The Celiac Ganglion (Artery) Compression Syndrome (CGCS)
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TEMA: ULTRALYD The Celiac ganglion (artery) compression syndrome (CGCS) av Thomas Scholbach Hospital for Children and Adolescents, Chemnitz Clinics, Chemnitz, Germany [email protected] Acknowledgement I thank Dr. Annegret Klimas for insightful discussions about CGCS. A preface openness of mind in many Norwegian colleagues with angiographic evidence of celiac compression[4], This article is solicited by the journal’s editor and it is whom I had the privilege to make friends with. This epigastric pain, postprandial pain and weight loss a joy for me (as well as an opportunity to present a still moves me and the whole story reminds me to of more than 5 kg [5], postprandial abdominal pain, personal view of the subject) to use this opportunity another deep experience related not only to Doppler weight loss, and vomiting [6]. Nevertheless, not to express my gratitude to the Norwegian Ultrasound and blood flow:πάντα ῥεῖ - everything flows …and rarely a shortcut constellation of three well defined Society who invited me to give some lectures at its thus changes. components has become a mantra to guide vascular annual congress in Trondheim in 2011. surgeons. Others extended the scope of symptoms to A clinical approach to CGCS include an abdominal bruit (primarily mentioned in We report here our experience with sonographic The clinical apprehension of CGCS has changed from Harjola’s pioneering paper) [7-9]. Some also describe diagnosis in more than 1000 cases with actually 53 its first description [1] – and still does. the change of this bruit’s volume with breathing – i.e. operated on. It was in Trondheim that some Norwegian diaphragms position and a decrease in inspiration [9] colleagues expressed their interest in my talk dealing Historical notes with abdominal vascular compression syndromes and Harjola and Lahtiharju [2] coined the term “celiac axis The coexistence of a variety of symptoms was seen by now inspired this contribution to this journal. But the syndrome” for a symptom complex consisting of some authors as clearly disqualifying for the diagnosis links to Norway and the Nordic countries in general postprandial epigastric pain, commencing 20 to 60 of celiac artery compression (“…Patients likely to go deeper - especially with respect to this topic. It is minutes after a meal, in addition of epigastric fullness benefit [from an operation –authors insertion] are not only that the Finnish surgeon Harjola described and vomiting or diarrhea in some of the reported 13 those with epigastric pain related to food or hunger for the first time – and successfully operated on – this patients (10 women and 3 men, 32–63 years old). who do not have a galaxy of other unrelated vascular compression syndrome but I draw a lot from symptoms”.)[10] the Norwegian Doppler pioneers as Liv Hatle, Bjørn In 3 of them, an epigastric bruit was audible prompting Angelsen and Sturla Eik-Nes, all from Trondheim, the suspicion of an arterial stenosis which was Diverging pathophysiological which were my remote teachers by means of their confirmed by preoperative angiography. The other concepts and therapeutic books and publications when we tried to catch up 10 patients underwent surgery for a variety of other conclusions with the western technique and technology while we reasons, and in all cases, celiac artery compression Not rarely the terms compression and stenosis were were shut away behind the “iron curtain” living in was found. Harjola’s description was refined later on used interchangeably or even in a joint manner [11, 12] the GDR. It was barely inconceivable to give back leading to the often repeated so called characteristic what may have further contributed to blur the something someday. Now we have got so many (clinical) triad. The constituents of this triad but apprehension of the exact nature of this syndrome [7, friends and colleagues just in Norway – in part due to changed: postprandial pain, vomiting, and weight 13, 14]. many German doctors living here in part due to the loss [3], abdominal pain, an epigastric bruit, and Figure 1. B-mode image of the longitudinal section of the abdominal aorta showing Figure 2. B-mode image of the longitudinal section of the abdominal aorta during clearly the compression of the celiac trunk (TC) by the inferior portion of the inspiration demonstrating the release of the celiac trunk (right arrow) by the retrac- diaphragm – the arcuate ligament (arrow). ting diaphragm (left arrow). 14 TEMA: ULTRALYD Figure 3. Color Doppler sonograms of the celiac trunk in different diaphragmatic positions during breathing. See the dilation behind the compression site and the filling of collaterals (blue) during deep expiration. examination and its irritation can only be concluded from the compression of the underlying celiac artery. This fact contributes much to concepts which focus on the vessel’s diameter and forget about the ganglion. The origin of pain was suspected in diminished blood Need to distinguish stenosis This is the reason why the author proposes a new flow thus causing ischemia downstream the stenosis from compression of the name for this condition: celiac ganglion compression (i.e. in the stomach, liver and spleen). Some celiac artery syndrome. questioned this causal connection and favored the Therefore, it seems worthwhile to highlight the neuropathic origin from the compressed neural tissue differences between both entities (Scholbach’s reply to Sonographic diagnosis of the celiac ganglion [1, 2, 15, 16]. [12]). The main and fundamental difference is the Color Doppler sonography is the method of choice to cause of lumen reduction of the celiac artery. In celiac rule in and rule out the diagnosis of CGCS. B-mode The longstanding uncertainties of the true origin of the ganglion compression syndrome (synonyms are sonography in many cases can clearly delineate all prevailing pain [17, 18] has much contributed to the Dunbar-syndrome, celiac artery compression relevant structures (figure 1) but is of limited value in diversification of operation techniques [19-24], recom- syndrome, celiac trunk compression syndrome, adipose patients and in cases with a slightly laterally mendations [25] and mixed results [13, 26, 27]. This median arcuate ligament syndrome [MALS]) the celiac directed CA. Color Doppler, often at a first glance, can is the main reason for disbelief of the very existence of artery and the straddling celiac ganglion are both show if there are turbulences and depicts the course of the syndrome at all [28] and the decades of debate to compressed by the arching arcuate ligament. So, the the CA quite clearly. Also aneurysms with a typically find the best operation technique with the longest compression is one sided, guillotine-like and often whirling flow patterns and post-compression dilation lasting results [29]. The main objection against the results in a sharp incision of the celiac artery from are easily recognized. Moreover, collateral arteries ischemia theory came from the frequent observation, above, which is easily visible in color Doppler and very from the superior mesenteric artery to distal portions that in severe cases of compression collaterals arising often, but substantially less clear in B-mode images of of the CA can be shown – nearly impossible with from the superior mesenteric artery bridged the the longitudinal section of the abdominal aorta (figure B-mode ultrasound. The compression is clearly visible narrowing [30]. For some authors it seemed thus 1). This compression is variable with breathing – in a midposition of the diaphragm (figure 3) and highly unlikely for ischemia to play a major role in the because while the diaphragm is moving the position diminished in inspiration. Expiration leads to the disease [31] or they even declined the entire concept of the arcuate ligament is changing thus causing strongest compression (figure 4). Often, this position [32, 33]. Others stressed that patency of the celiac changing degrees of squeezing the vessel (figure 2). cannot be held for a long time and many patients do artery (CA) in postoperative follow up was linked to The celiac ganglion is not visible with the conven- not expire deep enough to develop a pronounced persistence of pain relief [29]. Other reasons brought tional imaging techniques. Thus it evades the direct increase of flow velocity and a maximum reduction of forward against the clinical relevance of celiac artery compression were the discouraging results many observed after successfully restoring the vessel’s patency [26] or implementing venous bypasses. Rarely attempts were made to stent the compression site aiming at restoring the original lumen in order to ease the blood flow. The many recommendations often simply ignored the fundamental difference between an arterial stenosis and arterial compression from outside [34]. Often the argument was raised, that the many severe stenoses of the celiac trunk found at autopsies [35] which were completely asymptomatic during lifetime and thus ruled out a causal link of celiac compression and complaints[36]. The results were Figure 4a. Deep expiration in CGCS: Only the superior Figure 4b. Deep expiration in CGCS: Only the superior mixed and so the debate persists to date [37, 38]. mesentric artery is still visible. The course of the aorta mesentric artery is still visible. The coloration of the points to the strong diaphragmatic compression of ventral part of the aorta (white arrows) makes evident the celiac trunk bending the underlying vessels (i.e. that here the superior mesentric artery is pressed into superior mesentric artery and aorta). No flow in the the aortic surface. No flow in the celiac trunk (blue celiac trunk (arrow) arrow), Slower flow in the aorta itself (green arrow) 15 TEMA: ULTRALYD Figure 7. MR-Angiography in CGCS. Clearly visible increase of celiac trunk compression (arrows) from inspiration (left) to expiration (right) are necessary to demonstrate the characteristic change of compression degree, most pronounced in expiration (figure 7).