الفهرس 
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Abstract
Children with neuromuscular and mitochondrial disorders often have several signs and symptoms which may increase the risk associated with anesthesia and surgery. The term neuromuscular disease defines disorders of the motor unit and excludes influences on muscular function from the brain such as spasticity. The motor unit has 4 components a motor neuron in the brain stem or ventral horn of the spinal cord, its axon, which together with other axons forms the peripheral nerve and all muscle fibers that are innervated by a single motor neuron and the neuromuscular junction. Neuromuscular disorders consist of a heterogeneous group of diseases that can be classified into hereditary conditions and acquired syndromes as well as anatomically with regard to the site that the disease process affects (e.g. pre-junctional, junctional or synaptic, and post-junctional).
Diagnosis of children with neuromuscular disease is essential to assess the risk during surgery and anesthesia, the common presenting chief complaints from parents of children with suspected neuromuscular disorders may include infantile floppiness or hypotonia, delay in motor milestones, feeding and respiratory difficulties, abnormal gait characteristics, frequent falls, difficulty ascending stairs or arising from the floor, muscle cramps or stiffness. Children with neuromuscular disorders are at risk of many complications, respiratory, cardiac and musculoskeletal complications as scoliosis. Thus, preoperative assessment must include respiratory and cardiac assessment. Clinical signs of respiratory affection include rapid shallow breathing, weak cough, accessory muscle use, abdominal paradox, orthopnea, weakness of trapezius and neck muscles, cough after swallowing and tachycardia.
The anesthetic management of pediatric patients with neuromuscular disease can be very challenging and requires careful perioperative planning. These children commonly present for anesthesia for diagnostic procedures as muscle biopsy, or surgery relating to their underlying disorder as gastrostomy, corrective orthopedic procedures or strabismus surgery or incidental surgery.
Preoperative assessment must include a neurological examination to confirm the diagnosis and to identify the level of disease progression in each patient. Assessment of respiratory function should include an accurate medical history and physical examination, a chest X-ray, an evaluation of sleep disordered breathing and the measurements of respiratory function and cough effectiveness. Assessment of cardiac complication include detailed history, however many patients may not be aware of this since they are not capable of vigorous exercise, ECG is more useful as myocardial disease is often accompanied by QRS complex and T wave abnormalities, Echocardiography is commonly regarded as the standard investigation for patients with suspected myocardial disease.
The anesthetic implications of NMD are in part dictated by the stage and severity of the disease. In myopathies, Duchenne is more severe than Becker. The age of child is also important as during early childhood when skeletal muscle is being destroyed, rhabdomyolysis and hyperkalemia can occur in response to triggers, whereas during adolescence, progressive cardiac and respiratory failure are the overwhelming concerns. It is crucial to optimize the patient’s respiratory status before surgery. When respiratory function measurements and/or sleep studies are abnormal, non-invasive ventilation and manual or mechanically assisted cough techniques