Madelung's Deformity

Madelung's deformity with fracture

 

Pathology

 

Premature fusion of the volar & ulna aspects of the distal radius

- progressive ulnar & volar tilt of distal radial surface

 

Ulna becomes prominent through normal growth & subluxes dorsally

 

Lunate lies deeply between lower end of radius and ulna

 

Aetiology

 

1. Volar-ulnar radial physeal arrest

- focal physeal dysplasia

 

2. Carpal tether from same area to lunate

- Vicker's ligament

 

Anatomy Vickers' ligament 

 

Vickers describes fibrous band 

- runs from radius to lunate

- volar position

- large ligament ~ 0.5cm diameter

- may be the cause

- if excise early / before age 8 the radius improves in shape

 

Epidemiology

 

Idiopathic condition

- AD with incomplete penetrance

- F > M

- often bilateral

 

Association

 

Turner's Syndrome

 

Disorder of gonadal differentiation

- 45 X / XO

- female phenotype / sterile

- webbing of neck

- low posterior hairline

- cubitus valgus

- cardiac abnormalities

 

DDx Madelung Deformity

 

Post Traumatic

Enchondromatosis (Ollier's)

Hereditary Multiple Exostoses

Turner Syndrome

Idiopathic = Dyschondrosteosis

Infection

General dysplasia

 

Classification

 

Madelung's - ulnar, volar 

Chevron - ulnar, central 

Reverse Madelung - ulnar, dorsal

 

Clinical

 

Usually asymptomatic and good function

 

Wrist is in radial deviation

 

Dorsal subluxation of distal ulna

 

Reduced dorsiflexion / supination

 

NHx

 

Not apparent in childhood

Usually becomes obvious as hits adolescent growth spurt

 

X-ray

 

Madelungs APMadelung's Lateral

 

Volar-ulna tilt of the distal radius with dorsal subluxation of ulna / distal radius triangular shape

 

Proximal carpal row becomes wedge shaped

- lunate "falls into" the radio-ulna gap

 

DRUJ widened

 

Chevron 

- proximal carpal row distorted / V-shape

- lunate in apex between radius and ulna

 

MRI

 

Look for Vicker's ligament

 

Management

 

Mothers function is a good guide to management

 

Surgical Indications

 

1. Pain 

- must operate

- surgery very good at relieving pain

- in adults division Vickers' ligament almost always relieves pain 

 

2. Deformity 

- deformity starts ~ age 9 years (doesn't occur much before then) 

- best to operate early as will get good result

 

Options

 

1.  Excision Vicker's ligament + Epiphysiolysis of fused physis with fat graft

 

2.  Complete epiphysiodeses 

 

3.  Epiphysiodeses of distal Ulna

 

4. Ulna Shortening / Darrach

 

Madelung's with Darrachs

 

5.  Opening Wedge Osteotomy

 

Madelung's Osteotomy

 

6.  Wrist Arthrodesis