Upper Limb Ischaemia And Gangrene Introduction
Upper limb ischaemia (ULI) is a well-recognised clinical entity like lower limb ischaemia, though it is less common. There are certain specific conditions (such as a cervical rib, Raynaud’s disease, etc.) responsible for causing ULI.
Table of Contents
It is important to note that reconstructive surgery is rarely done in the upper limb, as opposed to the lower limb.
Read And Learn More: General Surgery Notes
Upper Limb Ischaemia And Gangrene Causes of Upper Limb Ischaemia
- Raynaud’s disease and Raynaud’s syndrome are vasospastic.
- Embolic causes.
- Thoracic outlet syndrome.
- Trauma.
- Buerger’s disease—vasospastic.
- Axillary vein thrombosis.
- Ergotism: These drugs are used for severe headaches due to migraine.
- Vinyl chloride exposure can give rise to vasospasm (Raynaud’s phenomenon).
- Vasculitis syndromes: Takayasu’s arteritis, giant cell arteritis, polyarteritis nodosa, systemic sclerosis scleroderma—CREST syndrome.
Raynaud’s Disease (Primary Raynaud’s Phenomenon)
- It occurs in young women, commonly.
- The upper limb is more involved than the lower limb.
- It is commonly seen in Western countries in white-skinned people. Cold climate is possibly a precipitating factor. It was 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 (associated disorder present) and primary Raynaud’s disease (no associated disorder).
- CREST syndrome: Calcinosis circumscripta, Raynaud’s phenomenon, (O) Esophageal defects, Sclerodactyly, Telangiectasia.
- Causes of Secondary Raynaud’s Phenomenon.
- Atherosclerosis
- Cervical rib
- Systemic lupus
- Carpal tunnel syndrome
- Scleroderma
- Vibrating tools—vibration white finger
- Currently, both primary and secondary varieties have been grouped together.
Raynaud’s Disease (Primary Raynaud’s Phenomenon) 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 and fill with deoxygenated blood, resulting in a bluish discolouration of the part (cyanosis).
- Stage of recovery or stage of rubor: As the attack passes off, the arterioles relax, circulation improves, and redness occurs. Because of capillary dilatation, there is red engorgement of the part and tingling, burning, or bursting pain in the fingers.
Raynaud’s Disease (Primary Raynaud’s Phenomenon) 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 that occur during the attack, which are accompanied by pain.
- In a few patients, because of recurrent attacks, gangrenous patches occur on the tip of the fingers (superficial necrosis).
Raynaud’s Disease (Primary Raynaud’s Phenomenon) Differential Diagnosis
- Cervical rib
- Vasculitis syndromes
- TAO affecting the upper limb usually affects male smokers. Peripheral pulses are feeble or weak.
Raynaud’s Phenomenon Investigations
- Blood counts, ESR: Increased ESR suggests autoimmune aetiology
- ANA: Antinuclear antibody (ANA) test for antinuclear antibodies
- Nailfold capillaroscopy: This is to analyse microvascular abnormalities in autoimmune rheumatic diseases. It can detect capillaries, haemorrhages, and angiogenesis in cases of scleroderma.
- Thermography: To differentiate primary and secondary Raynaud’s phenomenon. In the case of primary Raynaud’s disease, temperature gradients normalize.
- Wrist–Brachial Index
- Normal—0.85–1.0
- Abnormal <0.85
Raynaud’s Phenomenon Investigations Treatment
1. 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 performed.
2. 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 Horner’s syndrome.
- Indications for Cervical Sympathectomy
- Raynaud’s disease
- TAO
- Hyperhidrosis
- Cervical rib
- Causalgia
- 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
- All rami communicantes associated with the 2nd and 3rd ganglia are removed.
- The nerve of Kuntz, a grey ramus which springs from the 2nd thoracic ganglion to the 1st thoracic nerve, is also divided.
- It is commonly done through a supraclavicular or axillary route. Thoracoscopic sympathectomy is becoming popular.
- Sympathectomy raises the threshold at which spasm occurs (but the effect seems to be temporary). However, the severity of the disease is reduced.
Other Vasospastic Disorders
These are rare.
- Acrocyanosis
- Women with cyanosis of hands and feet
- Cutaneous vasoconstriction is the cause
- Livedo reticularis
- Spasm of arterioles and dilatation of venules
- Worsening by cold
- May be associated with systemic lupus erythematosus (SLE)
- Erythromelalgia
- Burning sensation of hands and feet due to heat
Thoracic Outlet Syndrome
Surgical Anatomy Of The Thoracic Outlet
The thoracic outlet is a tight space with bony structures all around, such as the manubrium sternum anteriorly, the spine posteriorly, and the first rib laterally.
- Introduction to Thoracic Outlet
- Tight space with bones all around
- The brachial plexus and subclavian artery are the chief contents
- The Scalenus antics muscle is the muscle for landmark
- Vascular compression is more dangerous
- Neurological symptoms are often undiagnosed
- At the root of the neck, the brachial plexus and subclavian artery pass through the scalene triangle into the axilla.
- The scalene triangle is the posterior compartment of the costoclavicular space. It is divided into anterior and posterior compartments by the scalenus anticus. The anterior compartment contains the subclavian vein.
Surgical Anatomy Of The Thoracic Outlet Boundaries of a Scalene Triangle
Base: First thoracic rib
Anteromedially: Scalenus antics
Posterolaterally: Scalenus medius
If the base (first thoracic rib) is raised by interposition of the cervical rib or any other cause, it results in compression of the subclavian artery.
- Thoracic Outlet Syndrome: Causes
- Transverse process of C7—long
- Hyperabduction syndrome—compression by pectoralis minor
- Operative scars—fibrous bands
- Rib—cervical rib
- Anomalous first rib—abnormal
- Costoclavicular syndrome—compression between the clavicle and first rib
- Insertion of scalenus—anomalous (Scalenus antics syndrome)
- Callus—malaligned fractured clavicle
- Remember as THORACIC
- The thoracic outlet can be divided into 3 anatomical spaces
- The scalene triangle is the most common site of brachial plexus compression
- cervical rib and anomalous 1st rib compresses the plexus in this location
- Costoclavicular space is the space between the 1st rib and clavicle traversed by all 3 structures (subclavian artery, vein, and brachial plexus)
- It is the most common site of subclavian vein compression
- Pectoralis minor and ribs of the chest wall, an extension of the thoracic outlet area. It is also called axillary compression syndrome.
- Types of thoracic outlet syndrome.
Types of thoracic outlet syndrome
Pathophysiology Of Cervical Rib With Compression
Pathophysiology Of Cervical Rib With Compression Subclavian artery occlusion effects
- 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 poststenotic dilatation (Venturi effect).
- In this dilated segment, multiple small thrombi develop, which, when dislodged, result in emboli and distal ischaemia. Vascular symptoms are strictly unilateral.
Pathophysiology Of Cervical Rib With Compression Cervical Rib
- This is an extra rib present in the neck in about 1–2% of the population.
- It is commonly unilateral but may be bilateral in some cases.
- 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.
Pathophysiology Of Cervical Rib With Compression 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, and 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 it is congenital, symptoms appear only at or after puberty. This is because of the development of the shoulder girdle muscles and sagging of the shoulder, which narrows the root of the neck. Nerve roots C8 and T1 are stretched by around 25 years of age (completion of growth).
- 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 apparent when the arm with muscle wasting is used. 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 or feeble. Oedema and venous distension are very rare. These are called vascular symptoms of the cervical rib.
- Features of ulnar nerve weakness (involvement of the lower nerve roots involvement, mainly T1) manifest as tingling and numbness or paraesthesia in the distribution of C8 and T1.
- The following tests confirm ulnar nerve weakness. It includes sensory disturbances and motor disturbances (performing fine actions—writing, buttoning, etc.,.)
Card test: The patient is asked to hold a thin paper or card between the fingers. In cases of ulnar nerve paralysis, due to weakness of the interossei muscles, the patient will not be able 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.
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 become feeble. This test indicates compression on the subclavian artery.
- 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 the brachial plexus, which gets compressed between the pectoralis minor and its attachment to the coracoid process.
- Military attitude test: When shoulders are set in backward and downward positions, the radial pulse becomes weak. This is due to compression of the subclavian artery between the clavicle and the first rib. This is seen in costoclavicular syndrome.
- Allen’s test: Ask the patient to clench his fist tightly. Compress the radial and ulnar arteries at the wrist with your thumbs. Wait for 10 seconds and ask the patient to open his hands. Pallor can be seen in the palm.
- Now release pressure on the radial artery and watch for blood flow. Repeat the test for the ulnar artery. If there is an occlusion in either artery, colour changes occur slowly in the fingers.
- Elevated Arm Stress Test (EAST): The patient is asked to abduct the shoulders to 90 degrees and flex the elbow. Then he is asked to pronate or supinate the forearms continuously. The appearance of symptoms suggests thoracic outlet syndrome.
- A hard mass may be visible or palpable in the root of the neck (Type 1).
- On palpation of the supraclavicular region, a thrill may be felt, and on auscultation, a bruit may be heard in cases of post-stenotic dilatation.
Pathophysiology Of Cervical Rib With Compression 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 >40 years.
- Cervical disc protrusion and spinal cord tumours may mimic cervical ribs with prominent neurological features.
- Carpal tunnel syndrome may occur due to various causes such as myxoedema, rheumatoid arthritis, and malunited Colles’ fracture. Predominant features of median nerve involvement, more so in menopausal women, give a clue to the diagnosis.
- Raynaud’s phenomenon
- Costovertebral anomalies
- Pancoast tumour
Differential Diagnosis Investigations
- X-ray neck may show a cervical rib (Types 1, 2, and 3). Interestingly, the Type 4 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 have prominent neurological features and, thus, may mimic the cervical rib. When in doubt, ask for an MRI.
- Duplex scan of the affected limb to detect an aneurysm. It may also detect a thrombus.
- CT angiography to define the exact nature of the arterial dilatation and thrombus.
Differential Diagnosis Treatment
1. Conservative
Patients with mild neurological symptoms are managed by shoulder girdle exercises or correction of faulty posture.
2. Surgery
- The presence of vascular symptoms and signs are the definite indications for surgery.
- Excision of the cervical rib including the periosteum is called extraperiosteal excision of the cervical rib (so that it will not regenerate). This is combined with cervical sympathectomy if vascular symptoms are predominant.
- If there is a thrombus in the subclavian artery, it is removed and the artery is repaired.
- At exploration, if a cervical rib is not found, the scalenus anterior muscle is divided. This is called scalenotomy. If hyperabduction syndrome is diagnosed, pectoralis minor is divided at its insertion into the coracoid process.
Differential Diagnosis Cervical Rib Surgery
- Remove cervical rib
- Repair subclavian artery
- Restore circulation
- Reduce vasospasm—sympathectomy
- Recognise other causes
Differential Diagnosis If the Cervical Rib is not Found
- Scalenotomy
- Division of pectoralis minor, scalenus anterior
- Extraperiosteal resection of the first rib
A summary of a cervical rib is given.
Axillary Vein Thrombosis
- Patients present with swelling of the arm after intense activity from the dominant hand
- Hypertrophy of the subclavius muscle may also cause compression of the subclavian-axillary vein (sportsman)
- Peripheral pulses will be normal
- Venography to diagnose thrombus
- Thrombolysis or, if necessary, venotomy and removal of the thrombus and 1st rib (if it is the cause of obstruction) are the treatment modalities.
- Axillary vein thrombosis is also a complication of axillary block dissection, especially where extensive nodal dissection has been done.
Vasculitis Syndromes Takayasus Arteritis
- It is of unknown aetiology.
- Commonly affects females (85%).
- It is a pan arteritis involving the aortic arch and its branches. The subclavian artery is involved in 85% of cases.
Takayasus Arteritis Clinical Features
- It starts as a generalised inflammatory disease—fever, body ache, malaise, and arthralgia.
- Upper limb claudication
- Absence of peripheral pulses
- Hypertension is common (occurs in 50% of cases) due to renal artery involvement.
- A bruit may be heard over the subclavian artery
- Visual disturbances may occur due to the involvement of retinal arteries. Late blindness may 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 they involve the limbs.
- Steroids are useful in controlling the disease.
- Immunosuppression should be tried carefully.
Takayasus Arteritis Pathology
It is a panarteritis, involving all layers of elastic arteries. Thrombosis and stenosis may occur later.
Takayasus Arteritis Investigations
- C-reactive protein is elevated as part of an acute phase response (nonspecific).
- Duplex-Doppler ultrasound and MR angiography may diagnose the site of obstruction and blood flow pattern.
Takayasus Arteritis Treatment
- Very early cases benefit from steroid therapy, for example. tablet prednisolone 30–50 mg/day (anti-inflammatory effect). Cyclophosphamide may be tried when other measures fail (immunosuppressive effect).
- Vascular reconstruction—is difficult.
Giant Cell Arteritis
- It is also called temporal arteritis.
- An elderly woman presenting with severe headache is the common presentation.
- Fever and malaise may also be present.
- Involvement of various arteries results in various symptoms.
- Palpable, pulsatile, tender temporal arteries clinch the diagnosis.
- Biopsy of the temporal arteries reveals giant cell granulomas, comprising mainly CD4+ T lymphocytes.
Giant Cell Arteritis Symptoms and Artery Involved
- Vessel involved
- Temporal artery
- Facial artery
- Retinal artery
- Upper limb artery
- Coronary artery
- Symptoms
- Headache
- Jaw pain
- Sudden blindness
- Claudication
- Myocardial infarction
- Treated with prednisolone 60–80 mg/day, which is slowly tapered over 1–2 years.
- Relapses and remissions are common.
Polyarteritis Syndrome
- This includes microscopic polyarteritis (more common) and polyarteritis nodosa (less common).
- This syndrome also has an inflammatory reaction.
- Ischaemia of the lower limbs and upper limbs may 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 the other diseases mentioned above.
Systemic Sclerosis
- It was earlier called ‘collagen vascular disorder’ because there is obstruction of the small vessels by collagen deposition.
- Now it is included under vasculitis syndromes because of its association with an inflammatory reaction.
- Ischaemic changes occur in the fingers and toes. Necrosis and ulceration are common.
- Oesophageal involvement results in dysphagia.
- Small bowel sclerosis results in disordered motility and malabsorption.
- Sympathectomy and vasodilators may be useful.
- Raynaud’s symptoms may be controlled with calcium channel blockers and nitrates.
Gangrene
Describe the types of gangrene.
Gangrene Definition
Macroscopic death of tissue with superadded putrefaction. It affects the limbs, intestines, appendix, etc. In this chapter, differential diagnoses of gangrene of the limbs are considered.
Gangrene Pregangrene
- Pregangrene Symptoms
- Rest pain is the main symptom
- Pallor on elevation
- Congestion on dependent position
- Guttering of veins
- Poor capillary filling
- Thickening or scaling of skin
Rest pain, colour changes at rest and with exercise, oedema, hyperaesthesia, and skin ulcerations are due to the inadequate blood supply to the limb. These changes are described as gangrenous changes in the limb.
Classification of Gangrene
- Cardiovascular causes
-
- TAO
- Atherosclerotic gangrene
- Acute embolic gangrene
- Syphilitic gangrene
- Raynaud’s disease
- Cervical rib
- Vasculitis syndrome
- Polycythaemia
- Neurological causes: Hemiplegia, paraplegia, bedsore.
- Traumatic gangrene: Direct—thrombosis; indirect crush injuries.
- Physical causes: Sunrays, radiation, corrosive acids.
- Drugs: Ergotamine.
- Diabetic gangrene.
- Acute infective gangrene: Boil, carbuncle, cancrum oris, gas gangrene.
Clinical Features of Gangrene
- Signs of Gangrene
- Loss of pulsation
- Loss of colour
- Loss of temperature
- Loss of sensation
- Loss of function
- A part which is gangrenous is a dead portion of the body. It has no arterial pulsations, venous return, or capillary filling.
- It has no sensation.
- The colour is initially pale and later changes to dusky grey and finally black. The black colour is due to the disintegration of haemoglobin and the formation of iron sulphide.
- The gangrenous part has to be treated with surgical excision or debridement, which may amount to disarticulation of the toe or even amputation.
- Gram-positive, gram-negative, and anaerobic organisms multiply in this segment and may produce septicaemia. Thus, this may precipitate multiorgan failure, including renal failure, adult respiratory distress syndrome (ARDS), cardiac failure, etc.
Gangrene Clinical Types
Comparison of dry gangrene and wet gangrene
Various Types Of Gangrene
Special Types Of Gangrene Cancrum Oris
It is an extensive ulcerative disease of the cheek mucosa occurring in malnourished children.
- Precipitating factors are:
-
- Malnourishment
- Major infectious diseases, such as diphtheria, whooping cough, typhoid, measles, and kala azar.
- As a result of these factors, opportunistic organisms, such as Vincent’s organisms (Borrelia vincentii and B. fusiformis) multiply and cause multiple ulcers, erosions, and later, fibrosis.
- Occasionally, as the disease progresses, the whole thickness of the cheek may be lost.
Treatment of Cancrum Oris
- Ryle’s tube-feeding
- Improve nutrition—IV fluids, parenteral nutrition (in severe cases)
- Appropriate antibiotics: Penicillin + Metronidazole 400 mg three times a day for 7–10 days.
- Reconstructive surgery may be necessary later.
- Chlorhexidine mouthwash
Complications of Cancrum Oris
- Fibrosis causes restriction of jaw movements.
- Septicaemia, toxaemia, and death.
Acrocyanosis
It is also called ‘hereditary cold extremities’.
- It presents as persistent cyanotic discolouration of the hands when exposed to cold due to intermittent spasms of small peripheral vessels.
- It commonly affects the hands and, rarely, the feet.
- Generally, it is mild and nonprogressive.
Drug Abuse And Gangrene
Abuse of drugs is an important cause of gangrene in modern days.
- Inadvertent injection of drugs into an artery may lead to thrombosis of the artery, resulting in acute ischaemia—commonly in the brachial artery.
- Emergency treatment in symptomatic cases includes heparinisation and infusion of dextran.
- In severe cases, emergency angiography and intraarterial thrombolysis are considered.
Gangrene Following Intra Arterial Drug Injection
Inadvertent intra-arterial injection of thiopentone into one of the high divisions of the brachial artery, (congenital anomaly)—usually the ulnar—will result in severe burning and blanching of the hand.
- After the injection, initial signs and symptoms occur very fast (within 15–20 seconds). It consists of intense forearm pain and mottling of the skin over the hand.
- Minutes later, discolouration and nail bed pallor become evident. Approximately 3 to 4 hours later, the symptoms progress to paraesthesias and pronounced hand weakness.
- The rapid development of signs indicative of necrosis (by the eighth day) requires the patient to undergo fasciotomies, multiple debridements, and skin grafts for cosmesis.
- If this complication is noticed, the following steps (measures) have to be taken immediately.
Gangrene Following Intra-Arterial Drug Injection Treating a Case of Inadvertent Intra-arterial Injection
- Step 1: If iatrogenic, maintain the intra-arterial catheter in place—do not remove it.
- Step 2: Identify the progression of the disease—colour changes, necrosis, gangrene.
- Step 3: Initiate anticoagulation—diluted heparin, intravenous and subcutaneous.
- Step 4: Institute symptomatic relief and plan for rehabilitation—analgesics, brachial plexus block (analgesia and vasodilatation) physiotherapy.
- Step 5: Elevation of extremity, antibiotics, antiplatelet anticoagulant therapy.
- Step 6: Perform specific interventions—angiogram, intra-arterial thrombolysis, vasodilators, prostacyclins, sympathectomy, corticosteroids.
- Step 7: Aim is to save the limb—the last resort is amputation.
Miscellaneous
Miscellaneous Ergot and Gangrene
- Ergot preparations are used over a long period in patients with migraine.
- Ergotamine gangrene occurs in those who eat bread infected with Claviceps purpurea. Example: Dwellers on the shores of the Mediterranean Sea and the Russian Steppes.
Subclavian Steal Syndrome
- It refers to the development of symptoms in either the brain or in the arm due to subclavian artery obstruction.
- Due to the obstruction, blood supply to the arm is provided by the collaterals which develop due to reversed blood flow from the basilar artery. The basilar artery is formed at the base of the skull by the joining of 2 vertebral arteries.
- The basilar artery supplies blood to the cerebellum, brain stem, and occipital lobes. Thus, symptoms may be vertebrobasilar or may occur in the arm.
- Subclavian Steal Syndrome
- It is more common on the left side
- Vertebrobasilar symptoms—dizziness, vertigo, imbalance
- Arm symptoms—fatigue, pain with exercise, paraesthesias, coolness and heaviness on the affected side
- Asymmetrical radial pulses or difference of 20 mmHg systolic pressure between the upper limbs.
- Javid test: Pressure on the common carotid artery produces a weak radial pulse on the ipsilateral side.
- Surgical correction is by endarterectomy, a bypass graft, or transluminal balloon angioplasty is another alternative.
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