Congenital Kyphosis

Definition

 

Kyphosis due to congenitally anomalous vertebrae

- characterized by severe angular deformity

- prominent gibbus at the apex of the curve

 

Classification

 

Type I - Failure of formation

 

Failure formation anteriorly + preservation of posterior elements

- hemivertebrae / wedge vertebrae

- most common

- produces the worst deformity

- NHx is one of relentless progression

- 7per year

- most likely to result in paraplegia

 

Congenital Kyphosis XrayCongenital Kyphosis CT

 

Type II - Failure of segmentation

 

Anterior unsegmented bar

- better prognosis

- produces more rounded kyphosis

- deformity progresses more slowly

- paraplegia is uncommon

 

Type III

 

Mixed pattern

 

Clinical Features

 

Severe deformity maybe obvious at birth

- less obvious deformities may not appear until later

- may be accentuated during adolescent growth spurt

 

Progression occurs to end of growth & often after growth complete

 

Mechanism

 

Due to

- growth differential

- erosion of vertebral body from mechanical pressure

 

Issues

 

Deformity can become very severe (Type I)

- breakdown of the overlying soft tissues

- compression of abdominal viscera

- impairment of pulmonary function

 

Paraplegia may occur (Type I)

 

Management

 

Non operative not effective

 

Work up

 

MRI / Neurosurgery

- exclude intraspinal pathology

 

Echocardiogram

- cardiac abnormality

 

Abdominal US

- renal abnormality

 

Operative

 

Type I

 

Ideal treatment is early detection & early posterior fusion

- best results if fusion by age 3

- can be done as early as 6/12

 

A.  < 5 years & curve < 50o

- posterior spinal fusion

 

B.  > 5 years and > 500

- anterior and posterior fusion

 

C.  Neurology

- anterior decompression first

 

Congenital Kyphosis CTCongenital Kyphosis Correction Lateral

 

Type II 

 

If early, can treat with anterior osteotomy of unsegmented bar

- i.e. epiphyseolysis

 

If late, requires posterior fusion