Upper Limb Ischaemia
Upper limb ischaemia (ULI) is as well a recognized clinical entity as lower limb ischaemia, though it is less common. There are certain specific conditions which are responsible for ULI such as cervical rib, Raynaud’s disease, etc. It is also important to note that reconstructive surgery is rarely done in the upper limb when compared to the lower limb.
Read And Learn More: Clinical Medicine And Surgery Notes
However, students should be able to identify the ischaemic features in the upper limb early and refer the case to a suitable specialist, so that it can be treated promptly and adequately, thereby avoiding a tragedy such as loss of the limb.
The following are the causes of upper limb ischaemia:
- Raynaud’s disease and Raynaud’s syndrome
- Embolic causes
- Thoracic outlet syndrome
- Trauma
- Buerger’s disease
- Atherosclerotic vascular disease of the upper limb (rare)
- Axillary vein thrombosis
- Vasculitis syndromes
-
- Takayasu’s arteritis
- Giant cell arteritis
- Polyarteritis nodosa
- Systemic sclerosis, scleroderma, CREST syndrome (see below)
Raynaud’s Disease (Primary Raynaud’s Phenomenon)
- It occurs in young women, commonly.
- The upper limb is more commonly involved than the lower limb.
- Commonly seen in Western countries in white-skinned people. Cold climate is possibly a precipitating factor. First described by Raynaud as bilateral episodic digital ischaemia of the upper limb on exposure to cold and emotions. It is also referred to as primary Raynaud’s phenomenon.
- Raynaud’s phenomenon is the blanket term used to describe cold-related digital vasospasm (see pathophysiology). Raynaud’s phenomenon is subdivided into Raynaud’s syndrome where there is an associated disorder, and primary Raynaud’s disease where there is none.
- Crest syndrome: Calcinosis circumscripta, Raynaud’s phenomenon, (O) Esophageal defects, Sclerodactyly, Telangiectasia.
- Causes of Secondary Raynaud’s phenomenon are given
Secondary Raynaud’s Phenomenon Causes
- Atherosclerosis
- Scleroderma
- Systemic lupus
- Cervical rib
- Carpal tunnel syndrome
- Vibrating tools—Vibration white finger
Pathophysiology
On exposure to cold1, some kind of discomfort and colour changes are observed. This is due to abnormal sensitivity of the arterioles to cold. Three stages have been described:
Stage of syncope: Arterioles undergo constriction as an abnormal response to cold. As a result of this, the part becomes blanched and severe pallor develops.
Stage of asphyxia: After a brief period of vasoconstriction, capillaries dilate, filling with deoxygenated blood resulting in bluish discolouration of the part (cyanosis).
Stage of recovery or stage of rubor: As the attack passes off, relaxation of the arterioles occurs, circulation improves and redness occurs. Because of the dilatation of capillaries, red engorgement of the part occurs, which causes tingling, burning or bursting pain in the fingers.
Pathophysiology Clinical Features
- Affects young women
- Typically causes bilateral episodic digital ischaemia on exposure to cold
- The thumb is usually spared
- Peripheral pulses are normal
- Pallor, cyanosis and rubor are the colour changes during the attack along with the pain In a few patients, because of recurrent attacks, gangrenous patches occur on the tip of the fingers (superficial necrosis).
1Cold refers to temperature—cold climate (winter) cold environment (refrigerator), or cold substance like cold water or ice.
Differential Diagnosis
- Cervical rib
- Vasculitis syndromes
- TAO affecting the upper limb usually affects male smokers. Peripheral pulses are feeble or weak.
Pathophysiology Treatment
- Conservative line of treatment:
- Reassurance
- Avoid unnecessary exposure to cold
- Avoid smoking
- Calcium antagonists such as nifedipine 10–20 mg, two times a day may be beneficial.
- If these measures fail, surgery is undertaken.
- Cervical sympathectomy:
- In this operation, the sympathetic trunk from the lower half of the stellate ganglion to just below the 3rd thoracic ganglion is removed.
- The upper ½ of the stellate ganglion is preserved to avoid Homer’s syndrome.
- All rami communicantes associated with the 2nd and 3rd ganglion are removed.
Indications For Cervical Sympathectomy
- Raynaud’s disease
- Cervical rib
- TAO
- Causalgia
- Hyperhidrosis
Complications Of Cervical Sympathectomy
- Perforation of pleura causing pneumothorax
- Lymph fistula due to injury to the thoracic duct
- Horner’s syndrome
- Injury to the accessory nerve
- Haemorrhage
-
- The nerve of Kuntz, a grey ramus which springs from the 2nd thoracic ganglion to the 1st thoracic nerve is also divided.
- Commonly done through a supraclavicular route and an axillary route. Laparoscopic sympathectomy is becoming popular.
- The effect seems to be temporary.
- However, the severity of the disease is reduced.
- Sympathectomy raises the threshold at which spasm occurs.
Thoracic Outlet Syndrome Causes
- Cervical rib
- Scalenus antics syndrome
- Costoclavicular syndrome
- Hyperabduction syndrome
- Abnormal rib and clavicle
- Malaligned fractured clavicle
- Long C7 transverse process
- Hypertrophy of the subclavius muscle as in butterfly swimmers.
Surgical Anatomy Of Thoracic Outlet And Pathophysiology Of Cervical Rib With Compression
- The thoracic outlet is a tight space with bony structures all around such as the manubrium sternum in the front, the spine posteriorly and the first rib laterally.
- At the root of the neck, the brachial plexus and subclavian artery pass through the scalene triangle into the axilla.
- The Scalenus triangle is the posterior compartment of the costoclavicular space. The division of anterior and posterior compartments is by scalenus anticus. The anterior compartment contains a subclavian vein.
- If the base (the first thoracic rib) is raised by the interposition of the cervical rib, it results in compression of the subclavian artery.
- Boundaries of scalene triangle.
- Base: First thoracic rib
- Anteromedially: Scalenus antics
- Posterolaterally: Scalenus medius
Causes Of Subclavian Artery Compression
- Cervical rib
- Clavicle
- Callus due to fracture
- Congenital abnormality—abnormal first rib
- Scalenus antics muscle
Subclavian Artery Occlusion—Effects
Lumen narrowing
↓
Fibrosis or thickening of the arterial wall
↓
Stenosis
↓
Post stenotic dilatation
↓
Multiple→ thrombi Embolism→ Ischaemia
- Due to slow compression, the artery distal to the compression dilates due to a jet-like effect and turbulence of blood flow. This is described as post-stenotic dilatation (Venturi effect).
- In this dilated segment, small multiple thrombi develop, which, when dislodged, result in ischaemia. Vascular symptoms are strictly unilateral.
Cervical Rib
- This is an extra rib present in the neck in about 1–2% of the population.
- Commonly unilateral and in some cases it is bilateral.
- It is more frequently encountered on the right side.
- It is the anterior tubercle of the transverse process of the 7th cervical vertebra which attains excessive development and results in a cervical rib.
Types Of Cervical Rib
- Type 1 The free end of the cervical rib is expanded into a hard, bony mass which can be felt in the neck.
- Type 2 Complete cervical rib extends from the C7 vertebra posteriorly to the manubrium anteriorly.
- Type 3 Incomplete cervical rib, which is partly bony, partly fibrous.
- Type 4 A complete fibrous band which gives rise to symptoms but cannot be diagnosed by X-ray.
Cervical Rib Clinical Features
- Common in young females: Even though congenital, symptoms appear only at or after puberty. This is because of the development of shoulder girdle muscles and sagging of the shoulder which narrows the root of the neck. Nerve roots C 8, T1 are stretched by completion of growth around 25 years.
- Dull aching pain in the neck is caused by the expanded bony end of the cervical rib.
- Features of upper limb ischaemia Claudication pain is brought about by the usage of an arm with muscle wasting. Low temperature, pallor, excessive sweating (vasomotor disturbances), splinter haemorrhages, ischaemic ulcers in fingers and gangrene of the skin of the fingers are the other features. Peripheral pulses may be absent/feeble. Oedema and venous distension are very rare. These are called vascular symptoms of the cervical rib.
- Features of ulnar nerve paralysis or weakness (lower nerve root involvement, mainly first thoracic nerve) manifest as paralysis of interosseus muscles, wasting of hypothenar muscles, tingling and numbness, or paraesthesia in the distribution of C8, T1.
The following are the tests1 which are done for ulnar nerve weakness. It includes sensory disturbances and motor disturbances (performing fine actions—writing, buttoning, etc.)—not seen in the cervical rib.
- Card test: The patient is asked to hold a thin paper or a card in between the fingers. In cases of ulnar nerve paralysis, due to weakness of interossei muscles, the patient is unable to hold the card tightly.
- Froment’s sign: The patient is asked to hold a book between the hand and the thumb. In cases of ulnar nerve paralysis, since the adductor pollicis is paralysed, there is flexion at the distal interphalangeal joint of the thumb. This is because the flexor pollicis longus which is supplied by the median nerve, contracts.
1 These tests can be asked in clinical examination, hence mentioned here.
5. A hard mass may be visible or may be palpable in the root of the neck. (Type I).
6. On palpation of the supraclavicular region, a thrill and on auscultation, a bruit can be heard in cases of post-stenotic dilatation.
7. Adson’s test: Feel the radial pulse, ask the patient to take deep inspiration and turn the neck to the same side. The pulse may disappear or it may become feeble. This test indicates compression on the subclavian artery.
8. Hyperabduction test (Halsted test): This test is done to rule out hyperabduction syndrome caused by pectoralis minor. The radial pulse becomes weak on hyperabduction due to the angulation of axillary vessels and brachial plexus, which gets compressed between pectoralis minor and its attachment to the coracoid process.
9. Military attitude test: When shoulders are set in backward and downward position the radial pulse becomes weak. This is due to the compression of the subclavian artery between the clavicle and the first rib. This is seen in costoclavicular syndrome.
10. Allen’s test: The patient is asked to clench his fist tightly, and compress the radial artery and ulnar artery at the wrist with the thumbs. Wait for 10 seconds, and ask the patient to open his hands. Pallor can be seen in the palm. Now release radial artery pressure and watch for the blood flow. If there is digital artery occlusion it will be evident when colour changes occur in the fingers slowly.
11. Elevated arm stress test (EAST) (Roos): The patient is asked to abduct the shoulders to 90° and to flex the elbow. Then he is asked to pronate/supinate forearms continuously. The appearance of symptoms suggests thoracic outlet syndrome.
Cervical Rib Differential Diagnosis
A patient who presents with a few neurological symptoms and signs in the upper limb with a cervical rib may have some other causes for those symptoms. Hence, it is important to exclude other causes.
- Cervical spondylosis: This should be considered as a possibility in patients above the age of 40 years.
- Cervical disc protrusion and spinal cord tumours may mimic cervical ribs with predominant neurological features.
- Carpal tunnel syndrome can occur due to various causes like myxoedema, rheumatoid arthritis, malunited Colle’s fracture, etc. Predominant features of median nerve involvement, more so in menopausal women give a clue to the diagnosis.
- Raynaud’s phenomenon
- Costovertebral anomalies
- Pancoast tumour.
Cervical Rib Differential Diagnosis Investigations
- X-ray neck may show a cervical rib (Type I, II and III). Interestingly Type IV variety, a fibrous band which cannot be diagnosed by X-ray or by any other investigation, usually gives rise to symptoms.
- Cervical disc protrusion and spinal cord tumours may mimic cervical rib with predominant neurological features.
Cervical Rib Differential Diagnosis Treatment
1. Conservative
- Patients with mild neurological symptoms are managed by shoulder girdle exercises or correction of faulty posture.
2. Surgery
Vascular symptoms and signs are the definite indications for surgery.
- Excision of cervical rib including periosteum: This is called extra-periosteal excision of the cervical rib (so that it will not regenerate). This is included with cervical sympathectomy if vascular symptoms are predominant.
- If there is a thrombus in the subclavian artery, it is explored and thrombus is removed and the artery is repaired.
Cervical Rib Surgery
- Remove cervical rib
- Repair subclavian artery
- Restore circulation
- Reduce vasospasm—sympathectomy
- Recognise other causes
- At exploration, if the cervical rib is not found, divide the scalenus anterior muscle. This is called scalenotomy.
- If hyperabduction syndrome is diagnosed, pectoralis minor is divided from its insertion into the coracoid process.
Axillary Vein Thrombosis
- Patients present with swelling of the arm after intense activity from the dominant hand.
- Hypertrophy of the subclavius muscle also can cause compression of the subclavian—the axillary vein (Sportsman).
- Peripheral pulses will be normal.
- Venography to diagnose thrombus
- Thrombolysis or if necessary venotomy, removal of the thrombus and 1st rib (if it is the cause of obstruction) are the treatment modalities.
If Cervical Rib Is Not Found
- Scalenotomy
- Division of pectoralis minor
- Extraperiosteal resection of the first rib
Vasculitis Syndromes Takayasu’s Arteritis (Pulseless Disease)
- It is of unknown aetiology.
- Commonly affects females (85%)
- It is a panarteritis involving the aortic arch and its branches—the subclavian artery is involved in 85% of the cases.
Vasculitis Syndromes Takayasu’s Arteritis Clinical features
It starts as a generalized inflammatory disease —fever, body ache, malaise, arthralgia, etc.
- Upper limb claudication
- Absence of peripheral pulses.
- Hypertension is common in 50% of the cases due to renal artery involvement.
- Bruit may be heard over the subclavian artery.
- Visual disturbances can occur due to the involvement of retinal arteries. Late blindness can occur.
Vasculitis Syndromes Takayasu’s Arteritis Pathology
It is a panarteritis, involving all layers of elastic arteries—Later thrombosis and stenosis can occur.
Vasculitis Syndromes
- Aetiology is inflammatory or immunological
- Uncommon causes of upper and lower limb ischaemia
- Women are affected more than men
- Multiple small vessel involvement
- Symptoms are confusing—depending upon organ involvement
- Ischaemic changes are minimal and superficial when it involve the limbs
- Steroids are useful in controlling the disease
- Immunosuppression should be tried carefully
Vasculitis Syndromes Investigations
- C-reactive protein is elevated as a part of acute phase response (nonspecific).
- Duplex-Doppler ultrasound and MR angiography can diagnose the site of obstruction and blood flow pattern.
Vasculitis Syndromes Treatment
- Very early cases benefit with tablet prednisolone 30 to 50 mg/day (antiinflammatory effect). Cyclophosphamide can be tried when other measures fail (immunosuppressive effect).
- Vascular reconstruction—is difficult.
Giant Cell Arteritis
- It is also called temporal arteritis
- Elderly women presenting with severe headaches is a common presentation.
- Fever and malaise may also be present.
- Involvement of various arteries will result in various symptoms.
- Palpable, pulsatile, tender temporal arteries will clinch the diagnosis.
- Biopsy of the temporal arteries will reveal giant cell granuloma, comprising mainly CD4 + T lymphocytes.
- Treated by prednisolone 60–80 mg/day slowly tapered over 1–2 years.
- Relapses and remissions are common.
Polyarteritis Syndrome
- This includes microscopic polyarteritis (commonly) and polyarteritis nodosa (less often).
- This syndrome also has an inflammatory reaction.
- Ischaemia of the lower limbs and upper limbs can occur due to the involvement of small vessels.
- Abdominal pain is due to the involvement of visceral vessels.
- Involvement of renal arteries causes loin pain, haematuria and hypertension.
- Treatment is similar to other diseases mentioned above.
Systemic Sclerosis—Scleroderma
- Earlier called collagen vascular disorder because of obstruction of the small vessels by collagen deposition.
- Now included under vasculitis syndromes because of their association with inflammatory reactions.
- Ischaemic changes occur in the fingers and toes—necrosis and ulceration is common.
- Oesophageal involvement results in dysphagia.
- Small bowel sclerosis results in disordered motility and malabsorption.
- Sympathectomy and vasodilators may be useful.
- Raynaud’s symptoms can be controlled by calcium channel blockers and nitrates.
Systemic Sclerosis—Scleroderma Vessel Involved
- Temporal artery
- Facial artery
- Retinal artery
- Upper limb artery
- Coronary artery
Systemic Sclerosis—Scleroderma Symptoms
- Headache
- Jaw pain
- Sudden blindness
- Claudication
- Myocardial infarction
Leave a Reply