MANAGEMENT OF CONGENITAL SCOLIOSIS

M.BERGOIN Aix en Provence France

Deformities of the spine caused by congenital abnormalites can induced

Congenital scoliosis

Congenital kyphosis

Scoliosis predominant

Kyphosis predominant p

Congenital kyphoscoliosis
Congenital scoliosis is C i l li i i a lateral curve of the spine. More frequent Congenital kyphosis is anabnormal posterior convex angulation of the spine. spine Less common but potentially devastating

EMBRYOLOGY
Congenital scoliosis and kyphosis are produced by some C it l li i dk h i d db disturbance in early development of the spine. The critical period being during the first six weeks of intrauterine life usually sporadic exceptionnally hereditary origin

TYPE OF ANOMALY TOPOGRAPHY AGE OF THE PATIENT NATURAL HISTORY

Winter Mac Master Nasca Touzet Duboussset

TYPE OF ANOMALY
Type I : Failure of formation Type II : Failure of segmentation Type III :Mixed and complex anomalies TYPE I : FAILURE OF FORMATION
Group 1: unilateral failure of V. formation
hemivertebra Wedge vertebra

Group 2: antero central failure of V. formation

Group 3: subtotal agenesis

Binucleate vertebra

HEMIVERTEBRAE

Full segmented H.V

Semi segmented H.V

Nonsegmented H.V

2 fully segmented H.V (Same side)

2 opposing f.segmented H.V

Incarcerated H.V

TYPE OF ANOMALY
Type I : Failure of formation Type II : Failure of segmentation Type III :Mixed and complex anomalies

TYPE II :FAILURE OF SEGMENTATION

BAR

Group 1:unilateral failure of V.segmentation Scoliosis ++

Group 2:complete anterior failure of V.segmentation V segmentation Kyphosis ++ Group 3:progressive anterior congenital fusion

TYPE OF ANOMALY
Type I : Failure of formation Type II : Failure of segmentation Type III :Mixed and complex anomalies

TYPE III : MIXED AND COMPLEX ANOMALIES

Ex: unilateral unsegmented bar With controlateral hemivertebra

Scoliosis +++

TYPE OF ANOMALY AND TOPOGRAPHY

OCCIPITO-CERVICAL
HV 56% Block vert2% e t2% Wedg.vert7% unsegm Bar16%

CERVICAL CERVICO-THORACIC UPPER THORACIC LOWER THORACIC THORACOLUMBAR

Complex malf.19%

LUMBAR LUMBOSACRAL (Junctional zones + + +)

TYPE OF ANOMALY AND NATURAL HISTORY

SEMI SEGMENTED HEMIVERTEBRA

12 YS OLD COBB angle: 22

16 YS OLD COBB angle :28

12 YS OLD COBB angle : 30

15 YS OLD COBB angle : 50

THORACIC

THORACOLUMBAR

TYPE OF ANOMALY AND NATURAL HISTORY

65

IMMEDIATE INSTABILITY
CONGENITAL DISLOCATED SPINE (J.DUBOUSSET)

POTENTIAL INSTABILITY

ASSOCIATED ANOMALIES
Lungs Neurologic Facial Digestive Cardiac Uro-genit Muscl-skelet ARNOLD CHIARI

SYRINGOMYELIA

DIASTEMATOMYELA

CONGENITAL SPINE DEFORMITIES

SURGICAL TREATMENT

LATE SURGERY
Adolescent and adult Ad l t d d lt

SEMI EARLY SURGERY

Between 5 and 10 years old

EARLY SURGERY
Before 5 years old

LATE SURGERY:THORACOLUMBAR BINUCLEATE VERTEBRA

KYPHO-SCOLIOSIS

15 yrs old girl

1st stage post

2nd stage ant

7 ys old

9 ys old

11 ys old

LATE SURGERY LEFT LUMBOSACRAL HV

29 ys old

30 ys old

29 yrs old woman ld

CONGENITAL SPINE DEFORMITIES

SURGICAL TREATMENT

LATE SURGERY
Adolescent and adult Ad l t d d lt

SEMI EARLY SURGERY

Between 5 and 10 years old

EARLY SURGERY
Before 5 years old

1991

1992

1995

10 yrs old boy Congenital scoliosis: COMPLEX ANOMALY SEMISEMI-EARLY SURGERY

Instrumented hemiepiphyseodesis

CONGENITAL SPINE DEFORMITIES

SURGICAL TREATMENT

LATE SURGERY
Adolescent and adult Ad l t d d lt

SEMI EARLY SURGERY

Between 5 and 10 years old

EARLY SURGERY
Before 5 years old

CONGENITAL SPINE DEFORMITIES

EARLY SURGERY
Before 5 years old

METHODS I In situ arthrodesis

(Circumferencial arthrodesis)

II Hemiepiphyseodesis pp y
(Convex side)

III Hemivertebral excision

EARLY SURGERY :HEMIEPIPHYSEODESIS

2 years old girl

Complex cervico-thoracic malformation. lf i

Post op cast

4 years old C6 obliquity : 25

C6 obliquity:39
Ant.and Ant and post hemiepiphyseodesis

EARLY SURGERY HEMIEPIPHYSEODESIS

2 years o d yea s old

C6 obliquity: 39 Cobb angle: 44

12 years old
C6 obliquity: 15 Cobb angle : 31

EARLY SURGERY HEMIEPIPHYSEODESIS

Ben.D 3 years old Bar on concave side H.V on convex side Cobb angle : 78 78 Post.and ant.instr. hemiepiphyseodesis

Ben.D 6 years old y Under traction 3 years post-op

EARLY SURGERY :HEMIEPIPHYSEODESIS

Ben.D Ben D 13 years old 10 years follow up

CERVICO THORACIC JUNCTION

COMPLEX ANOMALIES

HE.B 12.02

4 YEARS OLD GIRL C6 OBLIQUITY: 27

POST-OP

6 MONTHS F- UP C6 OBLIQUITY : 13

3 YEARS F-UP C6 OBLIQUITY : 6

INSTRUMENTED

HEMIEPIPHYSEODESIS

COMPLEX ANOMALIES

1999

2004

H.V. EXCISION
An effective hemivertebral resection consists of immediate correction of the deformity coupled with an anterior and p y p posterior usually y instrumented arthrodesis of the abnormal area : M.Bergoin (1976) In 1976 we began to use a new and safe technique : one stage first posterior and second anterior excision associated with an anterior fusion and posterior instrumented fusion

H.V. EXCISION (lumbosacral)

1978

4 years old

6 years old

8 years old

14 y. old

LUMBOSACRAL JUNCTION

4 months

4 years 4 months old

13 years old

H.V. L4-L5

LUMBOSACRAL JUNCTION

H.V.L4-L5 3 years old

H.V.L4-L5 2 years old

LUMBOSACRAL JUNCTION

2000

2005

Hemivertebral excision
THORACOLUMBAR JUNCTION

HV

EXCISION

1999

2004

CONCLUSIONS
-PROPHYLACTIC TREATMENT BEFORE SIGNIFICANT DEFORMITY -AVOID SALVAGE PROCEDURE -AVOID UNNECESSARY TREATMENT AVOID

-SURGERY BEFORE THREE YEARS OLD SURGERY IS RECOMMANDED