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Ben J. Bixenmann, Beth M. Kline-Fath, Karin S. Bierbrauer, and Danesh Bansal

Object

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).

Methods

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.

Results

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.

Conclusions

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.

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Smruti K. Patel, Brittany Staarmann, Alexander Heilman, Allie Mains, Jason Woodward, and Karin S. Bierbrauer

Spina bifida is the most common nonchromosomal birth defect, resulting in permanent disability of multiple organ systems, yet compatible with long-term survival. Important advances across various disciplines have now improved survival among the spina bifida population. Although the majority of individuals living with spina bifida are now adults, there are few publications in the neurosurgical literature regarding the care of adults with spina bifida, associated medical conditions, surgical interventions, and long-term complications. The major goals for transitioning adult patients with spina bifida are preservation of function and promotion of independence as well as general overall health. Nevertheless, many gaps exist in our knowledge and understanding of the complex needs of this aging patient population. The goal of this paper was to provide a comprehensive updated review of the literature regarding the challenges and considerations involved in the transitional care to adulthood for patients with spina bifida. Unique to this review, the authors provide a first-hand personal communication and interview with an adult patient with spina bifida that discusses many of these challenges with transition.

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Michelle C. Caruso, Margot C. Daugherty, Suzanne M. Moody, Richard A. Falcone Jr., Karin S. Bierbrauer, and Gary L. Geis

OBJECTIVE

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.

METHODS

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.

RESULTS

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).

CONCLUSIONS

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.

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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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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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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.

OBJECTIVE

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.

METHODS

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°).

RESULTS

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.

CONCLUSIONS

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.

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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.