Why neuromuscular conditions? The spastic hand Matthew Nixon Consultant orthopaedic hand surgeon Complex decision making MDT approach Often told nothing can be done Variety of surgical treatments Multilevel, tendon transfers, fusions Little need for complex / expensive kit Spastic CP arthrogryposis What is? FSHD OBPI Stroke hemiplegia CP hemiplegia Dystonic CP MPS Velocity dependent hypertonia Corticospinal tract Primary pathway of motor neurons Not directly affected in Rubrospinal tract Cyclical, non-voluntary movements 1 st catch - Fast stretch Reticulospinal tract Inhibitory function => dec tone Eg prevent flexor response to stimuli 2 nd catch - Slow stretch Vestibulospinal tract Excitatory function to maintain posture/balance 1
Kennard principle: Activation of unaffected hemisphere Pathophysiology of neuromuscular conditions Small lesion intermediate Large lesion Weakness / Imbalance of forces Correctable Fixed Kennard MA. Age and other factors in motor recovery from precentral lesions in monkeys. Am J Physiol 1936; 115: 137 46. Staudt M. Two types of ipsilateral reorganization in congenital hemiparesis. Brain. 2002 Skeletal deformity Incidence Upper limb involvement Cerebral palsy Most common physical disability in children 1 in 2000, many living into adulthood 92% Wrist 59 deg flexion 85 deg pronation 15% Shoulder Add & Int rotation Incidence of upper limb involvement 82% had some upper limb involvement 69% had reduced motor control 36% had a upper limb Only 12% had seen a specialist for treatment of UL 50% Hand 65% thumb in palm 35% finger deformity 77% Elbow 55 deg flexion Matthew Nixon. Prevalence and pattern of upper limb involvement in cerebral palsy. J Child Orthop. 2014. Matthew Nixon. Audit of cerebral palsy upper limb involvement at RMCH. 2014. Upper limb involvement Manual Ability Classification System Motor function Grasp Release Dexterity Mirror movements Carer Washing Dressing Hand hygiene 1 2 3 4 5 Appearance Pain Joint subluxations Easy manipulation Reduced speed Needs adaptations Poor despite adaptations Severe limitations 13% 33% 16% 19% 19% Nixon et al. Incidence UL involvement in CP. British Society Surgery to the Hand. 2011 2
Gross Motor Functional Classification System 1 2 3 4 5 Differences in management of upper and lower limb Widely used. Observer measured score 1-5. Measure of lower limb function and mobility. Strong correlation to MACS Lower limb Closed kinetic chain (Stabilised by ground reaction force) => Harder to over correct Power important Upper limb Open kinetic chain (No ground reaction force) => Very easy to over correct Fine motor control important Decision making factors Age Age dependent factors Active control Time since injury Functional Dystonia vs FFD Growth velocity Hägglund G.. Development of with age in children with CP. BMC Musculoskelet Disord. 2008 3-7 years Age High Very high risk of over correction Botox neurectomy Thumb in palm Wrist flexion Elbow flexion Most disabling 7-12 yrs >13 yrs Moderate more growth potential risk of over-correction Less Less growth potential Constrained procedure Unconstrained rebalancing comparison of AbliHand logit score vs normal all p<0.01 MWU Patterson J, Late deformities following the transfer of FCU to ECRB in children with CP J Hand Surg Am. 2010. M Nixon et al. Functional impact of CP hand s. J Children s Orthopaedics. 2014 3
One third of older children have cosmetic concerns vs Odds Ratio = 3.1 (1.1 to 8.6), Chi sq= 0.029 1 Mild tone Catch <50% ROM 2 Mod tone Catch >50% ROM 3 Severe tone Entire ROM 4 Fixed deformity Modified Ashworth classification Contracture Early disease Overactive muscle Head Injury Fibrosis & Nixon et al. Functional impact of CP hand s. J Children s Orthopaedics (in press). 2014 Botox, splinting Tendon transfers Poor response to botox Contracture release Joint stabilisation Is there active control? Management No active control Joint stabilisation -splints, arthrodesis Active control present Joint rebalancing - release -tendon transfer Upper motor neuron Muscle relaxants, ITB Hand therapy: CIMT Spine Selective dorsal rhizotomy Lower motor neuron Selective peripheral neurotomy Neuromuscular junction Botulinium toxin Muscle / Tendon Tendon transfer Joint Arthrodesis, splints Sakzewski L. RCT of upper limb CIMT versus standard care for children with unilateral cerebral palsy. Dev Med Child Neurol. 2015 Jan 4
Botulinium toxin Cochrane review Benefits for 3-6 months NICE (2012) Motor function, cosmesis, pain, hygiene FCR PT PT Sakzewski L. RCT of upper limb CIMT versus standard care for children with unilateral cerebral palsy. Dev Med Child Neurol. 2015 Jan Coghill J. Do Lycra garments improve function and movement in children with cerebral palsy? Arch Dis Child. 2010 May Thomson K. Commercial gaming devices for stroke upper limb rehabilitation: a systematic review. Int J Stroke. 2014 Role in my practice Diagnose which muscles misfiring Dystonia vs Buy time in young children Those not suitable for surgery B Hoare. Botulinium toxin as an adjunct for children with CP. Cochrane review. 2010 Selective Dorsal Rhizotomy Removes afferent feedback Has a place in lower limb Good for generalised reduction in No evidence for specific use in the upper limb Gigante P, M Reduction in upper-extremity tone after lumbar SDR in children with spastic cerebral palsy. J Neurosurg Pediatr. 2013 Selective peripheral neurotomy Young (4-10 years), high, good response to botox 50% reduction Reduction associated pain Improvement in function Benefits last up to 5 years A- median nerve B- AIN C- pronator teres muscle. D- pronator teres nerve E- flexor carpi radialis, F- flexor digitorum profundus G- flexor digitorum supercialis Maarrawi J, Long-term results of selective peripheral neurotomy for the treatment of spastic upper limb. J Neurosurg. 2006 Kyung Woo Kwak, Surgical Results of Selective Median Neurotomy for. J Korean Neurosurg. Aug 2011. Elbow Brachialis aponeurosis Biceps lengthening +- Brachioradialis/elbow capsule Wrist deforming forces Wrist vs finger flexors Pronator teres Long extensor weakness Outcome at 5 years 50 deg resting posture 20 deg active extension 5 deg active flexion No change supination Gong HS. Early results of anterior elbow release in patients with CP. J Hand Surg Am. 2014 5
Wrist Flexion Finger flexor Wrist flexion deformity FCU => ECRB FCU => EDC Wrist flexed, fingers flexed Wrist flexed, fingers extended Active control + Passively correctable FCU Rebalancing ECRB transfer EDC transfer Wrist flexors isolated Fingers flex as wrist extended Outcome at 17 years 90% cosmetic improvement 80% functional improvement Beach WR. Use of the Green transfer in treatment of patients with spastic CP: 17-year experience. JPO 1991. Wrist flexion deformity Fixed flexion Proximal row carpectomy Wrist fusion Wrist fixed flexion with clasp hand Outcome of 41 wrists - 98% union, 94% satisfiaction - Disability Assess. Scale from 9.6 to 5.5 - Improvement appearance VAS by 7.9 Van Heest AE, Strothman D. Wrist arthrodesis in cerebral palsy. J Hand Surg Am. 2009. 1. PRC + arthrodesis 2. Fractional lengthening 3. FCU to EDC transfer Hand Thumb in palm - classification 1 2 3 4 Swan neck deformity - Grasp and release Clasp hand - Hand hygiene MC adduction Adductor Policis MCPJ flexion Flexor Pol Brevis CMCJ Instability AP & FPB EPL/APL active MCPJ and IPJ AP &FPB FPL Thumb in palm - Manual dexterity 6
Swan neck deformity FDS and lumbrical Hyperlaxity Contracture release -1 st webspace -Intrinsic/extrinsic release Joint stabilisation -MCPJ arthrodesis -Sesamoid arthrodesis Rebalancing -EPL translocation -FPL lengthening +- BR transfer Tonkin MA, Sesamoid arthrodesis for hyperextension of the thumb metacarpophalangeal joint. J Hand Surg Am. 1995 Gwilym S, Giele HP. Sesamoid arthrodesis of the thumb: a technique using a Mitek anchor and wire suture. Ann R Coll Surg Engl. 2005 Central slip release Simple, effective Lateral band advancement 40% 5 year recurrence Carlson MG. J Hand Surg Am. 2007. Surgical treatment of swan-neck deformity in hemiplegic cerebral palsy. de Bruin M. J Pediatr Orthop. 2010. Long-term results of lateral band translocation in cerebral palsy. Acquired brain injury Glove and stocking Shoulder subluxation Skeletally mature High anaesthetic risk Botulinium toxin Hand hygiene Skin maceration Poor grasp and release Pre-op Distal FDS release Proximal FDP release STP transfer Mass anastamosis Post op The hemiplegic shoulder Flaccid paralysis Spastic paralysis rebalancing constrained Botulinium / neurectomy Therapy / CIMT Inferior subluxation: Biceps suspension procedure Reduces pain (mean VAS 1.5) Maintains passive ROM Contractures: Soft tissue release Pec major, Lat dorsi, subscap 0 2 4 6 8 10 12 14 16 Age (yrs) Nixon M, Manara J. Management of shoulder pain after stroke. J Shoulder elbow Surgery. Accepted 2014 Namdari S, Outcomes of the biceps suspension procedure for inferior glenohumeral subluxation in hemiplegia. JBJSAm. 2010 7
Summary Upper limb involvement in CP is common Many patients are not referred for treatment Cheap and effective treatments are available Specialist services are available locally 8