TY - JOUR
T1 - Incidence of Congenital Spinal Abnormalities among Pediatric Patients and Their Association with Scoliosis and Systemic Anomalies
AU - Passias, Peter G.
AU - Poorman, Gregory W.
AU - Jalai, Cyrus M.
AU - Diebo, Bassel G.
AU - Vira, Shaleen
AU - Horn, Samantha R.
AU - Baker, Joseph F.
AU - Shenoy, Kartik
AU - Hasan, Saqib
AU - Buza, John
AU - Bronson, Wesley
AU - Paul, Justin C.
AU - Kaye, Ian
AU - Foster, Norah A.
AU - Cassilly, Ryan T.
AU - Oren, Jonathan H.
AU - Moskovich, Ronald
AU - Line, Breton
AU - Oh, Cheongeun
AU - Bess, Shay
AU - Lafage, Virginie
AU - Errico, Thomas J.
N1 - Funding Information:
The KID is the largest publicly-available all-payer pediatric (age below 21 at admission) health care database in the United States, and was created in an effort to examine health care trends in disease incidence and outcomes. This database is created under the Agency for Health care Research and Quality’s Health care Cost and Utilization Project (HCUP). KID sampling includes complicated and uncomplicated births, as well as other pediatric inpatient procedures from community,
Publisher Copyright:
© 2017 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2019/9/1
Y1 - 2019/9/1
N2 - 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.
AB - 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.
KW - VACTERL
KW - concurrent anomalies
KW - congenital anomalies
KW - congenital scoliosis
KW - congenital spine anomalies
KW - pediatric scoliosis
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U2 - 10.1097/BPO.0000000000001066
DO - 10.1097/BPO.0000000000001066
M3 - Article
C2 - 31393300
AN - SCOPUS:85031498594
SN - 0271-6798
VL - 39
SP - e608-e613
JO - Journal of Pediatric Orthopaedics
JF - Journal of Pediatric Orthopaedics
IS - 8
ER -