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Incidence of Congenital Spinal Abnormalities Among Pediatric Patients and Their Association With Scoliosis and Systemic Anomalies

Passias, Peter G. MD*; Poorman, Gregory W. BA*; Jalai, Cyrus M. BA*; Diebo, Bassel G. MD; Vira, Shaleen MD*; Horn, Samantha R. BA*; Baker, Joseph F. MbChB, FRCS*; Shenoy, Kartik MD*; Hasan, Saqib MD*; Buza, John MD*; Bronson, Wesley MD*; Paul, Justin C. MD, PhD*; Kaye, Ian MD*; Foster, Norah A. MD*; Cassilly, Ryan T. MD*; Oren, Jonathan H. MD*; Moskovich, Ronald MD*; Line, Breton BSME; Oh, Cheongeun PhD*; Bess, Shay MD*; LaFage, Virginie PhD; Errico, Thomas J. MD*

Journal of Pediatric Orthopaedics: September 2019 - Volume 39 - Issue 8 - p e608–e613
doi: 10.1097/BPO.0000000000001066
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Background: Congenital abnormalities when present, according to VACTERL theory, occur nonrandomly with other congenital anomalies. This study estimates the prevalence of congenital spinal anomalies, and their concurrence with other systemic anomalies.

Methods: A retrospective cohort analysis on Health care Cost and Utilization Project’s Kids Inpatient Database (KID), years 2000, 2003, 2006, 2009 was performed. ICD-9 coding identified congenital anomalies of the spine and other body systems. Outcome Measures: Overall incidence of congenital spinal abnormalities in pediatric patients, and the concurrence of spinal anomaly diagnoses with other organ system anomalies. Frequencies of congenital spine anomalies were estimated using KID hospital-and-year-adjusted weights. Poisson distribution in contingency tables tabulated concurrence of other congenital anomalies, grouped by body system.

Results: Of 12,039,432 patients, rates per 100,000 cases were: 9.1 hemivertebra, 4.3 Klippel-Fiel, 56.3 Chiari malformation, 52.6 tethered cord, 83.4 spina bifida, 1.2 absence of vertebra, and 6.2 diastematomyelia. Diastematomyelia had the highest concurrence of other anomalies: 70.1% of diastematomyelia patients had at least one other congenital anomaly. Next, 63.2% of hemivertebra, and 35.2% of Klippel-Fiel patients had concurrent anomalies. Of the other systems deformities cooccuring, cardiac system had the highest concurrent incidence (6.5% overall). In light of VACTERL’s definition of a patient being diagnosed with at least 3 VACTERL anomalies, hemivertebra patients had the highest cooccurrence of ≥3 anomalies (31.3%). With detailed analysis of hemivertebra patients, secundum ASD (14.49%), atresia of large intestine (10.2%), renal agenesis (7.43%) frequently cooccured.

Conclusions: Congenital abnormalities of the spine are associated with serious systemic anomalies that may have delayed presentations. These patients continue to be at a very high, and maybe higher than previously thought, risk for comorbidities that can cause devastating perioperative complications if not detected preoperatively, and full MRI workups should be considered in all patients with spinal abnormalities.

Level of Evidence: Level III.

*Department of Orthopaedic Surgery, NYU Langone Medical Center Hospital for Joint Diseases

Department of Orthopaedic Surgery, Hospital for Special Surgery, New York, NY

Department of Orthopaedic Surgery, Rocky Mountain Spine Clinic, Denver, CO

P.G.P., G.W.P., C.M.J., B.G.D., S.B., V.L., and T.J.E.: active involvement in drafting and critical revision of manuscript, provided final approval of version to be published, study design, data acquisition, analysis and interpretation of data. S.V., J.C.P., I.K., R.T.C., and C.O.: active involvement in drafting and critical revision of manuscript, provided final approval of version to be published, study design, analysis and interpretation of data. S.R.H, K.S., and W.B.: active involvement in drafting and critical revision of manuscript, provided final approval of version to be published, analysis and interpretation of data. J.F.B., S.H., J.B., N.A.F., and J.H.O.: active involvement in drafting and critical revision of manuscript, provided final approval of version to be published, data acquisition, analysis and interpretation of data. R.M.: active involvement in drafting and critical revision of manuscript, provided final approval of version to be published, study design. B.L.: active involvement in drafting and critical revision of manuscript, provided final approval of version to be published, study design, data acquisition.

P.G.P.: reports nonpaid consultant position with Medicrea. S.B.: reports grants from Depuy Spine Synthes, paid consultancy with K2 Medical; NuVasive; Innovasis; and Allosource, and patents with K2 Medical; Pioneer; and Innovasis, outside the submitted work. V.L.: reports grants from Depuy Synthese Spine, during the conduct of the study; grants from SRS, grants from NIH, grants from DePuy Spine, personal fees from NuVasive, personal fees from Medicrea, personal fees from DePuy Spine, personal fees from Nemaris Inc., other from Nemaris Inc., outside the submitted work. T.J.E.: reports paid consultancy relationship with K2M and royalties from Fastenetix. The remaining authors declare that they have nothing to disclose.

Reprints: Peter G. Passias, MD, Department of Orthopaedic Surgery, New York Spine Institute, NYU Medical Center, Hospital for Joint Diseases, 301 East 17th Street, New York, NY 10003. E-mail: Peter.Passias@nyumc.org.

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