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Celiac trunk compression syndrome. A review / Una revision del sindrome de compresion del tronco celiaco.

PETRELLA, S. & PRATES, J. C. Celiac trunk compression syndrome. A review. Int. J. Morphol., 24(3):429-436, 2006.

SUMMARY: The purpose of the present review is to report the anatomic and the clinical-surgical aspects involved in the celiac trunk compression syndrome by the median arcuate ligament of the diaphragm, reviewing the major findings of the syndrome in the anatomic field during dissection of cadavers, followed by clinical-surgical findings of stenosis of the celiac trunk, the relationship of this stenosis with the patient's symptoms and healing after decompression of that artery; invasive and non-invasive methods used to diagnose compression; the stenotic effect of physiologic mechanisms of the median arcuate ligament, aorta and celiac trunk displacement during respiration; anatomy of the aortic channel and celiac plexus; the median arcuate ligament and the celiac plexus as constrict agents; skeletopy of the celiac trunk, the median arcuate ligament and predisposition to syndrome; association of the syndrome with morphological and metabolic aspects.

KEY WORDS: Celiac artery; Ligaments; Celiac plexus; Syndrome; Diaphragm; Arterial occlusive disease.

PETRELLA, S. & PRATES, J. C. Una revision del sindrome de compresion del tronco celiaco. Int. J. Morphol., 24(3):429-436, 2006.

RESUMEN: La presente revision tiene por objetivo conocer tanto los aspectos anatomicos como clinicos y quirurgicos relacionados con el sindrome de compresion del tronco celiaco causado por el ligamento arqueado mediano del diafragma. Se revisan los principales descubrimeinos del sindrome, tanto en el plano anatomico durante la disecacion de cadaveres, como en la clinica-quirurgica de la estenosis del tronco celiaco. Ademas, se revisa la relacion de esta estenosis con los sintomas del paciente y cura despues de la descompresion del tronco celiaco. Por otra parte, se explican los metodos no invasivos e invasivos utilizados en la descompresion; el efecto estenotico de los mecanismos fisiologicos del desplazamiento del ligamento arqueado mediano, aorta y tronco celiaco durante la respiracion; anatomia del canal aortico y plexo celiaco; el ligamento arqueado mediano y el plexo celiaco como agentes constrictores; la esquelotopia del tronco celiaco y del ligamento arqueado mediano y la predisposicion para el sindrome. Finalmente, se hace una asociacion del sindrome del tronco celiaco con anomalias morfologicas y metabolicas.

PALABRAS CLAVE: Arteria celiaca; Ligamentos; Plexo celiaco; Sindrome; Diafragma; Enfermedad Arterial Oclusiva.


A Relationship of the celiak trunk with diaphragm crura. The first investigations on acknowledge of the celiac trunk compression by diaphragm crura happened in the anatomic field during cadaver dissection (Rio Branco, 1912; Lipshutz, 1917; George, 1934; Michels, 1955). Dissections of 62 cadavers revealed that sometimes the origin of the celiac trunk is overlapped by the diaphragm (Lipshutz). In his study, George also reported eight cases of overlap by the diaphragm in 38 dissections of cadavers. Additionally, it was observed in these cases that the constrictive action of the diaphragm reduces the caliber of the celiac trunk at its origin (George, and Michels).

Discover of the celiac trunk compression syndrome. Later, this stenoic anatomical anomaly of the celiac trunk was angiographically individualized in a series with 15 patients and correlated etiologically with some clinical manifestations, such as postprandial abdominal pain and weight loss. Additionally, the clinical evaluation showed the symptoms healing in 12 individuals after surgical release of this vessel compression by sectioning the median arcuate ligament (Dunbar et al., 1965).

The surgical success of Dunbar et al. in the medical area, led many authors to confirm and attribute this arterial compression to the diaphragm (Marable et al., 1966; Stoney & Wylie, 1966; Terpstra, 1966; Lord et al., 1968; Marable et al., 1968; Taheri, 1968; Hivet & Lagadec, 1970; Ciscato et al., 1976; Warter et al., 1976).

Invasive and non-invasive diagnostic methods. The compression of the celiac trunk by the median arcuate ligament is an entity angiographically well characterized (Marable et al., 1966; Warter et al., 1973a) since its first observations by the routine practice (Dunbar et al.).

The stenosis visualization by the celiac trunk compression syndrome can also be performed according to non-invasive methods as the echo-Doppler (Tridico et al., 1988); color pulsed Doppler (De Pauw et al., 1992; Sproat et al., 1993) and computerized tomography (Patten et al., 1991; Sponza et al., 1933; Loffeld et al., 1995); digital subtraction angiography (Desmond & Roberts, 2004); magnetic resonance angiography (Dordoni et al., 2002; Alehan & Dogan, 2004); three-dimensional computerized tomography (Horton et al., 2005); as well as the visualization of the adequacy flow in the aorta and celiac trunk by means of Doppler ultrasound scanning (Roayaie et al., 2000; Dordoni et al. and Alehan & Dogan).

Sponza et al. published a study in which visibility of the celiac trunk, its arteries and the superior mesenteric artery as well as its anatomic variations and vascular anomalies were analyzed in 100 patients by means of computerized tomography. The results were in accordance with the angiographic data reported in the literature.

Patten et al. showed in five individuals submitted to a computerized tomography to evaluate several anomalies, occurrence of high-grade stenosis of the celiac trunk by the median arcuate ligament. The surgery and/or angiography confirmed the diagnosis (Patten et al. and Loffeld et al.).

Recently, the laparoscopy, a less invasive surgical maneuver became equally effective in the celiac trunk decompression (Kokotsakis et al., 2000; Roayaie et al.; Dordoni et al.; Desmond & Roberts and Carbonell et al., 2005).

Displacement of the median arcuate ligament and the celiac trunk during respiration. Arteriographic experiments showed the mechanisms of the median arcuate ligament, celiac trunk and aorta displacement during respiration in individuals with compression of the celiac trunk syndrome. According to the lateral incidence arteriography, it could be observed that during inspiration, the median arcuate ligament caudally moves an average of 8mm and ventrally 4.4mm. Concomitantly, the celiac trunk and the aorta caudally move an average of 3 to 6mm respectively, resulting in a distance between the orifice of celiac trunk and the median arcuate ligament (Reuter et al., 1971; Stanley & Fry, 1971; Reuter & Bernstein , 1973).

The "notch" in the emergency of the celiac trunk, caused by the median arcuate ligament compression is more accentuated during expiration and in a lesser degree or inexistent in inspiration (Curl et al., 1971; Williams et al., 1985 and Loffeld et al.).

Stenosis of the celiac trunk by the median arcuate ligament. Several studies showed that the constrictor agent of the celiac trunk compression is the median arcuate ligament (Dunbar et al.; Gautier et al., 1965; Stoney & Wylie; Deutsch, 1968; Lord et al.; Edwards et al., 1970; Hivet & Lagadec; Rubush, 1970; Bobbio & Zanella, 1971; Stanley & Fry; Auche et al.,1972; Dreze et al., 1972; Van De Berg et al., 1972; Watt, 1972; Conti et al., 1973; Meves & Beger, 1973; Warter et al., 1973a, 1973c; Ducellier et al., 1974; Ciscato et al.; Ferrandiz et al., 1976; Lynch, 1976; Datta & Vichery, 1979; Abate et al., 1980; Lord & Tracy, 1980; Mongelli et al., 1980; Pailler et al., 1982; Gutnick, 1984; Reilly et al., 1985; Aburahma, 1995; De Cecchis et al., 1996).

Stenosis of the celiac trunk by the median arcuate ligament and celiac plexus. Marable et al. (1966) described the celiac trunk compression by the median arcuate ligament and by the celiac plexus as a new clinical entity. Thus, the plexotomy alone does not seem to cause a symptom relief. Bobbio et al. (1967) observed intrasurgically that if the section of the fibrotic ganglion of the celiac plexus is performed, the pulsations of the celiac trunk and its branches do not return to normal, being necessary decompression of the celiac trunk by sectioning arciform fibers of the diaphragm, which also exert compression.

When the median arcuate ligament is sectioned during surgery, some nervous fibers need to be sectioned leading to an effective result (Joubaud et al., 1977). It is possible to figure out that relief of symptoms after division of the median arcuate ligament in patients with persistent stenosis could be due to increase in blood flow after periarterial sympathectomy and denervation of the celiac ganglion inevitably performed during sectioning of the median arcuate ligament (Carey et al., 1969).

After Marable et al. (1966), several authors reported the celiac plexus compression by both agents--the median arcuate ligament and the celiac axis (Rob, 1966; Stoney & Wylie; Bobbio et al.; Harjola & Lahtiharju, 1968; Tahery (1968); Carey et al.; Cormier & De La Fontaine, 1970; Olivier et al., 1970; Balmes et al., 1971; Lindner & Kemprud, 1971; Stanley & Fry; Tongio et al., 1971; Dreze et al.; Conti et al.; Beger et al., 1975; Joubaud et al.; Watson & Sadikali, 1977; Guibert et al., 1980; Daskalakis, 1982; Ghosn et al., 1982; Matesanz et al., 1982; Thevenet et al., 1985; Bacourt et al., 1984 and Roayaie et al.).

Stenosis of the celiac trunk by the celiac plexus. Harjola (1963) was the first to describe the extrinsic compression of the celiac trunk by fibrosis of the celiac ganglion. The angiogram showed stenosis of the celiac trunk and in the surgery a constriction of the artery by the fibrosed celiac ganglion was observed. The artery was released and the epigastric bruit disappeared and the patient remained with no symptoms. The author confirmed a retroperitoneal fibrosis and, according to them, in spite of the pain being caused by fibrosis, it could not explain its appearance after meals. Based on this clinical report, many authors agreed with this defined clinical entity, considering the celiac plexus as the etiologic agent in the celiac trunk compression syndrome, which is formed by an abundant neurofibrous tissue able to constrict this vessel (Drapanas & Bron, 1966; Debray et al., 1967a, 1967b; Snyder et al., 1967; Harjola, 1968; Harjola & Lahtiharju; Jamieson, 1970; Leger et al., 1970; Olivier et al.; Di Marino et al., 1972; Mc Sherry, 1977; Van Gossun et al., 1984; Jamieson, 1986).

Evidence of celiac ganglion compression was based on macroscopic observation that the ganglion abnormally dense and fibrosed constrict the celiac trunk as a ligature or in an annular manner, from a distance of 3 to 4 mm (Harjola & Lahtiharju).

Anatomy of the celiac plexus. The terms celiac, splanchnic and solar plexuses are equivalent for the same nervous structure. The etymology of these terms emphasizes the adjacent celiac trunk, the accessory splanchnic nerves and its location (Thompson et al., 1977).

The major part of the sympathetic system in the abdomen is formed by an autonomous nerve plexus extending front and sides all along the entire length of the abdominal aorta. Its superior part is designated celiac plexus (Ward et al., 1979). This is the largest of the three plexuses of the autonomous nervous system (cardiac, celiac, hipogastric); it supplies the abdominal organs and is situated at the T12-L1 level (Thompson et al.). It is located in front of the diaphragm crura, medially to the adrenal glands and upon the abdominal aorta surrounding the origin of the celiac trunk and the superior mesenteric artery, composed of dense strands of splanchnic nerve fibers (Snyder et al.).

The right and left semilunar celiac ganglions emerge from the celiac plexus and are found upon the diaphragm crura, in the origin of the celiac trunk and lateral to the aorta. Its fibers are interconnected around the celiac trunk and its branches, accompany these up to the respective superior abdominal organs (stomach, liver, gallbladder,pancreas, adrenal glands and kidneys (Lindner & Kemprud; Lowe, 1975; Joubaud et al. and Ward et al.). Both ganglions are immersed into an extensive network of nervous fibers and in one mass of connective tissue (Snyder et al. and Ward et al.).

Anatomy of the diaphragm crura. The aortic hiatus is a foramen in which the aorta passes from the thorax to the abdomen. It is delimited medially and laterally by tendons of the right and left crura of the diaphragm and its internal extensions consist of a triangle whose inferior margin forms the median arcuate ligament rounding the acute angle formed by the interconnection of the two crura (Bobbio et al.; Hivet & Lagadec and Furnemont, 1974). This leaning position of these two crura and their relationship with the pre-vertebral musculature forms the real "aortic channel" (Bobbio et al.).

Regarding the anatomic aspect, the median arcuate ligament is connective fibrous or thick fibromuscular, arciform structure, transversally crossing the aorta at the T12 vertebra level and attaching right in front of this vessel to these tendinous portions of both crura to form the anterior margin of the aortic hiatus (Reuter & Bernstein; Joubaud et al.; Langeron et al., 1980 and Patten et al.). This fibrous ligament is infiltrated by nervous formations of the celiac plexus (Guibert et al.).

Leger et al. described the median arcuate ligament as a fascia fulfilling the space resembling a triangle, formed by the two crura interconnection of the diaphragm, which in some cases may cover the origin of the celiac trunk. This of both crura (Ruiz Liard et al., 1964). Also Lindner & Kemprud identified the median arcuate ligament as a structure variable in size, shape and position usually presented a round fibrous of 1 to 3 mm in width formed by condensing the fibrous margin of the two crura of diaphragm decussating to form the ventral border of the aortic hiatus easily observed by naked eye. Also, there was evidence that this connective margin is occasionally inexistent or difficult to be identified, which seems to produce little or no compression on the celiac trunk.

Di Marino et al. concluded that the median arcuate ligament is a thin fibrous connective formation recovering the deep face of the diaphragm, adhering to the tendinous-muscular contour of the aortic hiatus and that sometimes overlapping the celiac trunk and is not able to play the role of stenosis. However inferior border of the diaphragmatic aortic ring, mainly the fibrous border, may provoke compression of the celiac trunk.

Anomalous display of the celiac trunk and/or median arcuate ligament. Several authors believe that the anatomic substrate of the celiac trunk compression syndrome is represented by the more caudal anomalous display of the median arcuate ligament, varying from the intervertebral disk T12-L1 towards the inferior third of the L2 vertebra (Curl et al.; Stanley & Fry; Warter et al., 1976) sometimes reaching the level of the L3 and L4 vertebras (Rubush et al.). The anomalous insertion of the diaphragm crura, the hypertrophy or lowering its fibrous arcade, hardly constricts the proximal segment of the celiac trunk when it crosses the aortic hiatus (Marable et al., 1966; Stoney & Wylie; Harjola & Lahtiharju; Marable et al., 1968; Olivier et al.; Rubush; Bobbio & Zanella; Curl et al.; Conti et al.; Kieny & Dietz, 1973; Furnemont; Loffeld et al.). Other authors observed that the celiac trunk might have an intrathoracic origin (Fadhli, 1968; Warter et al., 1970b; Gutnick). Due to these situations, this celiac trunk compression syndrome was denominated celiac-phrenic disorder (Warter et al., 1976)


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* Selma Petrella & ** Jose Carlos Prates

* Arbovirus Division, Adolfo Lutz Institute, Sao Paulo, Brazil.

** Descriptive and Topographic Anatomy Division, Federal University of Sao Paulo, Brazil.

Correspondence to:

Prof. Dra. Selma Petrella

Rua Rio Grande, 180 Apto. 62

Sao Paulo--Vila Mariana

CEP 04018-000

Sao Paulo--SP, BRASIL

Received: 14-10-2005 Accepted: 28-06-2006
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Author:Petrella, Selma; Prates, Jose Carlos
Publication:International Journal of Morphology
Date:Sep 1, 2006
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