Surgery

Upper extremity surgery can improve hand function in a few selected cases. The ideal surgical candidate must be a motivated, intelligent child who has good sensation in the handand uses the extremity. Those children with satisfactory handeye coordination can benefit from surgery even when hand sensation is poor.

The surgeon must be careful in patient selection because some children develop adaptive mechanisms to compensate for lost hand movements as they grow. Functional loss occurs after surgery in such patients because surgery prevents the adaptive movements they developed over the years. Consider surgery between 6- 12 years of age when the child will cooperate with postoperative rehabilitation. Set goals that fit with the expectations of the child and the parents.

Before surgery should be considered the followings

• Voluntary hand use
• Sensation
• Intelligence
• Athetosis

The shoulder Adduction – internal rotation contracture is the most common problem. Provide a program of stretching exercises. Consider surgical lengthening of the muscles if the deformity is severe.

The elbow Flexion contractures of more than 45 are functionally disabling. Try botulinum toxin injection to elbow flexors and stretching exercises in dynamic deformities and even for cosmetic reasons. Consider surgery for elbow only if the hand is functional, if there is skin breakdown at the elbow or if hygiene in the antecubital fossa is poor. Deformities greater than 60o require surgical lengthening of the biceps tendon, be aware of the fact that this procedure worsens the forearm pronation deformity. Maximum range of motion is gained 3 months postoperatively.

Forearm The main problem is a pronation contracture because of spasticity in the pronator teres and pronator quadratus muscles [B]. Activities that require supination like grasping a walker or a cane, balancing objects in the palm, washing the face are impossible. Severe pronation causes radial head dislocation but it is generally painless and does not cause functional problems. Consider pronator teres transfer to the supinator if the child can voluntarily pronate the forearm. Pronator release gives satisfactory results if the child has active supination. Longstanding pronation contracture of the forearm leads to relative shortening of the biceps aponeurosis. Release this structure to allow the biceps to be a more effective supinator.

Wrist The wrist usually is held in a position of flexion and ulnar deviation because of flexor carpi radialis and flexor carpi ulnaris spasticity [D, E]. The digital flexors also contribute to wrist flexion. Finger flexors are inefficient and the grasp is weak when the wrist is flexed [F]. Grasping is essential for function. Correct flexion contractures of wrist and fingers and adduction of thumb if they interfere with grasp. Macerations and mycotic infections are common in severe flexion contractures of the hand. Surgery becomes necessary for hygienic purposes. Options for surgery [G] include wrist flexor lengthening, flexor origin slide, tendon transfer to improve wrist extension, proximal row carpectomy, and wrist fusion with or without carpal shortening [H]. Avoid wrist arthrodesis because the patient loses the tenodesis effect of wrist extension that results in finger flexion and facilitates grasp and release. Consider wrist arthrodesis only to relieve the pain and improve the cosmesis of the hand when there is no or limited hand function. Wrist and digital flexor muscles can be selectively lengtheneddistally. Do not release or transfer both flexor carpi ulnaris and radialis as this eliminates active wrist flexion.Consider tendon transfers to augment wrist extension when it is weak or absent. Transfer the flexor carpi ulnaris to extensor digitorum communis when both finger and wrist extension is weak. This transfer improves wrist extension and does not impair finger extension and release.

Fingers Finger flexion deformity is a result of spasticity and contracture in the flexor digitorum superficialis and profundus muscles [1]. It becomes more obvious when the wrist and metacarpophalangeal joints are held in neutral position. Consider surgical intervention when flexion deformity is severe [2]. The flexor-pronator origin release effectively lengthens the flexor digitorum superficialis, pronator teres and flexor carpi radialis. Correct finger flexion deformity by direct Z-lengthening of involved tendons. If there is spasticity of intrinsic hand muscles, releasing the finger flexors will increase the deformity. Excessive lengthening weakens flexor power, impairs grasp, and can produce swan neck deformities. In this case, transfer the flexor digitorum superficialis tendon to augment wrist, finger or thumb extension instead of lengthening. Swan-neck deformity [3] is hyperextension deformity of the proximal interphalangeal joints. It is because of over-activity of the intrinsic muscles, and increases with the pull of the extensor digitorum communis when the wrist is in flexion. Consider surgical intervention if there is severe hyperextension, or when the proximal interphalangeal joints lock in extension. The thumb The thumb-in-palm deformity [4] is characterized by metacarpal flexion and adduction, metacarpophalangeal joint flexion or hyperextension and usually interphalangeal joint flexion. The causes are spasticity and contracture of the adductor pollicis, first dorsal interosseous, flexor pollicis brevis, and flexor pollicis longus . The extensor pollicis longus, extensor pollicis brevis, and/or abductor pollicis longus are often weak or ineffective. The thumb-in-palm deformity impairs the ability of the hand to accept, grasp, and release objects. The goals of surgery  are to release the spastic muscles to position the thumb, to create a balance in the muscles around the thumb, and to provide articular stability for grasp and pinch.

Hemiplegia
Hemiplegic children have involvement of the arm and leg on  one side of the body. The upper extremity is more severely involved than the lower. Spastic hemiplegia constitutes 20% of cases with spastic CP. These children generally have very few associated problems. Communication is unimpaired most of the time. They may have seizures, learning and behavioural problems. Functional prognosis is good compared to other types because one side of the body is normal. All hemiplegic children learn to walk by the age of three. They become independent in the activities of daily living. Seizures, mild mental retardation,  learning difficulties and behavioural disturbances may complicate the management and integration into the society.

Common musculoskeletal problems

The shoulder is adducted and internally rotated, the elbow is flexed and pronated, the wrist and fingers are flexed, the thumb is in the palm. The hip is flexed and internally rotated, the knee is flexed or extended, the ankle is in plantar flexion. The foot is generally in varus, although valgus deformity may also be  seen. The hemiplegic side is short and atrophic depending on the severity of involvement.

Musculoskeletal problems in hemiplegia

Upper extremity

• Shoulder -  Internal rotation, Adduction
• Elbow -  Pronation, Flexion
• Wrist – Flexion
• Hand – Flexion , Thumb-in-palm

Lower extremity

• Hip – Flexion, Internal rotation
• Knee – Flexion, Extension
• Ankle – Plantar flexion
• Foot – Varus

Treatment consists of physiotherapy, occupational therapy, bracing, botulinum toxin injections and orthopaedic surgery. Some children may need speech therapy and antiepileptic medication.

Treatment in hemiplegia

• Physiotherapy – Prevent contractures, Strengthen weak muscles, Establish a better walking pattern
• Occupational therapy – Functional use of upper extremity, Activities of daily living
• Bracing
  Lower extremity  –>  Solid or hinged AFOs
  Upper extremity  –>   Functional or resting hand splints
• Botulinum toxin A
  Lower extremity – Rectus femoris and gastroc spasticity
  Upper extremity – Pronator flexor spasticity
• Orthopaedic surgery – Correction of Pes equinovarus, Stiff knee, Femoral anteversion

Physiotherapy & occupational therapy

Motor problems of the hemiplegic child are usually mild. Physiotherapy is prescribed to prevent contractures of the involved side, to strengthen the weak muscles, to enable functional use of the upper extremity and to establish a better walking pattern. The basic program for the lower extremity consists of hip, knee, ankle range of motion exercises; rectus femoris, hamstring and gastrocnemius muscle stretching and agonist muscle strengthening. Do not neglect the back extensors and pelvic girdle muscles. Prescribe occupational therapy to gain hand function. Activities that involve both hands may improve the use of the involved side. Inhibiting the sound extremity and forcing the involved one to work is a novel method called constraint induced therapy. This method has certain beneficial effects but it is frustrating for most children. Children with hemiplegia do not need physiotherapy for ambulation. Prognosis for independent walking is very good. Physiotherapy is beneficial to prevent contractures of the ankle. In most of the cases the physiotherapy and occupational therapy can be accomplished on an outpatient basis or home program.

Botulinum toxin A

Botulinum toxin injections are used for upper and lower extremity spasticity in the young child. The toxin reduces gastrocnemius-soleus and rectus femoris spasticity in the lower extremity. The child uses his braces more efficiently and may develop a better walking pattern. Early relief of spasticity may prevent shortening of the gastrocnemius muscle and delay or eliminate the need for surgical intervention. In the upper extremity, inject botulinum toxin to relax wrist, finger and thumb flexors so that the child may gain forearm supination and wrist stabilization. Relaxing the spastic muscles with botulinum toxin injections may aid the treatment team to visualize how the child will function when his spastic muscles are surgically lengthened. However, the toxin cannot show its real effect in some older children with already shortened muscles. Botulinum toxin may be combined with surgery in the older child. Inject muscles which have mild spasticity and no shortening with Botulinum toxin and surgically lengthen the severely spastic short muscles. This combination approach adopted in the recent years enables a swifter return of function, less complications and less muscle weakness because of less extensive orthopaedic surgery.

Bracing

Upper extremity bracing
There are two indications for hand splints in hemiplegia. One is to prevent deformity and the other is to improve function. Night splints help stretch muscles and maintain range of motion.Tone usually decreases at night, therefore the use of resting splints at night to prevent deformity is questionable. The child’s compliance with night splints is generally poor. Use day splints to increase function by either supporting the wrist in 10o extension, the thumb in opposition or both. Keep in mindthat day splints prevent sensory input in the already compromised hand.

Lower extremity bracing
AFOs stabilize the ankle and foot and keep it in the plantigrade position for weight bearing. They are set in 5o dorsiflexion to avoid genu recurvatum or at neutral to prevent knee flexion. If the foot remains fixed the child has to extend the knee. Correct all fixed contractures before giving braces. Use hinged AFOs for mono and hemiplegic patients especially when they have active dorsiflexion.

Type of brace   <–>  Indication

• Solid AFO                        <–> Equinus and equinovarus
• AFO in 5 dorsiflexion <–> Equinus & genu recurvatum
• Hinged AFOs                  <–> Equinus and equinovarus if the child:
  a.     can tolerate the hinge
  b.     has varus-valgus control
  c.     has 5o passive dorsiflexion
• Supramallleolar orthoses (SMO)  <–> Mild varus – valgus deformity
  without equinus

Classification

CP encompasses a spectrum of motor disorders of varying tone, anatomical distribution and severity. Clinicians classify patients to describe the specific problem, to predict prognosis and to guide treatment. Classification is based on the change in muscle tone, anatomical region of involvement and severity of the problem. Classification provides a clearer understanding of the specific patient and directs management. Spastic CP Spasticity is defined as an increase in the physiological resistance of muscle to passive motion. It is part of the upper motor neuron syndrome characterized by hyperreflexia, clonus, extensor plantar responses and primitive reflexes. Spastic CP is the most common form of CP. Approximately 70% to 80% of children with CP are spastic. Spastic CP is anatomically distributed into three types.

Clinical classification

Tonus              Lesion site
Spastic                        Cortex
Dyskinetic                    Basal ganglia – extrapyramidal system
Hypotonic / Ataxic         Cerebellum
Mixed                          Diffuse

Anatomical classification
Location                     Description

Hemiplegia           Upper and lower extremity on one side of body
Diplegia                 Four extremities, legs more affected than the arms
Quadriplegia        Four extremities plus the trunk, neck and face
Triplegia                Both lower extremities and one upper extremity
Monoplegia          One extremity (rare)

Below is shortly described each type of CP , for more information on exact type please visit section ( types of cp )

Spastic CP

Spasticity is defined as an increase in the physiological resistance of muscle to passive motion. It is part of the upper motor neuron syndrome characterized by hyperreflexia, clonus, extensor plantar responses and primitive reflexes. Spastic CP is the most common form of CP. Approximately 70% to 80% of children with CP are spastic. Spastic CP is anatomically distributed into three types.

Hemiplegia

With hemiplegia, one side of the body is involved with the upper extremity generally more affected than the lower. Seizure disorders, visual field deficits, astereognosis, and proprioceptive loss are likely. Twenty percent of children with spastic CP have hemiplegia. A focal traumatic, vascular, or

infectious lesion is the cause in many cases. A unilateral brain infarct with posthemorrhagic porencephaly can be seen on magnetic resonance imaging (MRI).

Diplegia

With diplegia, the lower extremities are severely involved and the arms are mildly involved . Intelligence usually is normal, and epilepsy is less common. Fifty per cent of children with spastic CP have diplegia. A history of prematurity is usual. Diplegia is becoming more common as more low- birth-weight babies survive. MRI reveals mild periventricular leukomalacia (PVL).

Quadriplegia (Total body involvement – tetraplegia)

With quadriplegia, all four limbs, the trunk and muscles that control the mouth, tongue, and pharynx are involved . When one upper extremity is less involved, the term triplegia is used. Thirty percent of children with spastic CP have quadriplegia. More serious involvement of lower extremities is common in premature babies. Some have perinatal hypoxic ischemic encephalopathy. MRI reveals PVL.

Dyskinetic CP

Abnormal movements that occur when the patient initiates movement are termed dyskinesias. Dysarthria, dysphagia, and drooling accompany the movement problem. Mental status is generally normal, however severe dysarthria makes communication difficult and leads the observer to think that the child has intellectual impairment. Sensorineural hearing dysfunction also impairs communication. Dyskinetic CP accounts for approximately 10% to 15 % of all cases of CP. Hyperbilirubinemia or severe anoxia causes basal ganglia dysfunction and results in dyskinetic CP.

Ataxic CP

Ataxia is loss of balance, coordination, and fine motor control. Ataxic children cannot coordinate their movements. They are hypotonic during the first 2 years of life. Muscle tone becomes normal and ataxia becomes apparent toward the age of 2 to 3 years. Children who can walk have a wide-based gait and a mild intention tremor (dysmetria). Dexterity and fine motor control is poor. Ataxia is associated with cerebellar lesions.

Mixed CP

Children with a mixed type of CP commonly have mild spasticity, dystonia, and/or athetoid movements. Ataxia may be a component of the motor dysfunction in patients in this group. Ataxia and spasticity often occur together. Spastic ataxic diplegia is a common mixed type that often is associated with hydrocephalus.

Exceptions

Some children with CP cannot be fitted into these CP groups because they present with many different impairments. Dystonia may be seen in the spastic child, and anatomical classification may not be fully explanatory because clinical findings may overlap. An example is the hypotonic total-body-involved baby who stays hypotonic throughout childhood. Define the pathological abnormalities observed in these children according to the anatomical, and clinical involvement, as described above.