed up with serial MR imaging studies (in five cases, the AVM is now obliterated). During the follow-up period (1918 patient-months) there were eight hemorrhages in five patients, with a cumulative posttreatment hemorrhage rate of 3.2%/patient/year in the 1st year and a rate of 4.3%/patient/year over the first 3 years. There were two permanent neurological complications (6%) and no deaths in this study.
Conclusions. The lower overall obliteration rate reported in this series is most likely due to the larger mean AVM volumes treated at UCSF as well as conservative dose—volume prescriptions delivered to children. Significantly higher obliteration rates were observed when a marginal radiation dose of at least 18 Gy was delivered. The permanent complication rate is low and should encourage those treating children to use doses similar to those used in adults.
Matthew B. Potts, Sunil A. Sheth, Jonathan Louie, Matthew D. Smyth, Penny K. Sneed, Michael W. McDermott, Michael T. Lawton, William L. Young, Steven W. Hetts, Heather J. Fullerton and Nalin Gupta
Stereotactic radiosurgery (SRS) is an established treatment modality for brain arteriovenous malformations (AVMs) in children, but the optimal treatment parameters and associated treatment-related complications are not fully understood. The authors present their single-institution experience of using SRS, at a relatively low marginal dose, to treat AVMs in children for nearly 20 years; they report angiographic outcomes, posttreatment hemorrhage rates, adverse treatment-related events, and functional outcomes.
The authors conducted a retrospective review of 2 cohorts of children (18 years of age or younger) with AVMs treated from 1991 to 1998 and from 2000 to 2010.
A total of 80 patients with follow-up data after SRS were identified. Mean age at SRS was 12.7 years, and 56% of patients had hemorrhage at the time of presentation. Median target volume was 3.1 cm3 (range 0.09–62.3 cm3), and median prescription marginal dose used was 17.5 Gy (range 12–20 Gy). Angiograms acquired 3 years after treatment were available for 47% of patients; AVM obliteration was achieved in 52% of patients who received a dose of 18–20 Gy and in 16% who received less than 18 Gy. At 5 years after SRS, the cumulative incidence of hemorrhage was 25% (95% CI 16%–37%). No permanent neurological deficits occurred in patients who did not experience posttreatment hemorrhage. Overall, good functional outcomes (modified Rankin Scale Scores 0–2) were observed for 78% of patients; for 66% of patients, functional status improved or remained the same as before treatment.
A low marginal dose minimizes SRS-related neurological deficits but leads to low rates of obliteration and high rates of hemorrhage. To maximize AVM obliteration and minimize posttreatment hemorrhage, the authors recommend a prescription marginal dose of 18 Gy or more. In addition, SRS-related symptoms such as headache and seizures should be considered when discussing risks and benefits of SRS for treating AVMs in children.
Jakub Godzik, Michael P. Kelly, Alireza Radmanesh, David Kim, Terrence F. Holekamp, Matthew D. Smyth, Lawrence G. Lenke, Joshua S. Shimony, Tae Sung Park, Jeffrey Leonard and David D. Limbrick
Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I–associated syringomyelia. A secondary objective was to explore the role of craniovertebral junction (CVJ) characteristics as additional risk factors for scoliosis.
The authors conducted a retrospective review of pediatric patients evaluated for CM-I with syringomyelia at a single institution in the period from 2000 to 2012. Syrinx morphology and CVJ parameters were evaluated with MRI, whereas the presence of scoliosis was determined using standard radiographic criteria. Multiple logistic regression was used to analyze radiological features that were independently associated with scoliosis.
Ninety-two patients with CM-I and syringomyelia were identified. The mean age was 10.5 ± 5 years. Thirty-five (38%) of 92 patients had spine deformity; 23 (66%) of these 35 patients were referred primarily for deformity, and 12 (34%) were diagnosed with deformity during workup for other symptoms. Multiple regression analysis revealed maximum syrinx diameter > 6 mm (OR 12.1, 95% CI 3.63–40.57, p < 0.001) and moderate (5–12 mm) rather than severe (> 12 mm) tonsillar herniation (OR 7.64, 95% CI 2.3–25.31, p = 0.001) as significant predictors of spine deformity when controlling for age, sex, and syrinx location.
The current study further elucidates the association between CM-I and spinal deformity by defining specific radiographic characteristics associated with the presence of scoliosis. Specifically, patients presenting with larger maximum syrinx diameters (> 6 mm) have an increased risk of scoliosis.
Manish N. Shah, Jeffrey R. Leonard, Gabrielle Inder, Feng Gao, Michael Geske, Devon H. Haydon, Melvin E. Omodon, John Evans, Diego Morales, Ralph G. Dacey, Matthew D. Smyth, Michael R. Chicoine and David D. Limbrick
This study describes the pediatric experience with a dual-multifunction-room IMRIS 1.5-T intraoperative magnetic resonance imaging (iMRI) suite and analyzes its impact on clinical variables associated with neurosurgical resection of intracranial lesions, including safety and efficacy.
Since the inception of the iMRI–guided resection program in April 2008 at both Barnes-Jewish and St. Louis Children's Hospital, a prospective database recorded the clinical variables associated with demographics and outcome with institutional review board approval. A similarly approved retrospective database was constructed from February 2006 to March 2010 for non–iMRI resections. These databases were retrospectively reviewed for clinical variables associated with resection of pediatric (age 20 months–21 years) intracranial lesions including brain tumors and focal cortical dysplasia. Patient demographics, operative time, estimated blood loss, additional resection, length of stay, pathology, and complications were analyzed.
The authors found that 42 iMRI–guided resections were performed, whereas 103 conventional resections had been performed without the iMRI. The mean patient age was 10.5 years (range 20 months–20 years) in the iMRI group and 9.8 years (range 2–21 years) in the conventional group (p = 0.41). The mean duration of surgery was 350 minutes in the iMRI group and 243 minutes in the conventional group (p < 0.0001). The mean hospital stay was 8.2 days in the iMRI group, and 6.6 days in the conventional group, and this trended toward significance (p = 0.05). In the first 2 weeks postoperatively, there were 8 reoperations (7.77%) in the conventional group compared with none in the iMRI group, which was not significant in a 2-tailed test (p = 0.11) but trended toward significance in a 1-tailed test (p = 0.06). The significant complications included reoperation for hydrocephalus or infection: 6.8% (conventional) versus 4.8% (iMRI).
Intraoperative MR imaging–guided resections resulted in a trend toward reduction in the need for repeat surgery in the immediate 2-week postoperative period compared with conventional pediatric neurosurgical resections for tumor or focal cortical dysplasia. Although there is an increased operative time, the iMRI suite offers a comparable safety and efficacy profile while potentially reducing the per-case cost by diminishing the need for early reoperation.
Rowland H. Han, Dennis C. Nguyen, Brent S. Bruck, Gary B. Skolnick, Chester K. Yarbrough, Sybill D. Naidoo, Kamlesh B. Patel, Alex A. Kane, Albert S. Woo and Matthew D. Smyth
The authors present a retrospective cohort study examining complications in patients undergoing surgery for craniosynostosis using both minimally invasive endoscopic and open approaches.
Over the past 10 years, 295 nonsyndromic patients (140 undergoing endoscopic procedures and 155 undergoing open procedures) and 33 syndromic patients (endoscopic procedures in 10 and open procedures in 23) met the authors’ criteria. Variables analyzed included age at surgery, presence of a preexisting CSF shunt, skin incision method, estimated blood loss, transfusions of packed red blood cells, use of intravenous steroids or tranexamic acid, intraoperative durotomies, procedure length, and length of hospital stay. Complications were classified as either surgically or medically related.
In the nonsyndromic endoscopic group, the authors experienced 3 (2.1%) surgical and 5 (3.6%) medical complications. In the nonsyndromic open group, there were 2 (1.3%) surgical and 7 (4.5%) medical complications. Intraoperative durotomies occurred in 5 (3.6%) endoscopic and 12 (7.8%) open cases, were repaired primarily, and did not result in reoperations for CSF leakage. Similar complication rates were seen in syndromic cases. There was no death or permanent morbidity. Additionally, endoscopic procedures were associated with significantly decreased estimated blood loss, transfusions, procedure length, and length of hospital stay compared with open procedures.
Rates of intraoperative durotomies and surgical and medical complications were comparable between endoscopic and open techniques. This is the largest direct comparison to date between endoscopic and open interventions for synostosis, and the results are in agreement with previous series that endoscopic surgery confers distinct advantages over open surgery in appropriate patient populations.
Jacob K. Greenberg, Margaret A. Olsen, Chester K. Yarbrough, Travis R. Ladner, Chevis N. Shannon, Jay F. Piccirillo, Richard C. E. Anderson, John C. Wellons III, Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.
Chiari malformation Type I (CM-I) is a common and often debilitating pediatric neurological disease. However, efforts to guide preoperative counseling and improve outcomes research are impeded by reliance on small, single-center studies. Consequently, the objective of this study was to investigate CM-I surgical outcomes using population-level administrative billing data.
The authors used Healthcare Cost and Utilization Project State Inpatient Databases (SID) to study pediatric patients undergoing surgical decompression for CM-I from 2004 to 2010 in California, Florida, and New York. They assessed the prevalence and influence of preoperative complex chronic conditions (CCC) among included patients. Outcomes included medical and surgical complications within 90 days of treatment. Multivariate logistic regression was used to identify risk factors for surgical complications.
A total of 936 pediatric CM-I surgeries were identified for the study period. Overall, 29.2% of patients were diagnosed with syringomyelia and 13.7% were diagnosed with scoliosis. Aside from syringomyelia and scoliosis, 30.3% of patients had at least 1 CCC, most commonly neuromuscular (15.2%) or congenital or genetic (8.4%) disease. Medical complications were uncommon, occurring in 2.6% of patients. By comparison, surgical complications were diagnosed in 12.7% of patients and typically included shunt-related complications (4.0%), meningitis (3.7%), and other neurosurgery-specific complications (7.4%). Major complications (e.g., stroke or myocardial infarction) occurred in 1.4% of patients. Among children with CCCs, only comorbid hydrocephalus was associated with a significantly increased risk of surgical complications (OR 4.5, 95% CI 2.5–8.1).
Approximately 1 in 8 pediatric CM-I patients experienced a surgical complication, whereas medical complications were rare. Although CCCs were common in pediatric CM-I patients, only hydrocephalus was independently associated with increased risk of surgical events. These results may inform patient counseling and guide future research efforts.