Congenital Scoliosis Flashcards
1
Q
Define congential scolisosi?
A
- Failure of the normal vertebral development during 4th-6th wk of gestation
- caused by developmental defect in the formation of the mesenchymal anlage
2
Q
What is the epidemiology of congenital scoliosis?
A
- Prevalence in general population 1-4%
3
Q
What are the causes of congential scoliosis?
A
- Most occur spontaneously
-
Maternal exposure
- diabetes
- alcohol
- Valproic acid- in anticovulsants/ mood stabilisers
- Hypethermia
-
Genetics
- Notch gene
4
Q
What are the associated conditions of congential scoliosis?
A
- Associated conditions in 61%
- Cardiac defects- 10%
- Genitourinary defects- 25%
- Spinal cord malformations
- w underlying syndrome/dhromosomal abnormality
- VACTERL syndrome
- 38-55%
- Vertebral malformations, anal atresia, cardiac malformations, tracheo-oephageal fistula, renal and radial abnormalities, limb defects
-
Klippel- Feil sydrome
- short neck, low hairline, fusion c cpine
-
Jarcho- Levin syndrome/Spondylocostal Dysostosis
- Short trunk dwarfism, multiple vertebral, rib defects and fusion
- most commonly autosomal recessive
- often assoc w thoracic insufficiency syndrome
- shortening of thorax/rib fusion
- results in thorax unable to support lung growth/resp decompensation
5
Q
What is the progression with congenital scoliosis?
A
- Most rapid in first 3 years of life
- Rate of progression- **greatest to least
- Unilateral Unsegmental Bar with contralateral hemivertebra ** 5-10o pa
- Unilateral unsegmental bar
- Fully segmented Hemivertebra
- Unsegmented Hemivertebra
- Incarcerated hemivertebra
- Unicarcerated hemivertebra
- Block vertebra- least chance of progression
- Presence of fused ribs increases risk of progression
6
Q
Can you describe the classification of congenital scoilosis?
A
-
Failure of formation
- Fully Segmented Hemivertebra
- Semisegmented Hemivertebra
- Unsegemented Hemivertebra
- Incarcerated Hemivertebra
- Unicarcerated hemivertebra
- Wedge Vertebra
-
Failure of Segmentation
- Block Vertebra- bilat bony bars
- Bar Body- unilat unsegemented bar
-
Mixed
- Unilateral unsegmented bar with contralateral hemivertebra
7
Q
What investigations are helpful in dx of congenital scoliosis?
A
- X rays
- AP and lateral films usually enough for dx
- CT
- 3d CT better to delinate bony anatomy
- MRI
- in all pt with congential scoliosis prior to surgery to evaluate neural axis abnormality and
- chiari malformation
- tethered cord
- syringomyelia
- diastematomyelia
- intrdural lipoma
- Renal USS
- Echo- cardiac manifestations
- in all pt with congential scoliosis prior to surgery to evaluate neural axis abnormality and
8
Q
What is the tx for congenital scoliosis?
A
Non operative
-
Observation
- for non progression
- incarcerated hemivertebra
- nonsegmental hemivertebra
- some partially segmented hemivertebra
- bracing not indicated in primary tx of congential scoliosis
Surgery
-
Anterior/posterior spinal fusion +/- Vertebrectomy
- pt w failure of formation with contral failure of segmentation- any age
- fusion is short distance after hemivertebrectomy
- young pt with significant progression, neurological deficits or declining respiratory function
-
Growing rod construct
- used in an attempt to control deformity during spinal growth/delay arthrodesis
- may need to be lengthened every 6 months
- Osteotomy of bar
-
Hemiepiphysiodesis
- intact growth plates on concave side
-
Hemivertebrectomy- with progressive curve causing truncal imbalance and oblique take off
- pt <6 yrs, flexible curve <40o
-
Spinal column shortening resection
- those present late and fixed
9
Q
What are the complications of congenital scoliosis?
A
-
Crankshaft phenomenon
- caused with posterior surgery alone
-
Short stature
- growth of spinal column affected by fusion
- younger pt more affected
-
Neurologic injury
- overdistraction
- overcorrection
- harvesting of segmental vessels
-
Soft tissue compromise
- nutritional aspects of care essential to esnure soft tissue healing
