Vascular Compression Syndromes © 2015 Hans Huber Publishers, Hogrefe AG, Bern DOI 10.1024/0301

Home , Nutcracker syndrome, Popliteal fossa

Review 419

Vascular compression syndromes

Michael Czihal1, Ramin Banafsche2, Ulrich Hoff mann1, and Th omas Koeppel3

1 Division of Vascular Medicine, Medical Clinic and Policlinic IV, Vascular Center, Hospital of the Ludwig-Maximilians-University Munich, Germany 2Division of Vascular and Endovascular Surgery, Vascular Center, Hospital of the Ludwig-Maximilians-University, Munich, Germany 3Department of Vascular Surgery, Asklepios Hospital St. Georg, Hamburg, Germany

Summary: Dealing with vascular compression syndromes is individuals. Th is implies important diffi culties in diff erentiating one of the most challenging tasks in Vascular Medicine prac- physiological from pathological fi ndings of clinical examination tice. Th is heterogeneous group of disorders is characterised by and diagnostic imaging with provocative manoeuvres. Th e level external compression of primarily healthy arteries and/or veins of evidence on which treatment decisions regarding surgical as well as accompanying nerval structures, carrying the risk of decompression with or without revascularisation can be relied on subsequent structural vessel wall and nerve damage. Vascular is generally poor, mostly coming from retrospective single centre compression syndromes may severely impair health-related studies. Proper patient selection is critical in order to avoid over- quality of life in aff ected individuals who are typically young treatment in patients without a clear association between vascular and otherwise healthy. Th e diagnostic approach has not been compression and clinical symptoms. With a focus on the thoracic standardised for any of the vascular compression syndromes. outlet-syndrome, the median arcuate ligament syndrome and Moreover, some degree of positional external compression of the popliteal entrapment syndrome, the present article gives a blood vessels such as the subclavian and popliteal vessels or the selective literature review on compression syndromes from an celiac trunk can be found in a signifi cant proportion of healthy interdisciplinary vascular point of view.

Key words: Vascular compression syndrome, thoracic outlet syndrome, median arcuate ligament syndrome, Dunbar`s syndrome, popliteal entrapment syndrome

Introduction the popliteal entrapment syndrome than men. Th e onset of symptoms is (PES). May-Th urner-Syndrome and typically observed between 20 and Vascular compression syndromes, the Hypothenar-Hammer-Syndrome 50 years of age [6]. characterised by clinical symptoms have been recently reviewed in this arising from the external compres- journal [1, 2] and thus were not in- Pathoanatomy sion of normal, non-diseased blood cluded in this work. More rare vas- Th e upper thoracic outlet consists vessels, belong to the most controver- cular compression syndromes are of three compartments, namely the sially discussed disorders in Clinical briefl y discussed. interscalene triangle, the costocla- Medicine. Being relatively infrequent, vicular space and the retropectoralis reliable diagnostic criteria have not yet minor space [7]. Within this con- been established. Moreover, positio- Thoracic outlet syndrome tainer, the brachial plexus, the sub- nal extrinsic compression of vascular clavian artery, the subclavian vein structures can be found in a substan- Deﬁ nition and epidemiology and also sympathetic nerve fi bres can tial proportion of healthy individuals, Th e term “TOS” was introduced by be compressed by various anatomi- resulting in serious diagnostic uncer- Peet et al. in 1956 [3] and is used cal abnormalities, either congenital http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 tainties. Finally, the level of evidence today to describe a heterogeneous or developmental (Tab. I, Fig. 1) regarding the treatment of vascular disease complex characterised by [3 – 7]. Th e most common location compression syndromes is generally upper extremity symptoms resulting of compression is the costoclavicular poor. Th e present review article aims from (positional) compression of space. Of note, the subclavian vein to critically review the available litera- the neurovascular bundle at the up- does not cross the interscalene tri- ture to provide a scientifi c basis for per thoracic outlet [4, 5]. Th e scarce angle and instead runs in front of the clinical decision making. Th e main epidemiological data available should anterior scalene muscle. Th us, hyper- focus of this paper lies on the thoracic be interpreted with caution. Reported trophied scalene muscles or cervical outlet syndrome (TOS), the median incidences range from 3 to 80 cases ribs usually do not cause subclavian arcuate ligament syndrome (MALS, per 1,000 population, and women vein compression [8]. In addition to also called Dunbar`s syndrome) and seem to be more frequently aff ected anatomical factors, it is assumed that M. Czihal et al.: Vascular compression syndromes Vasa 2015; 44: 419 – 434 © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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static factors may play an aetiologi- (e.g. whiplash injury) may contribute surgical series patients with disputed cal role, e.g. postural abnormalities to the development of TOS via post- nTOS constituted up to 90 % of the of the spine and the shoulder girdle, traumatic scarring and shortening of study population. or hypertrophy of the scalene muscles the aff ected musculature [4, 5]. Th e incidence of vTOS was much low- due to body building [9]. Moreover, er in these series (3 – 5 % of all TOS local trauma to the shoulder or neck Classiﬁ cation and clinical cases), with primary upper extrem- presentation ity deep venous thrombosis (also en- Th e term TOS neither specifi es the titled “Paget-Schroetter-Syndrome” components of the neurovascular or “eff ort thrombosis”) being the bundle being compressed nor the fi rst manifestation of vTOS in most compressing anatomical structures aff ected subjects. It typically occurs [5]. Th erefore, according to the clin- in the dominant arm aft er strenuous, ical symptoms, TOS should be dis- unusual or repetitive physical activ- criminated in arterial TOS (aTOS), ity and presents with sudden onset venous TOS (vTOS, also referred to oedematous arm swelling and/or as “thoracic inlet syndrome”) and upper extremity discomfort or pain neurogenic TOS (nTOS) [4 – 7]. [8]. vTOS presenting with intermit- Based on the presence or absence of tent arm swelling and cyanosis due to objective diagnostic fi ndings, nTOS positional subclavian vein obstruc- can be further categorised into true tion (McCleery-Syndrome) is quite and disputed nTOS [6]. While aTOS, rare [11]. vTOS and true nTOS are well defi ned Less than 1 % of TOS cases are arte- and broadly accepted, disputed nTOS rial. In these cases, repetitive arterial is subject of a highly controversial in- compression has led to vessel wall terdisciplinary debate. Sceptics even damage, resulting in scarred stenosis Figure 1: Left-sided cervical rib argue that if this disease entity really or the development of ectasia/aneu- in a patient with TOS (arrow), exists it is quite rare [10]. On the rysm (Fig. 2). Structural vessel wall (TOS = thoracic outlet syndrome). contrary, proponents consider it to damage predisposes to mural throm- be a common disorder and in many bus formation, being a hazardous source of arterial embolism into the arm and digital arteries (Fig. 3) [5]. Table I: Aetiology of the thoracic outlet syndrome Acute or subacute digital ischemia Anatomical abnormalities is the leading clinical manifestation Bony structures of aTOS and the clinical spectrum Complete or incomplete cervical rib ranges from secondary Raynaud`s Elongated C7 transversal processus phenomenon to fi nger necrosis due to critical ischemia. Arm claudication is Abnormal ﬁ rst rib (e.g. exostosis) a less frequent manifestation of aTOS. Abnormal clavicle (e.g. callus or malunion after fracture) Symptoms of nTOS are non-radicular Soft tissue abnormalities in nature and typically infl uenced by Scalene muscle hypertrophy the position of the upper limb and/ http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 Minor scalene minor muscle or neck. Th e most frequent symptoms Pectoralis minor muscle are pain and paresthesia of the arm, Subclavian muscle hand and fi ngers (with paresthesia Various ligamentous abnormalities (e.g. costoclavicular ligament) restricted to the ulnar aspect of the forearm and hand in most cases due Postural abnormalities to predominant aff ection of the lower Hypotension of the shoulder girdle musculature plexus) [4 – 7]. Further symptoms include pain of the shoulder, neck Trauma and supraclavicular fossa as well as Neck trauma (e.g. whiplash injury resulting in muscle scarring and shortening) occipital headache. In advanced dis- Repetitive upper extremity activities related to work or athletics ease, patients may experience a loss of Vasa 2015; 44: 419 – 434 M. Czihal et al.: Vascular compression syndromes © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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strength and impairment of fi ne motor skills of the hand [6]. Raynaud`s phenomenon secondary to aff ection of sympathetic fi bres can also be a clinical feature of nTOS and can hamper the discrimination of nTOS and aTOS [5]. Among the multitude of disorders to be considered in the diff erential diagnosis of nTOS, some of the most important are cervical ra- diculopathy, ulnar or median nerve entrapment and shoulder patholo- gies [6]. Noteworthy, bilateral symptoms (particularly in nTOS and vTOS) as well as combined vascular and neurologic symptoms are not uncommon [12, 13]. Figure 2: Bilateral subclavian artery aneurysms (arrows) with mural thrombus in a patient with aTOS. (aTOS = arterial thoracic outlet syndrome). Diagnostic workup Diagnosis of TOS is challenging. Th is is underlined by the results from a cohort study reporting on patients who underwent surgery for established TOS. Patients reported a mean of 3.1 ± 1.1 physician-consultations prior to diagnosis and a mean la- tency between symptom onset and diagnosis of 46.0 ± 83.5 months [14]. A detailed medical history and thorough physical examination are of utmost importance in the clinical evaluation of suspected TOS. Visual inspection may reveal predisposing postural variants such as drooping shoulders [7] as well as sequelae of the syndrome (e.g., hand muscle atrophy in nTOS, digital necrosis in aTOS, prominent subcutaneous col- Figure 3: Embolic axillary artery occlusion (arrow) originating from sub- lateral veins or livid discoloration and clavian artery ectasia (arrowhead) in a patient with aTOS. (aTOS = arterial oedema of the upper limb in vTOS) http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 thoracic outlet syndrome). [4, 8]. Bilateral pulse palpation, arterial auscultation and Allen`s Test are essential for detection of peripheral arterial complications in aTOS. described in the literature (Tab. II). unclear as a diagnostic gold standard Neurological assessment has been Tests are considered to be positive is missing. Th e main problem is the described elsewhere in detail [6, 7]. when the patient`s symptoms can substantial rate of positive test results Clinical provocative manoeuvres are be reproduced and/or arterial pulse in healthy individuals, implying the the key elements in the diagnosis of loss or arterial bruits appear. Unfor- risk of over-diagnosis [9, 15 – 17]. A TOS. Various tests aiming to uncover tunately there is (a) no consensus diagnostic approach with a combina- compression at diff erent compart- on standardised testing and (b) the tion of several tests (arm abduction, ments of the thoracic outlet have been diagnostic accuracy of the tests is elevation and external rotation as well M. Czihal et al.: Vascular compression syndromes Vasa 2015; 44: 419 – 434 © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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Table II: Clinical provocative manoeuvres for the diagnosis of TOS (modiﬁ ed according to [5])

Test Proposed Procedure* Diagnostic** mechanism accuracy Adson`s test Compression at The patient breathes in deeply, then extends and rotates the neck Sensitivity 79 % the interscalene towards the side being tested. triangle Specifi city Positive test: loss of radial pulse, supraclavicular bruit and/or 74 – 100 % pain/paresthesia Costoclavicular Costoclavicular The patient breathes in and retracts both shoulders, while the Sensitivity: manoeuvre compression examiner draws the patient’s arm down. unknown (Military Positive test: loss of radial pulse, subclavian bruit and/or pain/ Specifi city exercise-test) paresthesia 53 – 100 % Wright`s test Costopectoral Passive retraction of the hyperabducted, externally rotated arm Sensitivity 70 – 90 % compression Positive test: loss of radial pulse, axillary bruit and/or pain/ Specifi city 29 – 53 % paresthesia Elevated arm Not specifi ed 90° abduction and full external rotation of both arms with the head Sensitivity 52 – 84 % stress test in neutral position. While holding this position, the Patient opens (Roos test) and closes the hands repeatedly for 3 minutes. Specifi city 30 – 100 % Positive test: pain and/or paresthesia, early discontinuation of the test for relief of symptoms Modifi ed upper Compression (1) Active arm abduction with extended elbows Sensitivity: 98% limb tension test of the brachial (Elvey test) plexus in nTOS (2) Dorsifl exion of both wrists Specifi city: unknown (limited) (3) Tilting the head to each shoulder Positive test: Pain down the arm or paresthesia (ipsilateral in position 1 and 2; contralateral to the side the head is tilted in position 3) *All tests are performed with the patients in a seated position and the radial pulse being palpated by the examiner. **Diagnostic accuracy according to the fi nal clinical diagnosis.

as shoulder retraction and head rotation) seems to increase the diagnostic accuracy and is thus recommended for clinical practice [18]. Non-invasive vascular laboratory testing is the next step in objectifying TOS with vascular compression. Th e diagnostic approach should include acral photoplethysmography for detection of digital artery obstructions and colour duplex sonography for http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 detection of morphological abnormalities of the subclavian arteries (mural thrombus, aneurysm) and subclavian veins (post-thrombotic sequelae). Complete colour duplex sonography of the upper extremity Figure 4: Intra-arterial angiography showing focal luminal narrowing arteries also enables the investigator (arrow) of the left subclavian artery in neutral position (A) and complete to detect important alternative rea- occlusion (arrowhead) with arm abduction in a patient with TOS (B). sons for digital artery occlusions such (TOS = thoracic outlet syndrome). as the hypothenar hammer syndrome [2]. Dynamic testing with provoca- Vasa 2015; 44: 419 – 434 M. Czihal et al.: Vascular compression syndromes © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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tive manoeuvres performed in a sit- compression at the thoracic outlet is Conservative treatment includes pa- ting position is helpful in confi rming universally found in normal subjects tient education to avoid provocative positional arterial compression. With with arm abduction/external rotation arm positions (e.g., overhead use of this regard, pulse volume recording, [24]. When interpreting the results of the upper extremities), pain control, continuous wave Doppler sonogra- vascular imaging, the examiner should and individually tailored physical phy and colour duplex sonography take into account that passive arm ab- therapy aimed to strengthen the can be applied. However, it should duction in a supine position may fail muscles of the pectoral girdle and be noted that the sensitivity of colour to replicate the true pathophysiology to restore a normal posture [6, 29]. duplex sonography is probably not of TOS. Cornelis et al. found that ar- With conservative treatment, rates superior to clinical provocative test- teriography revealed a signifi cantly of clinical improvement between 50 ing with combined manoeuvres [18]. lower rate of severe positional arterial and 90 % have been reported [6, 9]. Confi rmation of arterial compression stenosis when performed in a supine Duration of symptoms and additional may also contribute to the diagnosis position (31 %) compared to the in- psychoemotional disturbances may of vTOS, and a study applying colour vestigation done in a sitting position negatively impact the treatment result duplex sonography revealed arterial (87 %) in patients with established [9]. Th e principles and controversies compression in every second patient TOS [25]. of conservative TOS-treatment are with nTOS [19]. It must be pointed It is of great importance to recognise outlined in detail in the reviews of out that the absence of arterial com- that the detection of postural vessel Hooper and Vanti et al. [9, 29]. pression does not allow ruling out compression by vascular imaging in Surgical decompression of the neuro- nTOS [5]. Th us, in suspected nTOS, an asymptomatic patient does not vascular bundle of the thoracic outlet further testing (nerve conduction justify the diagnosis of TOS. can include resection of bony struc- studies and electromyography) is re- tures (fi rst rib, cervical rib, callus required. A more detailed description Treatment principles sulting from a clavicle fracture), sca- of electrodiagnostic tests in suspected Treatment of TOS has two goals: lenotomy, neurolysis of the brachial nTOS is provided elsewhere [4,6]. relief of the patient`s symptoms plexus and resection of fi brous bands. Conventional X-ray imaging of the and prevention of complications. In retrospective studies, rates of sig- chest and neck easily allows the de- Depending on the type and severity nifi cant clinical improvement were tection of cervical ribs (Fig. 1) [5]. of TOS, conservative and invasive between 45 and 92 % [4]. Th e rate of Computed tomography (CT) and surgical approaches can be applied. severe complications (particularly magnetic resonance imaging (MRI) Th e level of evidence on which treat- nerve and vessel injury) has been re- including CT- and MR-angiography ment decisions can be based is still ported to be very low in experienced in neutral position and under provo- very low, with the majority of the centres [13, 14, 30, 31]. Of note, re- cation are helpful for uncovering the published reports being retrospec- current symptoms of TOS seem to be site of vascular compression and for tive single centre experiences [9, common in the long-term follow-up characterisation of the compressing 26]. Some factors particularly im- and were the reason for second surgi- structure when surgical decompres- pede the interpretation of the cur- cal procedures in about 30 % of pa- sion is intended [20 – 24]. Intra-arteri- rently available literature: hetero- tients in the large series published by al angiography still has a limited role in geneous diagnostic criteria, lack of Urschel et al. [30]. Th us, when refer- the diagnostic workup of patients with characterisation of the TOS-type in ring a patient for surgical treatment of suspected aTOS, mainly in assessing many studies, diffi culties in report- TOS, one should be aware that (a) the the digital arteries for embolic occlu- ing changes in subjective symptoms procedure carries a minor risk for sig- http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 sions, in investigating the subclavian and diff erences in the outcome defi - nifi cant neurovascular complications arteries in neutral and provocative po- nitions. As of today, only two small and (b) that a substantial percentage sition in cases of suspected aTOS re- prospective, randomised trials deal- of patients will have no benefi t from maining ambiguous aft er non-invasive ing with the treatment of TOS have the surgical procedure. diagnostics, and for planning arterial been published, but there is no ran- Decompressive surgery can be per- reconstruction (Fig. 4) [5]. Similarly, domised trial comparing surgical formed via a transaxillary or an an- contrast venography with postural vs. conservative treatment [27, 28]. terior supraclavicular approach. In- manoeuvres can be a useful diagnostic How to treat the condition is there- troduced by Roos in 1966 [32], the tool in suspected vTOS [8]. However, fore one of the most controversial transaxillary approach has become venography results should be inter- points in the scientifi c debate sur- the most popular procedure in the preted with caution, as partial venous rounding TOS. surgical treatment of TOS [6, 13, M. Czihal et al.: Vascular compression syndromes Vasa 2015; 44: 419 – 434 © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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14, 31]. It provides a good exposure treatment goals are prevention of (a) when required (e.g., catheter directed of almost the entire fi rst rib. As the the post-thrombotic syndrome and thrombolysis in upper limb ischemia incision is hidden in the axilla, the (b) of recurrent thrombosis. With this secondary to arterio-arterial embo- cosmetic result is usually excellent. regard it is noteworthy that severe lism). Following decompression, re- A shortcoming of this approach lies post-thrombotic syndrome is virtu- construction of the subclavian artery in its limited exposure of the neural ally unknown aft er primary upper ex- must be performed in case of mor- structures. Th e supraclavicular ap- tremity deep venous thrombosis [35]. phological alterations (particularly proach off ers a good exposure of the Moreover, there seems to be no clear aneurysms). Th is frequently requires dorsal part of the fi rst rib and of cervi- correlation between morphological a combined transaxillary and supra- cal ribs when present. It allows wide sequelae (i.e. residual venous stenosis clavicular approach. exposure of the brachial plexus but, and occlusion) and the presence and Most patients with nTOS should pri- on the contrary, carries a considerable severity of the post-thrombotic syn- marily receive conservative therapy risk of nerve injury (phrenic nerve, drome [36]. Some authors advocate for a few months [6, 29]. With this brachial plexus, cervical sympathetic decompressive surgery for the treat- regard, a double-blind, randomised, chain). When indirectly comparing ment of Paget-Schroetter-syndrome, controlled trial failed to prove a posi- the results of cohort studies applying usually embedded into a multimod- tive eff ect of botulinum toxin injec- either the transaxillary or the supra- al approach with catheter-directed tions vs. placebo injections to the clavicular approach, clinical success thrombolysis and angioplasty with scalene muscles on pain in subjects rates appear quite similar [4]. In a or without stent placement to re- with nTOS [28]. When conserva- small prospective randomised trial store venous patency [37]. Although tive treatment does not result in an comprising 55 patients with nTOS, an abundance of mainly retrospective improvement of patient´s symptoms Sheth et al. compared neuroplasty single centre studies reported impres- and related disability, surgical decom- (without fi rst rib resection) through a sive results of thrombolysis alone or pression of the brachial plexus can supraclavicular approach with trans- in combination with surgical de- be considered. Noteworthy, limited axillary resection of the fi rst rib. Vas- compression, high-quality evidence data from cohort studies comparing cular TOS and the presence of a cervi- proving the concept that invasive conservative and surgical treatment cal rib were exclusion criteria. During treatment reduces the frequency of yielded contradictory results but a mean follow-up of 37 months, sig- the post-thrombotic syndrome and raised concerns about the effi cacy of nifi cantly more patients in the group of recurrent thrombosis is lacking. surgical treatment in nTOS [39 – 41]. randomised to transaxillary fi rst rib Th e current ACCP-guidelines recom- An observational study indicated that resection reported clinical improve- mend anticoagulation treatment for ment (75 % vs. 48 %) [27]. Th e deci- a minimum duration of 3 months for a highly selective algorithm with ini- sion on which approach to use in an all patients. Catheter-directed throm- tial TOS-specifi c physical therapy individual patient should take into bolysis should be considered only for in all nTOS-patients and surgical account the pathoanatomy, the pres- a subgroup of patients with extensive decompression performed only in a ence of structural arterial damage thrombosis suff ering from severe subset of patients depending mainly and how familiar the surgeon is with symptoms for less than 14 days and on the success of conservative treat- the specifi c procedure. Endoscopic having a very low bleeding risk [38]. ment may improve the clinical success and computer-assisted (Da-Vinci- According to the guidelines, surgical rate of surgery [42]. Only in patients system) procedures for transaxillary decompression should be confi ned to with true nTOS suff ering from severe fi rst rib resection have been proposed exceptional circumstances [38]. On muscle weakness or atrophy a surgery http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 as alternatives to traditional open the contrary, fi rst rib resection and fi rst-strategy may be warranted [6, 29]. surgery [33, 34]. Whether these tech- venolysis appear to be reasonable In a study focusing on long-term niques bear the potential to reduce lo- treatment options in patients suff er- quality of life (QOL), Rochlin et al. cal complications needs to be clarifi ed ing from McCleery-Syndrome [11]. identifi ed several factors that pre- in future studies. Despite a lack of systematic data, at- dicted the clinical outcome of decom- tributable to the rarity of this disease pression surgery in nTOS patients. Treatment approach stratiﬁ ed variant, surgical decompression is Signifi cantly poorer scores on vali- according to the type of TOS generally accepted in aTOS to prevent dated QOL-assessment-instruments In vTOS presenting with primary up- (recurrent) ischemic events [6, 29]. were associated with comorbid per extremity deep venous thrombo- Surgery should be performed early chronic pain syndromes, opioid use, sis (Paget-Schroetter-Syndrome), the aft er restoration of distal blood fl ow smoking, age ≥40 years, neck and/or Vasa 2015; 44: 419 – 434 M. Czihal et al.: Vascular compression syndromes © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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shoulder disease and surgical com- most authors argue that obstruction Diagnostic workup plications [12]. of the celiac trunk secondary to ex- Th e optimal diagnostic workup is ternal compression is able to cause highly disputed. First of all, MALS is symptomatic ischemia of the upper a diagnosis of exclusion and should be Median arcuate gastrointestinal tract. Interestingly, considered in cases with abdominal ligament syndrome a more recent study applying gastric symptoms remaining unclear aft er (Dunbar`s syndrome) exercise tonometry challenged this thorough gastroenterological diag- theory as it documented a gradient nostic testing including endoscopy between gastric and arterial blood of the upper and lower gastrointesti- Deﬁ nition and epidemiology carbon dioxide level aft er exercise, nal tract. Physical examination may MALS is probably is the most con- indicating gastric ischemia second- reveal epigastric tenderness or an troversial vascular compression ary to blood redistribution in patients abdominal bruit. Th e most reason- syndrome. MALS was broadly rec- considered as having typical MALS able fi rst step in diagnostic imaging ognised as a clinical syndrome aft er [48]. A postprandial steal phenom- is functional colour duplex sonogra- publication of a series comprising enon via collaterals to the superior phy with respiratory manoeuvres. In 15 patients treated with surgical mesenteric artery may contribute to MALS, a signifi cant increase in PSV decompression by Dunbar et al. in gastric ischemia. Another disputed with deep expiration can be observed. 1965 [43]. Epidemiologic data are mechanism is compression and irri- A case-control study limited by its not available. Th e female to male ra- tation of the celiac plexus resulting very small sample size suggested a tio is estimated at 3:1 and the typical in alteration of gastric myoelectrical peak systolic expiratory fl ow veloc- patients are women with an asthenic activity [49]. ity of more than 350 cm/s together habitus aged between 20 and 40 years. Fixed stenosis with post-stenotic with a celiac trunk defl ection angle However, MALS has been reported in dilatation of the celiac trunk is fre- during expiration of more than 50° as patients of any age group [44]. quently seen in MALS and a result a diagnostic criterion for MALS (sen- of structural vessel wall alterations sitivity 83 %, specifi city 100 %) [51]. Pathoanatomy secondary to chronic compres- Computed tomography and magnetic Th e median arcuate ligament is a sion. Some cases with aneurysms resonance tomography allow excel- musculofi brous arch bridging the of the pancreaticoduodenal artery lent visualization of external com- crura of the diaphragm to form the in association to MALS have been pression of the celiac trunk, but intra- aortic hiatus. It traverses the aorta described, possibly related to the arterial angiography, performed with above the origin of the celiac trunk, increased collateral blood fl ow via respiratory manoeuvres remains the and the celiac ganglion is adjacent to this artery [50]. reference standard of diagnostic im- the ligament. Imaging studies showed aging in MALS. A hook-shaped ap- some degree of external compression Clinical presentation pearance of the celiac trunk observed of the celiac trunk resulting from the MALS may present with a variety of in the sagittal plane during expiration median arcuate ligament in about symptoms, with the classical triad is the typical angiographic fi nding in one of fi ve asymptomatic individuals consisting of postprandial abdominal MALS (Fig. 5). Provocation of a steal [45]. Low insertion of the diaphragm pain, weight loss and nausea/vomit- phenomenon by direct vasodilator and a high origin of the celiac trunk ing. However, presentation with un- injection into the superior mesen- are considered to increase the risk specifi c complaints such as chronic, teric artery has been suggested as a of celiac artery compression [46]. continuous or intermittent, vague diagnostic criterion for MALS [52]. http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 Compression is mostly pronounced pain epigastric pain, sometimes ra- Intra-arterial pressure measurement in expiration and can aff ect also the diating to the fl anks or back, seems and intravascular ultrasound may superior mesenteric artery and rarely to be common. A more characteris- further elucidate the dynamic charac- the renal arteries. tic presentation is exercise-induced ter of celiac artery stenosis secondary In MALS, positional compression abdominal pain in young athletes to external compression [53] of the celiac artery is associated who may experience symptom relief Gastric exercise tonometry may with clinical symptoms. However, when leaning forward [46]. In case of substantially add to the diagnosis the actual pathomechanism behind compression of the renal arteries by of MALS. According to a proto- the clinical syndrome still remains the diaphragmatic crura, the leading col published by Otte et al., arterial to be elucidated. Given the abun- clinical consequence is renovascular blood gas analysis and gastric pCO2- dant splanchnic collateral circulation, hypertension [47]. measurement via a nasogastric tube M. Czihal et al.: Vascular compression syndromes Vasa 2015; 44: 419 – 434 © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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is frequently required due to fi brous stenosis, occlusion or even aneu- rysmal degeneration. Open surgery allows direct reconstruction (patch or aorto-coeliac bypass), whereas laparascopic treatment requires an additional endovascular approach with stenting of the celiac artery. Robot-assisted laparoscopic surgery has been performed successfully in a limited number of MALS-patients [56]. In a systematic review of 21 retrospective studies on 400 patients, complete Figure 5: Selective intra-arterial angiography of the celiac trunk in a fe- postoperative symptom relief was male patient suffering from median arcuate ligament syndrom (MALS): achieved in 96 % and 78 % aft er laparo- Fixed stenosis of the celiac trunk origin visible during inspiration (arrow) scopic and open surgery, respectively (A). Further luminal narrowing resulting in subtotal stenosis after expi- [57]. Additional endovascular proce- ration (arrow) (B). Courtesy of Prof. Gunnar Tepe, Rosenheim, Germany. dures were required in 12 % of patients who underwent laparoscopic surgery. Th e frequency of late symptom reoc- are performed at rest and aft er 10 [55]. Independent validation of these currence did not diff er between the minutes of submaximal exercise on promising results by other working treatment modalities (6 – 7 %). Th us, a cycle ergometer. Eff ective gastric groups is pending. the individual patient needs to be in- acid suppression prior the test with formed explicitly that there remains a proton pump inhibitors is manda- Treatment small chance of symptom persistence tory. Th e main criterion for a positive When the diagnosis of MALS is or recurrence despite proper surgical (pathological test) is a post-exercise considered to be very likely, surgical treatment. In 9.1 % of subjects who gradient between gastric and arterial decompression is the treatment of underwent laparoscopic treatment, blood carbon dioxide of > 6 mmHg choice. Both open surgical decom- bleeding complications forced con- (0.8 kPa) [54]. Th e combination of pression, performed via a short upper version to open surgery [57]. Recent- colour duplex sonography and gas- midline laparotomy, and laparoscopic ly, Klimas et al. showed similar rates tric exercise tonometry has been decompression comprise transection of immediate postoperative symp- shown to have excellent diagnostic of the median arcuate ligament and tom relief (100 %) and recurrence of accuracy in the diagnosis of chronic the crus of the diaphragm proximal to symptoms (6.9 %) in a large series of gastrointestinal ischemia and hence the celiac artery as well as transection children and adolescents who under- may be a reasonable fi rst line diag- of the ganglionic tissue over the aorta. went laparoscopic decompression of nostic approach in suspected MALS Revascularisation of the celiac artery the celiac trunk for MALS [58].

Table III: Popliteal Vascular Entrapment Forum classifi cation for popliteal entrapment syndrome [60, 61] http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 Classification Pathomechanism Type I Popliteal artery displaced medially by the medial head of the gastrocnemius muscle Type II Medial head of the gastrocnemius head attached laterally to the popliteal artery Accessory muscle strings/fi brous bands arising from the medial head of the gastrocnemius Type III muscle Popliteal artery compressed by muscle strings/fi brous bands arising from the popliteal Type IV muscle Type V Entrapment of the popliteal vein Type VI Other variants Type F Functional entrapment Vasa 2015; 44: 419 – 434 M. Czihal et al.: Vascular compression syndromes © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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Endovascular treatment by means of nal development. A detailed descrip- 61]. Repetitive compression may lead celiac artery stenting without prior tion of the embryological fundamen- to intimal damage, mural thrombus release of external compression is not tals of PES is provided elsewhere [60, formation, stenosis or aneurysm recommended due to the risk of stent fracture/crush [46].

Popliteal entrapment syndrome

Deﬁ nition and epidemiology PES describes a group of conditions characterised by clinical symptoms arising from compression of the popliteal artery, the popliteal vein, and/ or the tibial nerve in the popliteal fossa by surrounding musculoskel- etal structures [59 – 62]. Although anatomic abnormalities obviating the course of the popliteal vessels have been reported much earlier, the term PES was fi rst introduced in 1965 [63]. Th e prevalence of PES in the general population is basically unknown. According to a systematic review the mean age at the time of diagnosis was 32 (range 20.7 – 41) years, with a median percentage of 83 % male patients [62].

Pathoanatomy A considerable heterogeneity in the use of diff erent classifi cation systems in published studies on PES must be acknowledged [62]. Nowadays broadly accepted is the classifi cation system proposed by the popliteal vascular entrapment forum in 1998 (Tab. III) [60, 61]. Types I to IV refer to anatomical en- http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 trapment of the popliteal artery. Th e basis for PES types I-III is an abnormal medial migration of the medial head of the gastrocnemius muscle during embryogenesis, resulting in medial Figure 6: Eleven-year old girl presenting with acute occlusion of the right deviation and/or compression of the popliteal artery. Magnetic resonance -imaging documents medial devia- popliteal artery (Fig. 6,7). Type IV tion of the occluded artery and compression between the medial head (popliteal artery entrapment by the of the gastrocnemius muscle (#) and the semimembranosus muscle (*), popliteal muscle) can be explained corresponding to popliteal entrapment syndrome (PES) type I. The left by the persistence of a segment of the popliteal artery has a regular anatomical course. primitive axial artery during embryo- M. Czihal et al.: Vascular compression syndromes Vasa 2015; 44: 419 – 434 © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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of intermittent claudication during walking or running, but acute limb ischemia secondary to local thrombosis or distal embolism occurs is present at the time of diagnosis in 11 % of symptomatic limbs [62]. If the diagnosis is initially missed, aff ected limbs are at risk of developing critical limb ischemia due to recurrent embolism. Th e frequency of bilateral symptomatic PES varies considerably between studies (0 – 100 %, median 38 %) [62]. Diagnosis of unilateral symptomatic PES should necessarily prompt investigation of the asymptomatic contralateral leg. Th e main symptom of venous entrapment is calf swelling, whereas tibial nerve entrapment typically goes along with pain in the popliteal Figure 7: Young male patient complaining of bilateral calf claudication. fossa and paresthesia/numbness of MR-angiography without provocative manoeuvres reveals no pathologi- the foot. cal fi ndings (A). Computed tomography-angiography performed during In a systematic review, the median plantar fl exion shows fi liform narrowing of the popliteal artery on both duration of symptoms until the cor- sides (B,C). Intraoperatively, an abnormal lateral insertion of the hyper- rect diagnosis could be established trophied medial head of the gastrocnemius muscle (#) is confi rmed, was 12 months. Noteworthy, a cor- corresponding to popliteal entrapment syndrome (PES) type II (D). relation between duration of symptoms and the presence of irreversible structural vascular damage could not formation. Structural arterial dam- to 88 % of asymptomatic individuals, be established [62]. age, as seen in approximately 10 % of clearly indicating that the phenom- Th e neuromuscular symptoms (calf patients, carries the inherent risk of enon per se does not imply a clinically cramps, plantar paresthesia) of func- local thrombotic occlusion and distal relevant disease [65, 66]. If the popli- tional PES may resemble those of embolisation. teal vein is additionally involved, the chronic recurrent exertional compart- Functional entrapment of the pop- pathology is classifi ed as type V. Th e ment syndrome (CRECS), a disorder liteal artery occurs in the absence true rate of popliteal vein entrapment, also typically occurring in young phys- of anatomical abnormalities and is with or without concomitant arterial ically active adults [64]. By contrast to found in about one out of four PES- involvement, is basically unknown, as classical claudication, lower limb pain cases [62, 64]. Hypertrophy of the is the rate of tibial nerve compression in CRECS usually persists over hours gastrocnemius muscles, as frequently in the popliteal fossa. and days aft er exercise [67]. seen in competitive athletes and per- http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 sons abusing anabolic steroids, has Clinical presentation Diagnostic approach been attributed to be causative for Among other disorders such as large As in other vascular compression positional compression [59]. How- vessel vasculitis, persistence of the syndromes, the diagnostic approach ever, Hoff mann et al. observed no sciatic artery, adventitial cystic dis- has not been standardised. Th e fi rst diff erences in the rate of signifi cant ease and iliac artery endofi brosis, PES diagnostic step is non-invasive vas- popliteal artery compression during is an important diff erential diagnosis cular laboratory testing, including plantar fl exion in normally vs. highly of symptomatic lower leg ischemia in systolic ankle pressure measurement, trained individuals [65]. In this and younger patients. Th e absence of car- pulse volume recording, and colour other studies, some degree of popli- diovascular risk factors and an athlet- duplex sonography. When performed teal artery compression with maxi- ic habitus should heighten the index with adequate provocation manoeu- mal plantar fl exion was found in 53 of suspicion. Most patients complain vres by means of forced active plantar Vasa 2015; 44: 419 – 434 M. Czihal et al.: Vascular compression syndromes © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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fl exion, sensitivities between 90 and should be reserved for cases remain- situation, a medial approach is pref- 100 % have been reported for each of ing ambiguous despite previous cross erable, facilitating harvesting of the the aforementioned techniques in a sectional imaging studies or when great saphenous vein and also off er- limited number of studies which used endovascular treatment of acute leg ing quicker return to physical activity surgical confi rmation as the reference ischemia is intended. [61]. In case of popliteal artery wall standard [62]. Colour duplex sonog- Popliteal artery occlusion, found in a lesions without thrombotic occlusion raphy off ers the opportunity to depict median of 24 % of patients at the time or distal embolic occlusions, inter- both morphological changes of the of diagnosis [62], may hamper the di- position of a short popliteo-popliteal vascular structures (focal wall thick- agnostic eff orts to uncover a vascu- saphenous vein graft is the method of ening, stenosis with post-stenotic an- lar compression syndrome. In these choice. When acute thromboembolic eurysm, thrombotic occlusion) and cases, evidence of popliteal artery occlusions of the below the knee- haemodynamic changes provoked by compression on the contralateral leg arteries are present, consideration forced active plantar fl exion. In some may further underline the suspected should be given to catheter-directed cases, the underlying anatomic ab- diagnosis of PES. thrombolysis in order to improve the normality may also be detected, for arterial outfl ow. Subsequent surgical instance a muscular slip between Treatment revascularization and decompression the popliteal artery and vein [68]. It is generally accepted that anatomi- of the popliteal artery are mandatory, Treadmill testing is useful for further cal PES requires surgical treatment, as otherwise recurrent ischemia is characterisation of the functional rel- including decompression of the pop- very likely. In case of popliteocrural evance of the disorder when present- liteal vessels and, if structural vessel obstructions not approachable by ening with intermittent claudication. damage has occurred, arterial or ve- dovascular techniques, femorocrural In recent years, cross-sectional im- nous revascularisation [59 – 62]. Th is vein bypass surgery is required. In the aging techniques (MR- and CT-an- recommendation, however, is mainly young, otherwise healthy patients, the giography) have been increasingly based on evidence coming from ret- saphenous veins are usually eligible using in the diagnostic workup of rospective cohort studies. Interpre- as graft material. Patency rates have suspected TOS. Th ese techniques tation of these studies is limited by been assumed to be generally better allow excellent visualisation of the several factors, including diff erences than in arteriosclerotic peripheral popliteal fossa`s anatomy and detec- in cohort characteristics and the lack arterial disease, but Sinha et al. ob- tion of abnormal musculotendinous of standardised outcome measures. served a median failure rate of 27 % structures (Fig. 6, 7). In neutral posi- Indeed, half of the studies included (range 0 – 88 %) in their systematic tion, structural abnormalities of the in a systematic review did not clearly review [62]. popliteal vessels and, in PES type I, describe symptom resolution aft er Treatment success as indicated by a medial deviation of the artery can surgery [62]. postoperative resolution of symp- be found. Repeated imaging during For surgical treatment, either a dor- toms was reported in a median of forced plantar fl exion has been shown sal or a medial approach can be used. 77 % (range 70 – 100 %) of patients to have excellent sensitivity for the Th e choice between both techniques with symptomatic arterial compres- diagnosis of PES [62]. MRI has the is primarily guided by the presence or sion and in 53 % (range 48 – 57 %) of advantage of being free of radiation absence of structural vessel damage. patients with symptomatic venous exposure, but movement artefacts In the absence of arterial injury, the compression [62]. Minor or major during image acquisition in provoca- posterior access is more favourable amputations had to be performed tion position may limit its diagnostic as it allows excellent visualisation of only in exceptional cases. Data on http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 accuracy [59]. the muscular/ligamentous structures the long-term outcome of revascu- Traditionally, PES has been diagnosed being causative for entrapment [61]. larisation operations in the treatment by conventional digital subtractive ar- Th ere is no consensus on whether or of PES are scarce. In the currently terio- or venography with provoca- not to perform reattachment of the largest retrospective cohort study, tion of vascular compression (forced medial head of the gastrocnemius comprising 88 limbs of 48 patients, active plantar fl exion). Although still muscle to the medial femur condyle the primary patency rate of 15 re- considered the reference imaging aft er transection [62]. versed saphenous vein graft s was method, conventional angiography When arterial damage has occurred, 100 % aft er a median follow-up of do not provide anatomical informa- standard techniques of reconstruc- 4.2 years (range 1 – 10 years), as was tion on the perivascular structures re- tion must be employed in addition the primary patency rate of the pop- sponsible for compression, and thus to surgical decompression. In this liteal arteries in all limbs managed M. Czihal et al.: Vascular compression syndromes Vasa 2015; 44: 419 – 434 © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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Table IV: Rare vascular compression syndromes [73-80]

Syndrome Pathoanatomy Symptoms Diagnosis Treatment Eagle syndrome Internal carotid artery Neck pain, dysphagia, compression by an abnor- cranial nerve palsies, mally elongated styloid Horner`s syndrome. process Rare: transient ischemic attack and stroke in the Resection of the anterior cerebral circu- Intra-arterial angiogra- abnormal structure(s); lation phy with provocative Anticoagulation with Rotational Cervical spine patholo- Dizziness or Syncope manoeuvres; spiral CT of or without surgical re- vertebral artery gies, e.g. osteophytes and occurring with head the head and neck with vascularization in case ischemia discus prolapse, leading to rotation. 3D-reconstruction of structural arterial positional vertebral artery damage. (Bow hunter`s compression Rare: posterior circulation syndrome) stroke secondary to vertebral artery dissection/ occlusion (“Bow hunter`s stroke”) Langer`s axillary Accessory muscular band Symptoms of intermit- Colour duplex sonogra- Resection of the aber- arch crossing the axilla, found tent upper extremity phy; venography; magne- rant muscle bundle in up to 8% of the general venous congestion; tic resonance imaging population. Axillary vein Upper extremity deep obstruction and or throm- venous thrombosis bosis is a very uncommon complication. Axillary artery Repetitive compression Typically occurring in History of professional Staged revascularizati- / posterior cir- of the third portion of overhead athletes (base- overhead sports activities; on including throm- cumfl ex humeral the axillary artery or the ball pitchers, volleyball Intra-arterial angiography bectomy or throm- artery (PCHA) PCHA against the head of players). Arm claudicati- bolysis, exclusion of injury the humerus on secondary to axillary PHCA aneurysm and/ artery occlusion; acral or partial axillary artery ischemia due to periphe- resection with vein ral embolism from poste- graft interposition rior circumfl ex humeral artery aneurysms Brachial artery Compression of the bra- Intermittent claudication Muscular men with Surgical release of the compression syn- chial artery in the cubital of the forearm; hand and hypertrophied forearm lacertus fi brosus drome (Popeye fossa due to hypertrophy fi nger ischemia following muscles; pain and disap- syndrome) of forearm muscles or the local arterial thrombosis pearance of the radial lacertus fi brosus with or without periphe- pulse with forearm pro- ral embolism nation and resisted elbow fl exion; colour duplex sonography and intra-arterial angiography with provocative manoeuvres

http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 External iliac ar- Multifactorial compres- Typically occurring in Competitive athletes, par- Adjustment of cycling tery endofi brosis sion and tethering of the competitive cyclists. ticularly professional or posture, reduction external iliac artery (main Thigh pain, weakness or top amateur cyclists. Iliac of cycling intensity. factors: repetitive hip hy- cramps at (sub)maximum artery bruit; ankle pres- Surgical or endovascu- perfl exion, psoas muscle exercise or during hip hy- sure drop after cycle-er- lar treatment should hypertrophy), resulting in perfl exion, predominantly gometer based exercise be avoided if possible. vessel tortuosity, kinking left leg. (standard treadmill testing Surgical release of the and endofi brosis. usually without abnormal iliac artery is the only results); colour duplex invasive procedure sonography; magnetic supported by low level resonance imaging with evidence. provocative manoeuvre (hip hyperfl exion) Vasa 2015; 44: 419 – 434 M. Czihal et al.: Vascular compression syndromes © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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Syndrome Pathoanatomy Symptoms Diagnosis Treatment Adductor canal Intimal damage with Unilateral superfi cial fe- Diffi cult with diagnostic Surgical decompression compression subsequent arterial moral artery obstructions imaging; evidence for ex- during femoropopliteal syndrome thrombosis at the outlet of occurring in younger ternal compression during graft interposition the adductor canal where male patients without surgical revascularization the neurovascular bundle obvious cardiovascular crosses the vastoadductor risk profi le, particularly membrane competitive athletes Dorsalis pedis Compression of the Foot claudication, blue Cessation of the dorsa- Transection of the artery entrapment dorsalis pedis artery by the toe syndrome lis pedis artery Doppler extensor hallucis brevis syndrome extensor hallucis brevis signal during dorsifl exion tendon and transpositi- tendon of the foot on to extensor hallucus longus tendon Left renal vein Compression of the Left fl ank and abdominal Urological investigations Dependent on the entrapment syn- renal vein between the pain; haematuria; clinical to confi rm unilateral severity of symptoms drome aorta and the steep-angled manifestations attri- haematuria and rule out and patient`s age: Sur- origin of the superior me- butable to left renal-to other sources of haema- veillance vs. surgical or (Nutcracker syn- senteric artery (anterior gonadal vein refl ux, turia; colour duplex so- endovascular revascula- drome) nutcracker syndrome) or i.e. symptoms of pelvic nography; cross sectional rization between the aorta and the congestion syndrome, imaging; retrograde veno- vertebral column (poste- varicocele, lower limb graphy with assessment rior nutcracker syndrome varices. of the reno-caval pressure in case of retroartic course gradient of the left renal vein)

by surgical decompression only (me- No clear recommendations can be comes from retrospective case series dian follow-up 3.9 years) [69]. More made concerning primarily asymp- [73 – 80]. Th is is particularly true recently, study groups from Japan tomatic patients with arterial com- for compression syndromes of the and Korea published follow-up data pression. It seems reasonable to extremities, with less than 20 cases aft er vein graft interposition/bypass perform preventive decompressive each reported in the literature. A con- surgery, demonstrating primary graft surgery of the asymptomatic contra- cise overview is provided in Table IV. patency rates at 5 years between 74 lateral leg in patients with symptom- Recently, a contribution of external and 100 % [70 – 72]. Th e patency rate atic PES to avoid arterial ischemic compression between the sternum was negatively infl uenced by outfl ow complications. Refl ecting current and the aortic arch to the develop- obstruction and increasing length of clinical practice, this approach was ment of left innominate vein stenosis the bypass in the study by Kim et al. applied in almost all studies reporting in haemodialysis patients has been [70]. Of note, bypass occlusions in the surgical treatment in PES [62]. suggested [81]. very long-term (12 and 23 years aft er In addition to classic compression surgery) have been reported [71]. An- syndromes, resulting from entrap- eurysmatic degeneration of venous Other vascular ment of blood vessels by adjacent interponates is another important compression syndromes anatomical structures, abnormali- complication in the long-term. In this ties in the course or morphology of case, persistent compression should As mentioned above, May-Th urner- large and medium sized arteries may http://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000465 - Monday, March 06, 2017 10:38:24 PM Harvard University IP Address:128.103.149.52 be ruled out. Th ese fi ndings under- Syndrome and Hypothenar-Hammer result in the compression of adjacent score the need for regular long-term Syndrome were subjected to dedi- organs. Superior mesenteric artery surveillance at least of patients who cated reviews in recent issues of this syndrome (Wilkie`s syndrome), for underwent revascularisation proce- journal [1, 2]. Th erefore, we did not instance, is characterised by compres- dures. include either condition in the pression of the third portion of the duode- Very limited data suggest that a con- ent work. num by the narrow-angled superior servative approach with cessation A variety of other vascular compres- mesenteric artery. Other examples of intense sports activities may be sion syndromes has been reported. are left main bronchial obstruction an appropriate treatment approach Related to the rarity of these disor- by large thoracic aortic aneurysms, in PES with functional entrapment ders, evidence for diagnostic and and a right aberrant subclavian artery [69]. therapeutic decision making at best (arteria lusoria) leading to oesopha- M. Czihal et al.: Vascular compression syndromes Vasa 2015; 44: 419 – 434 © 2015 Hans Huber Publishers, Hogrefe AG, Bern

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Proceedings of the staff meetings. Mayo computed tomography in 48 patients. Joint geal obstruction. Although primarily Clinic 1956; 31: 281 – 287. Bone Spine 2001; 68 (5): 416 – 24. caused by a vascular disease process, 4 Sanders RJ, Hammond SL, Rao NM. Th o- 19 Molina JE, D’Cunha J. Th e vascular com- the clinical manifestations implicate racic outlet syndrome: a review. Neurolo- ponent in neurogenic-arterial thoracic that patients with such disorders gist 2008; 14 (6): 365 – 73. outlet syndrome. Intern Angiol 2008; 17 primarily present to other medical 5 Sanders RJ, Hammond SL, Rao NM. Diag- (2): 83 – 87. nosis of thoracic outlet syndrome. J Vasc 20 Aralasmak A, Cevikol C, Karaali K, et al. subspecialties. Surg. 2007; 46: 601 – 604. MRI fi ndings in thoracic outlet syn- 6 Huang JH, Zager EL. Th oracic outlet drome. Skeletal radiology 2012; 41 (11): syndrome. Neurosurgery 2004; 55 (4): 1365 – 1374. 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