From Diagnosis to Recovery, The Comprehensive Cerebral Palsy Care at Upright Kids Ortho with Paediatric Orthopaedic Surg

1. FROM DIAGNOSIS TO RECOVERY: THE COMPREHENSIVE CEREBRAL PALSY CARE AT UPRIGHT KIDS ORTHO With Paediatric Orthopaedic Surgeon Bangalore |Dr. Jayanth S Sampath Cerebral palsy (CP) is a disorder that affects muscle tone, movement, and motor skills (the ability to move in a coordinated and purposeful way). Cerebral palsy can also lead to other health issues, including vision, hearing, and speech problems, and learning disabilities. CP is usually caused by damage to parts of the brain before or during a child’s birth, or during the first 2 years of a child’s life.

2. There is no cure for CP, but treatments such as physical therapy, splints or orthotics, medications such as Botulinum injections and Orthopaedic Surgery will help a child who is living with the condition. It is worthwhile pointing out that majority of common human ailments such as diabetes, high blood pressure, stroke, heart attack, kidney failure, etc are also incurable conditions. But, treatment of these conditions is essential to prevent complications. Cerebral palsy is no different in that it does not have a cure but treatment is essential. How does Cerebral Palsy work At Upright Kids Ortho? What are the symptoms and signs of cerebral palsy? Paediatric Orthopaedic Surgeon Bangalore | Dr. Jayanth S Sampath says the predominant symptoms and signs of cerebral palsy are related to difficulties with movements of the legs and arms. The extent and severity of the brain lesion is the leading factor in the magnitude of the motor deficit. For example, developmental motor delay, gait disorders, poor fine and gross motor coordination, swallowing disorders, or speech delay are all the result of the basic movement problem. The way they present varies from child to child. For that reason, it is difficult to describe a clinical picture that will satisfy every child with cerebral palsy. The clinical presentation, even though with many common features, is very much unique for a particular child. In addition, the comorbid conditions add more to the uniqueness of the presentation of the child with cerebral palsy. For example, some children may be blind, while others may have normal vision; some children may have a severe cognitive delay while others may have a normal or near normal cognitive level. What are the types of cerebral palsy? Based upon the form of motor impairment, cerebral palsy can be divided into types: •Spastic cerebral palsy •Dyskinetic cerebral palsy (according to the predominant symptoms dyskinetic CP may be either dystonic or choreo-athetoid) which includes ataxic cerebral palsy •Hypotonic cerebral palsy. These categories are not rigid, and the majority of patients most probably have a mixture of them. Cerebral palsy can also be classified into different types depending on the mobility level of the child. This system is called GMFCS (Gross Motor Functional Classification System) and is the most widely used classification system to describe children with cerebral palsy. The figure below easily

3. illustrates the different types from Level I to Level V. Kindly note that the mobility level changes slightly as the child goes through the teenage years and pubertal growth spurt. This is a well-described aspect of the natural history of cerebral palsy. Children who were walking with support until the age of 8 – 10 years lose some or all of their walking ability during early adolescence. This has been attributed to a reduction in the power: weight ratio of children. As muscles become bigger, they also become weaker relative to their weight. Causes of CP Paediatric Orthopaedic Surgeon Bangalore | Dr. Jayanth S Sampath says There is no identifiable cause in some children with cerebral palsy. Typical causes to look for include •Problems occurring before birth (e.g. exposure to radiation, infection, inadequate brain development), •Problems during birth such as asphyxia before birth, hypoxia (poor oxygen supply) of the brain, and birth trauma during labor and delivery •Premature and low birth weight babies •Complications occurring some time after birth or during childhood. •Genetic causes In the modern context, many premature babies (babies born earlier than 36 weeks and/ or less than 1.5 kg in weight at birth) can be saved thanks to advances in neonatal care but premature babies have an increased risk of CP. Many of the children that we see in our practice have spent some time in the NICU (neonatal intensive care unit). Fortunately, premature babies have spasticity as their predominant movement disorder, most will walk independently and their gait problems can be corrected by orthopaedic surgery. In the past, conditions such as kernicterus (uncontrolled jaundice in newborn babies causing damage to parts of the brain) were quite common. This group of disorders caused dyskinetic type of CP that was not readily amenable to surgery and prognosis for walking was poor. Due to early detection of jaundice in most neonates and prompt treatment, the incidence of Kernicterus is on the decline. Why do premature babies develop CP? Premature infants are vulnerable, in part because their organ systems and control mechanisms are not fully developed, increasing the risk of reduced oxygen supply to the brain that may manifest later as CP.

4. It is sometimes difficult to differentiate between cerebral palsy caused by damage to the brain that results from inadequate oxygenation and CP that arises from brain damage which occurs when the baby is still in the womb that then precipitates premature delivery. From the point of view of treatment, this distinction is academic and not of much relevance. MR scan of the brain may help in diagnosing this further but in terms of treatment, diagnostic tests such as MR scans do not help with orthopaedic treatment. After birth (postnatal), other causes include toxins, severe jaundice, lead poisoning, physical brain injury, shaken baby syndrome, incidents involving hypoxia to the brain (such as near drowning), and encephalitis or meningitis. The three most common causes of asphyxia in the young child are: choking on foreign objects such as toys and pieces of food, poisoning, and near drowning. In general, postnatal causes of CP (damage to the brain after birth) result in a more severe form of the condition with extreme muscle tone and postures, severe cognitive impairment and the prognosis for walking is less certain compared to CP caused by prematurity. Diagnosis of Cerebral Palsy: The initial suspicion of CP is typically raised by family members or noticed by the parents as a failure to achieve motor milestones. The child may not achieve head control by 6 months, sitting may be delayed or mild abnormalities in walking may be noticed. The child’s paediatrician is usually the first person to raise the possibility of CP. As mentioned earlier, cerebral palsy is NOT a condition resulting from a single cause. It is a clinical diagnosis made by doctors when a child has a number of different problems such as problems with walking, speech, hearing, sight, balance, co-ordination and fine motor control (using the hands for delicate tasks such as writing). No single factor can confirm or refute the diagnosis of CP. Rather; it is combination of various factors that taken together will help the doctor make the diagnosis of CP. For instance, a 2 year old child who has not yet started walking; was a premature baby born at 7 months with a birth weight of 1kg may turn out to have cerebral palsy. Though tests such as an MR scan of the brain are quite commonly done in the process of making the diagnosis of CP, there is no single test which will

5. confirm the diagnosis. As the causes of CP are highly varied, a series of investigations (tests) may need to be carried out. Who makes the diagnosis? According to Paediatric Orthopaedic Surgeon Bangalore | Dr. Jayanth S Sampath, Any qualified clinician with experience in the area of child development can make a diagnosis of cerebral palsy. Typical clinicians who may see children with a potential diagnosis of CP include paediatricians, physiotherapists, paediatric orthopaedic specialists, and paediatric neurologists, amongst others. surgeons, developmental Once the possibility of CP has been raised, it is prudent to seek the opinion of •A paediatric neurologist (to confirm the diagnosis) and •A paediatric orthopaedic surgeon (to discuss treatment options). It is advisable to seek expert advice early so that parents can be correctly counselled about the particular treatment options suitable for their child. Clinicians unfamiliar with the modern management of CP are a source of misinformation to parents. Words such as “untreatable” are sometimes used, causing uncertainty and distress to parents. CP is very much treatable with modern scientific methods and good outcomes are routinely obtained in the majority of children. Treatment for CP There are several factors which influence choice of treatment for a particular child. The principal among these are: 1.Age of the child The age of the child is the single most important factor which influences treatment planning. Cerebral palsy is a “static” encephalopathy, meaning that the primary problem in the brain does NOT change over time. It does not get better or worse. However, bones, muscles and joints of the legs can develop abnormalities due to growth. Any intervention such as physiotherapy, Botox injection or surgery should be employed after a thorough understanding of the natural history of cerebral palsy. Thanks to the pioneering work of Prof Rosenbaum and co- workers (from CanChild Centre for Childhood Disability Research, McMaster University, Canada), we have a detailed understanding of how children with CP develop in the first few years of life. Similar findings were seen in an independent Swedish study (Developmental Medicine & Child Neurology 2007, 49: 751–756).

6. The graph below shows that children with GMFCS Level I and II CP (children with independent walking ability) reach their peak motor ability around the age of 6 to 7 years. This NATURAL improvement occurs due to the maturation of brain function. It, therefore, stands to reason that any intervention such as orthopaedic surgery should be performed after the age of 7 years. The Ontario Motor Growth Study The Motor Growth Curves report patterns of gross motor development in children with cerebral palsy, classified according to each of the five levels of the Gross Motor Function Classification System (GMFCS) (Palisano et al., 1997). Children in this study were followed longitudinally for several years. The findings were published in a paper entitled ‘Prognosis for Gross Motor Development in Cerebral Palsy. Creation of Motor Growth Curves’, Rosenbaum et al., JAMA 2002; 288; 1357-63. In the first few years of life, most children with CP will benefit from supportive treatments such as physiotherapy and orthotics. Once the child reaches his or her maximum motor potential (typically between the ages of 8 and 10 years) and achieves a steady state in terms of growth, consideration can be given to whether orthopaedic surgery will be beneficial. The graph below illustrates common interventions undertaken at different ages in children with CP. It can be seen that peak age for non-surgical treatments is around 5 years and the peak age for surgery is around 10 years. 2.GMFCS Level: The walking ability of the child determines his/ her GMFCS level. Children with GMFCS levels I and II and some children with Level III CP may benefit from Gait Improvement Surgery (also called Single Event Multi-level Surgery or SEMLS). Children with GMFCS Levels IV and V CP do not benefit from surgery to release contractures or muscle tightness in the legs, as the children in this category are unlikely to gain the ability to walk even with surgery. However, children with GMFCS Levels IV and V CP have a high risk of gradual dislocation of the hip joint. This will require careful monitoring through regular hip x-rays. 3.Movement Disorder: Spasticity is the most common movement disorder seen in CP. Muscles affected by spasticity feel tight when stretched suddenly. Slow and gentle stretching of the muscle does not evoke spasticity.

7. Children with spasticity as their predominant movement disorder and who are able to walk either independently or with support have a better overall prognosis with regard to walking/ standing ability compared to children with other types of movement disorders. Children with dyskinetic movement disorders such as Dystonia, Athetosis, chorea, ataxia or hypotonia DO NOT show a predictable response to surgery. Scientific publications also advocate non-surgical treatment options in children with Dystonia. Dyskinetic movements are caused by damage to parts of the brain (called basal ganglia). Performing orthopaedic surgery by muscle releases and tendon lengthening cannot change the damaged area in the brain. It is highly likely that children with dyskinetic movement patterns will remain unchanged even after orthopaedic improvements noted after surgery are likely to be due to the physiotherapy provided post-operatively and these improvements will reverse once the intensive physiotherapy is stopped. surgery. Any minor Spasticity is caused by damage to nerve fibres leaving the higher centres of the brain (cerebral cortex). Therefore, spasticity does not change after muscle release surgery. What orthopaedic surgery corrects is the permanent muscle stiffness which occurs as a consequence of spasticity. In fact, some experts believe that spasticity is beneficial in that it maintains muscle strength to a certain extent. Orthopaedic surgery is therefore recommended only in those children who have permanent shortening of the muscle rather than pure spasticity. If regular physiotherapy and other treatments are provided at an early stage, muscle shortening does not typically develop in younger children (less than 6 years of age). Even in those younger children where contractures do develop, this can be managed without the need for surgery, through the use of Botox, serial casting, physiotherapy and use of splints. There are treatments such as Botulinum toxin injection; Selective Dorsal Rhizotomy and Intrathecal Baclofen pump which directly reduce spasticity. More information about these anti-spasticity treatments is provided in a later section. As the child grows, bones and muscles normally grow in almost perfect synchrony. In children with CP, muscle growth is unable to keep up with bone growth. Therefore, muscles become progressively shorter over time. This causes joint tightness and eventually contractures (permanent joint tightness).

8. Bones can develop abnormal twists (called torsional problems) in children with CP. When babies develop inside the mother’s womb, bones of the legs develop with an inward twist. This is normal and happens in all children. Typically developing children start walking by the age of 1 year. The forces applied to the legs during walking causes the inwardly twisted bones to straighten out. When walking is delayed (in conditions such as CP), the embryonic alignment of the leg bones persists. Persistent femoral anteversion and internal tibial torsion together contribute to intoeing gait (feet turned inwards) in children with CP. The main orthopaedic issues in children with CP are: 1.Muscle shortening 2.Joint contractures 3.Bony torsion Medications Medications that can lessen the tightness of muscles may be used to improve functional abilities, treat pain and manage complications related to spasticity. It’s important to talk about the risk of drug treatments with your doctor and discuss whether medical treatment is appropriate for your child’s needs. The selection of medications depends on whether the problem affects only certain muscles (isolated) or the whole body (generalized). •Focal spasticity •Generalized spasticity Therapies A variety of nondrug therapies can help a person with cerebral palsy to enhance functional abilities. These include the following: •Physical therapy: Muscle training and exercises may help your child’s strength, flexibility, balance, motor development and mobility. Braces or splints may be recommended for your child. Some of these supports are used to help with function, such as improved walking. Others may stretch stiff muscles to help prevent contractures. •Occupational therapy: Using alternative strategies and adaptive equipment, occupational therapists work to promote your child’s independent participation in daily activities and routines in the home, the school and the community. •Speech therapy:Speech therapists help improve your child’s ability to speak clearly or to communicate using sign language. They can

9. also teach your child to use special communication devices — such as a board covered with pictures of everyday items and activities. Sentences can be constructed by pointing to the pictures. Speech therapists may also address difficulties with muscles used in eating and swallowing. Surgical or other procedures Surgery may be needed to lessen muscle tightness or correct bone abnormalities caused by spasticity. These treatments include: •Orthopaedic surgery: Children with severe contractures or deformities may need surgery on bones or joints to place their arms and legs in their correct positions. Surgical procedures can also lengthen muscles and tendons that are proportionally too short because of severe contractures. These corrections can lessen pain, improve mobility, and make it easier to use a walker, braces or crutches. •Severing nerves: In some severe cases, when other treatments haven’t helped, surgeons may cut the nerves serving the spastic muscles. This relaxes the muscle and reduces pain, but can also cause numbness. For more information visit- https://www.uprightkidsortho.com/blog/managing-cerebral- palsy-in-children-a-doctor-explains/ Our website - https://www.uprightkidsortho.com/ Our other treatment- Perthes disease- https://www.uprightkidsortho.com/perthes- disease/ Clubfoot – https://www.uprightkidsortho.com/clubfoot-or- congenital-talipes-equinovarus/ Leg length discrepancy- https://www.uprightkidsortho.com/leg- length-discrepancy/

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