Pediatric Disabilities / Children Orthopedic Problem
There are number of pediatric disabilities and orthopedic problem which affect a person since childhood. Some of them are congenital and others are acquired. We can group them in following major parts—
1. Neuro-developmental disabilities (NDD): —-
- Congenital developmental disabilities (autism, intellectual disabilities, attention deficit disorder & learning disabilities,)
- Metabolic and genetic condition eg. Down syndrome ( Trisomy of 21)
- Vision loss & blindness
- Hearing loss
- Speech & language disorder
- Nerve & muscle disorder (eg. Muscular dystrophy, congenital neuropathies, spina bifida, polio )
- Neurological disorder due to brain insult- cerebral palsy
2. Congenital Musculo-skeletal disorders
- Congenital dislocation of hip (CDH)
- Congenital talipus equanovarus (CTEV) & vertical talus
- Congenital limb deficiency like PFFD, Tibial hemimelia , Fibular hemimelia, Radial club hand
3. Acquired disorder- infection, trauma, Ricket & scurvy etc
Autism spectrum disorder: it is a developmental condition which affects individuals in two main areas. First thing, that they have impaired communication and social interaction and second, that they have restricted, repetitive patterns of behaviour, interest or activities. Cause of autism is not known but one thing is clear that it is not caused by parenting, or side effect of vaccination. This disorder arises most probably because of multiple factor including some genes, environmental impacts in early pregnancy. Two individuals with autism can be entirely different. Although there is no cure but with early intervention they can be helped to live lives with their full potential.
Pediatric Intellectual disabilities: Intellectual disability or learning disability was formerly known as mental retardation. It is generalized neuro-developmental disorder with significantly impaired intellectual and adaptive functioning defined as IQ level below 70. It affects about 2-3% of the general population and two third of them have only mild intellectual pediatric disability. Children with intellectual disability learn more slowly than others. The causes can be genetic. Down’s syndrome being the most common, problems during pregnancy like alcohol intake, rubella or other infection, birth hypoxia, exposure of infant to various diseases or toxins , iodine deficiency, malnutrition etc. Although currently there is no cure for established disability but most of the individuals can live socially acceptable life with appropriate teaching, training and support since early age.
Attention deficit hyperactivity disorder (ADHD)—– Attention deficit hyperactivity disorder is a neurodevelopmental psychiatric disorder in which there is significant problem in executing the work given to them that cause attention deficits along with hyperactivity which is not appropriate for a person’s age. This problem in school going children often results in poor school performance. Treatment in this problem involves some combination of counselling, life style changes & behavioural therapy.
Learning Disability- Learning disability is an umbrella term for variety of learning problem. Most common type of learning disability is problem in maths, reading, writing, reasoning, listening, time planning and speaking. Learning disability can impact relationship with family & friends. Before leveling learning disability, we should always rule out problem in hearing, vision & mental retardation. These children can come out from this problem with right approach of teaching, training and intervention.
Down’s syndrome (Trisomy of 21): Down’s syndrome or trisomy of 21 is a genetic disorder. It is associated with physical growth delays, characteristic facial features, and mild to moderate intellectual disability. The parents are typically normal. The parent’s diet or activity before and during pregnancy does not affect its occurrence. Although women of any age can have baby with Down’s syndrome but advance age mothers are at higher risk of it. Down’s syndrome can be identified during pregnancy with prenatal screening and pregnancy with final diagnosis of Down’s syndrome is terminated. People with Down’s syndrome may have small chin, slanted eyes, flat nasal bridge, a single palmer crease or protruding tongue. Usually face is flat and wide with short neck; there is extra space between first and second toe and short stature. Although the individuals with mild to moderate intellectual impairment, their understanding of language is better than ability to speak. Usually they are good at social interactions. Some of them also can have associated disorders like eyes, ear, epilepsy etc. Early diagnosis, screening for common problems and good family environment, education and proper care can give a good quality of life to the child and family.
Vision impairment / blindness: vision impairment may cause difficulty in recognizing the object clearly in activity of daily life. Impairment can be incomplete loss or complete loss of vision. Complete loss of vision in children occurs most commonly because of brain involvement, corneal opacity, cataract & retinopathy etc. Partial loss can occur because of strabismus, refractive error. Early recognition & early intervention helps the children in getting good outcome.
Hearing Loss: hearing loss can affect a Child ability to develop communication, language & social skill. Congenital hearing loss can occur because of genetic causes or some acquired causes like infection during pregnancy, trauma during birth. Acquired causes can occur because of recurrent ear infection & noise pollution. Early recognition & treatment of causes and hearing aid can helps these children in developing their skill. When a person has trouble understanding others (receptive language), or sharing thoughts, ideas, and feelings completely (expressive language), then he or she has a language disorder. A stroke can result in aphasia, or a language disorder.
Speech language disorder: Difficulty in pronouncing sounds, articulation disorder and stuttering are the example of speech problem. Difficulty in expressing thought, idea, need, information by language (expressive language) or trouble in understanding what others say (receptive language) are a few example of language disorder. Speech language pathologist helps these children by giving individualize treatment plan of rehabilitation.
Muscular Dystrophy : muscular dystrophy is a group of genetic disorder where abnormal gene (mutation) interfere with the production of protein required for muscle formation led to progressive weakening & loss of muscle mass. There is variety of muscular dystrophy in which some variety run in family and others caused by sporadic mutation of genes. It primarily affected boys. There is no treatment but still few medicines, exercise and brace helps in prolonging life of child. Stem cell treatment is in research phase but still it is not proven till date.
Hereditary Neuropathy: It is group of inherited disorder which affect peripheral nervous system (sensory, motor & autonomous system in different combination). Charcot Marie tooth disease is commonest one. Symptom can appear at birth or in late age to any age group. There is no standard treatment. Orthopaedic surgery is required in cases with foot or other skeletal deformity. Therapy and proper bracing helps in maintain correct posture.
Spin Bifida: This is a congenital development disorder caused by incomplete closure of Embryonic neural tube that leads to the bony defect in vertebral canal through which spinal Card, nerve root and meanings came out of canal or protrude. They can present in variety of manner from spina bifida occulta to to meningo-myelocoel. Spina bifida occulta does not require any intervention but careful observation is requiring to see the impact of tethered card syndrome. In cases where there is defect in spinal card associated with protuberance/ extrusion of spinal segment out of vertebral body then they need surgical closure at early age followed by treatment of residual orthopedic problem in extremity by brace/ therapy & surgery.
Polio- Polio was the commonest cause of severe physical disability in children few years back but now it is the history. But still few of our adult who was affected with polio years back , now they have complex problem from shortening, deformity, muscle wasting and weakness. We cannot do much for the muscle wasting and weakness but we can give them well align extremity by deformity correction & limb lengthening.
Congenital Acquired musculoskeletal disorder:
Club Foot (CTEV) – in this problem foot of new born baby are deviated medially. It can present in one or both side. Treatment of CTEV should be started at earliest after birth to get best outcome. Club foot is being managed with corrective plaster application by ponseti technique (5-6 time) at interval of 7-10 days & percutaneous tenotomy of tendoachillis with 1 month plaster followed by CTEV brace with abduction bar till the age of 1.5 year. If properly managed then you can have normal looking fully mobile & supple foot in future. Rarely recurrence & residual deformity need surgical intervention along with plaster application.
Sever deformity of Foot- sever foot deformity can occur because of trauma, CTEV and congenital nerve disorder. Sever foot deformity is being managed by various foot osteotomy & soft tissue procedure followed by brace application.
Fibular Hemimelia- in this condition fibula is absent partially or completely along with foot deformity. Management plan in Fibular hemimelia is excision of fibular analogue, foot reconstruction & lengthening of tibia with Ilizarov (Paley super ankle surgery).
Tibial hemimelia- in this condition tibia is absent partially distally or proximally to complete absence. Management plan in tibial hemimelia is based on paley type of tibial hemimelia. In complete absence of tibia we can reconstruct the knee joint by the concept of Weber arthroplasty or we can amputate the limb from knee joint for better prosthesis. Partial absence tibia distally managed initially by plaster application & exercise to stretch out soft tissue followed by brace till the age of 1.5 yr then by distal transport of fibula by ilizarov followed by tibialization of fibula and tibiotalar arthrodesis
Proximal focal femoral deficiency (PFFD) – In this condition underdevelopment to complete absence of thigh segment can occur. They can have present in variety of manner from minor shortening of femur to complete absence of femur bone with associated anomaly of leg & knee. Management plan in PFFD is also based on Paley classification. Standard treatment plan in proximal focal femoral deficiency is reconstruction of missing segment by Paley super hip & knee surgery. In first stage Hip joint is being reconstructed with proximal femoral & pelvic osteotomy (super hip surgery) & knee ligament reconstruction followed by limb lengthening by ilizarov. This children require one or two lengthening with growth modulation.
Angular Deformity – Angular limb deformity near to growth plate can be managed by growth modulation (8 plate) in early age till the age of 13 year but in late age they need corrective osteotomy.
Genu Vara- Sever deformity of proximal tibia and shortening managed by osteotomy & limb lengthening by ilizarov.
Genu Valgus – a) in younger age during growth period (<13 year age) this deformity managed with application of 8 plate on medial side of distal femoral physis.
b) after completion of growth (>15 year age) this deformity is being managed by corrective osteotomy
Congenital hip dislocation- congenital hip dislocation is also known as development dysplasia of hip (DDH). It means incomplete or complete displacement of femoral head from hip joint to dysplastic changes in acetabular cavity. Around 1% of hip is subluxatable at birth but most of them became stable with passage of time. Only .2% of hip have some dysplastic changes. Hip instability can be recognised at birth with ortolany / Barlow manoeuvre. Early intervention gives good outcome. Management plan is based on reducibility and age of presentation.
a) DDH in early infancy is being managed by pavlik harness.
b) between 3 to 9 month of age they need close reduction and hip spica application
c) After 1 year age these children need Open reduction +_ acetabular osteotomy.
d ) After 18 month of age they need open reduction and femoral shortening +- Acetabular osteotomy
Perthes ds (leg calve Perthes diseases) – in this problem blood circulation of femoral head in children is disturbed temporarily. So without blood supply bony tissue start dying and with passage of time rounded surface became flatten if not protected. This condition can last for years. Treatment plan is based on stage of disease process & roundness of femoral head. Aim of treatment is protection of femoral head from collapse and containment of femoral head in acetabular cavity so with passage of time they can remodel according to shape of acetabular roof. containment can done by abduction hip spica & proximal femoral reorientation osteotomy, coverage of femoral head can be improve by acetabular self procedure and residual head deformity can be given reshape by head reduction osteotomy by safe sugical dislocation of hip .
- child with pain, restriction of movement managed by adductor tenotomy & skin traction / walking single hip spica.
- containment of extruded femoral head by proximal varus osteotom
Cubitus varus- cubitus varus deformity is commonly develop after malunion of supracondylar fracture in childhood. cubitus deformity can be managed by corrective osteotomy
Osteomyelitis – Osteomyelitis of bone require urgent attention otherwise part of bone can dead and sequestrate . In acute Osteomyelitis they need good coverage of antibiotic with drainage of pus but in late cases they need sequestrectomy and reconstruction of bone. Deformity should be managed after detail evaluation & careful planning.
A, in forearm with Osteomyelitis radius we can reconstruct limb by making one bone forearm
B, in lower limb limb reconstruction can be done by ilizarrow
Non-union lateral humeral condyle- Lateral humeral condyle fracture if not managed in early period then these children are going to develop sever cubitus valgus deformity & ulnar nerve palsy . non union can be managed by osteosynthesis and corrective osteotomy
Tortricolis- tortricolis in children primarily occur because of sternomastoid muscle contracture which occurs because of birth trauma. Vertebral anomaly should always be ruled out before planning for treatment. Standard protocol of management is Z lengthening of sternomastoid.
Limb shortening- Limb shortening can occur because of many factors like epiphysial damage due to trauma or infection, congenital shortening & bone loss. Shortening of 1- 2 Cm can be managed by shoe raise, more than 2 cm in early age can be mange by temporary epiphysiodesis by the use of staple/ 8 plate/ screw but in late age managed by limb lengthening by ilizarrov bone transport.