What are Neuromuscular Diseases?
Neuromuscular diseases are disorders of the muscles and nerves associated with the nervous system. The nervous system comprises the brain, spinal cord, and nerves that connect the brain and the spinal cord to the rest of your body. Neuromuscular diseases affect normal body function, causing loss of sensation, muscle weakness, pain, improper coordination, and even paralysis. They can also affect any bone or joint in the body. When extended to the bones, these diseases may lead to abnormal bone development such as a missing, fragile, or curved bones.
What are Orthopedic Complications of Neuromuscular Diseases?
Orthopedic complications are abnormalities in the development and functioning of the bones, muscles, tendons, and ligaments due to neuromuscular diseases. The injured nerves impact the functioning of the related structures, thereby creating challenges with daily activities.
Treatment and management of orthopedic complications is especially challenging in children with neuromuscular disorders.
Signs and Symptoms of Orthopedic Complications of Neuromuscular Diseases in Children
The various orthopedic complications of neuromuscular diseases that affect children include:
Cerebral palsy is caused by damage to the brain and leads to stiffness and weakness in the muscles, abnormal muscle movement, and poor coordination. In many cases, hearing, vision, speaking, swallowing, and sensation are also affected.
Congenital Myasthenia Gravis
Congenital myasthenic syndrome is caused by a genetic defect and leads to muscle weakness that worsens upon physical exertion. Symptoms appear during early childhood. The facial muscles are affected, disrupting eye movements, chewing, and swallowing. Other symptoms include delayed or improper development, difficulty crawling, walking, and breathing problems.
Muscular atrophy refers to the degeneration of the muscles due to lack of physical activity. It makes joint and muscle movement difficult, including lifting the arms, bending, or moving the legs.
Scoliosis is the abnormal curvature of the spine causing a deviation to one side. It can affect either the mid or lower back. Scoliosis causes a physical deformity, making the spine look like the letter “C” or “S” instead of the letter “I”.
Hip dysplasia is a hip disorder in which the acetabulum - the hip socket - doesn't cover the femoral head properly. As a result, the patient suffers from abnormal hip development, which typically includes either partial or complete hip dislocation.
Charcot-Marie-Tooth disease (CMT) is a neurological disorder due to a genetic defect. It is characterized by degeneration of the peripheral nervous system (PNS). The PNS transmits signals from the brain to various parts of the body and vice versa. Symptoms include muscle weakness, stiffening of joints, hardening of muscles, and loss of sensation in the hands, feet, legs, and forearms.
What are the Treatment Options for Orthopedic Complications of Neuromuscular Diseases?
Treatment can involve a conservative approach which may later lead to surgery, depending on the child’s age, health condition, and the severity of the bone defect.
Conservative management may include one or more of the following:
- Diet modification
- Medications to treat individual symptoms
- Vitamin or calcium supplements to strengthen the bones
- Immobilization with a brace, cast, or splint to help stretch the tissues near the problem area
- Physical therapy to increase joint mobility and muscle strength
- Low-intensity exercises such as swimming to build muscle and bone strength
- Walking aids
- Occupational therapy to help children manage their symptoms during daily activities
Surgery is recommended for children with severe orthopedic complications and those that do not respond well to conservative management. Surgical procedures are usually performed under local or general anesthesia. Care is taken to perform the surgery through minimal incisions. However, sometimes open surgery is necessary.
Surgery may involve any of the following:
- Bone, tendon, or ligament repair
- Nerve release to relieve pressure on a compressed nerve
- Osteotomy, in which a section of the bone is cut and removed
- Partial/complete joint replacement