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Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study.
J Neurosurg Pediatr. 2019 Oct 18 [Online ahead of print]JN

Abstract

OBJECTIVE

Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction.

METHODS

Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx.

RESULTS

Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications.

CONCLUSIONS

This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.

Authors+Show Affiliations

1Department of Neurological Surgery, University of Wisconsin, Madison, Wisconsin.1Department of Neurological Surgery, University of Wisconsin, Madison, Wisconsin.2Department of Neurosurgery, University of Utah, Salt Lake City, Utah.3Department of Neurosurgery, Children's of Alabama, Birmingham, Alabama.4Section of Neurosurgery, Department of Surgery, University of Chicago, Chicago, Illinois.5Department of Pediatric Neurosurgery, Stanford Health Care, Palo Alto, California.6Department of Neurosurgery, Children's National Health System, Washington, DC.7Department of Neurosurgery, Duke University Medical Center, Durham, North Carolina.3Department of Neurosurgery, Children's of Alabama, Birmingham, Alabama.8Department of Neurosurgery, UPMC Children's Hospital of Pittsburgh, Pennsylvania.9Department of Neurological Surgery, Oregon Health and Science University, Portland, Oregon.3Department of Neurosurgery, Children's of Alabama, Birmingham, Alabama.10Department of Neurosurgery, Johns Hopkins All Children's Hospital, Tampa, Florida.11Department of Neurosurgery, Boston Children's Hospital, Boston, Massachusetts.Departments of12Biostatistics and Medical Informatics and.Departments of12Biostatistics and Medical Informatics and.13Radiology, University of Wisconsin-Madison, Wisconsin; and.13Radiology, University of Wisconsin-Madison, Wisconsin; and.14Department of Neurosurgery, Dell Medical School, Austin, Texas.1Department of Neurological Surgery, University of Wisconsin, Madison, Wisconsin.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31628281

Citation

Koueik, Joyce, et al. "Outcomes in Children Undergoing Posterior Fossa Decompression and Duraplasty With and Without Tonsillar Reduction for Chiari Malformation Type I and Syringomyelia: a Pilot Prospective Multicenter Cohort Study." Journal of Neurosurgery. Pediatrics, 2019, pp. 1-9.
Koueik J, Sandoval-Garcia C, Kestle JRW, et al. Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study. J Neurosurg Pediatr. 2019.
Koueik, J., Sandoval-Garcia, C., Kestle, J. R. W., Rocque, B. G., Frim, D. M., Grant, G. A., Keating, R. F., Muh, C. R., Oakes, W. J., Pollack, I. F., Selden, N. R., Tubbs, R. S., Tuite, G. F., Warf, B., Rajamanickam, V., Broman, A. T., Haughton, V., Rebsamen, S., George, T. M., & Iskandar, B. J. (2019). Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study. Journal of Neurosurgery. Pediatrics, 1-9. https://doi.org/10.3171/2019.8.PEDS19154
Koueik J, et al. Outcomes in Children Undergoing Posterior Fossa Decompression and Duraplasty With and Without Tonsillar Reduction for Chiari Malformation Type I and Syringomyelia: a Pilot Prospective Multicenter Cohort Study. J Neurosurg Pediatr. 2019 Oct 18;1-9. PubMed PMID: 31628281.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study. AU - Koueik,Joyce, AU - Sandoval-Garcia,Carolina, AU - Kestle,John R W, AU - Rocque,Brandon G, AU - Frim,David M, AU - Grant,Gerald A, AU - Keating,Robert F, AU - Muh,Carrie R, AU - Oakes,W Jerry, AU - Pollack,Ian F, AU - Selden,Nathan R, AU - Tubbs,R Shane, AU - Tuite,Gerald F, AU - Warf,Benjamin, AU - Rajamanickam,Victoria, AU - Broman,Aimee Teo, AU - Haughton,Victor, AU - Rebsamen,Susan, AU - George,Timothy M, AU - Iskandar,Bermans J, Y1 - 2019/10/18/ PY - 2019/04/10/received PY - 2019/08/01/accepted PY - 2019/10/20/entrez PY - 2019/10/20/pubmed PY - 2019/10/20/medline KW - ASAP = American Syringomyelia and Chiari Alliance Project KW - CM-I = Chiari malformation type I KW - Chiari malformation decompression KW - PFD = posterior fossa decompression KW - PFDD = PFD and duraplasty KW - PFDD-T = PFDD with tonsillar reduction KW - surgical outcomes KW - syrinx improvement KW - tonsillar resection SP - 1 EP - 9 JF - Journal of neurosurgery. Pediatrics JO - J Neurosurg Pediatr N2 - OBJECTIVE: Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction. METHODS: Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1-2 weeks, 3-6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx. RESULTS: Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications. CONCLUSIONS: This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations. SN - 1933-0715 UR - https://www.unboundmedicine.com/medline/citation/31628281/Outcomes_in_children_undergoing_posterior_fossa_decompression_and_duraplasty_with_and_without_tonsillar_reduction_for_Chiari_malformation_type_I_and_syringomyelia:_a_pilot_prospective_multicenter_cohort_study_ DB - PRIME DP - Unbound Medicine ER -