CASE REPORT
Ar t e r ia l com plica t ion s of t h or a cic ou t le t syn dr om e
Fe r na ndo Th om a zin h o, W a nde r Edu a r do Sa r din h a , Jose M a noe l da Silva Silve st r e , D om ingos de M or a is Filh o, Fe r na ndo M ot t a*
*Depart m ent of Surgical Clinic, Vascular Surgery Service, Hospit al Universit ário Est adual do Nort e do Paraná ( HURNP) , Universidade Est adual de Londrina, Londrina, PR, Brazil.
Correspondence
J Vasc Bras. 2008; 7( 1) : 150- 154.
ABSTRACT
The clinical m anifest at ions of t horacic out let syndrom e are m ainly neurological. Alt hough art erial com plicat ions are rare, t hey are pot ent ially severe. Am ong t hese are aneurysm s associat ed wit h em bolism and t hrom bosis. The aut hors report a case of a 37 year- old wom an wit h bilat eral cervical rib t hat developed em bolism in t he right upper lim b from a post st enot ic right subclavian art ery aneurysm and dilat at ion of t he left subclavian art ery, bot h due t o com pression.
Ke yw or ds: Thoracic out let syndrom e, cervical rib, art erial com plicat ions.
RESUM O
As m anifest ações clínicas da síndrom e do desfiladeiro t orácico são predom inant em ent e neurológicas, sendo as com plicações art eriais raras, m as pot encialm ent e graves. Ent re elas, devem os cit ar os aneurism as com com plicações em bólicas e a t rom bose. Os aut ores relat am o caso de um a m ulher de 37 anos com cost ela cervical bilat eral que apresent ou em bolia no m em bro superior direit o originada de um aneurism a pós- est enót ico da art éria subclávia direit a, além de apresent ar ect asia da subclávia esquerda t am bém por com pressão.
Pa la vr a s- cha ve : Síndrom e do desfiladeiro t orácico, cost ela cervical, com plicações art eriais.
The expression " t horacic out let syndrom e" ( TOS) was first used by Peet1 t o describe pat ient s w it h
neurovascular sym pt om s relat ive t o possible sit es of com pression of t he neurovascular bundle, which are basically t hree: int erscalene t riangle, cost oclavicular space and ret ro- pect oralis m inor space.
I t is believed t hat t he com binat ion of t wo fact ors is required t o cause t his syndrom e: 1) anat om ical narrowing; 2) som e t ype of t raum a t hat t riggers sym pt om s.2
Com pression in t he int erscalene t riangle, known as scalene syndrom e, has neurological and art erial sym pt om s, t here are no venous sym pt om s, since t he subclavian vein is not cont ained in t his
t riangle. Cost oclavicular com pression syndrom e can com press any bundle st ruct ure. Com pression in t he ret ro- pect oralis space, or sm all pect oral syndrom e, is rare, and t reat m ent is essent ially clinical.
There are ot her possible com pression sit es,3 wit h sim ilar m anifest at ions. Am ong t hem we can cit e t he m edian syndrom e, in which t here is com pression of t he axillary art ery bet ween brachial plexus bundles, and Langer's syndrom e, in which t here is com pression of t he vasculonervous bundle by an anom alous m uscle, t he axillopect oral m uscle.
The m ain sym pt om s are pain and parest esias, which occur spont aneously or are caused or exacerbat ed by m ovem ent s t hat reduce t he dim ensions of pot ent ial com pressions spaces. I t s dist ribut ion is m ore frequent in t he volar aspect of t he forearm and of t he last t wo fingers.
I nit ial hist oric publicat ions described alm ost exclusively vascular m anifest at ions.4 Throughout t im e,
m ore knowledge of t his syndrom e was acquired; it has been observed t hat neurological sym pt om s were m uch m ore frequent , and current ly it is known t hat t hey account for m ost cases,
corresponding t o approxim at ely 95% of clinical com plaint s. Presence of changes in art erial pulses, such as m aneuvers t hat sim ulat e com pression, do not necessarily indicat e t hat t here is art erial lesion.
Art erial com plicat ions are rare, but pot ent ially m ore severe t han neurological m anifest at ions, and m ay lead t o significant sequelae.
The aut hors report t he case of a pat ient wit h acut e art erial occlusion in t he right upper lim b due t o em bolism secondary t o a post st enot ic aneurysm as TOS com plicat ion.
Ca se r e por t
A 36- year- old fem ale pat ient present ed w it h com plaint of cyanosis, pallor and parest esias in t he right upper lim b for 3 days. She denied com plaint of previous parest esia in t he upper lim bs. She had no com orbidit y, was not a sm oker and was not using oral cont racept ives.
On init ial physical exam inat ion, t he pat ient had finger pallor and right hand w it h react ive hyperem ia. She had no radial and ulnar pulses in t he right upper lim b, norm al pulses in t he cont ralat eral lim b and in t he lower lim bs. Adson's m aneuvers perform ed bilat erally had posit ive result s.
Doppler ult rasound of t he upper lim bs was perform ed, showing occlusion of t he radial and ulnar art eries in t he proxim al t hird of t he right forearm . I t also showed posit ivit y for art erial com pression t est s t hrough scalene m aneuvers, wit h expressive reduct ion in peak syst olic velocit y in t he axillary art ery.
Digit al angiography t hrough fem oral punct ure showed right subclavian art ery aneurysm , wit h em bolic occlusion of forearm art eries, and left subclavian art ery ect asia (Figure 2).
Thirt y days lat er, she was subm it t ed t o resect ion of t he left cervical rib using ant erior scalenect om y. She had good evolut ion and no com plicat ions.
D iscu ssion
Cervical ribs and t he first anom alous r ib are rare condit ions, present in approxim at ely 1% of t he populat ion and in 4.5% of pat ient s w it h TOS.5 They are usually findings of im aging exam inat ions,
such as chest x - rays. Most are com plet ely asym pt om at ic, and rarely require t reat m ent . Cervical ribs and t he first anom alous rib m ay have com plicat ions t hat are pot ent ial t hreat s t o t he upper lim b, requiring regular follow- up.
According t o Sanders et al.,5 sym pt om onset is preceded by cervical t raum a in 80% of cases, and
20% of cases occur spont aneously. However, in a subgroup of 12 pat ient s who had com plet e cervical rib, t he incidence of spont aneous sym pt om s reached 50% . Of a t ot al of 40 pat ient s w it h cervical rib, it m easured 1- 2 cm in 11 cases; 2- 5 cm in 17 cases; and were com plet e in t he ot her 12 pat ient s. I n cases of com plet e rib, t hree were art iculat ed direct ly wit h t he first rib, and t he ot hers were insert ed in t he last rib t hrough a fibrous ligam ent .
The t ype of cervical r ib is of great significance in art erial com plicat ions. I t has been well est ablished, aft er a st udy by Gruber,6 t hat short ( t ype I ) and incom plet e ribs ( t ype I I )
preferent ially produce neurological com plicat ions, while long or com plet e ribs ( t ype I I I ) have art erial com plicat ions.6 Sanders et al.7 confirm ed t he sam e findings. Short et al.4 showed t hat 75% of pat ient s wit h incom plet e cervical rib had t heir sym pt om onset associat ed wit h cervical t raum a, while in pat ient s wit h com plet e cervical rib only 50% of cases had t his associat ion.
Our pat ient had com plet e bilat eral cervical rib ( t ype I I I ) , wit h art erial com plicat ions, right subclavian art ery aneurysm and left subclavian art ery ect asia. The m ost severe dilat at ion t o t he right was possible due t o t he fact t hat t he pat ient is right- handed, since m ost st udies at t em pt t o correlat e TOS wit h upper lim b t raum a.8
occipit al headache. Report s of finger parest esias are com m on, m ore frequent ly in t he fourt h and fift h fingers, due t o com pression of lower root s of t he ulnar nerve, reduct ion in hand and arm st rengt h, and t here is usually sym pt om worsening wit h lim b elevat ion. There m ay also be reduced t em perat ure, Raynaud's phenom enon and increased hand sweat ing. The incidence of each
sym pt om is sim ilar t o t hat of pat ient s who have no cervical r ib.
Art erial lesions in TOS are usually due t o bone abnorm alit ies. Findings in t he lit erat ure correlat e wit h presence of cervical rib in 70- 100% of cases. I n t hese pat ient s t he subclavian art ery passes over t he cervical rib ( which com presses it in it s inferior aspect ) and produces an int im al lesion wit h or wit hout post st enot ic dilat at ion.9 Such pat ient s oft en have em bolic com plicat ions in t heir course,
which are t he m ost disabling aspect of t his disease and it s t reat m ent is difficult . Art erial
com plicat ions t end t o be t reat ed lat e in relat ion t o t heir neurological equivalent s. I n fact , m ost of t he t im es t he basal problem is not recognized unt il t he t hrom boem bolism has occurred, whet her due t o an int im al lesion of t he subclavicular segm ent or due t o post st enot ic dilat at ion or
aneurysm .1 0
Presence of anom alous first rib and m uscle changes increases probabilit y of art erial lesion and em bolic phenom ena. The first anom alous cervical rib leads t o changes sim ilar t o t hose of t he cervical rib, but it affect s t he art ery in it s upper aspect . Ant erior scalene m uscle hypert rophy in at hlet es is associat ed wit h subclavian art ery im pairm ent , and it s occlusion wit h dist al em bolism .1 0
The need of early det ect ion of lesions caused by em boli at an early st age is ext rem ely im port ant , and all pat ient s wit h first rib abnorm alit ies or wit h cervical r ib should be regularly followed using Doppler ult rasound of t he subclavian- axillary segm ent .1 0 Every st enot ic segm ent or presence of
aneurysm should be lat er assessed by angiography t o program a surgical int ervent ion.
Many pat ient s w it h severe ischem ia who seek for m edical assist ance have previous hist ory of warning episodes. Pat ient s wit h hist ory of unilat eral Raynaud's phenom enon ( especially in t he radial t errit ory)1 1 and pat ient s experiencing sudden pain in t he digit al pulp wit h vasom ot or changes
( pallor or cyanosis) should be suspect ed of having an em bolic event and Doppler ult rasound and angiography of t he t horacic out let should be perform ed.1 2
Chest and cervical spine x- rays are ext rem ely im port ant t o reveal bone changes. Com put ed t om ography can be useful in t he TOS diagnosis, especially in pat ient s in whom x- rays are
norm al.1 3 Magnet ic resonance has proved t o be efficient in showing fibrot ic bands and deviat ion of t he brachial plexus in pat ient s wit hout cervical r ib.1 4 Elect rodiagnost ic assessm ent is useful for t he
diagnosis of com pression in t he carpus and Guyon's canal, but it is alt ered for t he diagnosis of TOS due t o t he posit ion in which t his t est is perform ed.1 5 , 1 6 Kom anet sky et al., in a st udy of 21 pat ient s
wit h TOS and 23 cont rols, in a relaxed and forced posit ion, did not consider t his t est useful for diagnosis.1 7
The t reat m ent of t his disease can be conserved t hrough physical t herapy, rest of t he act ivit y t hat caused t he sym pt om s and analgesia when t he syndrom e is m ainly of neurological im pairm ent .1
Surgical t reat m ent should be indicat ed in cases of failure in clinical t reat m ent ( aft er 6 m ont hs wit hout im provem ent or sym pt om recurrence) ,1 uncont rolled pain and parest esia, in addit ion t o vascular im pairm ent ( pallor, reduced pulses, cyanosis, Raynaud's phenom enon,1 1 lim b claudicat ion or pain at rest or ulcers) .1
Studies have shown that success rates have been similar between both techniques,5,7although the
supraclavicular approach allows for a better visualization of the scalene muscle. In addition. when the scalene muscle has any abnormality that could be responsible for the syndrome, it is possible to incise it at this moment; on the other hand, using the transaxillary approach, such procedure would be performed at a different surgical time.
One of the main complications of the surgery has been the phrenic nerve lesion,5 which usually
regressed after 3 months in most cases. Other complications that have been reported are subclavian artery and vein lesions. The first is much more frequent than the second.5,7,19,20
Pneumothorax has also been reported. It is more frequent when the transaxillary approach is used,7,19,21 but thoracocentesis was rarely required.
As the patient of the present report had a vascular TOS (pure arterial impairment) due to compression of a real cervical rib, a supraclavicular approach was used with resection of the cervical rib associated with right scalenectomy. Furthermore, aneurysm resection was performed using an interposed saphenous vein graft. That approach was adopted due to the unrestricted exposure of the interscalene triangle and good access for cervical rib resection.7,12 The same
technique was used for cervical rib resection on the left side, with no need of arterial resection, since the subclavian artery in that side was only dilated.
TOS is a common disease that has complex anatomy and a large anatomical variation of
structures, which should be managed by a specialized team and vascular surgeon experienced in this type of surgery.
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