INTRODUCTION
Pain takes a central position in a varied group of disorders, due to insufficient blood supply to the extremities. It results in ischemia of the peripheral tissues, which causes pain and often functional limitation in the patient.

Two important groups of disorders can be distinguished: critical vascular disease and the Raynaud’s phenomenon.
The latter can be subdivided into a primary and a secondary type.

The context and the cause of each of these three groups is different, which means that good diagnostics are essential to identify and influence the prognosis.

Critical ischemic vascular disease is most common in patients over 55 years old as a result of arterial vascular disease. The annual incidence is 0.25 to 0.45 patients per 1,000 population. The disease initially presents as vague pain in the extremities, but ends in necrosis and amputation of the extremity in the course of 5 years.

Raynaud’s phenomenon occurs frequently in our society with an incidence of 3% to 21%.
There is a PRIMARY form, also termed Raynaud’s disease, in which no underlying cause for the symptoms can be found.

The SECONDARY form, indicated with the general term Raynaud’s syndrome, does have an underlying cause. It is usually associated with systemic pathology, in particular rheumatic pathology.

The main pathophysiology is impaired perfusion of the peripheral parts of the extremities. Initially, it manifests itself as white discoloration of the fingers or toes and later as blue discoloration leading to ulcers.

The most important diseases to consider include systemic diseases such as generalized sclerosis and scleroderma. In 90% of the cases with these diseases, Raynaud’s phenomenon is the first symptom.

Thromboangiitisobliterans or Buerger’s disease can also be classified under secondary Raynaud’s. The age at onset is usually under 45 years. It is an immune-mediated arteritis of which the pathology is not fully known, but smoking or smoke cessation can seriously affect the symptom- atology.

Critical ischemic disease is often caused by arteriosclerosis due to hypertension or diabetes. Prevention by means of proper health hygiene is important and can influence the incidence and severity as well as the prognosis.

Primary Raynaud’s is idiopathic and will be diagnosed as such if underlying systemic pathology has been excluded.

Secondary Raynaud’s is often a manifestation of a systemic disease. It is essential to try to establish a diagnosis as soon as possible in order to influence the evolution of the disease.

A sclerotic disease can indeed have a large impact on the functioning of vital organs such as the lungs, liver, or kidneys.

There is often a disorder of the connective tissue, collagen or a rheumatic disease, often with autoimmune features (scleroderma, Sjo¨gren’s disease, rheumatoid arthritis, systemic lupus erythematosus, polymyositis) or a peripheral vascular disease (thromboangiitisobliterans or Buerger’s dis- ease). In rare cases, it occurs in combination with a malignancy or chemotherapy (cisplatinum, bleomycin, and vincristine).

Buerger’s disease appears to be an immune-mediated pathology, occurring both in men and in women. The symptoms already present at an early age, but are predominantly determined by smoking behavior. The first step in the treatment is therefore to refrain from tobacco use.

SYMPTOMS
Pain in the extremities.
In case of critical ischemic disease due to arteriosclerosis, patients often indicate evolution of nonspecific pain in the extremities while walking that disappears at rest.
The first symptom is usually intermittent claudication followed by an increasingly serious symptomatology over the years.
Eventually, slow-healing ulcers will develop.
Critical ischemic disease predominantly occurs in the older population.
Buerger’s disease in which the first symptoms are also atypical pain with eventual discoloration and ulceration.

Patients with Raynaud’s phenomenon mostly complain of pain in the distal parts of extremities, often accompanied by white discoloration of the extremities. At a later stage, the discoloration darkens and ulcers may eventually develop.
CLASSIFICATION of Perfusion Disorders in Peri- pheral Arterial Vascular Disease according to Fontaine
Stage I No symptoms (sufficient peripheral circulation)
Stage II Pain upon exertion, intermittent claudication
IIa ability to walk > 100 m
IIb ability to walk < 100 m
Stage III Pain at rest in the extremity concerned and in the supine position due to a poor muscle perfusion. The pain often temporarily decreases if the leg is dependent
Stage IV Trophic disorders such as necrosis/gangrene
TREATMENT
A CONSERVATIVE MANAGEMENT FOR ISCHEMIC VASCULAR DISEASE
Pharmacological therapy that aims at treating the underlying cause.
If the symptoms persist, it may be decided to perform vascular surgery.
Inoperable, vascular patients with pain at rest and/or ulcers can go for interventional pain procedures.
B INTERVENTIONAL MANAGEMENT FOR ISCHEMIC VASCULAR DISEASE
The treatment of these patients is aimed at pain reduction and cure of the ulcers in order to prevent amputation.
Two methods: 1. Sympathectomy
2. Spinal cord stimulation

Sympathectomy primarily has a vasodilatatory effect on the collateral circulation resulting from a reduced sympathetic tone.
Improved oxygenation of the tissues leads to less tissue damage, which results in decreased pain and increased healing of the ulcers. Pain reduction also occurs due to the interruption of sympathetic nociceptive interaction.

Spinal cord stimulation (SCS) has been used to treat a variety of chronic pain syndromes since 1967.
SCS resulted in better pain reduction than treatment with analgesics.

A Cochrane Review of 2005 concluded that SCS in critical ischemic vascular disease: (1) leads to fewer amputations;
(2) provides better pain relief; and
(3) restores more patients to Fontaine stage II.

PROTOCOL

A sympathetic nerve block can be considered in patients with critical ischemic vascular disease after extensive conservative treatment. If this has insufficient effect, SCS can be considered in a selected patient group.

Sympathectomy can be considered in the treatment of Raynaud’s phenomenon, but only after multidisciplinary evaluation of the patient and in close consultation with the patient’s rheumatologist, vascular surgeon or internist.

Botulinum Toxin A Injections
A study by Van Beek et al.

11 patients with rest pain and finger ulcers who received perivascular injections with botulinum toxin A. There was an immediate favorable effect on the pain in 100% of the patients. In nine patients (82%), the ulcers healed spontaneously and this effect was still present in these patients after follow-up of as long as 30 months.