CEREBRAL PALSY prevalence of cerebral palsy is approximately 1.0–2.5 per 1000 live births strongly influenced by gestational age and birthweight risk factors - preconceptional, antepartum, intrapartum, and postneonatal periods Classification 1. Monoplegia 2. Hemiplegia 3. Diplegia 4. Tetraplegia 5. Quadriplegia Also spasticity, flaccidity and athetosis Team approach Surgery is reparative not curative Goals 1. Improve function 2. Improve hygiene 3. Improve cosmesis Problems: 1. 2. 3. 4. 5. 6. shoulder internal rotation elbow flexion forearm pronation wrist flexion intrinsic spasticity thumb in palm deformity Dystonia Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions result in twisting and repetitive movements, abnormal postures, or both. associated with abnormalities of the basal ganglia Despite its name children with dystonia may not have abnormal muscle tone at rest Spasticity velocity-dependent increase in muscle resistance to stretch that contributes to the impairment of function and reduced muscle growth. Defined by classic characteristics. 1. selective a. predominant in flexor and adductor muscles b. responsible for the characteristic flexion-pronation deformity of the upper limb c. more severe in the distal part of the upper limb 2. 3. 4. 5. d. wrist flexors, especially the flexor carpi ulnaris (FCU), are frequently the muscles most involved by spasticity in the upper limb, leading to a characteristic hyperflexion and ulnar deviation of the wrist. e. Spasticity of the finger extrinsic muscles is tested by tapping briefly on the pulps (flexors) or the nails (extensors), which produces an exaggerated response of the tested muscles. f. intrinsic-plus deformity (MP joint flexion and IP joint extension) results from spasticity of the interossei. g. swan neck deformity may be the result of either excessive traction on the extensor tendons caused by excessive wrist flexion (extrinsic swan neck) or to spasticity of the interossei muscles (intrinsic swan neck), in which case it is often associated with MP joint flexion - passively flexing the wrist usually reduces the intrinsic swan neck, whereas it increases the extrinsic swan neck. h. A claw-type deformity with the MP joints hyperextended and the PIP joints flexed usually is caused by a combination of excessive traction on the extensor tendons and paralysis of the intrinsic muscles. i. Thumb spasticity involves mainly the adductor pollicis, resulting in an adducted position. When the flexor pollicis longus (FPL) also is involved, a thumb-in-palm deformity develops. elastic resistance a. attempts at stretching the involved muscles meet with resistance, which increases with the amount of strength applied and which correlates with the degree of spasticity. b. Unlike plastic contractures, the joint returns to its initial position as soon as the opposing force is stopped. If the opposing force is maintained long enough, however, the muscles usually yield, sometimes abruptly (this is referred to as the "penknife blade" phenomenon). present at rest and exaggerated with voluntary movement, emotion, fatigue, and pain. Osteotendinous reflexes are exaggerated, brisk, diffuse, and polykinetic. Clonus is infrequent in the upper limb. Synkineses may be associated. Clinical Considerations 1. Evaluate spasticity – differentiate from contracture 2. Evaluate the motor and sensory deficit in the upper limb 3. Evaluate the existing function and functional needs of the upper limb 4. Perform a complete general examination to seek associated neurologic disorders and seek contraindications to surgery Motor Examination Examine resting posture a. spasticity usually leads to a resting posture in shoulder adduction and internal rotation, elbow flexion, forearm pronation, and wrist flexion b. Fingers i. - most often they are curled in a clenched fist, but they also can display a swan neck deformity or a claw type deformity ii. boutonniere type of deformity is less common. c. thumb i. can assume either an adducted posture or an adducted and flexed posture. ii. adducted thumb is often in a slight retroposition with the metacarpophalangeal (MP) and interphalangeal (IP) joints extended. iii. flexus-adductus thumb, often referred to as "thumb-in-palm," is embedded in the palm with full opposition and full flexion of MP and IP joints. often the clenched fist is curled around the thumb. Examine degree of spasticity Examine voluntary control weakness generally predominates in the distal muscles spared from spasticity (wrist and finger extensors, abductor, and extensor pollicis longus, and supinator muscles). proximal muscles (shoulder external rotators and abductors and elbow extensors) are involved to a lesser degree. difficult when the antagonist muscles are severely spastic. In some cases, these pseudo-paralyzed muscles may be present but made ineffective by the spastic antagonist or by elongation caused by a severe deformity. In such cases, an accurate evaluation of individual muscles can be performed only after spasticity of the antagonists has been suppressed Examine fibrous contracture Fibrous (muscular) contracture involves only spastic muscles. Unlike spasticity, fibrous contracture is permanent and cannot be overcome. Test with tenodesis effect Motor Blocks differentiate spasticity from fibrous contracture in flexor, adductor, and pronator muscle groups allow assessment of individual muscles after removing spasticity of the antagonists or co-contractions of adjacent muscles blocks include anesthetics (lidocaine – paralysis starts in 15 mins), diluted alcohol, phenol localization facilitated greatly with the use of nerve stimulators. In spastic muscle – test for contractures In antagonistic muscles – test for voluntary control botulinum toxin has partially supplanted motor blocks. It produces the same effects but with a much easier technique of injection, a lack of side effects, and a long lasting effect paralysis that starts 10–15 days after the injection Joint ROM Passive range of motion rarely is affected in isolated cerebral palsy in children. It is impaired more frequently in adults and, when severe, associated neurologic lesions coexist. The precise range of motion of many joints may be difficult to assess, not so much because of spasticity, but because of muscle fibrous contractures If fibrous contracture is present, motor blocks do not assist in assessment of the passive range of joint motion. Sensory examination basic sensory functions (light touch, pain, temperature) are essentially intact in cerebral palsy complex sensations (epicritic sensibility, proprioception, gnosis) are affected more severely. Gnosis is most affected. Placing an object in the child's hand and asking him or her to identify it tests stereognosis. Functional examination Grasping tests o Thumb-finger pinch often is limited to a lateral (key) pinch because of the lack of fine voluntary control and because of the adducted posture of the thumb o pick up and release test Bimanual activities EMGs often helpful. EMG studies can help in determining the most appropriate muscles for transfer. Static and dynamic studies are necessary, but they require cooperation on the child's part This may be difficult to achieve in children younger than 5 years of age. In spastic muscles, EMG studies give information on the voluntary control, the possibility of relaxation, and possible co-contractions with another active movement. This is of the utmost importance when planning a tendon transfer, as a spastic muscle may be considered for potential transfer only if it displays satisfactory relaxation at rest and no co-contraction with other voluntary movements Medical Therapies 1. Serial casting 2. Splinting 3. muscle stretching, muscle training, 4. reeducation and rehabilitation 5. Electrical stimulation stimulation of the antagonist muscles to oppose a spastic muscle group followed by vigorous ROM exercises, which led to a dramatic decrease in the muscle tone. 6. Botox 7. Pharmacological aim of systemic pharmacologic management is to reduce the muscle overactivity seen with spasticity and dystonia. four mainstream antispasticity enteral medications: baclofen, diazepam, dantrolene sodium, and tizanidine Botox choice of appropriate spasticity management often is dictated by the topographic distribution of cerebral palsy and associated comorbidities. o In spastic monoplegia and hemiplegia, focal or regional spasticity management with injections of botox to the appropriate target muscles is a widely used intervention. o In spastic diplegia, selective dorsal rhizotomy may be indicated for lower limb spasticity. o In spastic quadriplegia, continuous intrathecal administration of baclofen by implantable pump may be appropriate. Indications 1. correction of spastic posturing that interferes with function in the absence of fixed deformity 2. preoperative planning - to determine the child's response to weakening of the elbow flexors, wrist flexors, and finger flexors; and to assess strengths of the antagonists 3. for relieve of severe postoperative pain and spasm o may complicate muscle–tendon surgery in many children with spastic cerebral palsy. most common target muscles, in descending order of frequency, have been: 1. 2. 3. 4. 5. 6. Pronator teres (93%) and pronator quadratus (2.9%) Wrist flexors: flexor carpi ulnaris (86%) and flexor carpi radialis (82%) Elbow flexors: biceps (78%), brachialis (72%), and brachioradialis (6%) Adductor pollicis and flexor pollicis brevis (76%) Long finger flexors (31%) and flexor pollicis longus (43%) Pectoralis major (16%) and subscapularis (12%) When the aim is to decrease typical spastic posturing and to improve function, the usual combination is biceps and brachialis, pronator teres, flexor carpi ulnaris, flexor carpi radialis, and adductor pollicis. The long finger flexors should be avoided carefully to prevent weakening grip strength. When the aim is improved palmar hygiene, injection of the finger flexors (flexor digitorum superficialis and profundus) is always required. Surgical Management (Zancolli, Hand Clinics 2003) Goals of surgical procedures 1. Rebalance the muscles to improve function 2. Decrease hygiene problems 3. Improve cosmesis Options available for surgery Muscle release Origin release Insertion release Lengthening through musculotendinous junction, Z lengthening Fasciotomy Tendon transfers Capsuloplasty Tenodesis Arthrodesis Osteotomy Neurotomies Timing of surgical procedures Delay until clear evaluation of the function of the hand is possible 6-12 years SHOULDER Usually internally rotated and adducted Adduction and internal rotation treated with tenotomy-in-continuity of lattisimus, teres major, subscapularis and/or pectoralis major to improve hygiene A proximal humeral external rotation osteotomy may be required Recurrent dislocation in athetoid patients may need glenohumeral arthrodesis ELBOW Position is usually one of flexion Can be significant functional and aesthetic improvement Release achieved in 2 ways 1. Release or lengthen the muscles crossing 2. Denervation of these muscles Musculocutaneous neurectomy Spastic deformity of less than 30 degrees Must have full passive range Contraindicated in elbows whose function depends on biceps and function Leaves a sensory deficit in the lateral arm Division of the musculocutaneous nerve through an axillary approach, identified as it enters the biceps muscle Elbow flexor lengthening Increase extension by 40 degrees S incision over the antecubital fossa Z lengthening of the biceps tendon Zancolli favors tenotomy-in-continuity at the musculotendinous junction Occasionally the lacertus fibrosus may be sectioned. Aponeurotic release or myotomy of brachialis Post op splinting is important Flexor-pronator muscle slide Used in combination with the above Zancolli aponeurotic fascial release of the medial epicondyle will also improve the contracture FOREARM Spasticity of pronator teres and pronator quadratus Makes manipulation of objects between hands difficult Important not to release PQ in conjunction with PT as lose pronation Mild cases of forearm pronation spasticity that reduce passively preoperatively are improved by simple division of the distal tendon of the pronator teres In moderate cases, pronator teres may be step-lengthened and then passed through the interosseous membrane to be reinserted to its original attachment to act as a supinator. Flexor-pronator muscle slide o Helpful with pronator contraction, elbow and wrist flexor contractures o Incision 5 cm prox to the medial epicondyle and down to the midpoint of the ulnar border of the forearm o All released on mass Release of pronator insertion o Useful for milder cases. o Midpoint of the radius, 3 cm incision, protect the lateral cutaneous nerve of forearm and superficial radial nerve. Pronator teres rerouting o 2 methods - Take a periosteal extension or step lengthen the tendon o Pass through the interosseous membrane in a volar to dorsal direction and reattach to the original point of insertion WRIST AND DIGITAL EXTENSION Zancolli's classification of hand deformity Type I spastic intrinsic-plus hands, in which spasticity of the interossei and lumbrical muscles causes flexion of the MP joints and extension of the IP joints, sometimes associated with a swan neck deformity. In this type a wrist flexion deformity is rare. Type II spastic flexion-pronation hands with (hyper)flexion of the wrist and pronation of the forearm. Three groups are individualized depending on the degree of active finger extension. 1. full active extension of the fingers with wrist in neutral 2. nearly complete active extension of the fingers, but only with some degree of wrist flexion. This group is subdivided further based on the presence (subgroup A) or absence (subgroup B) of active wrist extension. 3. no active finger extension, even with maximum wrist flexion. Principal goals in surgical reconstruction of the spastic hand 1. improving grasp and release patterns between the wrist and digits, 2. hand appearance 3. psychologic status of the patient and the family. All groups usually are associated with thumb deformity—either in adduction or flexion-in-palm with elbow flexion contracture, and pronation contracture of the forearm of variable severity. The degree of sensory impairment varies between groups. Swan-neck deformity may be present in all groups but particularly in group II. This deformity reduces substantially after the flexion contracture of the wrist is surgically corrected Flexion contracture of the elbow is common in the spastic upper limb and frequently is influenced by emotional stimuli. Correction of marked flexion contracture of the elbow improves significantly the appearance and function of the hand. Pronation contracture of the forearm is corrected when severe and when the patient has difficulty carrying out activities of daily living because of lack of supination. A mildly pronated forearm is a useful position for hand function and its correction is not essential. paramount importance that all the existing deformities of the upper limb (elbow, forearm, and hand) should be corrected at the same surgical procedure. A contracted thumb thus must be simultaneously corrected with the rest of the hand to allow better active extension of the fingers and lateral pinch grip. Severe swan-neck deformities are usually corrected in a secondary surgical stage. Treatment Outline Zancolli Group 1 principal spasticity is localized at the FCU muscle general appearance of the upper limb is satisfactory and the emotional influence on spasticity is usually mild or almost absent. Pronation spasticity of the forearm may be present. Zancolli Group 2 Principal spasticity is localized at the wrist and finger flexors and thumb. Group 2A a. extensor muscles of the wrist and fingers are active and voluntarily controlled and that the main spasticity is localized in the flexor muscles of the wrist and fingers. b. Thus unnecessary to perform tendon transfers to extend the wrist Group 2B a. flaccid paralysis of the wrist extensor muscles or because the wrist flexor muscles are severely contracted b. tendon transfers to extend the wrist are necessary in cases of subgroup IIB when the wrist extensor are paralyzed. Zancolli Group 3 a. spasticity of the hand is severe and localized at the flexor–pronator mass b. extensor muscles of the wrist and fingers are totally paralyzed c. surgery is indicated exclusively to improve appearance, hygiene, and comfort. Zancolli advocates partial release of all spastic muscles Surgical Options Group 1 principal muscular co-contraction is FCU aim is to lengthen the FCU through a simple tenotomy, preserving muscle continuity. Group 2 depends on severity of spasticity and functional condition of the wrist extensor muscles Aponeurotic release of the medial epicondyle muscles o deep attachments of the medial epicondyle muscles are released at the proximal third of the forearm o transverse excision of the superficial fascia is made around the whole muscular mass. This is followed by a complete excision of the septa that separate the muscles. Each septum is released deeply. o The muscular bellies are left intact, particularly the FCU if this muscle is to be transferred to the extensor tendons of the wrist (subgroup IIB) Selective lengthening of FDP, FDS and FCR is done at the musculotendinous junction Zancolli doesn’t perform the muscle slide o difficult to calculate the necessary degree of muscular release with risk of overcorrection o devascularise the muscles FCU to ECRB is the classic transfer o Zancolli passes it through the interosseous membrane proximal to PQ through a generous window o Green passed around the ulnar border of the wrist(helps with supination) Other tendon transfers – FCU to EDC, BR to ERCB, ECU to ERCB A) Most frequent surgical program indicated in subgroup IIB of a spastic hemiplegic hand with a thumb-inpalm deformity, mild flexion contracture of the elbow, and pronation contracture of the forearm. 1, Biceps lengthening with its muscle fibers in continuity; 2 and 3, excision of part of the superficial fascia and all septae of the medial epicondyle muscles; 4, pronator teres release (pronator quadratus remains active); 5, transference of the FCU through an ample window in the interosseous membrane to the extensor carpi radialis brevis tendon; 6, lengthening in continuity of the flexor pollicis longus tendon. It is necessary to avoid too much relaxation; 7, release of the adductor pollicis at its proximal end. When the metacarpophalangeal joint of the thumb is lax and deforms in hyperextension, a sesamoid-metacarpal fusion is indicated during the same surgical stage. (B) 1, Transference of FCU to extensor carpi radialis brevis with partial excision of the extensor retinaculum; 2, transference of brachioradialis to abductor pollicis longus; 3, transference of palmaris longus to extensor pollicis longus through the interosseous membrane (too much tension should be avoided). Balance of tension needs to be maintained between the lengthened flexor pollicis longus and the transferred extensor pollicis longus. In cases with a single adduction contracture of the thumb, the flexor pollicis longus is left unreleased and the extensor pollicis longus is translocated radially. (C) Transference of the pronator teres through the interosseous membrane to produce forearm supination. The tendon is fixed to the radius. Group III permanent and severe flexion contracture of the wrist and fingers, particularly under emotional stimuli, and it is impossible to voluntarily open the hand. The extensor muscles of the wrist and fingers are paralyzed Zancolli advocates release of all the upper limb contractures at the elbow, forearm, wrist, fingers, and thumb. This is accomplished by multiple tenotomies—with muscular continuity—of all the contracted muscles. If the tenotomies are insufficient because of the great severity of spasticity or myostatic contracture of the finger flexor muscles: o section the flexor superficialis tendons distally, near the wrist, and the flexor digitorum profundus tendons proximally at the mid-forearm level. o fingers are then extended to a median flexed position and the proximal end of the flexor superficialis tendons is sutured to the distal end of the flexor digitorum profundus tendons with latero–lateral tenorrhaphies o pronator teres is released and the flexor tendons of the wrist are "Z" lengthened THUMB IN PALM DEFORMITY Surgery is indicated to hold the thumb out of palm during grasp and to permit lateral pinch. House thumb deformities classification 1. Type I is an isolated adduction of the first metacarpal. a. Spasticity of the AP and first dorsal interosseous causes adduction of the thumb metacarpal and first web space contracture. The MCP and IP joints are mobile. 2. Type II is adduction of the first metacarpal with flexion of the MP joint. a. In addition to the contractures mentioned previously, FPB spasticity leads to flexion deformity at the MCP joint. The IP joint is mobile. 3. Type III is adduction of the first metacarpal with MP joint hyperextension. a. The long and short thumb extensors act across a mobile MCP joint to compensate for the adduction deformity of the metacarpal, but the result is hyperextension of the MCP joint. The FPL is not spastic. 4. Type IV is adduction of the first metacarpal with flexion of MP and IP joints. a. may arise from FPL spasticity alone or in concert with spasticity of the thumb intrinsics. Problems 1. 2. 3. 4. Web space contracture Adpb, FPB, 1st dorsal interosseous FPL spasticity Strength of AbPL, EPB (flaccid) Hypermobitiy in extension Operative procedures are directed at each of the four causes of deformity Neurectomy (for example, of the deep motor branch of the ulnar nerve) is not recommended; it is preferable to weaken a muscle rather than defunction it totally because this decreases the possibility of overcorrection. Although not ideal for tendon transfers, spastic muscles can be transferred and can maintain adequate function Pulp pinch is rarely achievable if not present preoperatively Objective should be to obtain lateral or key pinch grip between the middle phalanx of the index finger and the thumb during grasping activities; and thumb abduction and extension during release. Procedures Release of the spastic flexor and adductor muscles (AdPB, FPB, FPL and 1st dorsal interosseous) commonly Adductor Pollicis is released by sectioning its origin from the third metacarpal through a palmar incision, parallel to the proximal palmar crease (Mateu's technique) Release of the insertion of AP into the ulnar sesamoid may unduly weaken thumb flexion at the MCP joint and lead to a swan neck deformity of the thumb. deep branch of the ulnar nerve and deep palmar arch is seen in the depths of the wound and protected thenar branch of the median nerve is identified and preserved If a deforming force, FPB may be released by way of the same thenar crease incision, at its origin from the trapezium and the transverse carpal ligament The first dorsal interosseous may be released at its insertion or origin. Release from the first metacarpal origin is the preferred procedure, as release from the second metacarpal may lead to intrinsic dysfunction of the index finger. Augmentation of the weak extensor and abductor muscles APL reinforcement i. BR to APL transfer ii. Dividing APL, distal end APL end-to-side to FCR and proximal APL to EPB iii. tendon plication iv. tenodesis of APL to brachioradialis tendon. EPL reinforcement i. EPL lengthening at its musculotendinous junction A small incision is made in the distal volar forearm and the tendon is released; the thumb IP joint is hyperextended and the tendon should be seen to slide 1 cm distally ii. PL to EPL transfer iii. EPL translocation toward the radial aspect of the wrist (Manske Procedure) Stabilization of the thumb MPJ indicated in both types of thumb deformities when the joint is hypermobile in hyperextension (more than 20°). joint fusion or capsulodesis through sesamoid-metacarpal synostosis. Radial sesamoid is fixed to the neck of the first metacarpal in 10° of flexion Metacarpophalangeal joint fusion alone, without proper release of flexion and adduction spasticity and without reinforcement of the extensor muscles, does not in itself eliminate the thumb-in-palm deformity. Sesamoid-metacarpal synostosis. (A) Radial side incision in the thumb over the MP joint. (B) The radial sesamoid without its cartilage (1) is fixed to the neck of the first metacarpal with a thick suture. Cortical bone of the metacarpal is partially excised (2). The volar plate is preserved. (C) The metacarpophalangeal joint is maintained in a few degrees of flexion through the tension given to the volar plate (capsulodesis). Release of the skin web space contracture 4 flap Z plasty INTRINSIC MUSCLE SPASTICITY Swan neck deformity Pathogenesis Due to permanent and pronounced flexed position of the wrist and the pull of the long extensor tendons during the patient's efforts to open the hand In conjunction with tight intrinsics, the over exaggerated pull of the extrinsic extensor leads to stretching of the volar plate and laxity of the transverse retinacular ligaments excessive lengthening or surgical release of the FDS may unmask this Clinical Occurs in Group II hand deformities proximal interphalangeal joint frequently locks in extension and the ability and force of pinch and grasp are impaired. Surgical correction indicated when the deformity does not improve after surgery at the wrist (usually improves it) Surgical options include lateral band rerouting, lateral band tenodesis, superficialis tenodesis, spiral oblique ligament reconstruction, intrinsic muscle slide, or a resection of the ulnar nerve motor branch in Guyon's canal Zancolli lateral band rerouting procedure for Swan Neck Deformity Correction of severe swan-neck deformity (technique). (A) Mid-lateral longitudinal incision on the radial side of the finger. (B) The lateral band (1) is released between the middle of the proximal phalanx and the middle of the middle phalanx. The proximal end of the released band remains in continuity with the lateral slip of the extensor tendon and the lateral band of the intrinsic tendons. The flexor tendon sheath is opened at the proximal PIP joint level (2). (C) The released lateral band (3) is translocated to the volar part of the finger and placed between the volar plate (5) and the flexor superficialis tendon (6). Two strong stitches (4) join the volar plate and the chiasma of Camper, distal to the PIP joint level. These sutures maintain the lateral band volarly to the joint. (D) After the procedure, the finger must extend passively up to almost neutral extension. The suture of the volar plate to the flexor superficialis represents a pulley for the transferred lateral band (7). During active finger extension, the transferred band produces a simultaneous stabilization of the PIP joint and an extension of the distal interphalangeal joint. The lateral band in its new position is shortened. The normal use of the finger produces with time the relocation of the opposite lateral band to its normal position. A variation sutures the lateral band under A3, which is acting as the pulley Outcome Measures House scale measure of function (0-8) Bruce Johnstone has also designed scaling for Dressings, Hygiene and Cosmesis (0-4) Surgery shown to give significant improvement in function, cosmesis, hygiene, and ease of dressing. Hemiplegic patients tended to show larger improvements in cosmesis compared with quadriplegics.