Vertebral, intraspinal and other organ anomalies in congenital scoliosis

Abstract

Aims This study was undertaken to describe the pattern of vertebral, intraspinal and other organ anomalies in patients with congenital scoliosis and to determine the correlation between them. Methods Complete medical and radiological records of 227 consecutive patients with congenital scoliosis were analysed. The radiographs were examined for type of vertebral anomaly, location and severity of deformity. The median curve progression index (MCPI) was calculated in 198 patients. The magnetic resonance imaging (MRI) of the whole spine was analysed to detect the presence of cord abnormalities. The presence of other organ-system anomalies was also noted. The independent sample t test was used to compare severity of deformity between those with and without cord anomalies. The Chi-square test was used to compare frequency of cord abnormalities in different vertebral and organ-system anomalies. Results Hemivertebra with contralateral bar had the highest MCPI, while block vertebrae and wedge vertebrae had the lowest MCPI. Forty-eight patients had 83 cord anomalies. There was no statistically significant difference in severity of deformity, between those with and without cord anomalies. Failure of segmentation had the highest frequency of cord anomalies (p = 0.01). There was no significant difference in the frequency of cord anomalies between those with and without other organ defects. Conclusion Curve progression can be predicted by the underlying vertebral abnormalities. However, it cannot predict cord and other organ-system anomalies. Thus, all patients with congenital scoliosis must undergo MRI of the spine, electro- and echocardiography and ultrasonography of the abdomen to detect occult abnormalities and optimize the patient prior to deformity correction.

Cross-sectional analysis and trend of vertebral and associated anomalies in Chinese congenital scoliosis population: a retrospective study of one thousand, two hundred and eighty nine surgical cases from 2010 to 2019

“…In all CS patients, 49.1% were typed as failure of formation, 19.5% as failure of segmentation, and 31.4% as mixed defects. This distribution was similar to that in previous studies [7,14,16,18,19]. We also found that failure of formation was significantly more common in male patients, whereas failure of segmentation or mixed defects were significantly more common in female patients.…”

“…The incidence of intraspinal anomalies was 29.4%, which was in the range of previous studies [7,[12][13][14][16][17][18][19]. The most common associated anomaly of intraspinal defect was diastematomyelia, with an incidence of 19.3%, followed by syringomyelia (13.2%) and tethered cord (9.1%).…”

“…Among all kinds of congenital defects, intraspinal, cardiovascular, and genitourinary defects are the most common. Several previous studies have reported the incidences of different congenital defects in CS patients [7,[10][11][12][13][14][15][16][17][18][19]. However, the incidences varied greatly in different studies, and the interrelationship among these anomalies and the trend in recent years has not been fully clarified.…”

Congenital Osseous Torticollis that Mimics Congenital Muscular Torticollis: A Retrospective Observational Study

“…For these reasons, osseous torticollis is likely to be overlooked especially if vertebral anomalies are not accompanied by other discernible congenital defects, such as vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia (VATER) syndrome; Klippel–Feil syndrome (vertebral fusion with prominent features such as low hairline, short neck, facial asymmetry, scoliosis, or spina bifida and genitourinary problems, heart abnormalities, or lung defects); Alagille syndrome; or Müllerian duct aplasia, renal aplasia/agenesis, and cervicothoracic somite dysplasia (MURCS) association. Vertebral anomalies increase instability risk and spinal cord encroachment and therefore should be considered in pediatric patients with abnormal head and neck posture to avoid potential neurologic injury [ 4 , 5 , 6 , 7 , 8 ]. If a correct diagnosis is confirmed, patients can receive proper management and clinicians can avoid pursuing unnecessary physical therapy.…”

Geneticfactors of the Etiology and Pathogenesis of Congenital Spinal Deformities

“…Будучи эмбриологической аномалией, ВДП часто сочетается с пороками развития мочеполовой, сердечно-сосудистой, скелетно-мышечной систем. Эти факторы играют важную роль при принятии решений о тактике лечения детей с ВДП [5].…”