Madelung's Deformity

Original Editor - Shwe Shwe U Marma Top Contributors - Shwe Shwe U Marma, Oluwafikunayomi Wole-Bankole and Kim Jackson

Introduction

Madelung’s deformity is a condition of the wrist characterized by a shortened distal radius with volar–ulnar curvature and a dorsally prominent distal ulna.This condition could be congenital(multiple osteochondrom) or acquired as a result of a trauma to the growth plate (traumatic early closure of the epiphysis or Salter-Harris fracture (type V).

Aetiology

This condition is caused by an abnormal ligament discovered by Vickers and Nielsen. This ligament,5mm in diameter lies anterior to the volar-ulnar corner of the distal radial metaphysis, crosses the physis and inserts onto the lunate. It is found more in females and is usually associated with a mesomelic form of dwarfism known as Leri-Weill dyschondrosteosis or caused by mutation in the Short-stature homeobox (SHOX) gene (haploinsufficiency) which may lead to skeletal dysplasia such as the Madelung's Deformity. The volar ulnar corner of the distal radius is a critical point located between the radial calcar, distal ulna, and carpus and it is responsible for maintaining stability while transferring force from the carpus.Due to the position of the Vickman's ligament of crossing the volar-ulnar part of the distal radial metaphysis it restricts movements of the wrist and causing reduced stability of the wrist joint.

Madelung's deformity

A Vickers’ ligament is the feature to differentiate Madelung’s deformity from Madelung-like deformities.

Epidemiology

• Predominantly adolescent females are affected by a ratio of 4:1 and it does not usually manifest until 10-14 years of age, The deformity often occurs bilaterally Madelung deformity is relatively rare, with a prevalence of less than 2% of pediatric hand deformities, therefore less than 0.03% of the total population. It is common in patients with short stature as this stature is mostly attributed to the mutation in the SHOX gene. "In a study done, the mean age for patients who presented with Madelung's deformity was 22.5 years with a range from 10 years to 64 years and (77%) were female

Pathogenesis

Premature growth plate arrest at the medial volar aspect of the distal radius causes Madelung deformity. Repetitive traumatic pressure may result in Madelung-like deformity. Mutation or absence of the short stature homeobox (SHOX) gene is thought to be the cause of congenital Madelung deformity.Congenital Madelung deformity can occur as a part of Leri-Weill dyschondrosteosis (LWD) or Turner syndrome.

Clinical Features

• Initially asymptomatic, progressive clinical deformity
• Pain at wrist
• Limited range of motion
• Decreased wrist extension and supination; the distal ulna is prominent and dorsally dislocated
• Obvious hand-wrist deformity
• Distal radial abnormality or carpal subluxation

Diagnosis

Radiology investigations such as the X-ray and MRI

Key Features to note on an X-Ray are;

1. increased dorsal and radial convexity of the distal radius
2. increased volar and ulnar tilt of the distal radial articular surface
3. widened interosseous space
4. relative dorsal position of the ulnar head
5. pyramiding of the carpus

MRI

MRI is done on the patients who need the surgical release of Vickers’ ligament to prevent deformity progression.

Differential Diagnosis

• Turner syndrome
• Nail-patella syndrome
• Hereditary, multiple exostoses
• Ollier’s disease
• Achondroplasia
• Multiple epiphyseal dysplasias
• Mucopolysaccharidoses (Hurler and Morquio syndrome)

Treatment

Physiotherapy Management

The aim is physiotherapy's management is to relieve pain,improve the range of motion of the wrist joint, prevent deformity and increase the functional capacity and dexterity of the wrist.

1. Pain management
   • Cold
   • whirlpool immersion
   • Transcutaneous electrical nerve stimulation (TENS)
2. Volar splint is used to immobilize tissue injuries which could be traumatic or non-traumatic conditions of the hand and the wrist
3. Range of Motion exercises as limited pronation and supination suggests the need of exercise to help maintaining and/or increasing the power of involved muscles, i.e.- pronators and supinators
4. Exercises with Dexterity box
5. Passive movement and Stretching.
6. Muscle Strengthening

Medical and Surgical Management

• Radial or ulnar osteotomy
• Use of oral analgesics
• Vickers’ ligament resection
• Ulnar epiphysiodesis