290 - Incidence of Congenital Spinal Abnormalities among Paediatric Patients...

General Session: Pediatric Spine

Presented by: G. Poorman - View Audio/Video Presentation (Members Only)


P. Passias(1), G. Poorman(1), C. Jalai(1), B. Diebo(2), S. Vira(1), S. Horn(1), J. Moon(1), P. Zhou(1), J. Baker(1), K. Shenoy(1), S. Hasan(1), J. Buza(1), W. Bronson(1), J. Paul(1), I. Kaye(1), A. Atanda(1), N. Foster(1), R. Cassilly(1), J. Oren(1), R. Moskovich(1), B. Line(3), C. Oh(1), S. Bess(4), V. LaFage(5), T. Errico(1)

(1) NYU Hospital for Joint Diseases, Orthopaedic Surgery, New York, NY, United States
(2) SUNY Downstate, Orthopaedic Surgery, Brooklyn, NY, United States
(3) Rocky Mountain Spine Clinic, Orthopaedic Surgery, Denver, CO, United States
(4) Denver International Spine Center, Denver, CO, United States
(5) Hospital for Special Surgery, Spine Service, New York, NY, United States


Objective: Congenital abnormalities of the spine may have delayed presentations. When present, according to VACTERL theory, they occur non-randomly with other congenital anomalies. This study estimates the prevalence of congenital spinal anomalies, and their concurrence with other systemic anomalies and scoliosis.

Methods: Retrospective cohort analysis on Healthcare 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 all paediatric patients, and the occurrences of spinal anomaly diagnoses with scoliosis and 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 all other congenital anomalies, grouped by body system.

Results: Of 12,039,432 patients, rates per 100,000 cases were: 9.1 [8.56-9.64] Hemivertebra, 4.3 [3.93-4.67] Klippel-Feil, 56.3 [54.96-57.64] Arnold Chiari, 52.6 [51.3-53.9] Tethered Cord, 83.4 [81.77-85.03] Spina Bifida, 1.2 [1-1.4] absence of vertebra, and 6.2 [5.76-6.64] Diastematomyelia. Diastematomyelia had the highest concurrence rate of any spine diagnosis with 70.1% (67.8-73.4%) of diastematomyelia patients having at least one other congenital anomaly followed by 63.2% (60.8-65.6%) for hemivertebra, and 35.2% (32.0-39.0%) for Klippel-Feil. Overall, 19.9% (19.5-20.3%) of any spinal anomaly patients had a second other-system anomaly. Cardiac system anomalies had the highest concurrent incidence (6.5% [6.3-6.7%]). Hemivertebra had the highest co-occurrence rate of three or more anomalies (31.3% [29.0-33.6. In a detailed analysis of hemivertebra anomalies, Cardiac system had the highest incidence of concurrent anomalies with the most being Secundum ASD (14.49% [12.72-16.25%]).

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 should not be a target for cost cutting in current healthcare budget efforts. Levels of Evidence: III