Journal List > J Korean Soc Spine Surg > v.8(3) > 1035943

Park, Ahn, Kim, and Suh: Types and Associated Anomalies of Congenital Scoliosis

Abstract

Study design

A nalysis was based on radiographic appearance of 57 cases of congenital scoliosis and associated anomaly

Purpose

The aim of the present study was to assess the incidence, morphology and the associated anomalies of the congenital spinal scoliosis.

Summary of Literature Review

Hemivertebra is the most common type of congenital scoliosis and urogenital, musculoskeletal and cardiac anomalies are strongly associated.

Materials and Methods

The authors analysed the morphology and the associated anomalies of 57 cases of congenital scoliosis from 1994 to 2000.

Re su l ts

It was more common in male(32 males and 25 females). The bony anomalies were classified as failure of formation(40cases, 70.2%), failure of segmentation(11cases, 19.3%) and mixed type(6cases, 10.5%). Of the failure of formation, there were 36 cases(63.2%) of hemivertebra, 2 cases of posterior quadrant vertebra and 2 cases of wedge vertebra. We found associated anomalies in 26 patients(45.6%). A ssociated cardiac anomalies were 2 dextrocardia, ventricular septal defect, atrial septal defect and patent ductus arteriosus. A ssociated musculoskeletal anomalies were 5 rib fusion, 2 developmental dysplastic hip, 3 Klippel-Feil syndrome, A chondroplasia, A rnold- Chiari malformation, spinal dysraphism with sacral hair patch, cleft palate with congenital anklyloglossia. A ssociated neurogenic anomalies were 2 cases of syringomyelia and 3 mental retardation. There were unilateral renal agenesis and undescended testicle in urogenital anomalies.

Conclusion

Common type of the congenital scoliosis was hemivertebra(63.2%) caused by the failure of formation(70.2%). A ssociated anomalies were found in 26 patients(45.6%).

REFERENCES

1). Benard TN, Burke SW, Jhonston CE, Roberts JM. Congenital spinal deformities. A review of 47 cases. Orthopedics. 8:777–783. 1985.
2). Connor JM, Conner AN, Connor RAC, Tolmie JL, Yeung B, Goudie D. Genetic aspects of early child -hood scoliosis. Am J Med Genet. 27:419–424. 1987.
3). Day G, Upadhyay S, Ho E, Leong J, Ip M. Pulmonary function in congenital scoliosis. Spine. 19:1027–1031. 1994.
4). Drvaric DM, Ruderman RJ, Conrad RW, et al. Co ngen -ital scoliosis and urinary tract abnormalities: Are intravenous pyelograms necessary? J Pediatr Orthop. 7:441–443. 1987.
5). Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome. a constellation of associated anomalies. J Bone Joint Surg. 56-A:1246–53. 1974.
6). Jaskwhich D, Ali RM, Patel TC, Green DW. Congenital scoliosis. Curr Opin Pediatr. 12:61–66. 2000.
crossref
7). McMaster MJ, David CV. Hemivertebra as a cause of scoliosis, A study of 104 patients. J Bone Joint Surg. 68-B:588–595. 1986.
crossref
8). McMaster MJ and Ohtsuka. The natural history of con -genital scoliosis: A study of two hunderd and fifty-one patients. J Bone Joint Surg. 64-A:1128–1147. 1982.
9). Peterson HA, Peterson LFA. Hemivertebra in iden -tical twins with dissimilar spinal columns. J Bone Joint Surg. 49-A:938–942. 1967.
10). Prahinski JR, Polly DW, McHale KA, Ellebogen RG. Occult intraspinal anomalies in congenital scoliosis. J Pediatr Orthop. 20:59–63. 2000.
crossref
11). Reckles LH, Peterson HA, Bianco AJ, Weidman WH. The association of scoliosis and congenital heart defects. J Bone Joint Surg. 57-A:449–455. 1975.
crossref
12). Shapiro F, Eyre D. Congenital scoliosis. A histopathologic study. Spine. 6:107–117. 1981.
13). Suk SI, Yoon GS, Bin SI. The brace treatment of congenital scoliosis. J of Korean Orthop Surg. 20:545–553. 1985.
crossref
14). Tanaka T, Uhtoff H. The pathogenesis of congenital vertebral malformations. Acta Orthop Scand. 52:413–425. 1981.
15). Winter R, Moe J, Lonstein J. The incidence of Klip -pel-Feil syndrome in patient with congenital scoliosis and kyphosis. Spine. 9:363–366. 1984.
16). Wynne-Davies R. Congenital vertebral anomalies: Etiology and relationship to spina bifida cystica. J Med Genet. 12:280–288. 1975.

Figures and Tables%

Fig. 1-A.
Plain radiograph in 1988 shows right T6 hemivertebra fully segmented. Fig. 1-B. 10 years later(1998), the Cobb's angle was increased to 39 degrees.
jkss-8-202f1.tif
Fig. 2.
Whole spine anteriorposterior view shows hemimetameric shift.
jkss-8-202f2.tif
Fig. 3.
21-year-old-female with unilateral bar from T3-T11, the Cobb's angle was 122 degrees.
jkss-8-202f3.tif
Fig. 4-A.
Whole spine anteriorposterior shows right L2 unincarcerated hemivertebra partially segmented. Fig. 4-B. Cervical spine lateral view shows fusion of C1-2 and C4-6 spine.
jkss-8-202f4.tif
Fig. 5.
MRI shows left T12 hemivertebra with T7, 8 butterfly vertebra.
jkss-8-202f5.tif
Fig. 6.
Distribution of Cobb's angle of hemivertebra and unilateral bar shows 32.1 degrees of the mean Cobb's angle in hemivertebra and 55.4 degrees in unilateral bar.
jkss-8-202f6.tif
Table 1.
Associated anomalies in 26 patients with congenital scoliosis.
Cardiac anomalies(No. of patient)
Dextrocardia(2) Ventricular septal defect(1)
Atrioseptal defect(1) Patent ductus arteriosus(1)
Musculoskeletal anomalies(No. of patient)
Rib fusion(5) Developmental dysplastic hip(2)
Klippel-Feil syndrome(3) Arnold-Chiari malformation(1)
Spinal dysraphism & sacral hair patich(1) Achondroplasia(1)
Cleft palate & congenital anklyloglossia(1)
Urogenital anomalies(No. of patient)
Unilateral renal agenesis(1) Undescended testicle(1)
Neurogenic anomalies(No. of patient)
Mental retardation(3) Syringomyelia(2)
Table 2.
Summary of vertebral anomalies
Type of anomaly(No. of patient)
Failure of formation(40)
Hemivertebra(36)
Single hemivertebra(25)
unincarcerated fully segmented(12)
unincarcerated semisegmented(11)
fusion to upper vertebra(8)
fusion to lower vertebra(3)
unincarcerated nonsegmented(2)
Multiple hemivertebra(11)
hemimetameric shift(5)
butterfly vertebra+semisegmented hemivertebra(2)
butterfly vertebra+full segmented hemivertebra(2)
ipsilateral multiple hemivertebra(2)
Posterior quadrant vertebra(2)
Wedge vertebra(2)
Failure of segmentation(11)
Lateral unilateral bar with scoliosis(11)
Mixed type(6)
Posterior quadrant vertebra+block vertebra+unilateral bar(1)
Hemivertebra+block vertebra(3)
Hemivertebra+block vertebra+unilateral bar(1)
Hemivertebra+unilateral bar(1)
Table 3.
The mean of Cobb's angle of hemivertebra and unilateral bar
Type of anomaly location (No. of patient) The mean of Cobb's angle
Hemivertebra T1-T4(1) 40
(full segmented) T5-L1(8) 38.1
L2-L4(3) 41
Hemivertebra T1-4(1) 39
(semisegmented) T5-L1(5) 22.2
L2-L4(5) 24.4
Unilateral bar T1-4(2) 32.5
T5-L1(7) 66.3
L2-L4(2) 40

T∗ : thoracic spine; L: lum mbar spine

TOOLS
Similar articles