Thoracic insufficiency syndrome

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Thoracic insufficiency syndrome
Specialty Endocrinology

Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration. [1] It is frequently associated with chest and/or spinal abnormalities. Treatment options are limited, but include supportive pulmonary care and surgical options (thoracoplasty and/or implantation of vertical expandable prosthetic titanium rib (VEPTR) devices). [2] [3]

Thorax frontal part of an animals body, between its head and abdomen

The thorax or chest is a part of the anatomy of humans and various other animals located between the neck and the abdomen. The thorax includes the thoracic cavity and the thoracic wall. It contains organs including the heart, lungs, and thymus gland, as well as muscles and various other internal structures. Many diseases may affect the chest, and one of the most common symptoms is chest pain.

In physiology, respiration is the movement of oxygen from the outside environment to the cells within tissues, and the transport of carbon dioxide in the opposite direction.

Vertebral column bony structure found in vertebrates

The vertebral column, also known as the backbone or spine, is part of the axial skeleton. The vertebral column is the defining characteristic of a vertebrate in which the notochord found in all chordates has been replaced by a segmented series of bone: vertebrae separated by intervertebral discs. The vertebral column houses the spinal canal, a cavity that encloses and protects the spinal cord.

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Marfan syndrome genetic disorder of the connective tissue

Marfan syndrome (MFS) is a genetic disorder of the connective tissue. The degree to which people are affected varies. People with Marfan tend to be tall and thin, with long arms, legs, fingers and toes. They also typically have flexible joints and scoliosis. The most serious complications involve the heart and aorta, with an increased risk of mitral valve prolapse and aortic aneurysm. Other commonly affected areas include the lungs, eyes, bones and the covering of the spinal cord.

Long thoracic nerve Large nerve

The long thoracic nerve supplies the serratus anterior muscle. This nerve characteristically arises from the anterior rami of three spinal nerve roots: the fifth, sixth, and seventh cervical nerves (C5-C7) although the root from C7 may be absent. The roots from C5 and C6 pierce through the scalenus medius, while the C7 root passes in front of the muscle.

Laminectomy

A laminectomy is a surgical procedure that removes a portion of the vertebral bone called the lamina, which is the roof of the spinal canal. It is a major spine operation with residual scar tissue and may result in postlaminectomy syndrome. Depending on the problem, smaller alternatives, e.g., small endoscopic procedures, without bone removal, may be possible.

Thoracic outlet syndrome vascular disease that is characterized by compression at the superior thoracic outlet resulting from excess pressure placed on a neurovascular bundle passing between the anterior scalene and middle scalene muscles

Thoracic outlet syndrome (TOS) is a condition in which there is compression of the nerves, arteries, or veins in the passageway from the lower neck to the armpit. There are three main types: neurogenic, venous, and arterial. The neurogenic type is the most common and presents with pain, weakness, and occasionally loss of muscle at the base of the thumb. The venous type results in swelling, pain, and possibly a bluish coloration of the arm. The arterial type results in pain, coldness, and paleness of the arm.

Pancoast tumor tumor of the pulmonary apex. It is a type of lung cancer defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the ribs and vertebrae

A Pancoast tumor is a tumor of the pulmonary apex. It is a type of lung cancer defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the ribs and vertebrae. Most Pancoast tumors are non-small cell cancers.

Thoracic vertebrae vertebrae between the cervical vertebrae and the lumbar vertebrae

In vertebrates, thoracic vertebrae compose the middle segment of the vertebral column, between the cervical vertebrae and the lumbar vertebrae. In humans, there are twelve thoracic vertebrae and they are intermediate in size between the cervical and lumbar vertebrae; they increase in size going towards the lumbar vertebrae, with the lower ones being a lot larger than the upper. They are distinguished by the presence of facets on the sides of the bodies for articulation with the heads of the ribs, as well as facets on the transverse processes of all, except the eleventh and twelfth, for articulation with the tubercles of the ribs. By convention, the human thoracic vertebrae are numbered T1–T12, with the first one (T1) located closest to the skull and the others going down the spine toward the lumbar region.

Klippel–Feil syndrome physical disorder that has material basis in abnormal segmentation of the vertebra during fetal development which results in fusion located in cervical vertebra

Klippel–Feil syndrome (KFS), also known as cervical vertebral fusion syndrome, is a rare condition present at birth characterized by the abnormal joining (fusion) of any two of the seven bones in the neck. It results in a limited ability to move the neck and shortness of the neck resulting in the appearance of a low hairline.

Valvular heart disease is any disease process involving one or more of the four valves of the heart. These conditions occur largely as a consequence of aging, but may also be the result of congenital (inborn) abnormalities or specific disease or physiologic processes including rheumatic heart disease and pregnancy.

Scalene muscles Muscles on the sides of the neck

The scalene muscles are a group of three pairs of muscles in the lateral neck, namely the anterior scalene, middle scalene, and posterior scalene. They are innervated by the fourth, fifth, and sixth cervical spinal nerves (C4-C6).

Spinal fusion

Spinal fusion, also called spondylodesis or spondylosyndesis, is a neurosurgical or orthopedic surgical technique that joins two or more vertebrae. This procedure can be performed at any level in the spine and prevents any movement between the fused vertebrae. There are many types of spinal fusion and each technique involves using bone grafting—either from the patient (autograft), donor (allograft), or artificial bone substitutes—to help the bones heal together. Additional hardware is often used to hold the bones in place while the graft fuses the two vertebrae together.

Rib fracture break in a rib bone

A rib fracture is a break in a rib bone. This typically results in chest pain that is worse with breathing in. Bruising may occur at the site of the break. When several ribs are broken in several places a flail chest results. Potential complications include a pneumothorax, pulmonary contusion, and pneumonia.

Middle back pain, also known as thoracic back pain, is back pain that is felt in the region of the thoracic vertebrae, which are between the bottom of the neck and top of the lumbar spine. It has a number of potential causes, ranging from muscle strain to collapse of a vertebra or rare serious diseases. The upper spine is very strong and stable to support the weight of the upper body, as well as to anchor the rib cage which provides a cavity to allow the heart and lungs to function and protect them.

Traumatic asphyxia, or Perthes's syndrome, is a medical emergency caused by an intense compression of the thoracic cavity, causing venous back-flow from the right side of the heart into the veins of the neck and the brain.

Tricuspid insufficiency A tricuspid valve disease that is characterized by failure of the hearts tricuspid valve to close properly during systole. As a result, with each heart beat, blood is pumped out from the right side of the heart in the opposite direction to normal.

Tricuspid insufficiency (TI), also called tricuspid regurgitation (TR), is a type of valvular heart disease where there is failure of the heart's tricuspid valve to close properly when the ventricles contracts. This defect allows the blood to flow backwards, reducing its efficiency. Regurgitation may be due to a structural change of components of the tricuspid valve apparatus, a lesion can be primary or secondary.

Anterior spinal artery syndrome Human disease

Anterior spinal artery syndrome is syndrome caused by ischemia of the anterior spinal artery, resulting in loss of function of the anterior two-thirds of the spinal cord. The region affected includes the descending corticospinal tract, ascending spinothalamic tract, and autonomic fibers. It is characterized by a corresponding loss of motor function, loss of pain and temperature sensation, and hypotension.

Vascular myelopathy refers to an abnormality of the spinal cord in regard to its blood supply. The blood supply is complicated and supplied by two major vessel groups: the posterior spinal arteries and the anterior spinal arteries—of which the Artery of Adamkiewicz is the largest. Both the posterior and anterior spinal arteries run the entire length of the spinal cord and receive anastomotic (conjoined) vessels in many places. The anterior spinal artery has a less efficient supply of blood and is therefore more susceptible to vascular disease. Whilst atherosclerosis of spinal arteries is rare, necrosis in the anterior artery can be caused by disease in vessels originating from the segmental arteries such as atheroma or aortic dissection.

Asphyxiating thoracic dysplasia human disease

Asphyxiating thoracic dysplasia or Jeune syndrome is a ciliopathy.It is also known as "Jeune syndrome".

Spinal stenosis bone deterioration disease that has material basis in bony spurs, disc degeneration, or thickened ligaments which results in narrowing located in spinal cord

Spinal stenosis is an abnormal narrowing of the spinal canal or neural foramen that results in pressure on the spinal cord or nerve roots. Symptoms may include pain, numbness, or weakness in the arms or legs. Symptoms are typically gradual in onset and improve with bending forwards. Severe symptoms may include loss of bladder control, loss of bowel control, or sexual dysfunction.

Open aortic surgery surgical technique

Open aortic surgery (OAS), also known as open aortic repair (OAR), describes a technique whereby an abdominal or retroperitoneal surgical incision is used to visualize and control the aorta for purposes of treatment. OAS is used to treat aneurysms of the abdominal and thoracic aorta, aortic dissection, acute aortic syndrome, and aortic ruptures. Aortobifemoral bypass is also used to treat atherosclerotic disease of the abdominal aorta below the level of the renal arteries. In 2003, OAS was surpassed by endovascular aneurysm repair (EVAR) as the most common technique for repairing abdominal aortic aneurysms in the United States. In OAS for abdominal aortic aneurysm, the aneurysmal portion of the aorta is replaced with a graft, usually made of dacron or PTFE.

References

  1. The Characteristics of Thoracic Insufficiency Syndrome Associated with Fused Ribs and Congenital Scoliosis; Campbell, R. M. et al.; The Journal of Bone and Joint Surgery, Inc., 2003
  2. Kocher, Mininder; Millis, Michael B. (2011). Operative Techniques: Pediatric Orthopaedic Surgery E-BOOK: E-BOOK. Elsevier Health Sciences. p. 668. ISBN   1455711357.
  3. Akbarnia, Behrooz A.; Yazici, Muharrem; Thompson, George H. (2015). The Growing Spine: Management of Spinal Disorders in Young Children. Springer. p. 669. ISBN   9783662482841.