Deformities

Question 1

What is the Madelung deformity?

Answer 1

Madelung deformity is caused by epiphyseal growth plate disturbance at the ulnar side of distal radius, resulting in a short radius compared with the ulna.

The growth plate injury may be vascular, infectious, traumatic or muscular.

Question 2

What conditions are associated with Madelung deformity? “HIT DOC”

Answer 2

Hurler’s syndrome (mucopolysaccharidosis), achondroplasia
Infection
Trauma
Dyschondrosteosis (mesomelic dwarfism)
Osteochondromatosis (diaphyseal aclasia)
Congenital Turner’s syndrome

Question 2

What conditions are associated with Madelung deformity? “HIT DOC”

Answer 2

Hurler’s syndrome
Infection
Trauma
Dyschondrosteosis (mesomelic dwarfism)
Osteochondromatosis (diaphyseal aclasia)
Congenital Turner’s syndrome

Question 3

Features of Madelung deformity

Answer 3

1. Shortening and bowing of radius bones causing dorsal subluxation of the distal radioulnar joint.
2. V-shaped proximal carpal row
3. positive ulnar variance.
4. pisiform can impacted against the ulna

Question 4

What is pseudo-Madelung deformity?

Answer 4

Pseudo-Madelung deformity refers to increased radial inclination (i.e. ulnar tilt) of the distal radius but with negative ulnar variance and the absence of other typical features of Madelung deformity

Question 5

What are the causes of pseudo madelung deformity?

Answer 5

1. hereditary multiple exostoses
2. post-trauma, e.g. physeal injuries
3. rickets
4. enchondromatosis - Ollier disease
5. post-inflammatory or infectious arthritis
6. achondroplasia
7. mucopolysaccharidoses, e.g. Morquio syndrome, Hurler syndrome

Question 6

What is metaphyseal flaring/Erlenmeyer flask deformity? LEAD Gnome (gnome storing calcified blood)

Answer 6

1. Anaemias (thalassaemia, sickle cell anemia, pure red cell aplasia)
   fibrous dysplasia
2. Storage diseases (Gaucher disease , Niemann-Pick disease)
3. Bone dysplasias: fibrous dysplasia, multiple osteochondromatosis, enchondromatosis amongst many others
4. Osteopetrosis