Blount's Disease

 

BlountsBlounts

 

Definition

 

Infantile tibia vara

- progressive varus deformity of knees

- secondary to abnormality of medial upper tibial physis

 

Epidemiology

 

African descent / males / obesity

 

Etiology

 

Disruption of normal medial endochondral bone formation

 

Unknown 

- no consistent inheritance pattern

- ? due to abnormal compression on medial side of proximal tibial physis

 

Types

 

Infantile Adolescent

Onset 1 - 3 years

Bilateral

Most common

Onset > 6 years

Unilateral

Rare

 

Clinical

 

Bilateral & symmetrical bowing

- age 1 - 3

- walking

- normal physiological varus should resolve by age 2

 

Varus knee

Tibial torsion

 

X-ray

 

Blounts

 

Findings

- medial beaking of the epiphysis

- widened and irregular medial physis

- medial slope of the epiphysis

- metaphyseal varus

 

BlountsBlountsBlounts

 

Metaphyseal-Diaphyseal Angle Medial physeal slope

Line perpendicular to axis of tibia

Line through medial and lateral metaphyseal beaks

 

Line through medial physis

Line through lateral physis

Physiologic bow legs < 11°

Blount's > 11°

Definitive Blount's > 16o

High risk of progression if > 60°
MDA Metaphyseal angle

 

CT

 

Used to identify presence of physeal bar

 

Langenskiold Classification

 

Six stages

- stages I - III: reversible with bracing

- stages IV - VI: permanent damage to eiphysis / need surgery

 

Stage I Stage II Stage III
Medial beak Medial saucer shaped defect Develop step
Age 2 - 3 Age 2 - 4 Age 4 - 6

 

BlountsBlountsBlounts

 

Stage IV Stage V Stage VI

Narrow physis

Step deepens

Medial epiphysis splits into two

Physeal bar

Medial growth arrest

Develop severe varus

Age 5 - 10 Age 9 - 11 Age 10 - 13

 

BlountsBlountsBlounts

 

Differential diagnosis

 

Ricketsachondroplasia

Rickets                                                              Achondroplasia

 

Physiological varus - normal growth plate, metaphyseal-diaphyseal angle < 11°

Ricket's - widened physes / cupped metaphyses / flared distal distal

Metaphyseal chondrodysplasia

Achondroplasia

Trauma / tumour / infection

Osteogenesis imperfecta

Juvenile rheumatoid arthritis

 

Natural history

 

Progresses to severe osteoarthritis by early adulthood

 

Disease progression 

- metaphyseal-diaphyseal angle >16°  - 95% chance of progression

- metaphyseal-diaphyseal angle < 11° - 95% chance of spontaneous resolution

- metaphyseal-diaphyseal angle < 11 - 16° - close observation

 

Management 

 

Algorithm

 

Depends on

- age of child

- stage of disease

 

1.  <2 years 

 

Observe

 

2.  2 - 3 years & Medial Physeal Angle < 60°

 

KAFO Single Medial upright 

- free ankle with no knee hinge

- flexion limited

- knee cuff pulls it into valgus

 

Full-time bracing successful > 50%

 

3. Age > 3 years / Progression in Brace / Medial Physeal Angle > 60° 

 

Aim

- correct varus and internal rotation deformity

 

Options

 

A.  Lagenskiold I - IV

- osteotomy

- guide growith

 

B.  Lagneskiold V / VI

- take down bar and osteotomy or

- epiphysiolysis + medial metaphseal osteotomy

 

Langenskiold Stages I-IV Surgical Management

 

1.  Osteotomy

 

Aim

- restore alignment

- deformity reversible

- if restore physiological valgus (7o) then resolution is usual for I & II / possible for III & IV 

 

Type of osteotomy

 

A.  Opening / closing wedge

B.  "Smiley" upside down dome

C.  Oblique osteotomy

- Rab biplanar oblique osteotomy

- fix with single screw

 

Osteotomy Technique

 

Performed distal to TT

- closing wedge simplest but upside down dome has least shortening

- must osteotomise fibula

- usually want to correct IR deformity at same time

- must release anterior compartment to prevent compartment syndrome

- desired valgus & ER achieved

- fixation with K wires or screw

- POP post operatively

 

Recurrence after osteotomy

 

1. Obese

2. > Stage III

3. Medial physeal slope > 60°

4. Age

- > 5 y = 76%  

- < 5 y = 31%

 

2.  Guided growth / 8 plate

 

Now common mechanism of treating condition

 

3.  Osteotomy and external fixation

 

Langenskiold Stages V & VI 

 

Issue

 

Irreversible

- need to address physis as well as osteotomy

- usually total physiodesis

- overcorrection 10°

 

Surgery 

- must do fibula osteotomy as well

- usually perform total physeodesis of ipsilateral side

- always perform fasciotomy

- may need to realign epiphysis in severe forms with large medial-physeal slope

- consider epiphysiodesis of other side to address LLD

 

Options

 

1.  Medial Metaphyseal Elevation Osteotomy

 

Indications

- Grade V

 

Blounts Elevation

 

2.  Physeal Bridge Resection (physeolysis) + Osteotomy

 

Indications

- Grade VI

- bridge < 30% of physis

 

Technique

- excise bar where CT shows a bridge

- Insert fat into defect

 

3.  Lateral Hemi-epiphysiodesis + osteotomy

 

Indications

- grade VI

- bridge > 30%

 

Technique

 

All need fibula osteotomy

All need prophylactic compartment release

 

Complications

 

Compartment syndrome - must prophylactic release

Recurrence of varus - usually secondary to physeal bar

LLD

OA

 

Adolescent Type

 

Management

 

Wait till skeletal maturity, then HTO