Thursday 10 April 2014

Cerebral palsy

Cerebral palsy

It is a upper motor neuron disorder due to hypoxic or ischemic injury to the brain of a child during pregnancy or delivery or postnatal period. It results in altered tone in limbs. Early signs include delayed milestones, abnormal posture and gait. As child grows, spastic muscle tendon units fail to catchup with bone growth. So as age advances especially, during periods of rapid growth there can be a deterioration in ambulatory status due to contractures. 

Most of my patients are brought by parents who are aware of the name of the problem but don't know its implications. They continue to think that there is an issue with the upper limb or legs. I always make them understand that the real problem is, in the damaged brain which sends disturbed signals to hands and legs, which result in abnormal posture and gait. Joints and muscles are basically normal, its culprit is the damaged area in brain which controls them. Now that does not mean whole of the brain is damaged. Our brain is magnificently divided in such a way that each of the different part of brain  controls a specific organ or a part or a function. For this reason we may see many variations in presentation of our patients, depending on the part of brain involved.

The numerous causes of this condition are described in literature, a few important ones are mentioned below:

  •  Prenatal (75% cases) 

    Maternal infection during pregnancy ex: CMV, rubella
    Cerebral ischemia
    Maternal drug intake
    Congenital cerebral malformation or abnormalities
    Pre-eclampsiais a risk factor

  • Perinatal (6 - 8% cases)

    Cord prolapse
    Obstructed labour
    Abruptio placenta
    Meconium staining of amniotic fluid


  • Postnatal (10-18%cases)

    Jaundice
    Hypoglycemia
    Neonatal infection ex: meningitis, septicemia

    CP has been classified based on the extent of body involvement, tone, functional and ambulatory capacity. 

    Physiotherapy for keeping the joints free and mobile is mainstay during growth period.  Pharmacological therapy for muscle relaxation to reduce tone may be necessary. When there is a dynamic deformity Botox, casts and stretching might help. However once muscle groups become contracted our physiotherapy, botox and casts may not work efficiently. In severe cases selective posterior rhizotomy may become desirable.  Simultaneously bowel and bladder, speech, intellectual level, oromotor function, hearing, occupation, needs assessment and training to improve it.

    Muscle relaxants are generally used to decrease tone. Baclofen is commonly preferred.

    Botox injections reduce spasticity and effects are temporary and reversible. No major side effects are noted with it and is FDA approved. It must be realized that it is not an isolated treatment and must be integrated with proper rehabilitation. The effect kicks in about 2 weeks after injection and lasts for 3-6 months.

    Doses are again based on weight of the child and muscle group it is intended to be used on. 10 IU/kg per setting is maximum dose to be given. Larger muscles especially biarticular ones in lower limb need 3-6 IU/kg whereas for small muscles of arm and forearm, 1-2 IU/kg may suffice. Botox is available in a freeze dried vial, which needs normal saline for reconstitution. In India vials are available in 200, 100 and 50 IU dose. The site of injection is determined by a qualified physician either clinically, EMG or by ultrasound guidance. Paracetamol or topical local anaesthetic cream if given before botox injection may help reduce the pain after the procedure.

    Soft tissue surgery is key once contractures develop. In olden days single level surgery used to create imbalance as problems in other joints were not dealt with simultaneously. Present trend is to do a single time multilevel surgery (SEMLS), right from hips to ankles that will allow them to balance efficiently. It is necessary to have a video gait analysis done before surgery for better understanding of complex gait patterns.

    They include Psoas lengethening, Adductor release, Hamstrings fractional lengethening, rectus release with or without transfer to hamstrings, Gastronemius recession, Tendoachillis lengethening, Tibialis anterior or tibialis posterior split transfer.


    Bony procedures may be required in cases which do not yield to soft tissue surgery. 

    These include pelvic osteotomy, varus osteotomy, derotation osteotomy, Femur extension supracondylar osteotomy,calcaneal lengthening osteotomy, griece green subtalar arthrodesis.

    It is important to understand that all these will improve the child functionally but never cure the basic problem. Physiotherapy must be continued till skeletal maturity, as after that worsening of contractures rarely occurs.

    7 yrs old girl with spastic hemiplegia and equinus gait, she was treated with perutaneous tendoachillis lengthening.

    Postop cast in place


    AFO after cast removal for couple of weeks later only night time

    8 weeks postop without AFO, plantigrade foot

    Gait after 6 months

    Add caption

     

    Fixed knee deformity in CP in 15yr old boy with crouch gait

     

    Incision for Distal femoral extension osteotomy, inferiorly insicion for hamstring lengethening can be seen.
    AP view of supracondylar dital femoral extension osteotomy

     

    Lateral view of supracondylar extension osteotomy of femur



    Healed osteotomy after 8 weeks post op

     

    Calcaneal lengethening osteotomy with tricortical bone graft insitu for correction of pes planovalgus

     

     

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