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Kamlesh B. Patel, Cihat Eldeniz, Gary B. Skolnick, Paul K. Commean, Parna Eshraghi Boroojeni, Udayabhanu Jammalamadaka, Corinne Merrill, Matthew D. Smyth, Manu S. Goyal, and Hongyu An

OBJECTIVE

Head trauma is the most common indication for a CT scan. In this pilot study, the authors assess the feasibility of a 5-minute high-resolution 3D golden-angle (GA) stack-of-stars radial volumetric interpolated breath-hold examination (VIBE) MRI sequence (GA-VIBE) to obtain clinically acceptable cranial bone images and identify cranial vault fractures compared to CT.

METHODS

Patients younger than 18 years of age presenting after head trauma were eligible for the study. Three clinicians reviewed and assessed 1) slice-by-slice volumetric CT and inverted MR images, and 2) 3D reconstructions obtained from inverted MR images and the gold standard (CT). For each image set, reviewers noted on 5-point Likert scales whether they recommended that a repeat scan be performed and the presence or absence of cranial vault fractures.

RESULTS

Thirty-one patients completed MRI after a clinical head CT scan was performed. Based on CT imaging, 8 of 31 patients had cranial fractures. Two of 31 patients were sedated as part of their clinical MRI scan. In 30 (97%) of 31 MRI reviews, clinicians agreed (or strongly agreed) that the image quality was acceptable for clinical diagnosis. Overall, comparing MRI to acceptable gold-standard CT, sensitivity and specificity of fracture detection were 100%. Furthermore, there were no discrepancies between CT and MRI in classification of fracture type or location.

CONCLUSIONS

When compared with the gold standard (CT), the volumetric and 3D reconstructed images using the GA-VIBE sequence were able to produce clinically acceptable cranial images with excellent ability to detect cranial vault fractures.

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S. Hassan A. Akbari, Alexander T. Yahanda, Laurie L. Ackerman, P. David Adelson, Raheel Ahmed, Gregory W. Albert, Philipp R. Aldana, Tord D. Alden, Richard C. E. Anderson, David F. Bauer, Tammy Bethel-Anderson, Karin Bierbrauer, Douglas L. Brockmeyer, Joshua J. Chern, Daniel E. Couture, David J. Daniels, Brian J. Dlouhy, Susan R. Durham, Richard G. Ellenbogen, Ramin Eskandari, Herbert E. Fuchs, Gerald A. Grant, Patrick C. Graupman, Stephanie Greene, Jeffrey P. Greenfield, Naina L. Gross, Daniel J. Guillaume, Todd C. Hankinson, Gregory G. Heuer, Mark Iantosca, Bermans J. Iskandar, Eric M. Jackson, George I. Jallo, James M. Johnston, Bruce A. Kaufman, Robert F. Keating, Nicklaus R. Khan, Mark D. Krieger, Jeffrey R. Leonard, Cormac O. Maher, Francesco T. Mangano, J. Gordon McComb, Sean D. McEvoy, Thanda Meehan, Arnold H. Menezes, Michael S. Muhlbauer, Brent R. O’Neill, Greg Olavarria, John Ragheb, Nathan R. Selden, Manish N. Shah, Chevis N. Shannon, Joshua S. Shimony, Matthew D. Smyth, Scellig S. D. Stone, Jennifer M. Strahle, Mandeep S. Tamber, James C. Torner, Gerald F. Tuite, Elizabeth C. Tyler-Kabara, Scott D. Wait, John C. Wellons III, William E. Whitehead, Tae Sung Park, and David D. Limbrick Jr.

OBJECTIVE

The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM).

METHODS

The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups.

RESULTS

A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD.

CONCLUSIONS

PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

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Kaamya Varagur, Gary B. Skolnick, Sybill D. Naidoo, Matthew D. Smyth, and Kamlesh B. Patel

OBJECTIVE

Outcomes research on unilateral coronal synostosis is mostly limited to the early postoperative period. This study examines facial asymmetry, desire for revision, and patient-reported outcomes at school age in children who received either endoscopic strip craniectomy with helmet therapy or fronto-orbital advancement (open repair).

METHODS

Patients with repaired unilateral coronal synostosis born between 2000 and 2017, with 3D photographs taken when they were between 3.5 and 8 years of age, were eligible for study inclusion. Three pairs of bilateral linear measurements and two angular measurements were taken. Parent- and physician-reported desire for revision and patient-reported outcomes (Patient-Reported Outcomes Measurement Information System cognitive function and Quality of Life in Neurological Disorders stigma scores) were collected from patient charts.

RESULTS

Thirty-five patients qualified, including 25 with open repair and 10 with endoscopic repair. The median patient ages at repair were 3 months and 8 months in the endoscopic and open groups, respectively. The average ages at final 3D photography were 5.8 years and 5.5 years in the endoscopic and open groups, respectively. Digital anthropometry revealed no significant differences in measures of facial asymmetry between the repair groups (p ≥ 0.211). Midface depth (tragion to subnasale) was significantly less symmetric at school age than other linear measures (F(2,102) = 9.14, p < 0.001). Forehead asymmetry was significantly associated with parent- and physician-reported desire for revision (p ≤ 0.006). No significant associations were found between physical asymmetry and patient-reported stigma or cognitive function (p > 0.046, Holm-Bonferroni correction).

CONCLUSIONS

Children who underwent open or endoscopic repair for unilateral coronal synostosis have comparable facial symmetry at school age, but midface depth remains highly asymmetrical in both groups. Forehead asymmetry at school age correlates with parent- and physician-reported desire for revision.

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Afshin Salehi, Peter H. Yang, and Matthew D. Smyth

OBJECTIVE

Use of invasive stereoelectroencephalography (SEEG) has gained traction recently. However, scant research has investigated the costs and resource utilization of SEEG compared with subdural grid (SDG)–based techniques in pediatric patients. Here, the authors have presented a retrospective analysis of charges associated with SEEG and SDG monitoring at a single institution.

METHODS

The authors performed a retrospective case series analysis of pediatric patients with similar characteristics in terms of age, sex, seizure etiology, and epilepsy treatment strategy who underwent SEEG or SDG monitoring and subsequent craniotomy for resection of epileptogenic focus at St. Louis Children Hospital, St. Louis, Missouri, between 2013 and 2020. Financial data, including hospital charges, supplies, and professional fees (i.e., those related to anesthesia, neurology, neurosurgery, and critical care), were adjusted for inflation to 2020 US dollars.

RESULTS

The authors identified 18 patients (9 underwent SEEG and 9 underwent SDG) with similar characteristics in terms of age (mean [range] 13.6 [1.9–21.8] years for SDG patients vs 11.9 [2.4–19.6] years for SEEG patients, p = 0.607), sex (4 females underwent SDG vs 6 females underwent SEEG, p = 0.637), and presence of lesion (5 patients with a lesion underwent SDG vs 8 underwent SEEG, p = 0.294). All patients underwent subsequent craniotomy for resection of epileptogenic focus. SEEG patients were more likely to have a history of status epilepticus (p = 0.029). Across 1 hospitalization for each SDG patient and 2 hospitalizations for each SEEG patient, SEEG patients had a significantly shorter mean operating room time (288 vs 356 minutes, p = 0.015), mean length of stay in the ICU (1.0 vs 2.1 days, p < 0.001), and tended to have a shorter overall length of stay in the hospital (8.4 vs 10.6 days, p = 0.086). Both groups underwent invasive monitoring for similar lengths of time (5.2 days for SEEG patients vs 6.4 days for SDG patients, p = 0.257). Time to treatment from the initial invasive monitoring evaluation was significantly longer in SEEG patients (64.6 vs 6.4 days, p < 0.001). Neither group underwent readmission within the first 30 days after hospital discharge. Seizure outcomes and complication rates were similar. After adjustment for inflation, the average total perioperative charges were $104,442 for SDG and $106,291 for SEEG (p = 0.800).

CONCLUSIONS

Even though 2 hospitalizations were required for SEEG and 1 hospitalization was required for SDG monitoring, patients who underwent SEEG had a significantly shorter average length of stay in the ICU and operating room time. Surgical morbidity and outcomes were similar. Total perioperative charges for invasive monitoring and resection were approximately 2% higher for SEEG patients when corrected for inflation, but this difference was not statistically significant.

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Samuel J. Cler, Alexander Skidmore, Alexander T. Yahanda, Kimberly Mackey, Joshua B. Rubin, Andrew Cluster, Stephanie Perkins, Karen Gauvain, Allison A. King, David D. Limbrick Jr., Sean McEvoy, Tae Sung Park, Matthew D. Smyth, Ali Y. Mian, Michael R. Chicoine, Sonika Dahiya, and Jennifer M. Strahle

OBJECTIVE

Pilocytic astrocytomas (PAs) have a generally favorable prognosis; however, progression or recurrence after resection is possible. The prognostic value of histopathological qualifiers (defined below) or BRAF alterations is not well understood. The aim of this study was to identify the prognostic value of genetic and histopathological features of pediatric PAs.

METHODS

Patients treated for a WHO grade I PA at a single institution were analyzed for histopathological and genetic features and outcomes. “Histopathological qualifier” refers to designations such as "WHO grade I PA with increased proliferative index." BRAF alterations include gene fusions and point mutations. Patients with neurofibromatosis type 1 were excluded.

RESULTS

A total of 222 patients were analyzed (51% female, mean age 9.6 years). Tumors were located in the cerebellum/fourth ventricle (51%), optic pathway/hypothalamus (15%), brainstem (12%), and cerebral cortex (11%). BRAF alterations were screened for in 77 patients and identified in 56 (73%). Histopathological qualifiers were present in 27 patients (14%). Resection was performed in 197 patients (89%), 41 (21%) of whom displayed tumor progression or recurrence after resection. Tumor progression or recurrence was not associated with histopathologic qualifiers (p = 0.36) or BRAF alterations (p = 0.77). Ki-67 proliferative indices were not predictive of progression or recurrence (p = 0.94). BRAF alterations, specifically KIAA1549 fusions, were associated with cerebellar/fourth ventricular tumor location (p < 0.0001) and younger patient age (p = 0.03). Patients in whom gross-total resection was achieved had lower rates of progression and recurrence (p < 0.0001).

CONCLUSIONS

Histopathological features/qualifiers and BRAF alterations were not associated with tumor recurrence/progression in pediatric PAs. The extent of resection was the only factor analyzed that predicted outcome.

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Syed Hassan A. Akbari, Asad A. Rizvi, Travis S. CreveCoeur, Rowland H. Han, Jacob K. Greenberg, James Torner, Douglas L. Brockmeyer, John C. Wellons III, Jeffrey R. Leonard, Francesco T. Mangano, James M. Johnston, Manish N. Shah, Bermans J. Iskandar, Raheel Ahmed, Gerald F. Tuite, Bruce A. Kaufman, David J. Daniels, Eric M. Jackson, Gerald A. Grant, Alexander K. Powers, Daniel E. Couture, P. David Adelson, Tord D. Alden, Philipp R. Aldana, Richard C. E. Anderson, Nathan R. Selden, Karin Bierbrauer, William Boydston, Joshua J. Chern, William E. Whitehead, Robert C. Dauser, Richard G. Ellenbogen, Jeffrey G. Ojemann, Herbert E. Fuchs, Daniel J. Guillaume, Todd C. Hankinson, Brent R. O’Neill, Mark Iantosca, W. Jerry Oakes, Robert F. Keating, Paul Klimo Jr., Michael S. Muhlbauer, J. Gordon McComb, Arnold H. Menezes, Nickalus R. Khan, Toba N. Niazi, John Ragheb, Chevis N. Shannon, Jodi L. Smith, Laurie L. Ackerman, Andrew H. Jea, Cormac O. Maher, Prithvi Narayan, Gregory W. Albert, Scellig S. D. Stone, Lissa C. Baird, Naina L. Gross, Susan R. Durham, Stephanie Greene, Robert C. McKinstry, Joshua S. Shimony, Jennifer M. Strahle, Matthew D. Smyth, Ralph G. Dacey Jr., Tae Sung Park, and David D. Limbrick Jr.

OBJECTIVE

The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM).

METHODS

The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes.

RESULTS

A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively).

CONCLUSIONS

Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

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Jacob R. Lepard, Irene Kim, Anastasia Arynchyna, Sean M. Lew, Robert J. Bollo, Brent R. O’Neill, M. Scott Perry, David Donahue, Matthew D. Smyth, and Jeffrey Blount

OBJECTIVE

Pediatric stereoelectroencephalography (SEEG) has been increasingly performed in the United States, with published literature being limited primarily to large single-center case series. The purpose of this study was to evaluate the experience of pediatric epilepsy centers, where the technique has been adopted in the last several years, via a multicenter case series studying patient demographics, outcomes, and complications.

METHODS

A retrospective cohort methodology was used based on the STROBE criteria. ANOVA was used to evaluate for significant differences between the means of continuous variables among centers. Dichotomous outcomes were assessed between centers using a univariate and multivariate logistic regression.

RESULTS

A total of 170 SEEG insertion procedures were included in the study from 6 different level 4 pediatric epilepsy centers. The mean patient age at time of SEEG insertion was 12.3 ± 4.7 years. There was no significant difference between the mean age at the time of SEEG insertion between centers (p = 0.3). The mean number of SEEG trajectories per patient was 11.3 ± 3.6, with significant variation between centers (p < 0.001). Epileptogenic loci were identified in 84.7% of cases (144/170). Patients in 140 cases (140/170, 82.4%) underwent a follow-up surgical intervention, with 47.1% (66/140) being seizure free at a mean follow-up of 30.6 months. An overall postoperative hemorrhage rate of 5.3% (9/170) was noted, with patients in 4 of these cases (4/170, 2.4%) experiencing a symptomatic hemorrhage and patients in 3 of these cases (3/170, 1.8%) requiring operative evacuation of the hemorrhage. There were no mortalities or long-term complications.

CONCLUSIONS

As the first multicenter case series in pediatric SEEG, this study has aided in establishing normative practice patterns in the application of a novel surgical technique, provided a framework for anticipated outcomes that is generalizable and useful for patient selection, and allowed for discussion of what is an acceptable complication rate relative to the experiences of multiple institutions.

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Vijay M. Ravindra, Al-Wala Awad, Cordell M. Baker, Amy Lee, Richard C. E. Anderson, Barbu Gociman, Kamlesh B. Patel, Matthew D. Smyth, Craig Birgfeld, Ian F. Pollack, Jesse A. Goldstein, Thomas Imahiyerobo, Faizi A. Siddiqi, John R. W. Kestle, and for the Synostosis Research Group (SynRG)

OBJECTIVE

The diagnosis of single-suture craniosynostosis can be made by physical examination, but the use of confirmatory imaging is common practice. The authors sought to investigate preoperative imaging use and to describe intracranial findings in children with single-suture synostosis from a large, prospective multicenter cohort.

METHODS

In this study from the Synostosis Research Group, the study population included children with clinically diagnosed single-suture synostosis between March 1, 2017, and October 31, 2020, at 5 institutions. The primary analysis correlated the clinical diagnosis and imaging diagnosis; secondary outcomes included intracranial findings by pathological suture type.

RESULTS

A total of 403 children (67% male) were identified with single-suture synostosis. Sagittal (n = 267), metopic (n = 77), coronal (n = 52), and lambdoid (n = 7) synostoses were reported; the most common presentation was abnormal head shape (97%), followed by a palpable or visible ridge (37%). Preoperative cranial imaging was performed in 90% of children; findings on 97% of these imaging studies matched the initial clinical diagnosis. Thirty-one additional fused sutures were identified in 18 children (5%) that differed from the clinical diagnosis. The most commonly used imaging modality by far was CT (n = 360), followed by radiography (n = 9) and MRI (n = 7). Most preoperative imaging was ordered as part of a protocolized pathway (67%); some images were obtained as a result of a nondiagnostic clinical examination (5.2%). Of the 360 patients who had CT imaging, 150 underwent total cranial vault surgery and 210 underwent strip craniectomy. The imaging findings influenced the surgical treatment 0.95% of the time. Among the 24% of children with additional (nonsynostosis) abnormal findings on CT, only 3.5% required further monitoring.

CONCLUSIONS

The authors found that a clinical diagnosis of single-suture craniosynostosis and the findings on CT were the same with rare exceptions. CT imaging very rarely altered the surgical treatment of children with single-suture synostosis.

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Brooke Sadler, Alex Skidmore, Jordan Gewirtz, Richard C. E. Anderson, Gabe Haller, Laurie L. Ackerman, P. David Adelson, Raheel Ahmed, Gregory W. Albert, Philipp R. Aldana, Tord D. Alden, Christine Averill, Lissa C. Baird, David F. Bauer, Tammy Bethel-Anderson, Karin S. Bierbrauer, Christopher M. Bonfield, Douglas L. Brockmeyer, Joshua J. Chern, Daniel E. Couture, David J. Daniels, Brian J. Dlouhy, Susan R. Durham, Richard G. Ellenbogen, Ramin Eskandari, Herbert E. Fuchs, Timothy M. George, Gerald A. Grant, Patrick C. Graupman, Stephanie Greene, Jeffrey P. Greenfield, Naina L. Gross, Daniel J. Guillaume, Todd C. Hankinson, Gregory G. Heuer, Mark Iantosca, Bermans J. Iskandar, Eric M. Jackson, Andrew H. Jea, James M. Johnston, Robert F. Keating, Nickalus Khan, Mark D. Krieger, Jeffrey R. Leonard, Cormac O. Maher, Francesco T. Mangano, Timothy B. Mapstone, J. Gordon McComb, Sean D. McEvoy, Thanda Meehan, Arnold H. Menezes, Michael Muhlbauer, W. Jerry Oakes, Greg Olavarria, Brent R. O’Neill, John Ragheb, Nathan R. Selden, Manish N. Shah, Chevis N. Shannon, Jodi Smith, Matthew D. Smyth, Scellig S. D. Stone, Gerald F. Tuite, Scott D. Wait, John C. Wellons III, William E. Whitehead, Tae Sung Park, David D. Limbrick Jr., and Jennifer M. Strahle

OBJECTIVE

Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression.

METHODS

A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty.

RESULTS

In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion.

CONCLUSIONS

In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

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Brooke Sadler, Alex Skidmore, Jordan Gewirtz, Richard C. E. Anderson, Gabe Haller, Laurie L. Ackerman, P. David Adelson, Raheel Ahmed, Gregory W. Albert, Philipp R. Aldana, Tord D. Alden, Christine Averill, Lissa C. Baird, David F. Bauer, Tammy Bethel-Anderson, Karin S. Bierbrauer, Christopher M. Bonfield, Douglas L. Brockmeyer, Joshua J. Chern, Daniel E. Couture, David J. Daniels, Brian J. Dlouhy, Susan R. Durham, Richard G. Ellenbogen, Ramin Eskandari, Herbert E. Fuchs, Timothy M. George, Gerald A. Grant, Patrick C. Graupman, Stephanie Greene, Jeffrey P. Greenfield, Naina L. Gross, Daniel J. Guillaume, Todd C. Hankinson, Gregory G. Heuer, Mark Iantosca, Bermans J. Iskandar, Eric M. Jackson, Andrew H. Jea, James M. Johnston, Robert F. Keating, Nickalus Khan, Mark D. Krieger, Jeffrey R. Leonard, Cormac O. Maher, Francesco T. Mangano, Timothy B. Mapstone, J. Gordon McComb, Sean D. McEvoy, Thanda Meehan, Arnold H. Menezes, Michael Muhlbauer, W. Jerry Oakes, Greg Olavarria, Brent R. O’Neill, John Ragheb, Nathan R. Selden, Manish N. Shah, Chevis N. Shannon, Jodi Smith, Matthew D. Smyth, Scellig S. D. Stone, Gerald F. Tuite, Scott D. Wait, John C. Wellons III, William E. Whitehead, Tae Sung Park, David D. Limbrick Jr., and Jennifer M. Strahle

OBJECTIVE

Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression.

METHODS

A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty.

RESULTS

In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion.

CONCLUSIONS

In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.