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CEREBRAL PALSY

Introduction

Cerebral palsy (CP) is a nonprogressive upper motor neuron disease that occurs due to injury of the immature brain during the pre-, peri-, or postnatal period of life. As the most common cause of neurological disability in children, the disease affects a patient’s movement and posturing, primarily through weakness, poor coordination, and hypertonic positioning, but it may also include sensory and/or cognitive damage. The upper limbs are often affected; significant wrist and hand involvement typically are noted at an early age.  Its variable manifestations may change with growth and brain reorganization. Depending on the deformity or deformities present, conservative treatments like medications, bracing, botulinum toxin injections, and/or physical/occupational therapy may be effective for some patients.  Surgical interventions may be needed for more severe cases and those in which conservative treatment fails.1-4

Pathophysiology

  • CP has a range of etiologies that include neuronal migration abnormalities, periventricular leukomalacia, intracranial hemorrhage, and infarction, but the common feature is disturbed cerebral control of motor function. A large component is believed to relate to corticospinal tract damage, as it is the major descending tract controlling skilled, fractionated, voluntary hand movements.1
    • New investigations in brain function, however, have challenged the traditional concept of the pyramidal (corticospinal) and extrapyramidal as 2 independent motor systems. Rather, it appears that these systems are extensively interconnected and that they cooperate in the control of movement, and that most children have a combination of movement disorders regardless of what diagnosis they receive.2
    • The inciting injury or abnormal development—which can include a genetic abnormality, toxic or infectious etiology, or vascular insufficiency—may occur at any time in prenatal or neonatal brain development, and the stage at which this insult occurs will influence how CP presents.3
      • Cerebral injury before the 20th week of gestation can result in a neuronal migration deficit, while injury between weeks 26-34 can result in periventricular leukomalacia, and in focal or multifocal cerebral injury between weeks 34-40.3
      • In addition to the common motor dysfunction categories used such as spasticity and athetosis, the inciting injury can also disrupt the more complicated voluntary and postural control systems. These control systems, based largely on learning, rapidly adjust the contraction and relaxation of the numerous muscles required to maintain balance and to position the shoulder, arm, forearm, and wrist appropriately for hand use.2
      • Brain injury due to vascular insufficiency depends on various factors at the time of injury, including the vascular distribution to the brain, the efficiency of cerebral blood flow and regulation of blood flow, and the biochemical response of brain tissue to decreased oxygenation.3
      • The upper extremity effects of CP usually result in hypertonic posturing in elbow flexion, forearm pronation, wrist flexion, thumb adduction, and finger flexion.2

Related Anatomy

  • Metacarpophalangeal (MP) joints
  • Interphalangeal (IP) joints
  • Flexion-adduction muscles
  • Extensor-abduction muscles
  • Adductor pollicis muscle
  • Thenar muscles
  • Flexor pollicis longus (FPL) muscle
  • Brachioradialis muscle
  • Biceps muscle
  • Brachialis muscle
  • Pronator teres
  • Various classification systems may be used to classify CP.  These classifications help the physician and surgeon to make appropriate treatment decisions.
  • The Manual Abilities Classification System2 is a commonly used tool that evaluates a child’s ability to handle objects in daily activities:
    • Level I: handles objects easily
    • Level II: handles objects with reduced quality and speed
    • Level III: handles objects with difficulty requiring modification
    • Level IV: handles objects only in adapted situations
    • Level V: does not handle objects
    • The most established classification system is that described by Zancolli,5 which places patients in one of the following three groups based on active finger and wrist extension:
      • Group I: patient can actively and completely extend the fingers with <30° of wrist flexion
      • Group II: patient can actively extend the fingers, but only with >30° of wrist flexion; in severe cases, the wrist needs to flex completely to permit complete or partial finger extension
      • Group III: patient cannot actively extend the fingers, even with maximal flexion of the wrist
      • There is also a classification system devised specifically for thumb-in-palm deformity,4 the most complex upper extremity problem in CP; this deformity most commonly occurs because of spasticity and contracture of the flexion-adduction muscles, coupled with poor voluntary control and weakness of the extensor-abduction muscles.
        • Type 1: deforming of the adductor pollicis (AP) muscle results in a deformity of adduction of the thumb across the palm
        • Type 2: deforming of AP and thenar muscles results in a deformity of adduction of the thumb and flexion of the thumb MP joint
        • Type 3: deforming of the AP muscle results in a deformity of chronic adduction of the thumb with secondary volar plate laxity leading to MP secondary hyperextension
        • Type 4: deforming of the AP, thenar, and FPL muscles results in a deformity of thumb adduction, thumb MP joint flexion, and thumb IP joint flexion

Incidence

  • Incidence is 1.5-4 cases per 1,000 live births, making it the most common motor disability in childhood.
    • This incidence has not changed in >40 years, despite significant advances in the medical care of neonates.6,7
    • The prevalence of CP is higher in children born pre- or post-term compared to those born at 40 weeks.8
    • Hereditary spastic hemiplegia/hemiparesis/paraplegia

Related Conditions

  • Epilepsy/seizure disorder
  • Dysphagia
  • Intercranial hemorrhage
  • Jaundice
  • Strabismus
  • Autism
  • Attention Deficit Hyperactivity Disorder (ADHD)

Differential Diagnosis

  • Hereditary spastic hemiplegia/hemiparesis/paraplegia
  • Spinal muscular atrophy
  • Muscular dystrophy/myopathy
  • Familial/primary dystonia
  • Rett syndrome
  • Inherited metabolic disorders
  • Metabolic neuropathy
  • Tethered spinal cord
ICD-10 Codes

CEREBRAL PALSY

Diagnostic Guide Name

CEREBRAL PALSY

ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

DIAGNOSIS SINGLE CODE ONLY LEFT RIGHT BILATERAL (If Available)
CEREBRAL PALSY - CONGENITAL SPASTIC QUADRIPLEGIA G80.0      
- SPASTIC DIPLEGIC G80.1      
- SPASTIC HEMIPLEGIC G80.2      
- OTHER CEREBRAL PALSY G80.8      

ICD-10 Reference

Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
  • Cerebral Palsy Flexor Pronator Deformity
    Cerebral Palsy Flexor Pronator Deformity
  • Cerebral Palsy - Spastic Flexor Pronator Deformity
    Cerebral Palsy - Spastic Flexor Pronator Deformity
  • Cerebral Palsy - Spastic Thumb in Palm Deformity
    Cerebral Palsy - Spastic Thumb in Palm Deformity
Symptoms
Muscle imbalance/weakness/contracture with deficits in precision grasp, pinch, and dexterity
Cognitive issues
Skin conditions
Astereognosis
Oral motor impairment with or without speech impairment
Respiratory difficulties
Athetosis
Ocular abnormalities
Sleep disorders
Typical History

A typical patient is a 2-year-old boy, who initially presented with early hypotonia with reduced muscle strength ~9 months of age. At this time, the boy’s parents noticed that he also began showing a preference for his left hand and displayed difficulties crawling, using asymmetric movements when he rarely attempted to do so. Around 18 months of age, the boy’s hypotonia gradually transitioned to a more hypertonic state, with severe spasticity preventing him from being able to perform many basic upper extremity motor functions.

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
  • Cerebral Palsy - Spastic Flexor Pronator Deformity X-Ray with persistent contracture. During growth some skeletal deformities can occur.
    Cerebral Palsy - Spastic Flexor Pronator Deformity X-Ray with persistent contracture. During growth some skeletal deformities can occur.
Treatment Options
Treatment Goals
  • Improve function
  • Improve hygiene
Conservative
  • Most commonly used treatment options for CP of the upper extremity:3
    • Medications
      • Muscle relaxants
      • Benzodiazepines
      • Anticholinergic agents
      • Dopamine prodrugs
      • Anticonvulsant agents
      • Alpha-2 adrenergic agonist agents
    • Constant-induced movement treatment, targeted towards children with hemiplegic CP
    • Botulinum toxin injections
      • May reduce spasticity in targeted muscles and improve ROM at a particular joint, thus facilitating splinting and therapy.
      • Considered a time-limited intervention, as effects wear off and repeated administration is required.1
      • Can be useful for assessing potential effectiveness from tendon transfers.
    • Physical and/or occupational therapy
      • For children with mild thumb-in-palm deformities, treatment should include strengthening exercises for weak muscles and stretching exercises for spastic muscles, as well as splinting for thumb abduction and wrist extension with a hand or forearm splint.4
    • Orthoses/splinting
      • Often used with physical and/or occupational therapy.
      • Consideration must be given to possible discomfort or cosmetic problems with usage.3
  • Action Observation Treatment
    • Has also been advocated as an alternative or adjunct to operative management to help CP patients improve their motor skills.2
Operative
  • Indications for surgery depend on the type and severity of the upper extremity deformity or deformities and whether or not conservative treatment was successful.
  • Thumb-in-palm deformity
    • The main surgical indications are functional impairment due to an inability to grasp or pinch objects secondary to thumb-in-palm positioning, and hygiene for severe deformity in the nonfunctional hand.
    • Surgical considerations are typically dictated by the classification of the deformity:
      • Type 1: adductor pollicis release with possible first dorsal interosseous release and/or Z-plasty of the skin contracture in the first webspace
      • Type 2: adductor pollicis release and/or flexor pollicis brevis (FPB) release
      • Type 3: adductor pollicis release and/or fusion or capsulodesis of the MP joint
      • Type 4: adductor pollicis release and/or FPB and FPL release or lengthening
      • Other surgical options include FPL release and transfer to the extensor pollicis brevis (EPB) and brachioradialis transfer to the abductor pollicis longus.
      • Muscle origin release with tendon transfer surgery should be protected in a thumb spica cast for postoperative weeks 0-4, followed by a thumb spica splint and active ROM exercises for grasp, release, and pinch activities for weeks 4-8, and a nighttime splint with active strengthening and normal functional use at week 8.4
  • Elbow-flexion deformity
    • Lacertus fibrosis release
    • Biceps and brachialis lengthening
    • Brachioradialis origin release
  • Forearm pronation deformity
    • Pronator teres release or trasnfer (rerouting)
    • Flexor carpi ulnaris (FCU) transfer
    • One way to determine if surgery is needed to treat the forearm deformity is by measuring active range of supination and assessing the child’s function by observing the actual positioning the forearm during hand use.
      • If forearm posturing exceeds 25° of pronation, surgery is often needed.2
  • Wrist-flexion deformity
    • FCU or flexor carpi radialis (FCR) lengthening
      • Indicated when there is good finger extension and little spasticity on wrist flexion
    • FCU to extensor carpi radialis brevis (ECRB) transfer or FCU to extensor digitorum communis (EDC) transfer
      • A functional procedure indicated in patients with voluntary control, IQ of 50-70 or higher, and better sensibility
    • Flexor release is indicated when there is weakening of the wrist flexors
    • Wrist arthrodesis is indicated as a hygienic procedure in low-functioning patients
  • Finger flexion deformity
    • If the finger and thumb flexors remain tight, the sublimis tendons can be divided at the wrist, the profundus tendons are lengthened, and the thumb is released or in severe cases, a sublimitis to profundus (STP) transfer can be helpful.
    • Fractional tendon lengthening is done through a midline palmar forearm incision. After skin incision the superficial facia is incised, exposing the superficial layer of flexor muscles. Next, the neurovascular bundles are identified and protected while the flexor carpi radialis longus muscle (FCR), the flexor digitorum superficialis (FDS) and the flexor carpi ulnaris (FCU) are exposed. Next fractional lengthening is begun by making two parallel oblique or transverse incisions through the muscle facia.  The first incision is at a point 1 1/2 to 2 cm proximal to the proximal end of the tendon. After the fascial incisions are made, these three musculotendinous units are stretch (lengthened) by forceful passive but controlled finger, thumb and wrist extension.  Next the radial artery, radial nerve and FCR are retracted while fractional lengthening of the flexor pollicis longus is done.  Following this, the median is protected while retracting the FDS to expose the FDP muscle for its fractal lengthening.  Once all fascial incisions are completed, the fingers, thumbs and wrist are extended again. The musculotendinous units should not be not overlengthened. Postoperatively, the wrist is placed in 30 to 50 ° dorsiflexion with the thumb and fingers in neutral extension. Immobilization is carried on for four weeks.  Some surgeons advocate earlier active motion for the digits. Nighttime splinting is appropriate for an additional two months. A key success is that fractional lengthening should not be done if the fingers and thumb cannot be extended while the wrist is in maximum palmar flexion during the pre-operative exam.The most common age for surgical intervention in CP patients with functional impairment(s) is 7-10 years, since most children have adequate cognition and motivation to be able to learn grasp and pinch function after the procedure at this age. Conversely, surgery to correct hygiene in the hand usually occurs in adult patients after contractures develop.
    • Surgery is contraindicated if the CNS injury is severe enough to preclude adequate cognition for the child to participate in functional activities, if the child does not have sufficient voluntary control of the muscles around the thumb, or when the primary manifestation of CP is dystonia or extrapyramidal disease.4
Treatment Photos and Diagrams
Operative Treatment Options
  • Fractional tendon lengthening:. In the drawing note the neurovascular structures, the transvers incisions in the superficial musculotendinous units and the intact distal fascia just proximal to the tendons. An alternative to fractional lengthening is Z-lengthening of the tendons.
    Fractional tendon lengthening:. In the drawing note the neurovascular structures, the transvers incisions in the superficial musculotendinous units and the intact distal fascia just proximal to the tendons. An alternative to fractional lengthening is Z-lengthening of the tendons.
  • The first step of a Superficialis to Profundus Transfer is to suture all four FDS tendons together and all four FDP tendons together before cutting the FDS tendons distally and the FDP tendons proximally. The FPL ( not shown) requires z-lengthening.
    The first step of a Superficialis to Profundus Transfer is to suture all four FDS tendons together and all four FDP tendons together before cutting the FDS tendons distally and the FDP tendons proximally. The FPL ( not shown) requires z-lengthening.
  • In the second step of the STP Transfer the FDS tendons are sutured to the FDP tendons. Swan neck deformities are avoided by suturing the tendons together while the wrist is in neutral and the MP’s and PIP’s are flexed 45 degrees.
    In the second step of the STP Transfer the FDS tendons are sutured to the FDP tendons. Swan neck deformities are avoided by suturing the tendons together while the wrist is in neutral and the MP’s and PIP’s are flexed 45 degrees.
CPT Codes for Treatment Options

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Common Procedure Name
Capsulodesis three or four digits
CPT Description
Capsulodesis, metacarpophalangeal joint, 3 or 4 digits
CPT Code Number
26518
Common Procedure Name
Flexor digitorum superficialis tenodesis PIP joint
CPT Description
Tenodesis; of proximal interphalangeal joint, each joint
CPT Code Number
26471
Common Procedure Name
Tendon transfer
CPT Description
Tendon transplant or transfer flexor/extensor forearm and/or wrist, single each tendon
CPT Code Number
25310
CPT Code References

The American Medical Association (AMA) and Hand Surgery Resource, LLC have entered into a royalty free agreement which allows Hand Surgery Resource to provide our users with 75 commonly used hand surgery related CPT Codes for educational promises. For procedures associated with this Diagnostic Guide the CPT Codes are provided above. Reference materials for these codes is provided below. If the CPT Codes for the for the procedures associated with this Diagnostic Guide are not listed, then Hand Surgery Resource recommends using the references below to identify the proper CPT Codes.

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Complications
  • Infection
  • Excessive correction
  • Contracture
  • Adhesion
  • Tendon repair rupture or attenuation
  • Supination deformity
  • Recurrence
Outcomes
  • Several randomized controlled trials (RCTs) have demonstrated the short-term efficacy of botulinum toxin A for spasticity in the upper extremity, as measured by maximum elbow and thumb extension and several other tests.
    • The clinical response after botulinum toxin A injections is variable, with some patients making transient gains that deteriorate by 3 months and other studies demonstrating a durable efficacy extending up to 26 weeks.2
    • One RCT evaluated the short-term effects of botulinum toxin A injections for treating upper extremity spasticity, with each patient receiving an average of 3 injections.
      • A higher percentage of children treated with injections showed improvements compared to children receiving placebo.  The researchers concluded that when surgery is not indicated, repeated botulinum toxin A injections are safe and efficacious in providing short-term functional improvement of children’s upper extremity spasticity.3
  • One systematic review and meta-analysis evaluated the efficacy of hand orthoses in conjunction with therapy for children with CP.
    • Results suggested that this intervention offered a small benefit for manual skill development, but the effect diminished 2-3 months after discontinuing orthosis use.3
  • Another study reported that the most effective rehabilitative programs have the following elements in common: therapy is goal directed, measureable goals are identifiable by children and caregivers, motor training is focused on activities rather than individual movements, and a substantial amount of time is spent in training.3
  • Constraint-induced movement treatment and bimanual training have been found to demonstrate the most efficacy and clinical practicality for improving functionality among conservative intervention in another trial.9
Key Educational Points
  • While traditional surgical interventions have addressed secondary peripheral manifestations of the primary CNS insult, future treatments of CP-related upper extremity pathology may evolve by gaining a greater understanding of the primary CNS insults and CNS plasticity from such insults.3
  • Two treatment outcomes that have not often been evaluated in the surgical treatment of CP in the upper extremity are self-esteem and aesthetics.
    • It has been found that upper extremity deviations correlate most with lower self-esteem, and that even in high-functioning patients with mild CP, self-esteem may be adversely affected by such deviations, especially elbow flexion deformity.
    • Cosmetic appearance after surgical correction may also have a greater influence on patient’s satisfaction than functional outcome, especially in older children. Surgeons must therefore take this into consideration when making treatment decisions.3
  • The currently used outcome instruments for CP need to be refined and standardized for easier use, and long-term, multicenter studies are required to assess their effectiveness.2
    • Assessment of the patient’s cognition and motivation to use the hand is also imperative, because neglect is difficult to overcome and functional improvement requires active participation of the patient.4
  • Muscle imbalance can be common after surgery, particularly in growing children, and possible recurrent deformity can occur if it is performed between ages 7-10. Prolonged splinting and occupational therapy may therefore be necessary to avoid recurrence.4
  • Assessment of the dexterity of the contralateral dominant hands of children with hemiplegic CP may reveal opportunities for therapeutic intervention that improve fine motor function, as dexterity may be impaired in some CP patients.10
  • Within the last 2 decades, the comparably simple model of CP stemming solely from perinatal anoxic insult has been refuted, because antenatal factors have been identified as underlying factors for the majority of cases including prematurity, multiple pregnancy, thrombophilia, and genetic polymorphism in genes regulating inflammatory response.2
  • Splints and braces can sometimes simulate increased muscle spasticity and secondary discomfort.
References

Cited

  1. Basu AP, Pearse J, Kelly S, et al. Early intervention to improve hand function in hemiplegic cerebral palsy. Front Neurol 2015;5:281. PMID: 25610423
  2. Bunata R, Icenogle K. Cerebral palsy of the elbow and forearm. J Hand Surg Am 2014;39(7):1425-32. PMID: 24969499
  3. Leafblad ND, Van Heest AE. Management of the spastic wrist and hand in cerebral palsy. J Hand Surg Am 2015;40(5):1035-40. PMID: 25841769
  4. Van Heest AE. Surgical technique for thumb-in-palm deformity in cerebral palsy. J Hand Surg Am 2011;36(9):1526-31. PMID: 21816546
  5. Zancolli EA, Zancolli ER, Jr. Surgical management of the hemiplegic spastic hand in cerebral palsy. Surg Clin North Am 1981;61:395–406. PMID: 7233330
  6. Winter S, Autry A, Boyle C, et al. Trends in the prevalence of cerebral palsy in a population-based study. Pediatrics 2002;110(6):1220-5. PMID: 12456922
  7. Majnemer A, Mazer B. New directions in the outcome evaluation of children with cerebral palsy. Semin Pediatr Neurol 2004;11(1):11-7. PMID: 15132249
  8. Moster D, Wilcox AJ, Vollset SE, et al. Cerebral palsy among term and postterm births. JAMA 2010;304(9):976-82. PMID: 20810375
  9. Taub E, Uswatte G. Importance for CP rehabilitation of transfer of motor improvement to everyday life. Pediatrics 2014;133(1):e215-7. PMID: 24366987
  10. Tomhave WA, Van Heest AE, Bagley A, James MA. Affected and contralateral hand strength and dexterity measures in children with hemiplegic cerebral palsy. J Hand Surg Am 2015;40(5):900-7. PMID: 25754789

New Articles

  1. Ferre CL, Brandão M, Surana B, et al. Caregiver-directed home-based intensive bimanual training in young children with unilateral spastic cerebral palsy: a randomized trial. Dev Med Child Neurol 2017;59(5):497-504. PMID: 27864822
  2. Moon JH, Jung JH, Hahm SC, Cho HY. The effects of task-oriented training on hand dexterity and strength in children with spastic hemiplegic cerebral palsy: a preliminary study. J Phys Ther Sci 2017;29(10):1800-1802. PMID: 29184291

Reviews

  1. Jackman M, Novak I, Lannin N. Effectiveness of hand splints in children with cerebral palsy: a systematic review with meta-analysis. Dev Med Child Neurol 2014;56(2):138-47. PMID: 23848480
  2. Leafblad ND, Van Heest AE. Management of the spastic wrist and hand in cerebral palsy. J Hand Surg Am 2015;40(5):1035-40. PMID: 25841769

Classics

  1. Holt KS. Hand function in young cerebral palsied children. Dev Med Child Neurol 1963;5:635-40. PMID: 14086612
  2. Mortens J. Surgery of the hand in cerebral palsy. Acta Orthop Scand 1965;36(4):441-52. PMID: 5859809