62 Venous disorders

AXILLARY VEIN THROMBOSIS
Aetiology
CLINICAL FEATURES OF VENOUS HYPERTENSION OF THE LE
CONGENITAL VENOUS ANOMALIES
Classiﬁcation system
Clinical features
Cystic degeneration
Diagnosis
Epidemiology
FURTHER READING
Introduction
Investigation
Klippel–Trénaunay syndrome
Learning objectives
Leiomyoma and leiomyosarcoma of the vein wall
Management
PEL VIC CONGESTION SYNDROME
Pathology
Pathophysiology of ulceration
Prevention of recurrence
Prognosis
Prophylaxis
Signs
Symptoms
THE ANATOMY OF THE VENOUS SYSTEM OF THE LOWER LIMB
Treatment
VENOUS ENTRAPMENT SYNDROMES
VENOUS INJURY
VENOUS LEG ULCER
VENOUS PATHOPHYSIOLOGY
VENOUS THROMBOEMBOLISM
VENOUS TUMOURS Venous malformation cavernous angio

AXILLARY VEIN THROMBOSIS

Thrombosis of the axillary vein (Paget–Schrotter disease) may occur following excessive exercise in a patient with an anatomically abnormal thoracic outlet, but is also associated - with excessive muscle bulk as found in weight lifters. The vein may be compressed by a cervical rib if this is present early stage, the thrombus can be disrupted by thrombolysis delivered through one of the arm veins. The vein must then be imaged to see if there is any compression on elevation of the arm. If this is conﬁrmed, thoracic outlet decompression can be carried out by resecting the cervical rib or ﬁrst rib.

Aetiology

The three factors described by Virchow over a century ago are still relevant in the development of venous thrombosis. These are: /uni25CF contact of blood with an abnormal surface (e.g. endothelial damage); /uni25CF abnormal ﬂow (e.g. stasis); /uni25CF abnormal blood (e.g. thrombophilia). - There are many predisposing causes for VTE. These are listed in Table 62.2 . The most important factor is a hospital admission for treatment of a medical or surgical condition. Injury , especially fractures of the lower limb and pelvis, pregnancy and the oral contraceptive pill are other well - recognised predisposing factors. Endothelial damage is now known to be critically important. The interaction of the endothelium with inﬂammatory cells, or previous deep vein damage, renders the endothelial surface hypercoagulable and less ﬁbrinolytic. Stasis is a predisposing factor seen in man y of the conditions described in Table 62.2 , especially in the postoperative period, in patients with heart failure and in those with arterial ischaemia. - ) and

(a) diagnostic venogram; (b) therapeutic Figure 62.30 Post-thrombotic leg demonstrating features of eczema, pigmentation and mild lipodermatosclerosis.

/uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF A number of conditions are associated with increased coagulability of the blood (thrombophilia) ( Table 62.3 Deﬁciencies of antithrombin, activated protein C and protein S have all been shown to predispose to venous thrombosis in young patients. Activated protein C deﬁciency is associated with inheritance of the factor V Leiden gene and may account for the higher incidence of venous thrombosis in white populations (being present in 6–7%). It results in a small increase in the risk of VTE, although it may act in concert with some of the other predisposing factors. A thrombophilia should be excluded in any patient presenting with an episode of VTE who gives a family history of VTE or in whom there is no other predisposing factor. Although the development of DVT is probably multifac torial, immobility (and hence stasis) remains one of the most important factors. DVT is recognised as a complication of long-haul ﬂights and other forms of travel.

Patient factors Age Obesity Varicose veins Immobility Pregnancy Puerperium High-dose oestrogen therapy Previous deep vein thrombosis or pulmonary embolism Thrombophilia (see Table 62.3 ) Disease or surgical procedure Trauma or surgery, especially of pelvis, hip and lower limb Malignancy, especially pelvic, and abdominal metastatic Heart failure Recent myocardial infarction Paralysis of lower limb(s) Infection In /f_l ammatory bowel disease Nephrotic syndrome Polycythaemia Paraproteinaemia Paroxysmal nocturnal haemoglobinuria antibody or lupus anticoagulant Behçet’s disease Homocystinaemia

CLINICAL FEATURES OF VENOUS HYPERTENSION OF THE LE

CLINICAL FEATURES OF VENOUS HYPERTENSION OF THE LEG

The following clinical features are commonly seen: /uni25CF Varicose vein: subcutaneous dilated vein 3 /uni00A0 mm in diameter or larger. They are frequently elongated and tortuous, with intermittent ‘blowouts’, but are deﬁned by the presence of reﬂux and may be straight and uniform tubes morpho logically ( Figure 62.3 ). /uni25CF Telangiectasia (thread veins, spider veins and hyphen webs): represent tiny intradermal venules less than 1 /uni00A0 mm in diameter ( Figure 62.4 ). Maurice Klippel , 1858–1942, neurologist, La Salpêtrière, Paris, France. Paul Trénaunay , 1875–1938, French neurologist. Klippel and Trénaunay described this condition in a joint paper in 1900. A gaiter is a leather or cloth covering for the lower leg and ankle. The name is derived from the French ‘guetre’ for the same piece of clothing. /uni25CF Reticular vein: small dilated ‘bluish’ subdermal vein 1–2.9 /uni00A0 mm in diameter, usually tortuous, can be di ﬃ cult to distinguish this from a normal subdermal vein in someone with white thin transparent skin. /uni25CF Saphena varix ( Figure 62.5 ) is a (usually painless) groin swelling apparent on standing. /uni25CF Corona phlebectatica (malleolar ﬂare): a fan-shaped pat - tern of telangiectasia on the ankle or foot. This is an early , sign of advanced venous disease. /uni25CF Oedema: increased volume of ﬂuid in the skin and soft tis - sues of the leg. Commonly starts distally and moves more proximally with increasing venous dysfunction. Classically this is ‘pitting oedema’, with ﬁrm digital pressure leaving an indentation in the soft tissues. /uni25CF Eczema: an erythematous dermatitis, often appears minor, although it may be associated with signiﬁcant itching and discomfort. In extreme cases it may progress to blistering and weeping ( Figures 62.6–62.8 ). /uni25CF Pigmentation (haemosiderosis): a brownish discoloration of the skin, usually permanent. It is usually seen around the ankle, but is also seen in proximity to varicose veins and - incompetent perforators ( Figures 62.7 and 62.9 ). /uni25CF Lipodermatosclerosis (LDS): chronic inﬂammation and ﬁbrosis of the skin and subcutaneous tissues, resulting in a tight, contracted, ‘woody’ leg on examination. It occa - sionally results in signiﬁcant contractures of the Achilles

tendon. This is a sign of severe chronic venous disease ( Figures 62.6 and 62.9 ). /uni25CF Atrophie blanche: localised areas of atrophic, white skin, often surrounded by telangiectasia and pigmentation. Some authors distinguish this from the white scarring left by ulceration; others do not. Either way , this is a sign of severe chronic venous disease ( Figure 62.6 ). /uni25CF V enous ulcer: full-thickness skin loss, usually around the ankle, which fails to heal spontaneously and is propagated by continuing venous hypertension and the changes associ ated with chronic venous disease ( Figure 62.10 ).

Figure 62.4 Telangiectasia and reticular veins. Figure 62.5 A saphena varix.

CONGENITAL VENOUS ANOMALIES

There are four main types of anomaly: /uni25CF aplasia; /uni25CF hypoplasia; /uni25CF duplication; /uni25CF persistence of vestigial vessels. Aplasia is most commonly seen in the inferior vena cava and has a similar presentation to the post-thrombotic limb. Membranous occlusion of the left common iliac vein (May– Thurner syndrome) often develops where the v ein passes behind the right common iliac artery (iliac vein compression syndrome). This leads to an iliac vein thrombosis, which most commonly a ﬀ ects the left common and external iliac veins. Membranes may also narrow the hepatic veins, which can become totally occluded, leading to a Budd–Chiari syndrome. Hypoplasia results in a narrow vein, which frequently o ﬀ ers little signiﬁcant venous function and amounts to a functional venous occlusion, being circumvented by enlarged collateral venous tributaries. Duplications are quite common, with dou b le vena cava, femoral and renal veins; they often present as an incidental ﬁnding.

Classiﬁcation system

The descriptive CEAP (Clinical–aEtiology–Anatomy–Patho physiology) classiﬁcation for chronic venous disorders is widely utilised. - -

Figure 62.6 Advanced skin changes: lipodermatosclerosis, eczema and atrophie blanche. Figure 62.7 Pigmentation (haemosiderosis) and mild eczema. Figure 62.8 Severe eczema. Figure 62.9 Haemosiderosis and mild lipodermatosclerosis of the calf skin.

For clinical classiﬁcation: /uni25CF C0: no signs of venous disease; /uni25CF C1: telangiectasia or reticular veins; /uni25CF C2: varicose veins; /uni25CF C3: oedema; /uni25CF C4a: pigmentation or eczema; /uni25CF C4b: LDS or atrophie blanche; /uni25CF C4c Corona phlebectatica /uni25CF C5: healed venous ulcer; /uni25CF C6: active venous ulcer. Clinical class can be further characterised as symptomatic (s), asymptomatic (a) or recurrent following previous successful treatment or healing (r), e.g. C2a, C2s, C6r. For aetiological classiﬁcation: /uni25CF Ec: congenital; /uni25CF Ep: primary; /uni25CF Es: secondary (post-thrombotic); /uni25CF En: no venous cause identiﬁed. For anatomical classiﬁcation: /uni25CF As: superﬁcial veins; /uni25CF Ap: perforator veins; /uni25CF Ad: deep veins; /uni25CF An: no venous location identiﬁed. For pathophysiological classiﬁcation: /uni25CF Pr: reﬂux; /uni25CF Po: obstruction; /uni25CF Pr,o: reﬂux and obstruction; /uni25CF Pn: no venous pathophysiology identiﬁable. In clinical practice, patients are normally categorised as having ‘varicose veins’ or ‘venous ulcers’. Cases of varicose veins may be uncomplicated or complicated. Complications may be chronic (as discussed above) or acute, including superﬁcial vein thrombosis (thrombophlebitis) and bleeding. Uncomplicated varicose veins may be asymptomatic or symptomatic.

Figure 62.10 Venous ulcer.

Clinical features

The ulcer must be carefully examined. A venous ulcer usually has a gently sloping edge and the ﬂoor contains granulation tissue covered by a variable amount of slough and exudate. Any signiﬁcant elevation of the ulcer edge should indicate the need for a biopsy to exclude a carcinoma (usually a squamous cell). V enous leg ulcers characteristically develop in the skin of the gaiter region, the area between the muscles of the calf and the ankle. This is the region where many of the Cockett perfo rator s join the posterior tibial vein to the surface vein, known as the posterior arch vein. The majority of ulcers develop on the medial side of the calf, but may develop anywhere in the gaiter area. Extension onto the foot or into the upper calf is uncommon and, if there is ulceration at these sites, other diag noses should be seriously considered. Ulcers often develop in response to minor trauma; many patients notice some itch ing, perhaps associated with mast cell degranulation, before the ulcers develop . Almost all venous ulcers have surr ounding haemosiderosis (seen as pigmentation) and the more chronic ulcers develop LDS with associated ﬁbrosis of the subcuta neous tissue ( Figure 62.10 ). This is manifest as thickening, pigmentation, inﬂammation and induration of the calf skin. The pigmentation comes from haemosiderin and melanin and the haemosiderin itself may be an important factor in ulcer development. A full examination of the front and back of the limbs with the patient standing should be carried out to assess the presence of varicosities and truncal incompetence of the saphenous systems (note that venous ulcers are not always accompanied by varicose veins). All patients should hav e their pulses palpated and, if there is any doubt, their arterial Doppler pressures should be measured. Sensation and proprioception should be assessed to exclude neuropathy , especially in patients with diabetes. A careful examination of the hand and other joints may conﬁrm the presence of rheumatoid arthritis or osteoarthritis. Frank Bernard Cockett , 1916–2014, surgeon, St Thomas’s Hospital, London, UK. Jean-Nicholas Marjolin , 1780–1850, surgeon, Paris, France, described the development of malignant ulcers in scars in 1828. Most vascular surgeons will carry out a duplex scan when the patient with an ulcer is ﬁrst seen to assess the status of the deep and superﬁcial veins. The presence of reﬂux in these veins does not conﬁrm a venous ulcer, but supports the diagnosis in the absence of another cause and helps direct treatment. V enous ulcers are characteristically di ﬃ cult to heal; how - ever, persistence may indicate that there is another or coex - isting cause (e.g. malignancy , rheumatoid arthritis or arterial ischaemia). Biopsies are indicated if malignancy is suspected and it is important to remember that a Marjolin’s type of ulcer (a squamous cell or basal cell carcinoma) can develop in a chronic longstanding venous ulcer ( Figure 62.28 ). Patients with atypical or with ulcers not responding to treatment should have a full blood count, blood glucose, erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) assessment as well as a sickle cell test if the y have an appropriate ethnic background. Anaemia can both cause ulcers (e.g. sickle cell disease and pernicious anaemia) and be a result of ulceration (e.g. iron deﬁciency anaemia and the anaemia of chronic disease). Polycythaemia is a rare cause of ulceration. An antibody screen should be obtained if the ulcer appears ‘atypical’ or if there is any suggestion of joint disease (e.g. rheumatoid arthritis). All patients presenting with a new ulcer should have their Doppler pressures measured unless the - foot pulses are easily palpable and have been conﬁrmed as such by a vascular specialist.

Cystic degeneration

As in the peripheral arterial system, cystic degeneration of the vein wall is an uncommon cause of venous occlusion. It may be detected by ultrasound. The cyst may be deroofed or the venous segment excised. -

Diagnosis

The most common presentation of a DVT is pain and swelling, especially in the calf, usually in one leg; however, bilateral DVTs are common, occurring in up to 30%. When the swelling ) . - -

Figure 62.31 An organised thrombus.

is bilateral, DVT must be di ﬀ erentiated from other causes of systemic oedema, such as hypoproteinaemia, renal failure and heart failure. Some patients have no symptoms of thrombosis and may ﬁrst present with signs of a pulmonary embolus, e.g. pleuritic chest pain, haemoptysis and shortness of breath. Patients may also develop shortness of breath from chronic pulmonary hypertension. Sometimes the leg appears cellulitic and very occasionally it may be white or cyanosed: phlegmasia alba dolens and phlegmasia cerulea dolens ( Figure 62.32 indicates venous pressures that are so high they are impeding tissue perfusion. Patients who present with venous gangrene ( Figure 62.33 ) often have an underlying neoplasm. Clinical examination for DVT is unreliable. Physical signs may also be absent. Mild pitting oedema of the ankle, dilated surface veins, a sti ﬀ calf and tenderness over the course of the deep veins should be sought. Leg pain occurs in about 50% of patients with DVT but is non-speciﬁc. Homans’ sign – resistance John Homans , 1877–1957, Professor of Clinical Surgery , Harvard University Medical School, Boston, MA, USA, a founding member of the Society f Surgery . Philip Wells , contemporary , physician, University of Ottawa, Ottawa, ON, Canada. speciﬁc and should not be elicited. Tenderness occurs in 75% of patients but is also found in 50% of patients without objectively conﬁrmed DVT . The pain and tenderness associa ted with DVT does not usually correlate with the size, location or extent of the thrombus. Clinical signs and symptoms of pulmonary embolus occur in about 10% of patients with conﬁrmed DVT . A low-grade pyrexia may be present, especially in a patient who is having repeated pulmonary embolus. Patients may have signs of cyanosis, dyspnoea, raised neck veins , a ﬁxed split sec - ond heart sound and a pleural rub if they have pulmonary emboli causing right heart strain, although these signs may be subtle or absent.

Figure 62.32 Phlegmasia cerulea dolens. Figure 62.33 A foot with venous gangrene. The gangrene is symmet rical, involving all of the toes. There is no clear-cut edge and there is marked oedema of the foot.

Epidemiology

The adult prevalence of visible varicose veins is between 30% and 50%. Factors a ﬀ ecting prevalence include: /uni25CF Gender: the vast majority of studies report a higher preva - lence in women than in men, though community preva - lence may di ﬀ er. /uni25CF Age: the prevalence of varicose veins increases with age. In the Edinburgh V ein Study , the prevalence of trunk vari - cosities in the age groups 18–24 years, 25–34 years, 35–44 years, 45–57 years and 55–64 years was 11.5%, 14.6%, 28.8%, 41.9% and 55.7%, respectively . /uni25CF Ethnicity: does seem to inﬂuence the prevalence of vari - cose veins. /uni25CF Body mass and height: increasing body mass index and height may be associated with a higher prevalence of vari - cose veins. /uni25CF Pregnancy: increases the risk of varicose veins. /uni25CF Family history: evidence supports familial susceptibility to varicose veins. /uni25CF Occupation and lifestyle factors: there is inconclusive evi - dence regarding increased prevalence of varicose veins in smokers, in patients who su ﬀ er constipation and in those with occupations that involve prolonged standing.

Introduction

INTRODUCTION

Up to 40% of the adult population in resource-rich countries have diseases of the veins of the leg. This extraordinary prevalence along with the associated impairment in health- related quality of life make it a very important area of surgical practice. Surgical intervention has been revolutionised by /uni00A0 the development of endovenous techniques, and level 1 evidence has demonstrated that treatment can be associated with very high clinical- and cost-e ﬀ ectiveness. Despite the considerable importance placed on lower limb function during the manage ment of orthopaedic and arterial diseases, venous diseases are often forgotten or dismissed as cosmetic practice. An under standing of the nature and management of venous disease is critical to address this imbalance and improve the quality of patients’ lives.

Investigation

Tourniquet tests and the use of hand-held Doppler have now been abandoned. There is good evidence to support the policy of duplex ultrasound scanning for all patients with varicose veins prior to any intervention. The best clinical results come from clinicians who are personally very skilled in the use of duplex ultrasound and use it to design a bespoke treatment for each individual patient, based upon their unique anatomy . A high-frequency linear array transducer of 7.5–13 /uni00A0 MHz is appropriate for the majority of lower limbs in order to obtain good quality images. The B-mode settings (depth, focal zone, overall gain and dynamic g ain) should be optimised to ensure that the area of interest is in the centre and occupies the major ity of the image, and that the lumen of the vein appears as a dark void in the subcutaneous and deep tissues. The pulsed wave spectral or colour Doppler settings should be optimised for the low-ﬂow velocities encountered within veins. It is conventional to use blue to represent antegrade venous ﬂow towards the heart and red for the reverse. Visible venous ﬂow can be augmented by a calf squeeze. The aim of the duplex scan in a patient with varicose veins is to establish: /uni25CF the presence of reﬂux in the deep and superﬁcial venous system; /uni25CF the exact distribution and extent of reﬂux in the superﬁcial venous system, including a ﬀ ected junctions and perfora tors; /uni25CF the presence of obstruction in the deep venous system; /uni25CF the suitability of the incompetent superﬁcial veins for the di ﬀ erent treatments available (based upon diameter, extent, tortuosity , saphena varix); Christian Johann Doppler , 1803–1853, Professor of Experimental Physics, Vienna, Austria, enunciated the ‘Doppler principle’ in 1842. Antonio Valsalva , 1666–1723, anatomist, Bologna, Italy . Also described the Eustachian tube and aortic sinuses. /uni25CF - an indication of a pelvic source of reﬂux or obstruction. In order to standardise measurements of venous diameter and reﬂux, it is recommended that examination of the super - ﬁcial veins is performed with the patient standing. Superﬁcial or crural vein reﬂux is deﬁned as retrograde ﬂow in the r everse direction to physiological ﬂow lasting for 0.5 seconds or more. The pr oximal deep veins require a duration of 1 second or more to be classiﬁed as incompetent. Reﬂux may be elicited by release of a calf or foot squeeze for proximal or calf vari - cosities, respectively , manual compression over varicosity clus - ters, pneumatic calf cu ﬀ deﬂation, active foot dorsiﬂexion and relaxation or the Valsalva manoeuvre. The patient should stand facing towards the examiner with the leg rotated outwards, heel on the ground and w eight on the opposite limb ( Figure 62.11 ). The use of a platform, ideally - with a handle or support bar for the patient and a stool tha t can drop to a low height, will improve the ergonomic com - fort of both the sonographer and the patient. The scan should commence in the groin, using a transverse view to identify the GSV and CFV lying medial to the common femoral artery (the ‘Mickey Mouse’ sign; Figure 62.12 ). SFJ competence is assessed in the transverse view and potential destinations for reﬂux, including the GSV , the AAGSV and other major thigh tributaries superﬁcial to the saphenous fascia, are noted. Any indication of a pelvic source of reﬂux suggests the need for more proximal imaging. The full length of the GSV within its fascial compartment should be examined ( Figure 62.13 ), and its diameter measured if required. The groin is next examined - -

Figure 62.11 Patient position for venous duplex examination of the great saphenous system.

for reﬂux or obstruction in the CFV , superﬁcial femoral vein and SFJ using spectral and/or colour Doppler ( Figure 62.14 A loss of phasic ﬂow with respiration in the CFV suggests upstream obstruction and the need for proximal imaging. The presence and competence of thigh and calf perforators should be noted and the crural veins examined for reﬂux or obstruc tion. F or examination of the SSV and posterior thigh extension of the SSV (Giacomini vein), the patient is positioned facing away , knee slightly ﬂexed, heel on the ground and the weight taken on the opposite leg. If the SPJ is incompetent, the level of the SPJ in relation to the knee crease and whether the SSV joins the popliteal vein posteriorly , medially or laterally is noted if open surgical ligation is to be entertained. In the transverse view , the SSV vein is followed distally , checking its competence and diameter in the proximal, mid- and distal calf. Finally , the patency and competence of the popliteal vein is assessed. Pelvic and iliac veins may be investigated using transabdominal or transvaginal duplex. V ery occasionally investigations other than duplex are required, and these may be non-invasive, suc h as magnetic resonance (MR) venography , or invasive, such as contrast venography or intravenous ultrasound (IVUS). The use of varicography has become ). historical ( Figure 62.15 ).

Figure 62.12 ‘Mickey Mouse’ transverse B-mode image of the right (R) common femoral vein (CFV) and artery (CFA), great saphenous vein (GSV) and saphenofemoral junction. Figure 62.13 ‘Saphenous eye’ transverse B-mode view of the great saphenous vein in fascial compartments of the thigh. The fascial line above the vein is the saphenous fascia. A true great or small saphe nous vein will not cross this line, although the fascia may become discontinuous around the knee. The line deep to the vein is the fascia lata, with the muscle beneath. Figure 62.14 Spectral Doppler trace of the saphenofemoral junction showing antegrade and retrograde /f_l ow. The downward spike on the trace is the antegrade augmented /f_l ow and this is followed by approximately 4 seconds of retrograde /f_l ow.

Figure 62.15 Varicogram. (This is now a historical investigation.)

Investigation

The diagnosis of DVT and pulmonary embolus should be established by special investigations as the symptoms and signs are non-speciﬁc and may be absent. In addition, treatment with anticoagulation is not without risk and the diagnosis must be made with reasonable certainty . Many centres direct investigations based upon the modi - ﬁed Wells score ( Table 62.4 ) , with further imaging dictated by these results. These scores can be unreliable though, especially in hospital inpatients, and should only be considered a guide. V enous duplex ultrasound is commonly performed to look for evidence of thrombosis throughout the deep or superﬁcial venous system. Ideally , this should be performed by an expe - rienced vascular sonographer, but the v olume of cases is such that compression ultrasound is frequently being performed by non-specialists. Compression ultrasound involves applying pressure with the ultrasound probe over the common femoral ). This or Vascular

TABLE 62.4 Modi /f_i ed Wells criteria for predicting deep

vein thrombosis (DVT). Variable Score Lower limb trauma or surgery or immobilisation in a 1 plaster cast Bedridden for >3 days or surgery in last 4 weeks 1 Tenderness along the line of femoral or popliteal veins 1 Entire limb swollen 1 Calf >3 /uni00A0 cm larger circumference than other side 10 /uni00A0 cm 1 below tibial tuberosity Pitting oedema 1 Dilated collateral super /f_i cial veins (not varicose veins) 1 Previous DVT 1 Malignancy (including treatment up to 6 months ago) 1 Intravenous drug abuse 3 Alternative diagnosis more likely than DVT –2 Low probability (5%) of DVT (score –2 to 0); moderate probability (17%) of DVT (score 1 or 2); high probability (17–53%) of DVT (score

2).

will compress tightly shut. In the presence of DVT they will not fully compress. It is rapid to both learn and perform, but not ideal and most importantly misses calf vein thrombosis. Calf vein thromboses may propagate to form a more extensive thrombus, which may in turn embolise. The optimal manage ment of calf vein thrombosis when detected is not clear; some units use surveillance, with others anticoagulating such patients upon detection. Ascending venography , which shows a thrombus as a ﬁll ing defect, is now rarely r equired unless thrombolysis is being considered ( Figure 62.34 ). MR venography may also be used. Pulmonary embolus is diagnosed deﬁnitively by computed tomography (CT) pulmonary angiogram, which will demon stra te the presence of ﬁlling defects in the pulmonary arter ies ( Figure 62.35 ). Pulmonary angiography is rarely required unless thrombolysis is being considered. The di ﬀ erential diagnosis of a DVT includes a ruptured Baker’s cyst, a calf muscle haematoma, a ruptured plantaris muscle, a thrombosed popliteal aneurysm and arterial isch aemia. Duplex scanning will detect many of these conditions but often patients present with non-speciﬁc pain in the calf that resolves with no ﬁrm diagnosis being made. The di ﬀ eren tial diagnosis of a pulmonary embolism includes m yocardial infarction, pleurisy , pneumonia and aortic dissection.

Klippel–Trénaunay syndrome

This is a combined anomaly of a cutaneous naevus, persistent vestigial veins with varicose veins and soft-tissue and bone hypertrophy . The condition is a mesodermal abnormality that is not familial ( Figure 62.36 ). Segments of the deep veins are hypoplastic or aplastic and there may be an associated obstruction of the lymphatics. The condition must be distinguished from the Parkes-Weber syndrome, in which there ar e multiple arteriovenous ﬁstulae causing venous hypertension, ulceration and high-output cardiac failure. Virtually all patients with Klippel–Trénaunay syndrome should be treated conservatively with compression hosiery; however , some will beneﬁt from laser ablation of the naevus, stapling of the bones to avoid leg length discrepancy and occa sional removal of large superﬁcial varicose veins, provided the George Budd , 1808–1882, Professor of Medicine, King’s College Hospital, London, UK, described this syndrome in 1845. Hans Chiari , 1851–1916, Professor of Pathological Anatomy , Strasbourg, Germany (Strasbourg was returned to France after the end of the First World War, in 1918), gave his account of this condition in 1898. Frederick Parkes-Weber , 1863–1962, physician, The German Hospital, Dalston, London, UK. Sir James Paget , 1814–1899, English surgeon and pathologist, best known for his description of Paget’s disease of the bone. Leopold von Schrotter , 1837–1908, Austrian physician and laryngologist, Chair of Laryngology , University of Vienna, Vienna, Austria. deep veins are patent. LMWH should be given to all patients having surgery as this syndrome is associated with an increased risk of VTE. -

Figure 62.36 Two patients with Klippel–Trénaunay syndrome. (a) This patient has a longer leg and a capillary naevus; (b) this patient has a large lateral anomalous axial vein.

Learning objectives

To understand: Venous anatomy and physiology • The pathophysiology of venous hypertension • The clinical signi /f_i cance and management of super /f_i cial • venous re /f_l ux

Leiomyoma and leiomyosarcoma of the vein wall

These are extremely rare tumours that are usually slow growing. They present with pain and a mass with signs of venous obstruction, e.g. oedema and distended veins. Duplex scanning, CT ( Figure 62.41 ) and magnetic resonance imaging (MRI) show a ﬁlling defect within the vein wall. Treatment is by resection with replacement by autogenous vein taken from another site. Rarely , a PTFE graft is required. When the tumour a ﬀ ects the vena cava it must be resected and replaced with a prosthetic graft.

Management

Many patients with asymptomatic varicose veins do not progress - to develop complications, although a signiﬁcant proportion do, and there is no clear conﬁrmatory evidence that treating such patients prevents the development of future complications. There is clear evidence, however, that those with symptoms and/or complications see a signiﬁcant quality-of-life beneﬁt from treatment to remove or ablate reﬂuxing superﬁcial veins. When interventional treatment is planned there are con - siderable variations in practice and treatment strategies. A detailed description of the nuances, merits and criticisms of the various options is beyond the scope of this chapter; how - ever, a description of the basic treatment modalities available is presented below . An experienced surgeon will have his/her own preferred methods, but will frequently employ several or all methods in chosen circumstances, not infrequently in the same patient. Compression hosiery relies on graduated external pressure to improve deep venous return and reduce venous pressures. It may be knee length or thigh length; there is no evidence which length of stocking is more e ﬀ ective and hence below-knee stockings are usually prescribed as they are easier to don and have much better patient acceptance. Compression hosiery is classiﬁed according to the pressure it exerts: the British classiﬁ cation class 1 stockings exert pressure of 14–17 /uni00A0 mmHg, class 2 exert 18–24 /uni00A0 mmHg and class 3 exert 25–35 /uni00A0 mmHg. Compression hosiery signiﬁcantly improves varicose vein symptoms but is not popular with patients, with compliance rates and long-term tolerance being univ ersally poor. There is no evidence to suggest that compression hosiery prev the occurrence or progression of varicose veins. Further more, incorrect application of compression hosiery can have serious consequences (pressure necrosis, tourniquet e ﬀ ects); thus assessment, prescription and application of compression hosiery should be limited to those with the appropriate skills and training. Ther e are level 1 trial data to demonstrate that interventional treatment o ﬀ ers superior improvements in qual ity of life and is cost-e ﬀ ective. Compression is therefore to be regarded as an adjunct to assessment or treatment, unless by patient choice. Endothermal ablation Endothermal ablation technologies replaced surgical ligation and stripping as the gold standard treatment once randomised trials demonstrated that they were marginally safer, have extremely high technical e ﬃ cacy , o ﬀ er superior quality of life post procedure (with a rapid recovery) and equivalent improve ments in quality of life in the longer term. The techniques are cost-e ﬀ ective as they can be performed as an outpatient under local anaesthetic. The basic concept is that a treatment device is inserted into the incompetent axial vein percutaneously . The vein is surrounded by tumescent local anaesthetic solution. This compresses the vein onto the treatment device, emptying it of blood. It also hydro-dissects tissues such as nerves away from the zone of injury . Finally , it acts as a heat sink, mopping up excess thermal energy to prevent remote damage. The treat ment device then produces thermal energy that destroys the structure of the vein, resulting in permanent occlusion. Two broad technologies exist: laser ablation and radiofrequency ablation (RFA). Laser ablation Endovenous laser ablation (EVLA) utilises a small ﬂexible glass ﬁbre that is inserted into the vein. Laser energy (typically at a wavelength of 1470 /uni00A0 nm) is transmitted down the ﬁbre and is absorbed at the point of treatment at the end of the ﬁbre. Absorption of this radiation results in a vigorous generation of thermal energy . The tip of the ﬁbre may be bare, focusing the energy in a very small area; divergent forward ﬁring, spreading the energy over a larger area; or divergent side or radial ﬁring. It is postulated that the last two designs allow a more even distribution of energy , reducing vein wall perforations that Friedrich Trendelenburg , 1844–1924, Professor of Surgery successively at Rostock (1875–1882), Bonn (1882–1895) and Leipzig (1895–1911), Germany . The Trendelenburg position was ﬁrst described in 1885. bruising. There is no clear evidence to support one design over another. This procedure is very good for treatment of any v ein that will allow the passage of a guidewire. No technique has reported a higher technical e ﬃ cacy rate. The procedure begins with ultrasound-guided marking of the truncal vein to be treated and the site of proposed cannulation. T he varicosities are also marked at this stage if - concomitant treatment (phlebectomy or foam sclerotherapy) is to be undertaken. The patient is then positioned on the procedure couch in the reverse Trendelenburg position. For the GSV , the patient is supine with the hip of the leg to be treated externally rotated and slightly ﬂexed. A pillow under the contralateral hip/lower back may improve patient comfort. ents For the SSV the patient is positioned in the prone position. - The vein is then cannulated percutaneously under ultrasound guidance, at the lowest point of reﬂux. Some devices allow passage of the ﬁbre directly through a short sheath, while others use a wire ﬁrst, allowing passage of a catheter that then carries the laser ﬁbre. The former is slightly faster with fewer steps; the latter allows greater success with more tortuous - veins. Accurate positioning of the ﬁbre tip with ultrasound is crucial ( Figure 62.16 ), but the exact location is controversial, with some surgeons positioning the tip several centimetres distal to the junction and others aiming for a ﬂush occlusion. Proponents of the former cite that this strategy protects the deep vein from inadvertent damage and/or thrombosis. Proponents of the latter argue that neoreﬂux in junctional tributaries is a common pattern of recurrence and that in expert hands the rate of deep vein injury is no di ﬀ erent and the thrombosis rate may be lower (presumably as there is minimal patent stump - in which to form thrombus). Following the administration of perivenous tumescent anaesthesia ( Figure 62.17 ), the ablation can be performed. Practice varies as to the power of the laser and the withdrawal speed, but commonly an energy delivery -

Deep vein Figure 62.16 Endovenous laser ablation; B-mode longitudinal imaging during catheter tip positioning at the saphenofemoral junction. The saphenofemoral junction is highlighted (in blue) with an arrow pointing to the catheter.

There is no clear evidence to guide the optimal power and pullback speed. Compression is usually applied following treatment, but /uni00A0 there is no consensus over the method, degree or duration, and this is true of postprocedural compression with all techniques. Radiofrequency ablation RFA uses the same treatment principles, but an electromag netic current is used to create the thermal energy . A range of di ﬀ erent devices have been created but the most popular, which has the most supportive evidence, is the ClosureFast™ device (Medtronic) ( Figure 62.18 ). This device has a wire coil on the end of a treatment catheter. The generator passes an electrical current through the coil until the surrounding temperature reaches 120°C. This is then maintained for a treatment cycle of 20 seconds. The coil is then withdrawn for a set length and another treatment cycle is commenced. Coils of 3 /uni00A0 cm and 7 /uni00A0 cm are produced, with the latter increasing the speed of treatment, while still being suitable for most anatomies. RFA. The evidence is generally equivocal, with both treat - ments having relative advantages and disadvantages; choice often comes down to personal preference. Both are excellent treatment options and can be applied successfully to the major - ity of patients. There hav e been a range of studies comparing EVLA and RFA. The evidence is generally equivocal, with both treat - ments having relative advantages and disadvantages; choice - often comes down to personal preference. Both are excellent treatment options and can be applied successfully to the major - ity of patients. Some summary points: /uni25CF Both treatments have a very high e ﬃ cacy (>95% closure rate) and are suitable for treatment of the vast majority of patients presenting with superﬁcial vein reﬂux in associa - tion with superﬁcial axial incompetence. /uni25CF V eins that are very tortuous may still be suitable for endo - thermal ablation but require a guidewire and in the case of EVLA, a catheter-based, rather than direct ﬁbre system. Both techniques o ﬀ er this.

(a) (b) Figure 62.17 (a) Ultrasound-guided in /f_i ltration of perivenous tumes cent anaesthetic via a long spinal needle. The anaesthetic solution is in /f_i ltrated using an electronic foot-operated pump; (b) ultrasound image of a perivenous ‘halo’ of anaesthetic solution around the vein and catheter in transverse section.

Figure 62.18 Radiofrequency ablation with ClosureFast™ introducing the treatment catheter through a sheath. The distal 7 cm of this device comprises a metal coil.

allowing direct and targeted delivery of adjuvant foam sclerotherapy , e.g. to ablate areas of neovascularisation. This is more di ﬃ cult with radiofrequency catheters due to a smaller lumen (0.018 versus 0.035 /uni00A0 in), but it is still possible. Advantages in favour of RFA over EVLA include: /uni25CF The core skill set to plan and perform these procedures is the same; however, EVLA requires an understanding of power settings and pullback speeds, whereas a radio- frequency device typically has a set treatment cycle on a single button press. This reduces the device-speciﬁc learn ing curve and the possibility of a novice making a mistake with the energy delivery . /uni25CF The automatic treatment cycle also frees the surgeon’s fo cus, allowing better communication with the patient and with care, concurrent treatment, e.g. inﬁltrating local an aesthetic into the tributaries and performing phlebectomy , reducing procedural times . /uni25CF EVLA requires speciﬁc laser safety protocols including the design and function of the room as well as speciﬁc training for the operator and theatre team. This can have an im pact on the set-up and ﬂexibility of the service. /uni25CF RFA may be associated with a marginal reduction in pain and bruising, although this has not been shown to impact on periprocedural quality of life or recovery . Advantages in favour of EVLA over RFA include: /uni25CF When it comes to veins that are very large in diameter (>15 /uni00A0 mm) EVLA can be a better option, allowing an increase in energy delivery and higher e ﬃ cacy rates. /uni25CF EVLA consumables are typically much less expensive; however, costs vary by device and market and the price di ﬀ erence has reduced over time. /uni25CF A standard EVLA ﬁbre may be used to treat perforators, whereas a speciﬁc additional device is typically required for Lorenzo Tessari , b. 1949, physician, Trieste, Italy . for perforator management remain uncertain. As endothermal ablation treats only junctional and truncal incompetence, debate exists regarding the management of var - icosities. These can be managed concomitantly or sequentially by either phlebectomy or sclerotherapy . Concomitant phlebec - tomy ( Figure 62.19 ) results in a more rapid improvement in disease-speciﬁc quality of life, and allo ws the vast majority of patients to complete treatment in a single visit. Non-endothermal , non-tumescent ablation Endothermal ablation was a large step forwards in the - management of superﬁcial incompetence; however, all tech - niques require the injection of tumescent local anaesthetic solution and this can be uncomfortable for the patient. Other - techniques that avoid injection are being developed. - Ultrasound-guided foam sclerotherapy Sclerotherapy is the original non-endothermal, non-tumes - cent technique and has been performed for over 100 years. It involves the injection of a sclerosing agent directly into the superﬁcial veins. The most commonly used is sodium - tetradecyl sulphate. The direct contact with detergent causes cellular death and initiates an inﬂammatory response, aiming to result in thrombosis, ﬁbrosis and obliteration (sclerosis). Blood deactivates the action of the sclerosing agent and the doses administered need to be limited to avoid adverse e ﬀ ects, causing a trade-o ﬀ between poor e ﬃ cacy and safety . This led to the development of ultrasound-guided foam sclerotherapy (UGFS). The use of foam increases the e ﬀ ective volume of the agent, maximising endothelial contact and displacing any blood that deactivates it. The procedure commences with the patient standing, and the sites of venous cannulation are selected and marked using ultrasound. With the patient supine, the major venous trunks and superﬁcial varicosities to be treated are then all cannulated using ultrasound guidance prior to any injection ( Figure 62.20 ). Once all injection sites are cannulated the foam can be prepared. The most widely used method is that of Tessari; this utilises two syringes connected using a three-way

Figure 62.19 Phlebectomy performed under tumescent anaesthesia following endothermal ablation. Figure 62.20 Foam sclerotherapy; cannulation of veins during ultra

sound-guided foam sclerotherapy.

tap. A 1:3 or 1:4 ratio mixture of sclerosant and air is drawn into one syringe, and is then oscillated vigorously between the two syringes about 10 or 20 times ( Figure 62.21 ). The foam produced in this way is stable for about 2 minutes so it should be injected as soon as it has been made. The leg is then elevated to empty the veins of blood, and injection of foam commences ﬁrst with superﬁcial varicosities and ends with injection of the GSV or SSV . Only 1–2 /uni00A0 mL of foam should be injected at a time and the distribution of the foam should be monitored and massaged with the ultrasound probe. When the foam is visu alised at the site of junctional incompetence no further foam should be injected. The maximum volume of foam that should be injected at a single session should not exceed 10–12 /uni00A0 mL as the incidence of complications is dir ectly related to the volume of foam injected. Compression is then applied as following endothermal ablation. While it is postulated that compression may have a larger e ﬀ ect upon e ﬃ cacy for this treatment, prac tice is not informed by evidence and a wide variation exists. Outside of a small number of centres, the e ﬃ cacy of UGFS is signiﬁcantly worse than for endothermal ablation, leading to high reintervention rates, and the rates of complications such as phlebitis and pigmentation can be high. UGFS does how ever carry some signiﬁcant advantages: /uni25CF It avoids tumescent anaesthetic and is therefore a less pain ful procedure (although postoperative pain is probably similar). /uni25CF No axial or tributary veins are too tortuous. /uni25CF It also allows the treatment of calf veins with overlying skin damage or ulceration without the need to pierce through damaged skin. /uni25CF Consumable treatment costs are very low . These factors mean that many surgeons using endothermal techniques also use foam sclerotherapy as an adjunct in speciﬁc circumstances. Catheter-directed sclerotherapy and mechanochemical ablation The e ﬃ cacy of sclerotherapy relies on endothelial contact with fresh, undiluted sclerosant. Some have therefore experimented with catheter-delivered sclerotherapy rather than trying to milk the sclerosant down the vein lumen. There is no good evidence to date that this increases e ﬃ cacy and the technique is not in widespread use. A related technology that has shown more promise is mechanochemical ablation ( Figure 62.22 ). This involves a led wire from the end. treatment device that deploys an ang This attaches to a motorised handle. The catheter is placed within the vein lumen as for endothermal ablation. The trigger - on the handle is depressed, spinning the wire around and liquid sclerosant is inﬁltrated via the catheter simultaneously during catheter withdrawal. It is thought that the spinning wire causes physical damage to the endothelium and allows a deeper pen - etration of the sclerosant into the vein wall. The technique is possible in most cases without tumescent anaesthesia, although a small number of patients ﬁnd the procedure uncomfortable - and the device can ‘snag’ on the vein, tearing it or rarely strip - - ping it altogether. Comparative studies with endothermal abla tion suggest similar early e ﬃ cacy rates but increased medium-/ long-term recanalisation rates. The axial ablation is usually less painful than endothermal ablation, but this advantage is - lost when it is combined with phlebectomy of the tributaries; efore, it is uncertain whether it can replace endothermal ther ablation, unless axial ablation is to be performed in isolation. - Treating longer veins can also be challenging owing to limita - tions in catheter length and the safe dose of sclerosant. It is a good choice for a patient with needle phobia who is happy to forgo treatment of varicose tributaries. Endovenous glue The ﬁnal non-tumescent technique is the endoluminal appli - cation of cyanoacrylate adhesive ( Figure 62.23 ). Again, this involves a treatment catheter placed within the vein lumen. A handle is used to inﬁltrate the adhesive in 0.1-mL applications via the catheter. The vein is then compressed, sealing the results are similarly promising and lumen closed. Early e ﬃ cacy patients experience minimal intraprocedural pain. Long-term results and the optimal management of tributaries are unknown (similar to mechanochemical ablation). The consumable costs are currently the highest for any venous ablative technique.

Figure 62.21 Foam sclerotherapy; Tessari method of foam sclerosant preparation. Figure 62.22 Mechanochemical ablation device (reproduced with permission from Vascular Insights).

Open surgery The principles of traditional ligation and stripping are to fully dissect the point of junctional incompetence and to remove the reﬂuxing axial vein and dilated tributaries. The operation is usually performed under general anaesthesia but locoregional anaesthesia is used by some; the inﬁltration of tumescent local anaesthesia around the axial vein prior to stripping may have some advantages, but is not widely used. The role of open surgery as a primary treatment of a reﬂux ing superﬁcial axis has been considerably reduced with the development of the minimally invasive techniques described above, the long-term results of which are at least comparable to open surgery but with signiﬁcantly less morbidity and faster recovery . Experienced endovenous surgeons do still use open surgery in some circumstances and a venous surgeon needs to be trained and experienced in this area. Surgical adjuncts including phlebectomy and, occasionally , perforator ligation are much more commonly used, and the former has been shown to have a signiﬁcant impact upon out come. Saphenofemoral ligation and great saphenous stripping An oblique groin incision is made at the level of, and lateral to, the pubic tubercle, ideally above the groin crease. The GSV is identiﬁed and dissected to the SFJ, which should be clearly established before the vein is divided to avoid disastrous inadvertent transection of the superﬁcial femoral vein. The anatomy is often variable but six GSV tributaries may be encountered close to the SFJ: /uni25CF Laterally: /uni25CF superﬁcial inferior epigastric vein; /uni25CF superﬁcial circumﬂex iliac vein. /uni25CF Medially: /uni25CF superﬁcial external pudendal vein; /uni25CF deep external pudendal vein. /uni25CF Distally: /uni25CF anterior accessory of the great saphenous vein; /uni25CF posteromedial thigh vein. Classically , these are ligated distal to their divisions. A ﬂush SFJ ligation is then performed and the GSV retrogradely stripped to around the knee ( Figure 62.24 ). Phlebectomy is perfor med as discussed above. Closure of the cribriform fascia, with sutures or synthetic patches over the ligated SFJ, does not reduce groin recurrence. - - Stripping to the lowest point of reﬂux may improve results, but at a cost of increased saphenous nerve complications and is not widely performed. More recently , some surgeons have argued that surgical trauma and subsequent inﬂammation in the groin are associated with neovascularisation, which in turn may lead to recurrence. Furthermore, others hypothesise that it is the loss of the normal groin tributaries that may be responsible for driving the process of neovascularisation. These concepts have led some to believe that ligation of the reﬂuxing vein should be distal to the tributaries and that the junction itself should be left untouched. There is no clear clinical evidence to support these hypotheses.

Figure 62.23 Endovenous glue device (reproduced with permission from Medtronic Inc.). Femoral vein Ligature Point of division Great saphenous vein Figure 62.24 Saphenofemoral junction ligation and great saphenous vein stripping.

Saphenopopliteal junction ligation and small saphenous stripping Preoperative duplex to mark the position of the SPJ is highly recommended ( Figure 62.25 ). The patient is positioned in the prone position, a transverse incision is made over the premarked SPJ, the fascia is divided and the SSV is exposed. The SPJ can then be formally dissected with a ﬂush ligation or the SSV can be gently retracted and ligated as proximally as possible. No good evidence exists to favour one technique over the other; proponents of the ﬂush ligation would argue that it avoids leaving a stump of SSV , a common source of recur rence, while proponents of the simple SSV ligation technique argue that it reduces the incidence of the most common serious complications – nerve injury and popliteal vein injury . The SSV can then either be stripped or the proximal sec esected. Those who strip argue that tion of the vein can be r it reduces the incidence of recurrence, while opponents feel it increases the incidence of sural nerve injur y . There are no randomised trials comparing these techniques. Once again, phlebectomy is then performed. Adjunctive surgical techniques Phlebectomy This may be performed following treatment of junctional incompetence and axial vein reﬂux, or as a sole treatment under local anaesthetic in patients with isolated tributary incompetence, or possibly in very early axial reﬂux, which duplex ultrasound. Phlebectomy is usually performed through small stab incisions using small mosquito forceps and/or phle - bectomy hooks that have been demonstrated to be superior – in terms of bruising , pain and generic quality of life – to transilluminated-powered phlebectomy ( Figure 62.19 ). Perforator ligation The majority of studies assessing the role of perforator ligation have been in patients with venous ulcers, analysing the e ﬀ ects on ulcer healing; even in this situation randomised data are lacking. The role of perforator ligation in patients with uncom - plicated varicose veins is even less clear. In uncomplicated varicose veins perforators may be ligated through a small, duplex-guided incision, while in patients with skin changes subfascial endoscopic perforator ligation may be preferred, although the beneﬁts are unproven. Perforators can also be ablated with endovenous techniques. Complications of standard varicose vein surgery Complications (minor and major) are reported in up to 20% of patients who undergo traditional varicose vein surgery . Wound infections, the most common complication, are reduced by prophylactic antibiotics. Nerve injury is the most common serious complication. The incidence of saphenous nerve neuralgia is up to 7% following GSV stripping to the knee (the incidence is higher with stripping to the ankle). The incidence of sural nerve neuropraxia and common peroneal nerve injury may be as high as 20% and 4%, respectively , following SSV surgery . The incidence of venous thromboembolic complications is approximately 0.5% following varicose vein surgery; however, patient risk factors must be individually assessed and appropriate prophylaxis administered according to guidelines. Recurrent varicose veins Approximately 10–20% of patients who present to hospital with varicose veins have had previous intervention. Prospective data on long-term results following intervention for recurrent varicose veins are sparse and the criteria for deﬁning recur - - rence are variable. Signiﬁcant clinical recurrence 5–10 years following vari - cose vein surgery occurs in 10–35% of patients, but minor/ duplex-detected recurrence is much more common, being of - the order of 70%. Causes of recurrence include: neovascu - eﬂux in the residual axial vein, inadequate initial larisation, r surgery and new junctional reﬂux. Neovascularisation is the development of new veins within postsurgical tissue. These er time can span the tissue between veins lack valves and ov a ligated junction and nearby tributary veins. If signiﬁcant in size and/or number, these may contribute to recurrent venous hypertension. Recurrence is more common following SSV surgery than following GSV surgery , and in patients with high body mass index, while stripping of the incompetent axial vein reduces ence rates. Limited data suggest that recurrence rates recurr following endovenous thermal ablation may be lower than

Figure 62.25 Preoperative marking of the saphenopopliteal junction and small saphenous vein mapped using duplex scanning.

atypical distribution and duplex assessment is mandatory ( Figures 62.26 and 62.27 ). Open surgery for recurrent varicose veins is associated with a high (40%) complication rate, the most common being lymph leak and wound infection, thus endovenous interventions would seem to o ﬀ er an attractive alternative, where feasible. Summary box 62.1 Varicose veins /uni25CF /uni25CF /uni25CF /uni25CF

Are one of the most common conditions causing a physical impairment in quality of life Interventional treatment improves quality of life and is highly cost-effective Anatomical and physiological assessment using duplex ultrasound is invaluable in the diagnosis and planning of treatment Ultrasound-guided endovenous ablation has revolutionised treatment, minimising procedural morbidity while being highly effective Figure 62.26 Recurrent anterior abdominal wall varicose veins follow ing saphenofemoral junction ligation complicated by iliac deep vein thrombosis.

Management

The very best results are seen in specialist multidisciplinary ulcer services. The cause of a venous leg ulcer is venous hyper - - tension and the keystone of management is to decrease this hypertension using venous ablation and compression therapy . -

Figure 62.28 A Marjolin’s ulcer (a squamous cell cancer arising in a chronic venous ulcer).

In patients with venous leg ulcers, treatment of superﬁcial venous incompetence has been demonstrated to accelerate healing and reduce recurrence; therefore, expeditious referral to a vascular service for assessment is recommended. Compression The most clinical and cost-e ﬀ ective compression regimes are two-layer compression hosiery or four-layer compression bandaging. The latter includes: /uni25CF Orthopaedic wool: distributes the pressure and reduces undue pressure on sensitive areas susceptible to pressure damage. Also helps to absorb excess exudate that escapes the primary dressing. /uni25CF Cotton crepe: smooths the wool and holds it in place. /uni25CF Elastic bandage: ﬁrst compressive layer, contributes about one-third of the interface pressure. /uni25CF Cohesive bandage: second compressive layer, increases sti ﬀ ness and adds approximately two-thirds of the inter face pressure. The ideal interface pressure in pure venous ulceration is 35–40 /uni00A0 mmHg. Skilled application of these dressings is essential for both safety and e ﬃ cacy , and the best results come from specialist nursing teams based either in secondary care or in the community . Compression in mixed ulcers is controversial, but emerging evidence suggests that it is both safe and e ﬀ ective when performed and monitored appropriately . With an ankle–brachial pressure index (ABPI) of 0.5–0.8 modiﬁed compression with an interface pressure of 30 mmHg is safe and e ﬀ ective and pressures of up to 40 mmHg have been described in studies using inelastic bandages without ill e ﬀ ect. Contrary to conventional thinking, studies have shown an increase in perfusion in patients treated in this way , presumably by a reduction in capillary back pressure. Patients do see respectable healing rates in this group, but they remain lower than in those patients with an ABPI >0.8. It is not clear whether revascularisation followed by full compression yields better results, but this is common practice. Patients with an ABPI <0.5 or an ankle pressure <60 /uni00A0 mmHg must undergo revascularisation prior to any compression treatment. Other treatments Pentoxifylline, which increases microvascular perfusion by decreasing plasma cellular viscosity and cytokine inhibition, has been demonstrated to be a useful adjunct to compression by augmenting ulcer healing. Horse chestnut seed extract has been shown to be a safe and e ﬃ cacious treatment for chronic venous hypertension, improving symptoms and reducing leg volume. A number of biological dressings hav e been developed, including fetal keratinocytes and collagen meshes, which have been shown to improve healing; however, they ar cost-e ﬀ ective for the majority of ulcers. Pinch grafts and ulcer excision with mesh grafting have been shown to provide good early healing with moderate long-term results (50% healed at 5 years). cellulitis and all other speciﬁc ulcer-healing drugs are of dubi - ous validity . A large range of topical therapies and primary dressings have similarly failed to have an impact. Management

As with all traumatic injuries, the management priorities are the assessment and management of issues a ﬀ ecting the airway , then breathing and then circulation. V enous injuries have the potential to threaten life through massive bleeding and patients require vascular access, circulatory support and blood products. Trauma patients with life-threatening haemorrhage are at risk of hypothermia, acidosis, functional and consumptive coagu - lopathy and paradoxical thrombosis, and these issues need to be prevented where possible and managed when present. V enous pressures are low and so, where there is access to the site of injury , pressure will control bleeding and in most cases o ﬀ er deﬁnitive management. Intervention is required where pressure cannot be applied, or where the loss of venous function itself threatens life or limb. Intervention can include reduction and stabilisation of a fracture (e.g. pelvis), endo - venous embolisation or stent grafting. A small proportion of venous injuries will require formal exploration and ligation or repair. Di ﬀ erent types of repair are shown in Figure 62.39 ; the type of r epair carried out depends on the extent of the venous injury , including how much venous wall has been lost or damaged. Lateral sutures and vein patches are ideal methods of repair and end-to-end anastomosis is sat - isfactory , provided that it is not carried out under tension. A jump graft may be required. V ein replacement should be by autogenous tissue whenever possible, using vein harvested from another site, e .g. the internal jugular vein or the GSV from an undamaged limb. Artiﬁcial grafts, such as polytetraﬂuoroethylene (PTFE) g rafts, are at risk of infection and have given poor results in recent conﬂicts. The use of anticoagulants and an arteriovenous ﬁstula to reduce the risk of thrombosis in the vein graft are controversial and depend on the associated injuries that are present. In

contaminated wounds, tetanus toxoid and antibiotics should be given. A fasciotomy should always be considered if there is a concomitant arterial and venous injury .

(b) (c) (d) Figure 62.38 Types of venous damage: (a) incision; (b) transection; (c) irregular laceration; (d) avulsion of a tributary.

PEL VIC CONGESTION SYNDROME

Pelvic congestion syndrome (PCS) is among the di ﬀ erential diagnoses to be considered in female patients presenting with chronic pelvic pain and may be signiﬁcantly underdiagnosed. PCS su ﬀ erers are typically premenopausal, multiparous women aged 20–45 years, who present with severe dull aching pelvic pain thought to be the direct result of ovarian and pelvic varicosities. The pain is usually non-cyclical, and may be precipitated by prolonged standing. Other symptoms include dysmenorrhoea, menorrhagia, rectal discomfort or urinary frequency . Signs may include tenderness over the uterus/ovaries, vulval varicosities and haemorrhoids. There may be vulval and atypically distributed thigh varicosities. The road to a diagnosis of PCS is often a long and laborious one, usually only made following extensive investigations to exclude e not other more common causes of pelvic pain. Abdominal, pelvic and transvaginal duplex examination allows dynamic visualisation of pelvic blood ﬂow and should be the initial investigation of choice, as these are rapid, readily accessible outpatient procedures that are also valuable in excluding other pathologies. Alternatives include MR venography and diagnostic venography . Medical treatments for PCS include psychotherapy , progestins, danazol, gonadotropin receptor agonists (GnRH) with hormone replacement therapy , and non-steroidal anti inﬂammatory drugs (NSAIDs). Historical open surgical pr ocedures (extraperitoneal resection of ovarian veins) have now largely been superseded by percutaneous pelvic vein embolisation ( Figure 62.29 ), reducing peri- and post- procedural morbidity while maintaining high success rates.

Figure 62.29 (a, b) Left ovarian vein incompetence supplying the pelvic and pudendal varicosities: embolisation.

Pathology

The thrombus commences as a platelet aggregate. Subse quently , ﬁbrin and red cells form a mesh until the lumen of the vein wall occludes. The coralline thrombus then progresses Hulusi Behçet , 1889–1948, Turkish dermatologist, described a disease of inﬂamed blood vessels in 1937. /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF as a propagated loose red ﬁbrin clot containing many red cells ( Figure 62.31 ). This is likely to extend up to the next large venous branch and it is possible for the clot to break o ﬀ and embolise to the lung as a pulmonary embolus. In this situation the embolus arising from the lower leg veins becomes detached, passes through the large veins of the limb and vena cava, through the right heart and lodges in the pulmonary arteries. This may totally occlude perfusion to all or part of one or both lungs. This results in a clinical spectrum from tachycardia and pain, through respiratory failure (despite adequate ventilation) to cardiovascular collapse and death. Moderate-sized emboli can cause pyramidal-shaped infarcts on imaging.

(thrombophilia) that lead to an increased risk of venous thrombosis. Congenital De /f_i ciency of antithrombin III, protein C or protein S Antiphospholipid antibody or lupus anticoagulant Factor V Leiden gene defect or activated protein C resistance Dys /f_i brinogenaemias Acquired Antiphospholipid antibody or lupus anticoagulant

Pathophysiology of ulceration

The exact pathophysiology of ulcer development has not been established. Originally , it was thought that static blood within the superﬁcial veins led to hypoxia, which caused tissue death (stasis ulcers). This was not conﬁrmed by investigation of venous oxygen saturation, which was found to be higher in ulcerated limbs. This led to the concept of arteriovenous ﬁstulae, which were thought to develop in response to the high venous pressure; however, this could not be conﬁrmed. High venous pressure was found to be associated with a pericapil - lary inﬁltrate. This includes ﬁbrin and other proteins, which are known to lead to ﬁbrosis. It was hypothesised that these ‘cu ﬀ s’ could act as an impediment to di ﬀ usion of oxygen and nutrients. Leukocytes were found to be reduced in the blood retur ning - from legs with venous hypertension. This decrease in leukocyte passage was shown to increase if short-term venous hyperten - sion was induced by application of a tourniquet. This led to the conce pt of white cell ‘trapping’, which, however, has not been conﬁrmed by further investigation. Polymor phonuclear leuko - cytes were not found within the tissues, but increased numbers of mast cells, monocytes and lymphocytes have been found in - periulcer tissues. Reactive oxygen species are increased in the ulcer environ - ment and these may generate fr ee radicals, leading to tissue

the ﬁbroblasts in the ulcer surrounds are also abnormal, being in a ‘senescent’ state. Growth factors may be inhibited, leading to poor repair, and their absence may also lead to ulceration. It is debated whether these factors are the cause or e ﬀ ect of an ulcer. At present, ambulatory venous hypertension is the only accepted underlying cause of venous ulceration. This also explains why venous ulcers are never seen in the upper limb. It is important to try to deﬁne the exact mec hanism of ulcer development. V enous hypertension may be the result of primary valve incompetence of the saphenous veins, incompetence of the perforating veins or incompetence or obstruction of the deep veins.

Prevention of recurrence

Once an ulcer has healed the patient must be re-evaluated in an attempt to prevent recurrence. If not already performed, patients should undergo treatment for their superﬁcial venous incompetence. Class 2 below-knee graduated compression stockings should be prescribed for all patients with residual reﬂux or deep venous occlusion, or those with recurrent ulceration despite not being in this group. These should be worn for life.

Prognosis

Nearly all venous ulcers can be healed, but, even in those who have successful ablation or wear their stockings religiously , - there is a 20–30% incidence of reulceration by 5 years. The greatest risk of reulceration is in the post-thrombotic leg. Summary box 62.2 Venous leg ulcer /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF

Is associated with a profound impairment in quality of life Ulcers are not infrequently dif /f_i cult to heal and prone to recurrence The treatment of these chronic wounds is associated with high costs to healthcare systems and patients The mainstay of treatment is the reduction in venous hypertension, with ablation of super /f_i cial venous incompetence and compression Early endovenous ablation of super /f_i cial re /f_l ux almost halves the time to healing of venous leg ulcers, reduces ulcer recurrence rates and is cost-effective

Prognosis

It is now recognised that repair of a major vein can be carried out with a 70–80% success rate, reducing the morbidity of a combined arterial and venous injury considerably (especially limb loss). Complex repairs should not, however, be carried out if a patient’s life is at risk, when ligation may have to su ﬃ ce in the short term.

Prophylaxis

Prophylactic methods can be divided into mechanical and pharmacological. A variety of mechanical methods have been tried, but only the use of graduated elastic compression stockings and external pneumatic compression have been William Morrant Baker , 1839–1896, surgeon, St Bartholomew’s Hospital, London, UK, described these cysts in 1877. - - - - - shown to be worthwhile by reducing the incidence of throm - bosis. Newer devices, such as electronic nerve stimulators, lack evidence of e ﬃ cacy to date. More recent emerging evidence is - casting some doubt on the beneﬁt of mechanical prophylaxis in surgical patients and ther e are further studies underway . Compression-based prophylactic measures should be avoided in patients with peripheral vascular disease. Pharmacological methods are more e ﬀ ective than mechanical methods at risk reduction, although they carry an increased risk of bleeding. In the past, low-dose unfractionated heparin was used both intravenously and subcutaneously . In the absence of renal impairment, most centres currently use low-molecular-weight heparin (LMWH) given subcutaneously . This is given once daily , does not require monitoring and has a lower risk of bleeding complications. Patients who are being admitted for surgery may be graded as low , moderate or high risk for pulmonary embolism and VTE ( Tables 62.5 and 62.6 ). Patients in the medium- or high-risk groups should be considered for pharmacological

Figure 62.34 An ascending venogram of a deep vein thrombosis seen as /f_i lling defects (arrows) with contrast passing around the thrombus. Figure 62.35 A computed tomography pulmonary angiogram show

ing pulmonary emboli as /f_i lling defects (arrow) in the pulmonary artery. TABLE 62.5 Modi /f_i ed Wells criteria for predicting pulmonary embolism (PE). Variable Score Clinical signs and symptoms of DVT (minimum of leg 3 swelling and pain on palpation of deep veins) Alternative diagnosis less likely than PE 3 Heart rate >100 /uni00A0 bpm 1.5 Immobilisation >3 days or surgery within past 4 weeks 1.5 Previous DVT or PE 1.5 Haemoptysis 1 Malignancy (treatment or palliation within past 6 1 months) A score of <4 means PE is unlikely (12.4%); >4 is suggestive of PE (37.1%). bpm, beats per minute; DVT, deep vein thrombosis.

/uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF prophylaxis with an anticoagulant medication. Recent level 1 evidence suggests that the addition of mechanical prophylaxis in such patients a ﬀ ords no additional beneﬁt.

for venous thromboembolism. Low Minor surgery <30 minutes; any age; no risk factors Major surgery >30 minutes; age <40; no other risk factors Minor trauma or medical illness Medium Major surgery; age 40+ or other risk factors Major medical illness: heart/lung disease, cancer, in /f_l ammatory bowel disease Major trauma/burns Minor surgery, trauma, medical illness in patient with previous DVT, PE or thrombophilia High Major orthopaedic surgery or fracture of pelvis, hip, lower limb. Major abdominal/pelvic surgery for cancer Major surgery, trauma, medical illness in patient with DVT, PE or thrombophilia Lower limb paralysis (e.g. stroke, paraplegia) Major lower limb amputation DVT, deep vein thrombosis; PE, pulmonary embolus.

Signs

The presence of tortuous dilated subcutaneous veins is usually clinically obvious. These are conﬁned to the GSV and SSV systems in approximately 60% and 20% of cases, respectively . The distribution of varicosities may indicate which superﬁcial axis is defective; medial thigh and calf varicosities suggest GSV incompetence ( Figure 62.3a ), posterolateral calf varicosities are suggestive of SSV incompetence ( Figure 62.3b ), whereas anterolateral thigh and calf varicosities may indicate isolated incompetence of the AAGSV ( Figure 62.3c ). Any of the clin ical features above may be present. Large, dilated veins around the SFJ may present as a (usually painless) lump, emergent when standing and disappearing when recumbent. This is a saphena varix ( Figure 62.5 ). Gentle palpation over the varix during coughing may elicit an impulse and it ma y be mistaken for a groin hernia.

Symptoms

Varicose veins frequently cause symptoms. Patients describe aching, heaviness, throbbing, burning or bursting over a ﬀ ected areas and sometimes the whole limb. Such symptoms typically increase throughout the day or with prolonged standing, and are relieved by elevation or compression hosiery . Itching is also commonly described, though this is more frequent in the presence of complications, as is swelling of the ankle. V enous symptoms in the absence of complications can be vague and it may be di ﬃ cult to ascertain from history alone whether they are truly venous in origin and, therefore, whether treatment will help. A trial of compression hosiery can help as venous symptoms should show some beneﬁcial improvement. Symptoms can be very severe and interfere with a patient’s daily activities such as work, recreation and caring for children and adults. Such symptoms are independent of the degree of v enous incompetence or the presence of complications, including skin changes short of ulceration. Studies have also shown that symptoms are associated with a signiﬁcant deﬁcit in health-related quality of life, and signiﬁcant improvements are seen with treatment to remove or ablate the reﬂuxing veins. The maximal beneﬁt is seen in those with uncomplicated symptomatic varicose veins, as skin changes and a proportion of the associated morbidity are frequently irreversible. reticular veins occur very commonly in the absence of sig niﬁcant reﬂux or obstruction and in the vast majority do not cause any physical symptoms, though cosmetic treatment is commonly sought.

THE ANATOMY OF THE VENOUS SYSTEM OF THE LOWER LIMB

The venous system of the lower limb can be divided anatomi cally into the superﬁcial venous system , which is located within the superﬁcial tissues, and the deep venous system beneath the deep fascia of the leg, accompanying the arterial tree. The superﬁcial veins drain into the deep system, either at junctions or via fascial perforating veins, and the deep veins then return blood to the right atrium of the heart. V enous anatomy is characteristically variable. The terminology used below is consistent with international consensus. The deep veins of the lower limb ( Figure 62.1a ) include three pairs of venae comitantes, which accompany the three crural arteries (anterior and posterior tibial and peroneal arter ies). These six veins intercommunicate and come together in the popliteal fossa to form the popliteal vein, which also receives the soleal and gastrocnemius veins. The popliteal vein passes up through the adductor hiatus to enter the subsartorial canal as the femoral vein, w hich receives the deep (profunda) femoral vein (or veins) in the femoral triangle before passing behind the inguinal ligament to become the external iliac vein. The internal iliac vein combines with the external iliac vein in the pelvis to form the common iliac vein. The left common iliac vein passes behind the right common iliac artery to join the right common iliac vein on the right side of the abdominal aorta to form the inferior vena cava, which goes on to the right atrium. Far more anatomical variations exist within the superﬁcial veins of the lower limb, but there are almost always two trunks - or axes – the great and small saphenous veins ( Figur e 62.1b,c ). These lie superﬁcial to the fascia lata (deep fascia) but deep to - the saphenous fascia, in the saphenous ‘envelope’. As the sole of the foot is often placed under signiﬁcant pressure, the majority of the venous drainage of the foot is into the dorsal venous arch, running in the subcutaneous tissues over the metatarsal heads. T he medial end of this arch drains into the ﬁrst axis: the great saphenous vein (GSV). This is the longest vein in the body and is the most frequently a ﬀ ected by superﬁcial incompetence. The GSV passes anterior to the - medial malleolus and ascends the leg accompanied by the saphenous nerve in the superﬁcial tissues medial to the tibia, , looping posteriorly at the level of the medial condyle of the femur and continuing in the medial thigh. In the groin, it unites with tributaries corresponding to the arterial branches of the common femoral artery , before piercing the cribriform fascia covering the saphenous opening (approximately 2.5 /uni00A0 cm below and lateral to the pubic tubercle, but often somewhat higher) and terminates by draining into the common femoral vein (CFV) at the saphenofemoral junction (SFJ). Throughout its course the GSV unites variably with other superﬁcial - tributaries. /uni00A0 The anterior accessory of the great saphenous vein (AAGSV) is one of the most common. This is often seen originating around the lateral border of the knee, although it sometimes originates as low as the lateral end of the dorsal venous arch. Occasionally , this vein may also course up the medial aspect of the thigh, anterolateral to the GSV and

The management of venous ulceration • Venous thromboembolism •

following its course. In this instance, its origin is typically a conﬂuence of small tributaries around the knee. There is usually an in-line GSV axis passing uninterrupted from the foot (in some cases this may be hypoplastic), but this pattern of AAGSV is commonly mistaken for the GSV itself (some surgeons will call this a duplex GSV; a true duplex GSV is rare). The AAGSV may drain into the GSV in the thigh, but is typically at or near the junction itself. The small saphenous vein (SSV) originates from the lateral side of the dorsal venous arch and accompanies the sural nerve as it passes posterior to the lateral malleolus, then upwards in the posterior midline of the leg. In the proximal calf it is usually found sitting in the groove between the two muscular heads of gastrocnemius. Its termination commonly occurs by piercing the fascia of the popliteal fossa to drain into the popliteal vein at the saphenopopliteal junction (SPJ). However, this junction is highly variable and the vein may terminate as low as the mid-calf. The SSV may extend cranially beyond the SPJ, in which case it is known as either a cranial extension of the SSV , which terminates by piercing the fascia in the posterior thigh to drain into the deep system, or the Giacomini vein, which communicates with the GSV system occasionally joining the GSV at or about the SFJ. In some cases, the SSV does not terminate at or below the popliteal fossa at all, but continues on as described above. In the calf and thigh there are a number of valved perforat ing (communicating) veins that join the superﬁcial to the deep veins at inconstant sites and which allow blood to ﬂow from the superﬁcial to the deep venous system. The most important of these are the direct perforating veins of the medial and la calf and the communicating veins around the knee and in the mid-thigh. Carlo Giacomini , 1840–1898, anatomist, Turin, Italy . On his death he left his skeleton to the Anatomical Museum in Turin. Ernest Henry Starling , 1866–1927, physiologist, University College, London, UK.

femoral vein Saphenofemoral junction Super /f_i cial Profunda femoral femoral vein vein Popliteal vein Anterior tibial veins Posterior tibial veins (usually paired) (usually paired) Peroneal veins (usually paired) Figure 62.1 (a) Anatomy of the deep veins of the lower limb; (b) omy of the super /f_i cial veins of the lower limb (great saphenous axis); (c) anatomy of the super /f_i cial veins of the lower limb (small saphenous axis). Great saphenous vein Posteromedial thigh Anterior accessory tributary of the great saphenous vein Anterior tributary of the leg anat

Treatment

Deep vein thrombosis The management of DVT has in the past been focused upon reducing the risk of pulmonary embolus. Patients who are conﬁrmed to have a DVT on duplex imaging should be rapidly anticoagulated with a ‘treatment dose’ of subcutaneous LMWH. Patients with signiﬁcant renal impairment should be commenced on intravenous unfractionated heparin. Patients who have a sensitivity towards heparinoids, such as those with heparin-induced thrombocytopenia, should commence on another anticoagulant, such as fondaparinux (an indirect factor Xa inhibitor) or bivalirudin (a direct thrombin inhib itor). This will achieve rapid anticoagulation and reduce the risk of embolisation. Typically , patients will then commence on oral anticoagulation for at least 3 months (or longer depending upon the persistence of risk factors or in recurrent cases). Oral anticoagulation using new or ‘novel’ anticoagulants (NOACs), which directly inhibit either factor Xa (rivaroxaban and apixaban) or thrombin (dabigatran), is recommended as they are equally e ﬀ ective as vitamin K antagonists (warfarin) in preventing recurrent symptomatic VTE but are associated with less major bleeding complications. Patients who cannot be safely anticoagulated (usually because of bleeding risks) should be considered for a tempo rary inferior vena cava ﬁlter, until either they are safe to be Leo Buerger , 1879–1943, Professor of Urologic Surgery , The New Y ork Polyclinic Medical School, New Y ork, NY , USA, described thromboangiitis obliterans in 1908. the ﬁlter may be retrie ved. Endovascular surgery aiming to restore patency , including thrombus removal, lysis and stenting tec hniques, are increasingly /uni00A0 used in patients with acute DVT aiming to reduce the risk of chronic post-thr ombotic syndrome. Research suggests this may be beneﬁcial in selected patients, for example those with iliofemoral thrombosis. Pulmonary embolus Most pulmonary emboli can be treated by anticoagulation and observation, but severe right heart strain and shortness of breath indicate the need for thrombolysis or radiologically guided catheter embolectomy . Superﬁcial vein thrombosis This condition was previously known as thrombophlebitis. An abnormal endothelium is a much more common precip - itating factor than in most DVTs. Common causes include external trauma (especially to varicose veins), venepunctures and infusions of hyperosmolar solutions and drugs. The presence of an intravenous cannula f or longer than 24–48 hours often leads to local thrombosis. Some systemic diseases such as thromboangiitis obliterans (Buerger’s disease) and malignancy , especially of the pancreas, can lead to a ﬂitting thrombophlebitis (thrombophlebitis migrans), a ﬀ ecting di ﬀ er - ent veins at di ﬀ erent times. Finally , coagulation disorders such as polycythaemia, thrombocytosis and sickle cell disease are often associated, as is a concomitant DVT . The surface vein feels solid and is tender on palpation. The overlying skin may be attached to the v ein and in the early stages may be erythematous before gradually turning brown. A linear segment of vein of variable length can be easily palpated once the inﬂammation has died down. A full blood count, coagulation screen and duplex scan of the deep veins should usually be obtained. Any sugges - tion of an associated malignancy should be investig ated using Summary box 62.3 Venous thromboembolism - /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF -

May be unprovoked, in which case an association with an inherited thrombophilia should be considered Is much more commonly seen as a complication of illness or surgery Is associated with both quality-of-life impairment and a risk of mortality All healthcare professionals should actively assess the risk and consider preventative measures where this risk is increased Management should involve measures to reduce the risk of extension and/or embolisation, typically with systemic anticoagulation Early thrombus removal is increasingly being used aiming to prevent chronic post-thrombotic syndrome, and rarely for limb salvage

inal CT scan. Most patients are treated with NSAIDs and topical heparinoid preparations and the condition resolves spontaneously . Proximity to a deep venous junction or long a ﬀ ected length are indications for short-term anticoagulation and interv al duplex assessment. Rarely , infected thrombi require incision or excision. Ligation to prevent propagation into the deep veins is almost never required. Associated DVT or thrombophilia is treated with anticoagulation.

VENOUS ENTRAPMENT SYNDROMES

The axillary vein and the popliteal vein are the two veins that are most commonly compressed. The former is compressed at the thoracic outlet between the ï¬�rst rib and the clavicle, where it usually presents as an axillary vein thrombosis (see Axillary vein thrombosis ) ( Figure 62.37a ). The latter is compressed by an abnormal insertion of the gastrocnemius muscles. Entrapment may cause discomfort and swelling of the limb during exercise before thrombosis develops. Treatment is by surgical decompression, excising the ï¬�rst rib or dividing the abnormal musculature of the gastrocnemius insertion.

VENOUS INJURY

Blunt or penetrating trauma almost always damages some small and medium-sized veins, which can be safely ignored or ligated without causing any problems. Larger axial venous recognised that these axial veins should be repaired whenever possible to reduce subsequent morbidity (pain and swelling in the tissues being drained) and limb loss when associated with a concomitant arterial injury . Many venous injuries remain undiagnosed at the time of injury (e.g. crural vein damage associated with a fractured tibia) and only present many years later when post-thrombotic changes become apparent. V enous injuries occur from both civilian and military trauma but the incidence of venous military injuries has been particularly well documented. In total, 40–50% of arterial injuries have concomitant venous injuries, especially in the popliteal fossa. The mechanism may be laceration, contusion or avulsion ( Figure 62.38 ). Iatrogenic injuries result from damage at the time of surgery and from punctures caused by catheter inser - tion. Thrombosis , haemorrhage and embolisation are all com - mon complications and arteriovenous ﬁstulae may develop when there is a local concomitant arterial injury . Associated injuries to soft tissue, arteries and bones often overshadow the venous injury . Massive haemorrhage from the pelvic bones or the inferior vena ca va can rapidly lead to hypovolaemic shock and death if left untreated. Haematomas are common and engorgement, cyanosis and swelling are also indicative of a major venous injury .

(a) (b) Figure 62.37 Thoracic outlet syndrome: (a) cervical ribs on plain radiograph; (b) elevation of the arm causes occlusion of the axillary vein with collaterals. The patient has had previous surgery to decom press the left side (arrow in (a) ).

VENOUS LEG ULCER

V enous disease is responsible for around 85% of all chronic lower limb ulcers in resource-rich countries. Community-based prevalence is 0.1–0.3% in adults (2–4% in the elderly). V enous leg ulcer has a disproportionate cost to society , with profound impairment in health-related quality of life for both patients and their carers; the dressings alone account for 1–3% of west ern healthcare expenditure. Furthermore, 15–30% of patients with ‘venous’ leg ulcers have concomitant arterial occlusive disease. This is termed a ‘mixed’ ulcer. T here are many other causes of leg ulcers and these must be excluded in any patient presenting with ulceration. Causes of leg ulceration include: /uni25CF venous disease: superﬁcial incompetence, deep incompe tence or obstruction; /uni25CF arterial ischaemic ulcers; /uni25CF vasculitic ulcers; /uni25CF traumatic ulcers; /uni25CF neuropathic ulcers; /uni25CF neoplastic ulcers; /uni25CF infections, especially in low and middle income countries.

Figure 62.27 Recurrent varicose veins secondary to an incompetent thigh perforator.

VENOUS PATHOPHYSIOLOGY

The purpose of the venous system is primarily to return blood back to the heart so that it can be delivered into the pulmonary circulation. The venous system contains approximately 60% of the total blood volume, with an average pressure of around 5–10 /uni00A0 mmHg. Mechanical factors, alongside the autonomic nervous and endocrine systems, control the rate at which - blood is delivered to the right atrium. Through its e ﬀ ects upon myocardial contractility via the Starling mechanism, venous return is one of the factors responsible for determining cardiac output. teral Blood enters the lower limb through the femoral arteries before passing through arterioles into the capillaries, which have a pressure of about 32 /uni00A0 mmHg at their arterial ends. This pressure is reduced along the course of the capillaries and is approximately 12 /uni00A0 mmHg at the venular end of the capillary . The pressure continues to fall in the main veins, and is as low as 5 /uni00A0 mmHg at the upper end of the vena cava where it enters the right atrium. The venous pressure in a foot vein on standing is equiv alent to the height of a column of blood extending from the heart to the foot, e.g. approximately 100 /uni00A0 mmHg. To enable blood to be returned against gravity in the standing position a pressur e gradient must exist between the veins in the leg and those in the chest. This gradient is created in two ways. First, the increase in thoracic volume during inspiration decreases intrathoracic pressure. Second, the pressure in the veins of the leg is increased by compression by the surrounding mus cles (the ‘calf muscle pump’) and to a lesser extent the tone of the venous wall. The deep veins of the calf are capacious and are joined by blind-ending sacks called the soleal sinusoids, which force b lood into the popliteal and crural veins during calf muscle pump contraction, e.g. walking. The foot pump also ejects blood from the plantar veins during walking. As the calf muscles contract, the veins are compressed and the valves only allow blood to pass in the direction of the heart. The pressure within the calf compartment rises to 200–300 /uni00A0 mmHg during muscle contraction. Rapid blood ﬂow in the deep veins at junctions and perforators draws blood from the superﬁcial veins, driving this up the deep veins also. During muscle relax ation, the pressure falls and further blood from the superﬁcial veins enters the deep vein. Each time this occurs the pressure falls in the superﬁcial venous compartment until a thr is reached, when the venous inﬂow keeps pace with ejection from the deep veins. This is normally around 30 /uni00A0 mmHg, a fall of approximately two-thirds of the resting venous pressure. The net reduction in the pressure of the superﬁcial system is Rudolf Virchow , 1821–1902, pathologist, Charité Hospital, Berlin, Germany , was the ﬁrst to be credited with describing iliac vein compression. It was not until 1957 that May and Thurner (Innsbruck, Austria) clearly described compression of the left common iliac vein by the right common iliac artery . leg and the thorax and a patent and compliant venous system containing competent valves ( Figure 62.2 ). An absence of one or more of these results in venous hypertension, which leads to further vein wall damage, including loss of compliance, thickening, dilatation and valvular dysfunction. This venous damage goes on to reduce the function of the a ﬀ ected veins, worsening the venous hypertension in a vicious cycle. When exposed to high venous and capillary pressures chronically , the soft tissues of the leg will be damaged, causing a spectrum of damage that becomes irreversible. The causes of venous hypertension are listed in Table 62.1 . /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF - /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF - /uni25CF /uni25CF /uni25CF /uni25CF The majority of patients with venous disease have a problem primarily with the vein wall structure and in most this is conﬁned to the superﬁcial veins. Little is known about the mechanism of initiation of the changes in the vein wall. These changes are complex, but are typiﬁed by valvular failure allowing retrograde ﬂow within the vein with gravity - (venous incompetence). It is no longer thought that venous incompetence is caused by a primary mechanical valvular failure. eshold The vein wall changes include inﬂammatory cell inﬁltration and activation, dysfunctional smooth muscle cell proliferation, collagen deposition, decreased elastin content and increased matrix metalloproteinases. These e ﬀ ects typically lead to loss

limb Standing 80 70 60 50 40 30 Normal limb 20 Foot vein pressure (mmHg) 10 Walking Standing 0 0 10 20 30 40 Time (seconds) Figure 62.2 Effect of exercise on the super /f_i cial venous pressure in health and disease. The light blue line demonstrates the reduction in pressure primarily related to the action of the calf muscle pump. The dark blue line demonstrates how venous dysfunction is associated with reduced net antegrade /f_l ow during exercise, resulting in a relative increase in venous pressure when compared with normal (venous hypertension). TABLE 62.1 Factors causing venous hypertension. Pressure gradient dysfunction Increased abdominal or thoracic pressure COPD Pregnancy Obesity Large tumour Constipation Decreased calf muscle pump function Immobility Ankle joint fusion Paralysis Dysfunction of the venous system Venous structural de /f_i cit Valvular agenesis Valvular incompetence Venous dilatation Venous tortuosity Loss of vein wall compliance Loss of venous tone Arteriovenous /f_i stula Venous occlusion Agenesis Thrombosis Iatrogenic/trauma Venous compression May–Thurner syndrome Pelvic/abdominal tumour Pelvic/abdominal radiotherapy COPD, chronic obstructive pulmonary disease.

of compliance, dilatation, elongation (causing tortuosity) and secondary valvular dysfunction. This process can be initiated anywhere in the venous tree. Secondary varicose veins may develop in patients with post-thrombotic limbs and in patients with congenital abnormalities such as the Klippel–Trénaunay syndrome or multiple arteriovenous ﬁstulae. The extent and number of incompetent veins governs the extent of the venous hypertension and correlates with the severity of the soft-tissue complications seen. Importantly however, neither the reﬂux burden nor the presence of skin changes, short of ulceration, correlate with the presence or degree of symptoms.

Figure 62.3 Varicose veins: (a) left leg varicose veins in the distribution of an incompetent great saphenous vein (marked for intervention); /uni00A0 (b) right leg varicose veins in the distribution of the small saphenous system with a recent episode of phlebitis; distribution of an isolated incompetent anterior accessory of the great saphenous vein with associated gaiter area skin changes.

VENOUS THROMBOEMBOLISM

V enous thromboembolism (VTE) is an important condition within surgery , and autopsy studies suggest that it is the most common direct cause of death in surgical patients. V enous thrombosis is the formation of a semisolid coagulum within the venous system and may occur in the superﬁcial system (super ﬁcial vein thrombosis [SVT] or ‘thrombophlebitis’) or the deep system (deep vein thrombosis [DVT]). V enous thrombosis of the deep veins of the leg may be complicated by the immediate risk of pulmonary embolus and sudden death. Subsequently , patients are at risk of developing PTS ( Figure 62.30 venous ulceration. While DVT may occur in the upper limb, it is the leg that gives rise to the vast majority of the morbidity and subsequent complications of this condition.

VENOUS TUMOURS Venous malformation cavernous angio

VENOUS TUMOURS Venous malformation cavernous angioma/haemangioma

These malformations are common, representing one end of a spectrum of arteriovenous malformations. They often a ﬀ ect the skin but also extend into the deep tissues, including bones and joints. They usually present with variable swelling and dilated veins beneath the skin. Occasionally , there is no visible mass and the complaint is one of pain. Haemorrhage and thrombophlebitis may exacerbate the pain. A soft compressible mass, which is venous in colour especially if it is under the skin, is usually present ( Figure 62.40a ). A dark-blue tinge is often apparent, even if the malformation is deeply situated. Nodules within the mass usually represent previous episodes of thrombosis. The size and extent of the haemangioma are best visualised by nuclear MR with a short tau inversion recovery (STIR) sequence ( Figure 62.40b ) or CT scanning with contrast enhancement. V enography rarely shows an abnormality , but direct puncture with contrast injection shows the connections of the malformation. Treatment is a highly specialised area. Treatment options nowadays rarely initially involve surgical excision as once this Alexis Carrel , 1873–1944, a French surgeon who emigrated to work at the University of Chicago, Chicago, IL, USA. He was awarded the Nobel Prize in Physi ology or Medicine in 1912 for pioneering vascular suturing techniques. is done future embolisation and sclerotherapy are very di ﬃ cult. No treatment is entirely curative because it is di ﬃ cult to remove all of the angiomatous tissue or sclerose the angioma com - pletely . Sclerosis can be dangerous when the veins connect to the deep system, particularly near the central nervous system.

(b) (c) (d) (e) Figure 62.39 Types of venous repair: (a) lateral suture (risk of ste

nosis); (b) patch graft; (c) Carrel triangulation technique of venous anastomosis; (d) panel graft; (e) spiral graft.

62 Venous disorders

AXILLARY VEIN THROMBOSIS

Aetiology

CLINICAL FEATURES OF VENOUS HYPERTENSION OF THE LE

CONGENITAL VENOUS ANOMALIES

Classiﬁcation system

Clinical features

Cystic degeneration

Diagnosis

Epidemiology

FURTHER READING

Introduction

Investigation

TABLE 62.4 Modi /f_i ed Wells criteria for predicting deep

Klippel–Trénaunay syndrome

Learning objectives

Leiomyoma and leiomyosarcoma of the vein wall

Management

Figure 62.19 Phlebectomy performed under tumescent anaesthesia following endothermal ablation. Figure 62.20 Foam sclerotherapy; cannulation of veins during ultra

PEL VIC CONGESTION SYNDROME

Pathology

Pathophysiology of ulceration

Prevention of recurrence

Prognosis

Prophylaxis

Figure 62.34 An ascending venogram of a deep vein thrombosis seen as /f_i lling defects (arrows) with contrast passing around the thrombus. Figure 62.35 A computed tomography pulmonary angiogram show

Signs

Symptoms

THE ANATOMY OF THE VENOUS SYSTEM OF THE LOWER LIMB

Treatment

VENOUS ENTRAPMENT SYNDROMES

VENOUS INJURY

VENOUS LEG ULCER

VENOUS PATHOPHYSIOLOGY

VENOUS THROMBOEMBOLISM

VENOUS TUMOURS Venous malformation cavernous angio

(b) (c) (d) (e) Figure 62.39 Types of venous repair: (a) lateral suture (risk of ste