Incidence of Congenital Spinal Abnormalities among Pediatric Patients and Their Association with Scoliosis and Systemic Anomalies

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

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)e608-e613
JournalJournal of Pediatric Orthopaedics
Volume39
Issue number8
DOIs
StatePublished - Sep 1 2019
Externally publishedYes

Fingerprint

Scoliosis
Pediatrics
Incidence
Neural Tube Defects
Spine
Inpatients
Patient Acceptance of Health Care
Databases
Poisson Distribution
Spinal Dysraphism
Large Intestine
International Classification of Diseases
Health Care Costs
Comorbidity
Cohort Studies
Cross-Sectional Studies
Outcome Assessment (Health Care)
Weights and Measures

Keywords

  • concurrent anomalies
  • congenital anomalies
  • congenital scoliosis
  • congenital spine anomalies
  • pediatric scoliosis
  • VACTERL

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Orthopedics and Sports Medicine

Cite this

Incidence of Congenital Spinal Abnormalities among Pediatric Patients and Their Association with Scoliosis and Systemic Anomalies. / Passias, Peter G.; Poorman, Gregory W.; Jalai, Cyrus M.; Diebo, Bassel G.; Vira, Shaleen; Horn, Samantha R.; Baker, Joseph F.; Shenoy, Kartik; Hasan, Saqib; Buza, John; Bronson, Wesley; Paul, Justin C.; Kaye, Ian; Foster, Norah A.; Cassilly, Ryan T.; Oren, Jonathan H.; Moskovich, Ronald; Line, Breton; Oh, Cheongeun; Bess, Shay; Lafage, Virginie; Errico, Thomas J.

In: Journal of Pediatric Orthopaedics, Vol. 39, No. 8, 01.09.2019, p. e608-e613.

Research output: Contribution to journalArticle

Passias, PG, Poorman, GW, Jalai, CM, Diebo, BG, Vira, S, Horn, SR, Baker, JF, Shenoy, K, Hasan, S, Buza, J, Bronson, W, Paul, JC, Kaye, I, Foster, NA, Cassilly, RT, Oren, JH, Moskovich, R, Line, B, Oh, C, Bess, S, Lafage, V & Errico, TJ 2019, 'Incidence of Congenital Spinal Abnormalities among Pediatric Patients and Their Association with Scoliosis and Systemic Anomalies', Journal of Pediatric Orthopaedics, vol. 39, no. 8, pp. e608-e613. https://doi.org/10.1097/BPO.0000000000001066
Passias, Peter G. ; Poorman, Gregory W. ; Jalai, Cyrus M. ; Diebo, Bassel G. ; Vira, Shaleen ; Horn, Samantha R. ; Baker, Joseph F. ; Shenoy, Kartik ; Hasan, Saqib ; Buza, John ; Bronson, Wesley ; Paul, Justin C. ; Kaye, Ian ; Foster, Norah A. ; Cassilly, Ryan T. ; Oren, Jonathan H. ; Moskovich, Ronald ; Line, Breton ; Oh, Cheongeun ; Bess, Shay ; Lafage, Virginie ; Errico, Thomas J. / Incidence of Congenital Spinal Abnormalities among Pediatric Patients and Their Association with Scoliosis and Systemic Anomalies. In: Journal of Pediatric Orthopaedics. 2019 ; Vol. 39, No. 8. pp. e608-e613.
@article{0cb562874b2747eaac46cf6d12a864de,
title = "Incidence of Congenital Spinal Abnormalities among Pediatric Patients and Their Association with Scoliosis and Systemic Anomalies",
abstract = "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.",
keywords = "concurrent anomalies, congenital anomalies, congenital scoliosis, congenital spine anomalies, pediatric scoliosis, VACTERL",
author = "Passias, {Peter G.} and Poorman, {Gregory W.} and Jalai, {Cyrus M.} and Diebo, {Bassel G.} and Shaleen Vira and Horn, {Samantha R.} and Baker, {Joseph F.} and Kartik Shenoy and Saqib Hasan and John Buza and Wesley Bronson and Paul, {Justin C.} and Ian Kaye and Foster, {Norah A.} and Cassilly, {Ryan T.} and Oren, {Jonathan H.} and Ronald Moskovich and Breton Line and Cheongeun Oh and Shay Bess and Virginie Lafage and Errico, {Thomas J.}",
year = "2019",
month = "9",
day = "1",
doi = "10.1097/BPO.0000000000001066",
language = "English (US)",
volume = "39",
pages = "e608--e613",
journal = "Journal of Pediatric Orthopaedics",
issn = "0271-6798",
publisher = "Lippincott Williams and Wilkins",
number = "8",

}

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.

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 - concurrent anomalies

KW - congenital anomalies

KW - congenital scoliosis

KW - congenital spine anomalies

KW - pediatric scoliosis

KW - VACTERL

UR - http://www.scopus.com/inward/record.url?scp=85031498594&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85031498594&partnerID=8YFLogxK

U2 - 10.1097/BPO.0000000000001066

DO - 10.1097/BPO.0000000000001066

M3 - Article

C2 - 31393300

AN - SCOPUS:85031498594

VL - 39

SP - e608-e613

JO - Journal of Pediatric Orthopaedics

JF - Journal of Pediatric Orthopaedics

SN - 0271-6798

IS - 8

ER -