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; Baker, Joseph F.; Shenoy, Kartik; Hasan, Saqib; Buza, John A.; Bronson, Wesley H.; Paul, Justin C.; Kaye, I. David; Atanda, Abiola; Foster, Norah; Cassilly, Ryan T.; Oren, Jonathan H.; Lafage, Virginie; Errico, Thomas J.

doi:10.1016/j.spinee.2016.07.407

Cited by 7 publications

(11 citation statements)

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“…In our cohort, co-occurring anomalies were noted in 82% of patients while previous publications have estimated incidence rates of 30% to 70%. 1,4 We postulate that this higher incidence reported here is likely a reflection of including stillborn and terminated cases. Passias et al 4 reported cardiac, urinary, and digestive systems to be most commonly affected among patients with spinal abnormalities.…”

Section: Discussionmentioning

confidence: 64%

“…1,4 Cardiac system followed by urinary and gastrointestinal systems are most commonly affected. 4 Over 90% of congenital vertebral anomalies are sporadic, however, 5% to 10% have genetic impact and are associated with chromosomal anomalies. 8,9 Also, fetal alcohol syndrome has been reported to be associated with deformities of the cervical spine.…”

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confidence: 99%

“…Vertebral anomalies may be an isolated finding, associate with other malformation or be part of an underlying chromosome abnormality or syndrome, like Klippel-Feil6 or vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies and limb abnormalities (VACTERL) 7. Estimated frequency for associated malformations ranges from 30% to 70% depending on the type of vertebral anomaly 1,4. Cardiac system followed by urinary and gastrointestinal systems are most commonly affected 4.…”

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confidence: 99%

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Increasing Prevalence and High Risk of Associated Anomalies in Congenital Vertebral Defects: A Population-based Study

Heiskanen¹,

Syvänen²,

Helenius

et al. 2022

Journal of Pediatric Orthopaedics

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Background: Congenital vertebral anomalies are a heterogeneous group of diagnoses, and studies on their epidemiology are sparse. Our aim was to investigate the national prevalence and mortality of these anomalies, and to identify associated anomalies. Methods: We conducted a population-based nationwide register study and identified all cases with congenital vertebral anomalies in the Finnish Register of Congenital Malformations from 1997 to 2016 including live births, stillbirths, and elective terminations of pregnancy because of major fetal anomalies. Cases were categorized based on the recorded diagnoses, associated major anomalies were analyzed, and prevalence and infant mortality were calculated. Results: We identified 255 cases of congenital vertebral anomalies. Of these, 92 (36%) were diagnosed with formation defects, 18 (7.1%) with segmentation defects, and 145 (57%) had mixed vertebral anomalies. Live birth prevalence was 1.89 per 10,000, and total prevalence was 2.20/10,000, with a significantly increasing trend over time (P < 0.001). Overall infant mortality was 8.2% (18/219); 3.5% (3/86) in patients with formation defects, 5.6% (1/18) in segmentation defects, and 12.2% (14/115) in mixed vertebral anomalies (P = 0.06). Co-occurring anomalies and syndromes were associated with increased mortality, P = 0.006. Majority of the cases (82%) were associated with other major anomalies affecting most often the heart, limbs, and digestive system. Conclusions: In conclusion, the prevalence of congenital vertebral anomalies is increasing significantly in Finnish registers. Detailed and systematic examination is warranted in this patient population to identify underlying comorbidities as the majority of cases are associated with congenital major anomalies. Level of Evidence: Level III.

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Section: Discussionmentioning

confidence: 64%

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confidence: 99%

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confidence: 99%

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Increasing Prevalence and High Risk of Associated Anomalies in Congenital Vertebral Defects: A Population-based Study

Heiskanen¹,

Syvänen²,

Helenius

et al. 2022

Journal of Pediatric Orthopaedics

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“…They exhibit a wide canal, enlarged pedicles and dysraphic posterior arch which protects against neurologic compromise. Dislocations might be associated with tracheoesophageal fistula and unilateral renal agenesis [17].…”

Section: Development Of Vertebraementioning

confidence: 99%

Congenital spine deformities: timing of insult during development of the spine in utero

Nabizadeh

Dimar

2021

Spine Deform

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The development of the spine and spinal cord occurs at the earliest weeks of gestation. Their development not only affects each other but also are most likely associated with anomalies in other systems. It is essential to recognize the stages of spine development to understand the cause of congenital spinal deformities and their influences on the postnatal growing spine. A vast majority of congenital spinal problems are not evident clinically. For instance, the presence of neural axis abnormalities, such as spinal dysraphism or syringomyelia, may be so subtle that patients never seek medical care. Certain vertebral formation disorders such as hemivertebrae may remain asymptomatic throughout life if they are balanced while those with congenital bars may develop severe deformity. Major defects in the spine are often associated with abnormalities of the other organs such as cardiovascular and genital urinary system that warrants close attention by multidisciplinary specialists. A thorough understanding of the basics of embryology, which serves as a window into the development of the spine, is necessary to enable the practitioner to appreciate why, when, and where the numerous spine deformities develop in utero. Besides, certain developmental defects manifest in adulthood including spondylolysis, degenerative disc disease, congenital spinal stenosis, and even tumors like cordoma. Thus, understanding embryology can assist to establish the proper diagnosis and ensure optimal treatment.

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“…However, this may be misleading because of the imaging modalities available at the time; that is, an MRI scan was not routinely used to optimally detect tethered cords and Chiari malformations. Interestingly, more recent studies have indicated some regional differences as to which intraspinal anomaly is most commonly observed, as Shen et al 17 observed diastematomyelia as the most common anomaly in 226 Chinese patients with congenital scoliosis, whereas Passias et al 18 queried a large data set in the United States and found a tethered cord to be most common. In addition, the mean spinal cord diameter is diminished in patients with congenital scoliosis, increasing the likelihood of neurological sequelae both preoperatively and postoperatively 19 .…”

Section: Accompanying Anomaliesmentioning

confidence: 99%

Team Approach: Contemporary Treatment of Congenital Scoliosis

Karaarslan¹,

Gurel²,

Yücekul³

et al. 2019

JBJS Rev

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Congenital scoliosis is caused by developmental abnormalities such as failure of segmentation, failure of formation, or a combination of these conditions. » The multi-organ nature of congenital scoliosis necessitates a team approach in which spinal and intraspinal deformities are treated by orthopaedic surgeons, neurosurgeons, and physical therapists, and associated anomalies are treated by pediatricians, pediatric surgeons, cardiologists, pulmonologists, and urologists. » At the initial time of diagnosis, observation is appropriate for most cases. Although early arthrodesis may be appropriate for select cases, delaying tactics that are nonoperative (bracing and casting) or operative (growing rod and growth guidance) may preserve (or even stimulate) spine and chest cage development while controlling the deformity. » Early hemivertebra resection and short-segment arthrodesis provide excellent outcomes. » Patients with a neurological deficit may require neurosurgical intervention, which may be performed either before a deformity correction surgical procedure or simultaneously with the procedure.

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

Cited by 7 publications

References 0 publications

Increasing Prevalence and High Risk of Associated Anomalies in Congenital Vertebral Defects: A Population-based Study

Increasing Prevalence and High Risk of Associated Anomalies in Congenital Vertebral Defects: A Population-based Study

Congenital spine deformities: timing of insult during development of the spine in utero

Team Approach: Contemporary Treatment of Congenital Scoliosis

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