„Truncus-coeliacus-Kompressionssyndrom“ – Versionsunterschied

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Das '''Truncus-coeliacus-Kompressions-Syndrom''' <small>(im Folgenden TCS abgekürzt)</small> auch als Dunbar-Syndrom bezeichnet, äussert sich durch Bauchschmerzen, verursacht durch Einklemmung der Arteria coeliaca (oder [[Truncus coeliacus]] genannt]) und möglicherweise des [[Ganglion celiacum]] durch das [[Zwerchfell]] (genauer durch das Ligamentum arcuatum mediale) <ref>Horton</ref>. Die Bauchschmerzen können im Zusammenhang mit einer Mahlzeit stehen, evtl. durch einen Gewichtsverlust begleitet sein. Bei der [[Auskultation]] des Bauches hört man typische Stenosegeräusche.
{{QS-Medizin}}
{{Vorlage:Infobox ICD
| 01-CODE = I77.4
| 01-BEZEICHNUNG =Arteria-coeliaca-Kompressions-Syndrom
}}
Das '''Truncus-coeliacus-Kompressionssyndrom''', auch '''Dunbar-Syndrom''' genannt, wird durch eine Einengung des [[Truncus coeliacus]] (ist ein Ast der [[Hauptschlagader]] im Bauchraum) durch das [[Zwerchfell]] (genau: durch das Ligamentum arcuatum mediale) hervorgerufen.


Die Diagnose des TCS ist eine [[Ausschlussdiagnose]], denn viele Menschen haben diese Form der Stenose ohne jedoch an Beschwerden zu leiden. Deshalb kann die Diagnose erst nach Ausschluss allgemeiner Ursachen gestellt werden.
{{Doppeltes Bild|rechts|Normal median arcuate ligament and celiac artery.png|148|Median arcuate ligament syndrome anatomy.png|142|Links sieht man den anatomischen Normalbefund, das Ligamentum liegt oberhalb des Abganges der Arteria coeliaca. Rechts gut zu erkennen, wie das Ligamentum das Gefäß einschnürt||Normal|Krank}}
Zum [[Screening]] dient die [[Duplexsonographie]], die Bestätigung der Diagnose erfolgt mittels [[CT]] oder [[MRT]].


Die Behandlung erfolgt chirurgisch durch Abtrennen oder Eröffnen des Ligamentum zusammen mit einer Entfernung des [[Ganglion celiacum]]. Die Mehrzahl der Patienten profitiert von der OP, aber bei jüngeren Patienten, bei Patienten mit psychiatrischen Erkrankungen oder Patienten mit erhöhtem Alkoholkonsum ist die Erfolgsrate geringer, genauso bei Patienten die keinen Gweichtsverlust oder keine Schmerzen im Zusammenhang mit den Mahlzeiten haben.
Die Beschwerden (Bauchschmerzen) treten oft nach dem Essen auf. Sie werden wegen ihrer eher allgemeinen Art häufig sehr spät als durch die Einengung hervorgerufen erkannt. Behandelbar ist die Einengung durch eine OP.


== Anatomie und Pathogenese ==
==Quellen==
*{{Literatur
| Autor=B. Luther
| Herausgeber=Wolfgang Hepp, Helmut Kogel
| Titel=[http://books.google.de/books?id=GssVw6gnLnQC&lpg=RA2-PA225&ots=DRQ2xgkqHI&dq=truncus%20coeliacus%20Kompressionssyndrom&pg=RA2-PA225#v=onepage&q=truncus%20coeliacus%20Kompressionssyndrom&f=false Truncus-coeliacus-Kompressionssyndrom]
| Sammelwerk=Gefäßchirurgie
| Verlag=Elsevier, Urban und Fischer
| Ort=München; Jena
| Jahr=
| ISBN=3-437-21841-7
}}
*{{Literatur
| Autor=Horton KM, Talamini MA, Fishman EK
| Titel=Median arcuate ligament syndrome: evaluation with CT angiography
| Sammelwerk=Radiographics
| Band=25
| Nummer=5
| Jahr=2005
| Seiten=1177–82
| PMID=16160104
| DOI=10.1148/rg.255055001
}}
*{{Literatur
| Autor=Dunbar JD, Molnar W, Beman FF, Marable SA
| Titel=[http://www.ajronline.org/cgi/reprint/95/3/731.pdf Compression of the celiac trunk and abdominal angina]
| Sammelwerk=Am J Roentgenol Radium Ther Nucl Med
| Band=95
| Nummer=3
| Seiten=731–44
| Jahr=1965
| Monat=November
| PMID=5844938
}}




[[Kategorie:Krankheitsbild in der Inneren Medizin]]
[[Kategorie:Krankheitsbild in der Angiologie]]
[[Kategorie:Krankheitsbild in der Chirurgie]]



[[en:Median arcuate ligament syndrome]]
eIn [[medicine]], the '''median arcuate ligament syndrome''' ('''MALS''') is a condition characterized by [[abdominal pain]] attributed to compression of the [[celiac artery]] and possibly the [[celiac ganglia]] by the [[median arcuate ligament]].<ref name="mals-horton">{{cite journal |author=Horton KM, Talamini MA, Fishman EK |title=Median arcuate ligament syndrome: evaluation with CT angiography |journal=Radiographics |volume=25 |issue=5 |pages=1177–82 |year=2005 |pmid=16160104 |doi=10.1148/rg.255055001 |url=}}</ref> The abdominal pain may be related to meals, may be accompanied by weight loss, and may be associated with an abdominal [[bruit]] heard by a clinician. It is also called ''celiac artery compression syndrome''.

The diagnosis of MALS is one of exclusion, as many healthy patients demonstrate some degree of celiac artery compression in the absence of symptoms. Consequently, a diagnosis of MALS is typically only entertained after more common conditions have been ruled out. Once suspected, screening for MALS can be done with [[ultrasonography]] and confirmed with [[CT angiography|computed tomography]] (CT) or [[MR angiography|magnetic resonance]] (MR) [[angiography]].

Treatment is generally surgical, the mainstay being [[open surgery|open]] division, or separation, of the median arcuate ligament combined with removal of the [[celiac ganglia]]. The majority of patients benefit from surgical intervention. Poorer responses to treatment tend to occur in patients of younger age, those with a [[mental disorder|psychiatric condition]] or who use alcohol, have abdominal pain unrelated to meals, or who have not experienced weight loss.

==Anatomy and pathogenesis==
<div class="thumb tnone" style="width:300px; float:right; margin-left:10px; margin-bottom:10px"><div class="thumbinner" style="width:290"><div style="border:1px solid #ccc">[[Image:Normal median arcuate ligament and celiac artery.png|noframe|150px|]][[Image:Median arcuate ligament syndrome anatomy.png|noframe|140px|]]</div>
<div class="thumbcaption">Side views (sagittal plane) of the descending aorta and two of its branches, the [[celiac trunk]] and [[superior mesenteric artery]], demonstrate normal and MALS anatomy. ''Left'' The [[median arcuate ligament]] is normally several millimeters to centimeters superior to the origin of the [[celiac artery]]. ''Right'' In MALS, the ligament is anterior, rather than superior, to the celiac artery, resulting in compression of the vessel and a characteristic hook-shaped contour.</div></div></div>

The [[median arcuate ligament]] is a [[ligament]] formed at the base of the [[thoracic diaphragm|diaphragm]] where the left and right [[diaphragmatic crura]] join near the 12th [[thoracic vertebra]]. This fibrous arch forms the anterior aspect of the [[aortic hiatus]], through which the [[aorta]], [[thoracic duct]], and [[azygos vein]] pass. The median arcuate ligament usually comes into contact with the aorta above the branch point of the [[celiac artery]]. However, in up to one quarter of normal individuals, the median arcuate ligament passes in front of the celiac artery, compressing the celiac artery and nearby structures such as the [[celiac ganglia]].<ref name="mals-horton"/> In some of these individuals, this compression is pathologic and leads to the median arcuate ligament syndrome.<ref name="mals-horton"/>

Several theories attempt to explain the origin of pain caused by compression of the celiac artery.<ref name="duncan"/> One proposes that compression of the celiac artery causes [[ischemia]], or decreased blood flow, to abdominal organs, leading to pain. Another hypothesizes that there is compression not only of the celiac artery but also of the [[celiac ganglia]], and that pain results from compression of the latter.

==Epidemiology==
It is estimated that in 10-24% of normal, asymptomatic individuals the median arcuate ligament crosses in front of (anterior to) the celiac artery, causing some degree of compression.<ref name="mals-horton"/><ref name="pmid5117015">{{cite journal |author=Lindner HH, Kemprud E |title=A clinicoanatomical study of the arcuate ligament of the diaphragm |journal=Arch Surg |volume=103 |issue=5 |pages=600–5 |year=1971 |month=November |pmid=5117015 |doi= |url=}}</ref> Approximately 1% of these individuals exhibit severe compression associated with symptoms of MALS.<ref name="mals-horton"/> The syndrome most commonly affects individuals between 20 and 40 years old, and is more common in women, particularly thin women.<ref name="mals-horton"/>

==Signs and symptoms==
Patients with MALS reportedly experience [[abdominal pain]], particularly in the [[epigastrium]], which may be associated with eating and which may result in [[Anorexia (symptom)|anorexia]] and [[weight loss]].<ref name="mals-horton"/> Occasionally, [[physical examination]] reveals an abdominal [[bruit]] in the [[epigastrium|mid-epigastrium]].<ref name="mals-horton"/>

Complications of MALS result from chronic compression of the celiac artery. They include [[gastroparesis]]<ref name="pmid9068484">{{cite journal |author=Balaban DH, Chen J, Lin Z, Tribble CG, McCallum RW |title=Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis |journal=Am. J. Gastroenterol. |volume=92 |issue=3 |pages=519–23 |year=1997 |month=March |pmid=9068484 |doi= |url=}}</ref> and [[aneurysm]] of the [[pancreaticoduodenal arteries]].<ref name="pmid18541631">{{cite journal |author=Manghat NE, Mitchell G, Hay CS, Wells IP |title=The median arcuate ligament syndrome revisited by CT angiography and the use of ECG gating--a single centre case series and literature review |journal=Br J Radiol |volume=81 |issue=969 |pages=735–42 |year=2008 |month=September |pmid=18541631 |doi=10.1259/bjr/43571095 |url=http://bjr.birjournals.org/cgi/pmidlookup?view=long&pmid=18541631}}</ref>

==Diagnosis==
Median arcuate ligament syndrome is a diagnosis of exclusion.<ref name="mals-horton"/><ref name="duncan">{{cite journal |author=Duncan AA |title=Median arcuate ligament syndrome |journal=Curr Treat Options Cardiovasc Med |volume=10 |issue=2 |pages=112–6 |year=2008 |month=April |pmid=18325313 |doi= 10.1007/s11936-008-0012-2|url=http://www.treatment-options.com/1092-8464/10/112 |format={{dead link|date=April 2010}}}}</ref> That is, the diagnosis of MALS is generally considered only after patients have undergone an extensive evaluation of their [[gastrointestinal tract]] including [[esophagogastroduodenoscopy|upper endoscopy]], [[colonoscopy]], and evaluation for [[gallbladder disease]] and [[gastroesophageal reflux disease]] (GERD).<ref name="duncan"/>

The diagnosis of MALS relies on a combination of clinical features and findings on [[medical imaging]].<ref name="mals-horton"/> Clinical features include those signs and symptoms mentioned above; classically, MALS involves a triad of abdominal pain after eating, weight loss, and an abdominal [[bruit]], although the classic triad is found in only a minority of individuals that carry a MALS diagnosis.<ref name="duncan"/>

Diagnostic imaging for MALS is divided into screening and confirmatory tests.<ref name="duncan"/> A reasonable screening test for patients with suspected MALS is [[duplex ultrasonography]] to measure blood flow through the [[celiac artery]].<ref name="duncan"/><ref name="ultrasound">{{cite journal |author=Sproat IA, Pozniak MA, Kennell TW |title=US case of the day. Median arcuate ligament syndrome (celiac artery compression syndrome) |journal=Radiographics |volume=13 |issue=6 |pages=1400–2 |year=1993 |month=November |pmid=8290734 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=8290734}}</ref> Peak systolic velocities greater than 200 cm/s are suggestive of celiac artery stenosis associated with MALS.<ref name="duncan"/>

{|class="wikitable" style="width:300px; float:right; border-collapse:collapse" border="1" cellpadding="3"
|+ CT angiographic findings in MALS<ref name="mals-horton"/>
|
# Focal narrowing of proximal celiac artery with poststenotic dilatation
# Indentation on superior aspect of celiac artery
# Hook-shaped contour of celiac artery
|}

Further evaluation and confirmation can be obtained via [[angiography]] to investigate the anatomy of the celiac artery.<ref name="duncan"/> Historically, [[conventional angiography]] was used, although this has been largely replaced by less invasive techniques such as [[computed tomography angiography|computed tomography]] (CT) and [[magnetic resonance angiography|magnetic resonance]] (MR) angiography.<ref name="mals-horton"/><ref name="duncan"/> Because it provides better visualization of intraabdominal structures, CT angiography is preferred to MR angiography in this setting.<ref name="duncan"/> The findings of focal narrowing of the proximal celiac artery with poststenotic dilatation, indentation on the superior aspect of the celiac artery, and a hook-shaped contour of the celiac artery support a diagnosis of MALS.<ref name="mals-horton"/> These imaging features are exaggerated on expiration, even in normal asymptomatic individuals without the syndrome.<ref name="mals-horton"/>

Proximal celiac artery stenosis with poststenotic dilatation can be seen in other conditions affecting the celiac artery.<ref name="mals-horton"/>. The hook-shaped contour of the celiac artery is characteristic of the anatomy in MALS and helps distinguish it from other causes of celiac artery stenosis such as [[atherosclerosis]].<ref name="mals-horton"/> This hooked contour is not entirely specific for MALS however, given that 10-24% of normal asymptomatic individuals have this anatomy.<ref name="mals-horton"/>

==Treatment==
Decompression of the celiac artery is the general approach to treatment of MALS.<ref name="duncan"/> The mainstay of treatment involves an [[open surgery|open surgical approach]] to divide, or separate, the median arcuate ligament to relieve the compression of the celiac artery.<ref name="duncan"/> This is combined with removal of the [[celiac ganglia]] and evaluation of blood flow through the celiac artery, for example by intraoperative duplex ultrasound. If blood flow is poor, celiac artery [[revascularization]] is usually attempted; methods of revascularization include [[Coronary artery bypass surgery|aortoceliac bypass]], [[angioplasty|patch angioplasty]], and others.<ref name="duncan"/>

A [[laparoscopy|laparoscopic approach]] may also be used to achieve celiac artery decompression;<ref name="pmid15965588">{{cite journal |author=Carbonell AM, Kercher KW, Heniford BT, Matthews BD |title=Multimedia article. Laparoscopic management of median arcuate ligament syndrome |journal=Surg Endosc |volume=19 |issue=5 |pages=729 |year=2005 |month=May |pmid=15965588 |doi=10.1007/s00464-004-6010-x}}</ref> however, should the celiac artery require revascularization, the procedure would require conversion to an open approach.<ref name="duncan"/>

[[Endovascular surgery|Endovascular]] methods such as [[percutaneous transluminal angioplasty]] (PTA) have been used in patients who have failed open and/or laparoscopic intervention.<ref name="duncan"/> PTA alone, without deompression of the celiac artery, may not be of benefit.<ref name="pmid7787348">{{cite journal |author=Matsumoto AH, Tegtmeyer CJ, Fitzcharles EK, ''et al'' |title=Percutaneous transluminal angioplasty of visceral arterial stenoses: results and long-term clinical follow-up |journal=J Vasc Interv Radiol |volume=6 |issue=2 |pages=165–74 |year=1995 |pmid=7787348 |doi= 10.1016/S1051-0443(95)71087-9|url=}}</ref><ref name="duncan"/>

==Prognosis==
There are few studies of the long-term outcomes of patients treated for MALS.<ref name="duncan"/> According to Duncan,<ref name="duncan"/> the largest and more relevant late outcomes data come from a study of 51 patients who underwent open surgical treatment for MALS, 44 of whom were available for long-term follow-up at an average of nine years following therapy.<ref name="pmid3965762">{{cite journal |author=Reilly LM, Ammar AD, Stoney RJ, Ehrenfeld WK |title=Late results following operative repair for celiac artery compression syndrome |journal=J. Vasc. Surg. |volume=2 |issue=1 |pages=79–91 |year=1985 |month=January |pmid=3965762 |doi= 10.1067/mva.1985.avs0020079|url=}}</ref> The investigators reported that among patients who underwent celiac artery decompression and revascularization, 75% remained asymptomatic at follow-up. In this study, predictors of favorable outcome included:
* Age from 40 to 60 years
* Lack of [[mental disorder|psychiatric condition]] or alcohol use
* Abdominal pain that was worse after meals
* Weight loss greater than 20 lb (9.1 kg)

==History==
Celiac artery compression was first observed by Benjamin Lipshutz in 1917.<ref name="whonamedit">{{WhoNamedIt|synd|4106}}</ref> MALS was first described by Pekka-Tapani Harjola in 1963<ref name="pmid14083857">{{cite journal |author=Harjola PT |title=A rare obstruction of the coeliac artery. Report of a case |journal=Ann Chir Gynaecol Fenn |volume=52 |issue= |pages=547–50 |year=1963 |pmid=14083857 |doi= |url=}}</ref> and subsequently by J. David Dunbar and Samuel Marable in 1965.<ref name="pmid5844938">{{cite journal |author=Dunbar JD, Molnar W, Beman FF, Marable SA |title=Compression of the celiac trunk and abdominal angina |journal=Am J Roentgenol Radium Ther Nucl Med |volume=95 |issue=3 |pages=731–44 |year=1965 |month=November |pmid=5844938 |doi= |url=http://www.ajronline.org/cgi/reprint/95/3/731.pdf}}</ref> It has also been called '''Harjola-Marable syndrome''' and '''Marable syndrome'''.<ref name="whonamedit"/>

==See also==
*[[Superior mesenteric artery syndrome]]
*[[Nutcracker syndrome]]

==References==
{{reflist|2}}

[[Category:Gastroenterology]]
[[Category:Syndromes]]

[[de:Truncus-coeliacus-Kompressionssyndrom]]

Version vom 10. Dezember 2010, 23:01 Uhr

Das Truncus-coeliacus-Kompressions-Syndrom (im Folgenden TCS abgekürzt) auch als Dunbar-Syndrom bezeichnet, äussert sich durch Bauchschmerzen, verursacht durch Einklemmung der Arteria coeliaca (oder Truncus coeliacus genannt]) und möglicherweise des Ganglion celiacum durch das Zwerchfell (genauer durch das Ligamentum arcuatum mediale) [1]. Die Bauchschmerzen können im Zusammenhang mit einer Mahlzeit stehen, evtl. durch einen Gewichtsverlust begleitet sein. Bei der Auskultation des Bauches hört man typische Stenosegeräusche.

Die Diagnose des TCS ist eine Ausschlussdiagnose, denn viele Menschen haben diese Form der Stenose ohne jedoch an Beschwerden zu leiden. Deshalb kann die Diagnose erst nach Ausschluss allgemeiner Ursachen gestellt werden. Zum Screening dient die Duplexsonographie, die Bestätigung der Diagnose erfolgt mittels CT oder MRT.

Die Behandlung erfolgt chirurgisch durch Abtrennen oder Eröffnen des Ligamentum zusammen mit einer Entfernung des Ganglion celiacum. Die Mehrzahl der Patienten profitiert von der OP, aber bei jüngeren Patienten, bei Patienten mit psychiatrischen Erkrankungen oder Patienten mit erhöhtem Alkoholkonsum ist die Erfolgsrate geringer, genauso bei Patienten die keinen Gweichtsverlust oder keine Schmerzen im Zusammenhang mit den Mahlzeiten haben.

Anatomie und Pathogenese

eIn medicine, the median arcuate ligament syndrome (MALS) is a condition characterized by abdominal pain attributed to compression of the celiac artery and possibly the celiac ganglia by the median arcuate ligament.[2] The abdominal pain may be related to meals, may be accompanied by weight loss, and may be associated with an abdominal bruit heard by a clinician. It is also called celiac artery compression syndrome.

The diagnosis of MALS is one of exclusion, as many healthy patients demonstrate some degree of celiac artery compression in the absence of symptoms. Consequently, a diagnosis of MALS is typically only entertained after more common conditions have been ruled out. Once suspected, screening for MALS can be done with ultrasonography and confirmed with computed tomography (CT) or magnetic resonance (MR) angiography.

Treatment is generally surgical, the mainstay being open division, or separation, of the median arcuate ligament combined with removal of the celiac ganglia. The majority of patients benefit from surgical intervention. Poorer responses to treatment tend to occur in patients of younger age, those with a psychiatric condition or who use alcohol, have abdominal pain unrelated to meals, or who have not experienced weight loss.

Anatomy and pathogenesis

Side views (sagittal plane) of the descending aorta and two of its branches, the celiac trunk and superior mesenteric artery, demonstrate normal and MALS anatomy. Left The median arcuate ligament is normally several millimeters to centimeters superior to the origin of the celiac artery. Right In MALS, the ligament is anterior, rather than superior, to the celiac artery, resulting in compression of the vessel and a characteristic hook-shaped contour.

The median arcuate ligament is a ligament formed at the base of the diaphragm where the left and right diaphragmatic crura join near the 12th thoracic vertebra. This fibrous arch forms the anterior aspect of the aortic hiatus, through which the aorta, thoracic duct, and azygos vein pass. The median arcuate ligament usually comes into contact with the aorta above the branch point of the celiac artery. However, in up to one quarter of normal individuals, the median arcuate ligament passes in front of the celiac artery, compressing the celiac artery and nearby structures such as the celiac ganglia.[2] In some of these individuals, this compression is pathologic and leads to the median arcuate ligament syndrome.[2]

Several theories attempt to explain the origin of pain caused by compression of the celiac artery.[3] One proposes that compression of the celiac artery causes ischemia, or decreased blood flow, to abdominal organs, leading to pain. Another hypothesizes that there is compression not only of the celiac artery but also of the celiac ganglia, and that pain results from compression of the latter.

Epidemiology

It is estimated that in 10-24% of normal, asymptomatic individuals the median arcuate ligament crosses in front of (anterior to) the celiac artery, causing some degree of compression.[2][4] Approximately 1% of these individuals exhibit severe compression associated with symptoms of MALS.[2] The syndrome most commonly affects individuals between 20 and 40 years old, and is more common in women, particularly thin women.[2]

Signs and symptoms

Patients with MALS reportedly experience abdominal pain, particularly in the epigastrium, which may be associated with eating and which may result in anorexia and weight loss.[2] Occasionally, physical examination reveals an abdominal bruit in the mid-epigastrium.[2]

Complications of MALS result from chronic compression of the celiac artery. They include gastroparesis[5] and aneurysm of the pancreaticoduodenal arteries.[6]

Diagnosis

Median arcuate ligament syndrome is a diagnosis of exclusion.[2][3] That is, the diagnosis of MALS is generally considered only after patients have undergone an extensive evaluation of their gastrointestinal tract including upper endoscopy, colonoscopy, and evaluation for gallbladder disease and gastroesophageal reflux disease (GERD).[3]

The diagnosis of MALS relies on a combination of clinical features and findings on medical imaging.[2] Clinical features include those signs and symptoms mentioned above; classically, MALS involves a triad of abdominal pain after eating, weight loss, and an abdominal bruit, although the classic triad is found in only a minority of individuals that carry a MALS diagnosis.[3]

Diagnostic imaging for MALS is divided into screening and confirmatory tests.[3] A reasonable screening test for patients with suspected MALS is duplex ultrasonography to measure blood flow through the celiac artery.[3][7] Peak systolic velocities greater than 200 cm/s are suggestive of celiac artery stenosis associated with MALS.[3]

CT angiographic findings in MALS[2]
  1. Focal narrowing of proximal celiac artery with poststenotic dilatation
  2. Indentation on superior aspect of celiac artery
  3. Hook-shaped contour of celiac artery

Further evaluation and confirmation can be obtained via angiography to investigate the anatomy of the celiac artery.[3] Historically, conventional angiography was used, although this has been largely replaced by less invasive techniques such as computed tomography (CT) and magnetic resonance (MR) angiography.[2][3] Because it provides better visualization of intraabdominal structures, CT angiography is preferred to MR angiography in this setting.[3] The findings of focal narrowing of the proximal celiac artery with poststenotic dilatation, indentation on the superior aspect of the celiac artery, and a hook-shaped contour of the celiac artery support a diagnosis of MALS.[2] These imaging features are exaggerated on expiration, even in normal asymptomatic individuals without the syndrome.[2]

Proximal celiac artery stenosis with poststenotic dilatation can be seen in other conditions affecting the celiac artery.[2]. The hook-shaped contour of the celiac artery is characteristic of the anatomy in MALS and helps distinguish it from other causes of celiac artery stenosis such as atherosclerosis.[2] This hooked contour is not entirely specific for MALS however, given that 10-24% of normal asymptomatic individuals have this anatomy.[2]

Treatment

Decompression of the celiac artery is the general approach to treatment of MALS.[3] The mainstay of treatment involves an open surgical approach to divide, or separate, the median arcuate ligament to relieve the compression of the celiac artery.[3] This is combined with removal of the celiac ganglia and evaluation of blood flow through the celiac artery, for example by intraoperative duplex ultrasound. If blood flow is poor, celiac artery revascularization is usually attempted; methods of revascularization include aortoceliac bypass, patch angioplasty, and others.[3]

A laparoscopic approach may also be used to achieve celiac artery decompression;[8] however, should the celiac artery require revascularization, the procedure would require conversion to an open approach.[3]

Endovascular methods such as percutaneous transluminal angioplasty (PTA) have been used in patients who have failed open and/or laparoscopic intervention.[3] PTA alone, without deompression of the celiac artery, may not be of benefit.[9][3]

Prognosis

There are few studies of the long-term outcomes of patients treated for MALS.[3] According to Duncan,[3] the largest and more relevant late outcomes data come from a study of 51 patients who underwent open surgical treatment for MALS, 44 of whom were available for long-term follow-up at an average of nine years following therapy.[10] The investigators reported that among patients who underwent celiac artery decompression and revascularization, 75% remained asymptomatic at follow-up. In this study, predictors of favorable outcome included:

  • Age from 40 to 60 years
  • Lack of psychiatric condition or alcohol use
  • Abdominal pain that was worse after meals
  • Weight loss greater than 20 lb (9.1 kg)

History

Celiac artery compression was first observed by Benjamin Lipshutz in 1917.[11] MALS was first described by Pekka-Tapani Harjola in 1963[12] and subsequently by J. David Dunbar and Samuel Marable in 1965.[13] It has also been called Harjola-Marable syndrome and Marable syndrome.[11]

See also

References

Vorlage:Reflist

de:Truncus-coeliacus-Kompressionssyndrom

  1. Horton
  2. a b c d e f g h i j k l m n o p q Horton KM, Talamini MA, Fishman EK: Median arcuate ligament syndrome: evaluation with CT angiography. In: Radiographics. 25. Jahrgang, Nr. 5, 2005, S. 1177–82, doi:10.1148/rg.255055001, PMID 16160104.
  3. a b c d e f g h i j k l m n o p q r Duncan AA: Median arcuate ligament syndrome. In: Curr Treat Options Cardiovasc Med. 10. Jahrgang, Nr. 2, April 2008, S. 112–6, doi:10.1007/s11936-008-0012-2, PMID 18325313 (treatment-options.com [VORLAGE:TOTER LINK/!...NOURL (SEITE NICHT MEHR ABRUFBAR, FESTGESTELLT IM APRIL 2010.)'"`UNIQ--NOWIKI-0000002F-QINU`"']).
  4. Lindner HH, Kemprud E: A clinicoanatomical study of the arcuate ligament of the diaphragm. In: Arch Surg. 103. Jahrgang, Nr. 5, November 1971, S. 600–5, PMID 5117015.
  5. Balaban DH, Chen J, Lin Z, Tribble CG, McCallum RW: Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis. In: Am. J. Gastroenterol. 92. Jahrgang, Nr. 3, März 1997, S. 519–23, PMID 9068484.
  6. Manghat NE, Mitchell G, Hay CS, Wells IP: The median arcuate ligament syndrome revisited by CT angiography and the use of ECG gating--a single centre case series and literature review. In: Br J Radiol. 81. Jahrgang, Nr. 969, September 2008, S. 735–42, doi:10.1259/bjr/43571095, PMID 18541631 (birjournals.org).
  7. Sproat IA, Pozniak MA, Kennell TW: US case of the day. Median arcuate ligament syndrome (celiac artery compression syndrome). In: Radiographics. 13. Jahrgang, Nr. 6, November 1993, S. 1400–2, PMID 8290734 (rsnajnls.org).
  8. Carbonell AM, Kercher KW, Heniford BT, Matthews BD: Multimedia article. Laparoscopic management of median arcuate ligament syndrome. In: Surg Endosc. 19. Jahrgang, Nr. 5, Mai 2005, S. 729, doi:10.1007/s00464-004-6010-x, PMID 15965588.
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