Raynaud’s syndrome may be primary or secondary. The primary idiopathic form usually occurs in young women and affects the upper extremities more than the lower. The peripheral pulses are normal. The condition is attributable to abnormal sensitivity in the direct response of the arterioles to cold. When cooled, these vessels constrict and, as a result, the part (usually the fingers) becomes blanched and incapable of finer movements. The capillaries then dilate and fill with slowly flowing deoxygenated blood, the digits therefore becoming swollen and dusky. As the attack passes off, the arterioles relax, oxygenated blood returns into the dilated capillaries and the digits become red. Thus the condition is recognised by the characteristic sequence of blanching, dusky cyanosis and red engorgement, often accompanied by pain. In the idiopathic form, superficial necrosis is very uncommon. Early cases must be distinguished from chilblains and vascular disturbances sometimes associated with the costoclavicular syndrome, and from the other causes of secondary Raynaud’s syndrome.
Protection from cold and avoidance of pulp and nail-bed infections are part of the conservative regimen that is advised for mild cases. The use of calcium antagonists, such as nifedipine, may also have a role to play and electrically heated gloves can be useful in winter. Sympathectomy has been discredited in this condition.
Secondary Raynauds syndrome
This was previously called Raynaud’s disease (a term to be avoided). Although peripheral vasospasm may be noted in atherosclerosis, thoracic outlet syndrome, carpal tunnel, etc., the term secondary Raynaud’s syndrome is most often used for a peripheral arterial manifestation of the collagen diseases, especially progressive systemic sclerosis (scleroderma) and systemic lupus erythromatosis. It may also follow the use of vibrating tools (when it is commonly known as ‘vibration white finger’), e.g. pneumatic road drills, mining borers and chain saws, which vibrate at certain frequencies.
Treatment is directed primarily at the underlying condition, although the conservative measures outlined above are often helpful. The syndrome when secondary to the collagenoses leads frequently to necrosis of digits and multiple amputations. Sympathectomy yields disappointing results and is rarely used. Nifedipine, steroids and vasospastic antagonists may all have a place. Patients with vibration white finger should avoid vibrating tools.
Acrocyanosis, crurum puellarum frigidum4, may be confused with Raynaud’s disease, but it is painless and is not paroxysmal. Affecting young females, the cyanosis of the fingers and, especially, the legs may be accompanied by paraesthesia and chilblains. In severe cases, sympathectomy may be tried. If merely affecting the calves, a differential diagnosis is Bazin’s disease.
Preganglionic cervicodorsal sympathectomy
Supraclavicular method. Through a supraclavicular incision, the clavicular part of the sternomastoid, the posterior belly of the omohyoid and the scalenus anterior muscles are divided, the phrenic nerve being displaced medially. The subclavian artery is exposed and depressed; the suprapleural fascia is divided so that the dome of the pleura can be displaced downwards. The stellate ganglion is identified as it lies on the first rib. The sympathetic trunk is traced downwards and divided below the third thoracic ganglion. All rami communicantes associated with the second and third ganglia and the nerve of Kuntz, a grey ramus running upwards from the second thoracic ganglion to the first thoracic nerve, are meticulously divided. Occasionally, the approach is undera high arching subclavian artery.
Transthoracic method. This gives a greater exposure and facilitates the removal of the sympathetic chain from the fifth ganglion up to the lower fringe of the stellate ganglion. It tends to give better results than the supraclavicular method and can be employed when that has failed. In women where cosmetic effects are a consideration, the approach can be made via an axillary incision through the third space (Hedley Atkins). The sympathetic chain is easily seen and after dividing the pleura, it is dissected out, care being taken to avoid damage to the intercostal vessels, which may cause tedious haemorrhage. Care should also be taken, when making and suturing the approach wound, to avoid damage to the nerve to seratus anterior, giving rise to ‘winging’ of the scapula.
Endoscopic method. This seeks to achieve a sympathectomy via the transthoracic route using a suitable endoscope, e.g. a cystoscope or laparoscope. A Verres needle is passed via the axilla to induce a CO2 pneumothorax. A trochar and cannula are then employed to introduce the endoscope. The sympathetic chain is visualised and a coagulating electrode used to disrupt the ganglia. Some surgeons carry out the procedure without using CD2, the lung being simply deflated by the anaesthetist using a double-lumen endotracheal tube. The endoscopic method is now the procedure of choice for cervicodorsal sympathectomy.
Operative method. Using a transverse loin incision, an extraperitoneal approach is used in which the colon and peritoneum, to which the ureter clings, are stripped medially so as to expose the inner border of the psoas muscle. The sympathetic trunk lies on the sides of the bodies of the lumbar vertebrae; on the right side it is overlapped by the vena cava. Lumbar veins are apt to cross the trunk superficially. The sympathetic trunk is divided on the side of the body of the fourth lumbar vertebra. It is then traced upwards to be divided above the large second lumbar ganglion, which is easily recognised by the number of white rami which join it. Care should be taken not to mistake small lymph nodes, lymphatics, the genitofemoral nerve or the occasional tendinous strip of the psoas minor for the sympathetic chain. It is possible to perform the operation via an endoscope after the creation of a suitably expanded retroperitoneal tissue plane. Along with a decline in the recognised indications for sympathectomy there has been a move away from the operative approach in favour of the less hazardous chemical (phenol) sympathectomy.
Chemical method. This is contraindicated in patients taking anticoagulants. Under radiographic fluoroscopic control, with the patient in the lateral position, local anaesthetic is injected. A long spinal needle is then inserted to seek the side of the vertebral body and to pass alongside it to reach the lumbar sympathetic chain. After confirming the needle position by injection of contrast agent, approximately 5 ml of phenol in water (1:16) is injected. This is usually done at two sites: beside the bodies of the second and fourth lumbar vertebrae. Great care is needed to avoid penetrating the aorta, cava or ureter; the plunger of the syringe must always be drawn back before injection to exclude the presence of blood.
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