Volkmann Ischaemic Contracture

Volkmann ischaemic contracture is a fixed flexion contracture of the wrist and fingers (and sometimes the foot) caused by necrosis of muscle and nerve and fibrosis after ischaemia, most often from an unrecognised or untreated forearm compartment syndrome. The flexor digitorum profundus and flexor pollicis longus, in the deep volar compartment, are the most affected.

The classic cause is a supracondylar humerus fracture in a child (the brachial artery is compressed or in spasm), followed by forearm fractures, tight casts, crush injuries, vascular injury, burns, snake bite and prolonged limb compression. The incidence has fallen with better recognition and early fasciotomy.

Tsuge classified severity.

• Mild (localised) has a contracture of 2 or 3 fingers with a mild sensory loss.
• Moderate has all fingers and the thumb flexed with some wrist flexion and sensory loss.
• Severe (generalised) has involvement of all flexors and extensors with a severe fixed deformity and nerve palsy.

Complications are a clawed hand with loss of grasp, fixed wrist and finger flexion, median and ulnar nerve loss, muscle atrophy, joint stiffness, shortening of the forearm, and psychological and social handicap.

Pathophysiology.

Ischaemia lasting over 6 to 8 hours leads to muscle necrosis. The centre of the deep flexor muscle mass is affected most, since it is the farthest from collateral blood supply. The necrotic muscle is replaced by scar tissue that contracts, shortening the muscle tendon unit and pulling the fingers and wrist into flexion. Nerves running through the affected compartment (median and ulnar) are damaged by ischaemia and entrapped in scar tissue.

The deformity develops over weeks to months after the acute event: flexion of the wrist, flexion of the interphalangeal joints, hyperextension of the metacarpophalangeal joints (claw hand), pronation of the forearm, and a fixed thumb in the palm. Finger extension is blocked when the wrist is extended and improves when the wrist is flexed (the tenodesis effect shows muscle contracture).

Examine the skin (scars, atrophy), sensation (median and ulnar distributions), the strength of each muscle group, passive range of motion with the wrist flexed and extended, the pulses, and the forearm compartments. The Volkmann sign (finger extension only with wrist flexion) confirms the contracture.

• The diagnosis is clinical, based on the history of acute ischaemia and the typical deformity.
• Radiographs of the forearm, wrist and elbow show the fracture, malunion, bone shortening and joint contracture.
• MRI shows muscle fibrosis and the extent of necrosis. Ultrasound is useful in children.
• Nerve conduction studies and electromyography document nerve injury and direct nerve surgery.
• Vascular imaging (duplex, angiography) when the arterial supply is in doubt.
• Rule out other causes of contracture, such as cerebral palsy, Dupuytren contracture and congenital contracture.

Differential diagnosis.

• Cerebral palsy and spastic hand deformity.
• Dupuytren contracture and congenital arthrogryposis.
• Brachial plexus injury and Klumpke palsy.
• Tendon adhesion after trauma and complex regional pain syndrome.

Prevention is the key. Diagnose and treat acute compartment syndrome early with a fasciotomy (urgent decompression with release of all compartments). Monitor all children with supracondylar fractures for 24 hours.

Mild contracture (Tsuge mild).

• Physiotherapy with stretching, dynamic and static progressive splinting (night splints holding the wrist and fingers in extension), serial casting.
• Surgery for failure: slide of the flexor muscle origin (Scaglietti), or tendon lengthening (Z-lengthening of the flexor digitorum profundus and flexor pollicis longus).

Moderate contracture.

• Excision of the necrotic muscle with neurolysis of the median and ulnar nerves.
• Flexor origin slide, with tendon lengthening or tendon transfers (brachioradialis to flexor pollicis longus, extensor carpi radialis longus to flexor digitorum profundus).
• Postoperative: splinting in the safe position and early movement from 3 to 4 weeks.

Severe contracture.

• Excision of dead muscle with nerve grafting and tendon transfers.
• Free functioning muscle transfer (gracilis) with vascular and nerve anastomosis in the late stage, for those who lack active finger flexion.
• Proximal row carpectomy, wrist fusion, or forearm bone shortening to relieve tension.
• Rehabilitation by a hand therapist: splinting, sensory re-education, strengthening and functional training. Long term support for children.

Operative antibiotic prophylaxis. Cefazolin 2 g intravenously (3 g when weight is 120 kg or over) within 60 minutes before incision as a single dose (repeat after 4 hours or blood loss over 1500 mL).

With severe penicillin allergy, use vancomycin 15 mg/kg intravenously starting 90 to 120 minutes before incision as a single dose, with levels and creatinine checked in prolonged cases. In children use cefazolin 30 mg/kg intravenously (maximum 2 g) within 60 minutes before incision.

Analgesia.

• Paracetamol 1 g orally every 6 hours as needed for up to 5 to 7 days (maximum 4 g daily; 3 g daily when weight is under 50 kg, with chronic alcohol use or hepatic impairment).
• Ibuprofen 400 mg orally every 8 hours with food for up to 5 days (maximum 1200 mg daily); avoid with eGFR under 30 mL/min/1.73 m2, peptic ulcer, heart failure or anticoagulant use.
• Oral morphine 5 to 10 mg every 4 hours as needed for severe pain for up to 3 days (start 2.5 to 5 mg with age over 75 or eGFR under 30 mL/min/1.73 m2; avoid exceeding 60 mg in 24 hours without senior review). Monitor sedation and respiration and prescribe a laxative.
• Children: paracetamol 15 mg/kg orally every 4 to 6 hours (maximum 75 mg/kg daily and not exceeding 4 g daily) and ibuprofen 10 mg/kg orally every 6 to 8 hours with food (maximum 40 mg/kg daily and not exceeding 2400 mg daily). Give both for up to 5 days.
• Neuropathic pain: gabapentin 300 mg orally at night, increased by 300 mg every 1 to 3 days to 300 to 600 mg three times daily (maximum 1800 mg daily; reduce at creatinine clearance under 60 mL/min); monitor sedation and dizziness.

Monitoring. Hand function, range of motion, sensation and grip at each visit (every 6 weeks in the first year after surgery). Splint compliance and skin. Nerve recovery with electromyography at 3 to 6 months. Growth and deformity in children until skeletal maturity.

Referral and escalation. Refer every established contracture to hand surgery or paediatric orthopaedics. Emergency referral for any suspicion of acute compartment syndrome in a limb with a cast, a supracondylar fracture, or pain out of proportion.

Special groups.

• Children: the typical patient has a supracondylar fracture; late reconstruction is difficult and growth is affected.
• Adults after drug related prolonged compression or after tight casts: lower threshold for fasciotomy.
• Kidney impairment: avoid ibuprofen, reduce morphine doses and reduce gabapentin.