Vasculitis is a general term for a group of uncommon diseases that feature inflammation of the blood vessels. The blood vessels of the body are referred to as the vascular system. The blood vessels are comprised of arteries that pass oxygen-rich blood to the tissues of the body and veins that return oxygen-depleted blood from the tissues to the lungs for oxygen. Vasculitis is characterized by inflammation in and damage to the walls of various blood vessels.

Each of the vasculitis diseases is defined by certain patterns of distribution of blood vessel involvement, particular organ involvement, and laboratory test abnormalities. As a group, these diseases are referred to as vasculitides.

The word vasculitis is derived from the Latin “vasculum”, vessel + “- itis”, inflammation. Another term for vasculitis is angiitis. When arteries are the inflamed blood vessels, the condition is also referred to as arteritis. When the veins are inflamed, it is referred to as venulitis.

The actual cause of these vasculitis diseases is usually not known. However, immune system abnormality and inflammation of blood vessels are common features. Each form of vasculitis has its own characteristic pattern of symptoms, much of which depends on what particular organs are affected.

Examples of vasculitis include:

• Kawasaki disease,
• Behçet’s disease,
• polyarteritis nodosa,
• Wegener’s granulomatosis,
• Cryoglobulinemia,
• Takayasu’s arteritis,
• Churg-Strauss syndrome,
• Giant cell arteritis (temporal arteritis), and
• Henoch-Schönlein purpura.

The actual cause of these vasculitis diseases is usually not known. However, immune system abnormality and inflammation of blood vessels are common features. Each form of vasculitis has its own characteristic pattern of symptoms, much of which depends on what particular organs are affected.

Examples of vasculitis include:

• Kawasaki disease,
• Behçet’s disease,
• polyarteritis nodosa,
• Wegener’s granulomatosis,
• Cryoglobulinemia,
• Takayasu’s arteritis,
• Churg-Strauss syndrome,
• Giant cell arteritis (temporal arteritis), and
• Henoch-Schönlein purpura.

Vasculitis can also accompany:

• Infections (such as hepatitis B),
• Exposure to chemicals (such as amphetamines and cocaine),
• Medications,
• Cancers (such as lymphomas and multiple myeloma), and
• Rheumatic diseases (such as rheumatoid arthritis and systemic lupus erythematosus

Treatments:

The treatment of the various forms of vasculitis is based on the severity of the illness and the organs involved. Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, cortisone-related medications, such as prednisone , are used. Additionally, other immune suppression drugs, such as cyclophosphamide (Cytoxan) and others, are considered. Additionally, affected organs (such as the heart or lungs) may require specific medical treatment when the disease is active.

The management of vasculitis is an evolving field in medicine. The ideal programs for monitoring and treatment will continue to improve as disease patterns and causes are defined by medical research.

Craniosynostosis (kray-nee-o-sin-os-TOE-sis) is a birth defect in which one or more of the joints between the bones of your baby’s skull close prematurely, before your baby’s brain is fully formed. When your baby has craniosynostosis, his or her brain can’t grow in its natural shape and the head is misshapen.

Craniosynostosis can affect one or more of the joints in your baby’s skull. In some cases, craniosynostosis is associated with an underlying brain abnormality that prevents the brain from growing properly.

Treating craniosynostosis usually involves surgery to separate the fused bones. If there’s no underlying brain abnormality, the surgery allows your baby’s brain adequate space to grow and develop.

What is an aneurysm?

An aneurysm is an arterial condition in which the wall of an artery weakens, creating a bulge, or distension of the artery.

A comparison between an artery with an aneurysm and two different types of artery. Note the cross-section showing a thinner artery wall in the third diagram for aneurysm, compared with normal and narrowed arteries.

An aneurysm can occur in important arteries such as those supplying blood to the brain, and the aorta; the large artery that originates at the left ventricle of the heart and passes down through the chest and abdominal cavities.

The normal diameter of the aorta is around 0.8 inches. This width can bulge to beyond 2 inches with an aneurysm, a width that would typically necessitate surgical treatment.

An aneurysm can also occur in peripheral arteries – usually behind the knee (popliteal aneurysms) – although rupture of these is relatively uncommon.

The two most important common locations for aneurysms are:

• In the artery directly leaving the heart – an aortic aneurysm (including thoracic and, further down, abdominal aortic aneurysms)
• In an artery in the brain – a cerebral aneurysm.

Thoracic aortic aneurysm is often abbreviated to TAA, and abdominal aortic aneurysm to AAA. Brain aneurysms are often termed intracranial aneurysms, as well as “berry aneurysms” on account of their size and shape.

Two other examples of aneurysm are mesenteric artery aneurysm (affecting the artery supplying the intestines of the gut) and splenic artery aneurysm (occurring in the spleen, an abdominal organ).

Symptoms of aneurysm:

Most aneurysms do not themselves cause any symptoms.

Even if an aneurysm does not rupture, however, a large aneurysm may obstruct circulation to other tissues. An aneurysm can also contribute to the formation of blood clots that then obstruct smaller blood vessels, potentially causing ischemic stroke or other serious problems; this is known as thromboembolism.

Abdominal aneurysms are sometimes associated with symptoms if they grow rapidly. Some people report abdominal or lower back pain, or a pulsating sensation in the abdomen.

Similarly, thoracic aneurysms can cause symptoms by affecting nearby tissues, including nerves and other blood vessels.

If an aneurysm compresses the laryngeal or vagus nerve, it can cause chest or back pain and symptoms such as coughing, wheezing and difficulty swallowing. Compression of the coronary artery can also cause chest pain.

Otherwise, aneurysms tend to produce symptoms only when there are complications such as rupture.

Symptoms can also be related to the cause of the aneurysm rather than the aneurysm itself. In the case of infection or vasculitis (blood vessel inflammation), for example, there may be fever, malaise or weight loss.

Complications of aneurysm

If an aneurysm has remained undetected, the first sign of it could be when there is a complication – in particular, a rupture – with symptoms resulting from this rather than the aneurysm itself.

The majority of people living with an aneurysm do not suffer any of the complications in the following list. Managing the risk factors is important, however, because all of these possibilities are serious.

Complications of aneurysm include:

Brain aneurysm can lead to subarachnoid hemorrhage; a symptom of this stroke bleed is a sudden extreme headache.

• Thromboembolism – depending on where the clot has traveled to, thromboembolism can cause pain in the extremities or the abdomen. If a clot travels to the brain, it can cause a stroke
• Dissection of the aorta – see below for more detail
• Severe chest and/or back pain – if a silent or diagnosed aortic aneurysm in the chest ruptures, severe chest or back pain may arise. Such symptoms may help hospital medical staff diagnose an aneurysm.
• Angina – certain types of aneurysm can lead to angina, another type of chest pain; the pain is related to narrowed arteries supplying the heart itself (causing myocardial ischemia and possibly heart attack).
• Sudden extreme headache – if a brain aneurysm leads to subarachnoid hemorrhage (a kind of stroke), the main symptom is sudden extreme headache; often so severe that it is unlike any previous experience of head pain.
• Other symptoms – with any aneurysm rupture there may be pain, low blood pressure, a rapid heart rate, and light-headedness.

Again, most people with an aneurysm will not suffer any of these complications, and the risk can be reduced by taking preventive steps.

Treatments for aneurysm

Not all cases of unruptured aneurysm need active treatment, but when an aneurysm ruptures, emergency surgery is needed.

More details follow for the treatment options against the two main types of aneurysm.

Aortic aneurysm treatment options

An aortic aneurysm, whether abdominal or thoracic, may not need any active treatment and instead may just be monitored regularly. Medications and preventive measures may form part of conservative management or they may accompany active surgical treatment – through open or endovascular surgery.

Aneurysms that rupture require emergency surgery. Without immediate repair, a ruptured aneurysm is always fatal in the thoracic aorta, and almost always fatal in the abdominal aorta.

The decision to operate on an unruptured aneurysm in the aorta depends on a number of factors related to the individual patient, as well as to features of the aneurysm itself:

• Individual factors – age, general health, coexisting conditions and personal choice
• The aneurysm’s characteristics – size relative to location in the thorax or abdomen and the rate of growth
• Other factors – chronic abdominal pain or risk of thromboembolism may make surgery a good option.

An aortic aneurysm that has a larger diameter (about 2 inches or 5 cm) is more likely to prompt a recommendation of surgery, as is an aneurysm which is growing more quickly (a little less than 1/4 inch over the last 6-12 months).

For both elective unruptured and emergency ruptured cases, surgery can take one of two forms:

• Open surgery to fit a synthetic or stent graft
• Endovascular stent-graft surgery.

Endovascular surgery involves accessing the blood vessels through a small incision near the hip. In stent-graft surgery, an endovascular graft is inserted through this incision using a catheter and is positioned in the aorta to seal off the aneurysm.

In the emergency of a ruptured abdominal aortic aneurysm, the decision over which procedure to perform will be urgent. For elective operations to repair unruptured aneurysms, a review published in 2014 sought to determine the relative risks and benefits of open vs. endovascular surgery.

The review found that while there was a lack of robust studies comparing the two options directly, similar rates of complication were reported for both types of surgery.

The evidence suggested that endovascular aneurysm repair (EVAR) was “less invasive,” but the procedure did “not always significantly alter the postoperative course or length of hospital stay for patients.”

The authors concluded that open surgery still had a role for those patients who were not suitable for endovascular surgery.

Endovascular surgery has become the generally preferred method of aortic aneurysm surgery because of:

• Shorter operative times
• Often shorter hospital stays
• Better levels of surgical experience
• A perceived lower risk of disease or death surrounding surgery compared with laparoscopic surgery (although the review found this could be more perception than reality).

Endovascular surgery for the repair of aortic aneurysms does carry the following risks however, in addition to the usual risks of surgery:

• Bleeding around the graft (which requires additional surgery)
• Bleeding before or after the procedure
• Blockage of the stent
• Nerve damage, resulting in weakness, pain or numbness in the leg
• Kidney failure
• Reduced blood supply to the legs, kidneys or other organs
• Erectile dysfunction
• Unsuccessful surgery that then requires open surgery
• Slippage of the stent.

Cerebral aneurysm treatment options

Surgical treatment of an aneurysm in the brain is reserved for cases that present a high risk of rupture.

Aneurysms that are less likely to burst are not treated surgically because of the potential risk of brain-damage resulting from possible surgical complications.

In lieu of surgery, patients are given guidance on how to monitor and modify, where possible, the risk factors for rupture of a brain aneurysm. This will likely involve monitoring blood pressure – see prevention below.

Where a ruptured brain aneurysm has led to a subarachnoid hemorrhage (a medical emergency), a patient may be admitted to hospital and undergo brain surgery.

Such a procedure would aim to close off the artery that ruptured due to the aneurysm, in the hope of preventing another bleed.

Prevention of aneurysm

The single most important way a person can reduce their risk of developing an aneurysm is to not smoke or to quit if already smoking. Smoking is not only the strongest risk factor for the development of an aortic aneurysm, it is also one of the greatest risk factors for an aneurysm growing and rupturing.

Smoking is a greater risk factor for aneurysm than it is for atherosclerosis, the cardiovascular disease where fatty deposits accumulate on the arterial wall and which can weaken artery walls.

The fact that smoking is also a big risk factor for atherosclerosis is directly relevant, however, because most aneurysms are caused by weakening of the artery wall brought on by atherosclerosis:

Studies have shown that continued smoking induces a significantly faster expansion of aortic aneurysms – by about 0.4 millimeters a year).

Brain aneurysms are not always possible to avoid to the same extent as aortic aneurysms because ruptured brain aneurysms causing subarachnoid hemorrhage stroke can be congenital – present at birth.

Brain aneurysm has both genetic and environmental risk factors, and a history of brain aneurysms in the family is an established risk factor, as is increasing age. However, known risk factors also include, alongside hypertension (high blood pressure), smoking.

Cigarette smoke is also a major source of free radicals which cause oxidative stress. Oxidative stress and resulting tissue damage can occur due to higher production or reduced removal of free radicals. Oxidative stress can also increase inflammation, which contributes to the formation and rupture of cerebral aneurysms.

Thoracic outlet syndrome (TOS) is a condition whereby symptoms are produced (such as numbness in fingers, pain in shoulder, arm, and neck) due to compression of nerves and/or blood vessels in the upper chest. The passageway for these nerves and blood vessels to exit the chest and supply the upper extremities is referred to as the thoracic outlet. Muscle, bone, and other tissues border the thoracic outlet. Any condition that results in enlargement or movement of these tissues of or near the thoracic outlet can cause the thoracic outlet syndrome. These conditions include muscle enlargement (such as fromweight lifting), injuries, an extra rib extending from the neck (cervical rib), weight gain, and rare tumors at the top of the lung. Often no specific cause is detectable.

Thoracic outlet syndrome is a condition whereby symptoms are produced from compression of nerves or blood vessels, or both, because of an inadequate passageway through an area (thoracic outlet) between the base of the neck and the armpit.

Thoracic outlet syndrome symptoms include

• neck pain,
• shoulder pain,
• arm pain,
• numbness and tingling of the fingers, and
• impaired circulation to the extremities (causing discoloration).

An inadequate passageway for nerves and blood vessels as they pass through an area (thoracic outlet) between the base of the neck and the armpit causes thoracic outlet syndrome. This can be constant or intermittent. Thoracic outlet syndrome can be caused by weight lifting, obesity, tumors in the chest, and extra ribs extending from the seventh cervical vertebra at the base of the neck.

Risk factors include occupations that involve heavy usage of the upper extremities against resistance, including jack-hammer operators and dental hygienists, weight lifting, pregnancy, and obesity. Any condition that causes encroachment of the space for the brachial plexus at the thoracic outlet can lead to thoracic outlet syndrome, including poor posture.

Symptoms include neck, shoulder, and arm pain, numbness in the fingers, or impaired circulation and flushed sensations to the extremities (causing discoloration). The involved upper extremity can feel weak. Often symptoms are reproduced or worsened when the arm is positioned above the shoulder or extended. Patients can have a wide spectrum of symptoms from mild and intermittent to severe and constant. Pains can extend to the fingers and hands, causing weakness.

Doctors who treat thoracic outlet syndrome include general physicians, such as general-medicine doctors, family medicine doctors, and internists, as well as rheumatologists, physical-medicine doctors, and chest surgeons.

What is the prognosis for thoracic outlet syndrome?

Most people with thoracic outlet syndrome can have complete resolution of symptoms with conservative measures, including exercises specific for thoracic outlet syndrome, physical therapy, and avoiding stressing the tissues of the thoracic outlet. It can be helpful to avoid sleeping with the arms extended above the head. Rarely, surgical intervention can be necessary to take pressure off of involved nerves and blood vessels. Complications include embolization to the hand and nerve damage to the extremity involved.

Is it possible to prevent thoracic outlet syndrome?

It’s possible to prevent thoracic outlet syndrome by maintaining relaxed tissues of the upper chest. This can involve prevention exercises, stretches, and therapies designed to loosen the tissues around the shoulders and neck.