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Congenital contractural arachnodactyly (CCA), also known as Beals–Hecht syndrome, is a rare autosomal dominant congenital connective tissue disorder. [1] As with Marfan syndrome, people with CCA typically have an arm span that is greater than their height and very long fingers and toes. [2]
Dupuytren's contracture of the right little finger. Arrow marks the area of scarring. Typically, Dupuytren's contracture first presents as a thickening or nodule in the palm, which initially can be with or without pain. [11] Later in the disease process, which can be years later, [12] there is increasing loss of range of motion of the affected ...
Bethlem myopathy 2 also differs by including the possibility of scapula winging, pectus excavatum, stooped posture, kyphosis (hunchback), micrognathia, retrognathia, and a high-arched palate. [14] Childhood muscle weakness improves in teen years, but muscle weakness returns by the third decade of life. [11]
In pathology, a contracture is a shortening of muscles, tendons, skin, and nearby soft tissues that causes the joints to shorten and become very stiff, preventing normal movement. [ 1 ] [ 2 ] A contracture is usually permanent, but less commonly can be temporary (such as in McArdle disease ), [ 3 ] or resolve over time but reoccur later in life ...
Adhesions and fibrosis are made of dense fibrous tissue, which are strong and supportive, helping to prevent the injury or micro-injury from reoccurring. However, the fibrotic scar tissue causes the muscle tissue to become stuck together which restricts movement, causing pain, weakness, and limited joint mobility. [15] [16] [17] [18]
Often, every joint in a patient with arthrogryposis is affected; in 84% all limbs are involved, in 11% only the legs, and in 4% only the arms are involved. [4] Every joint in the body, when affected, displays typical signs and symptoms: for example, the shoulder (internal rotation); wrist (volar and ulnar); hand (fingers in fixed flexion and thumb in palm); hip (flexed, abducted and externally ...
Fibrous bands are the most common cause of deep gluteal syndrome, [2] and this frequently seen intraoperatively during endoscopic sciatic nerve explorations. [6] Fibrous tissue, also known as scar tissue, is dense, inelastic tissue that can form after the body heals from an injury. Fibrous bands are fibrous tissue with a long, thin shape like a ...
The average lifespan after the onset of symptoms in patients with MSA is 6–10 years. [3] Approximately 60% of patients require a wheelchair within five years of onset of the motor symptoms, and few patients survive beyond 12 years. [3] The disease progresses without remission at a variable rate.