From the Midwestern Vascular Surgical Society
Isolated celiac artery dissection Paul D. DiMusto, MD, Molly M. Oberdoerster, BS, and Enrique Criado, MD, Ann Arbor, Mich Objective: Spontaneous celiac artery dissection is rare, and its natural history is not well studied. The objective of this study was to review our experience with the evaluation and management of this condition. Methods: During the last 8 years, 19 patients (14 men, five women) presented with the diagnosis of spontaneous celiac artery dissection. Each patient’s clinical course was retrospectively reviewed, and patients were contacted for assessment of current symptoms. Results: All patients had computed tomography scans documenting a celiac artery dissection without concomitant aortic dissection. Ages ranged from 39 to 76 years. Seven patients presented with abdominal pain, and 12 were diagnosed incidentally. All patients were initially treated with observation because none had threatened end organs. Patients presenting with aspirin or clopidogrel therapy were continued on these medications, but no patients were prescribed any medications due to their dissection. Three patients continued to have abdominal pain and eventually underwent celiac artery stenting. Pain improved after the intervention in all three. One patient with aneurysmal degeneration of the celiac artery underwent surgical repair. No other patients required intervention. Eighteen patients had follow-up within a year of data collection in the clinic or over the phone. The average time from the initial diagnosis to follow-up for the entire cohort was 46 months. None had abdominal or back pain related to the celiac dissection, had lost weight, or had to change their eating habits. Conclusions: Celiac artery dissection can be safely managed initially with observation. If abdominal pain is persistent, endovascular stenting may stabilize or improve the pain, and surgical reconstruction can be done for aneurysmal degeneration or occlusion, both unusual events. Long-term anticoagulation does not appear necessary in these patients. (J Vasc Surg 2015;61:972-6.)
Spontaneous isolated celiac artery dissection without associated aortic dissection is a rare clinical condition. Fewer than 100 cases have been reported in the literature in the last 12 years, most as isolated case reports.1 The long-term course of celiac dissections is uncertain, and there is no consensus on the optimal strategy of management for this rare problem. Some authors recommend operative or endovascular intervention, whereas others recommend oral anticoagulation. However, whether there is significant benefit from any of these strategies is unclear. We have used a strategy of initial conservative management for patients presenting with spontaneous celiac artery dissection without end-organ malperfusion. Patients were observed clinically, and none were immediately prescribed anticoagulation or taken for operative intervention. We report the results of our experience with 19 patients treated with this approach. METHODS Patients with celiac artery dissection presenting to the senior author (E.C.) through inpatient consultation or From the Section of Vascular Surgery, University of Michigan. Author conflict of interest: none. Presented at the Rapid Fire session of the Thirty-eighth Annual Meeting of the Midwestern Vascular Surgical Society, Coralville, Iowa, September 4-6, 2014. Reprint requests: Enrique Criado, MD, 1500 E Medical Center Dr, 5463 Cardiovascular Center, Ann Arbor, MI 48109-5867 (e-mail: ecriado@ umich.edu). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2015 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvs.2014.10.108
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outpatient referral during the last eight years were prospectively entered into a database that was retrospectively reviewed. Data on patients presenting with threatened end organs needing emergency intervention are not available. Demographic information, presenting complaint, comorbidities, and initial management strategy for each patient were gathered. The clinical course of each patient was also reviewed, and any interventions, including medication changes, endovascular interventions, or open surgical repair, were documented. Patients were enrolled in annual surveillance with celiac duplex ultrasound imaging; however, those who had not been seen in the clinic within the last year were contacted by phone to obtain long-term follow-up. They were asked about any symptoms of abdominal or back pain at rest, pain after eating, weight loss, or change in eating habits since the diagnosis of the celiac artery dissection, any current anticoagulant use, and treatment that may have been received at another institution. Similar data points were abstracted from the medical record of patients who had been seen in the clinic within the last year. The data were organized into a database using Excel software (Microsoft Corp, Redmond, Wash). Given the relatively small number of patients, no statistical analysis was conducted. This study was given exempt status by the University of Michigan Medical Institutional Review Board, including a waiver of informed consent. RESULTS Isolated spontaneous celiac artery dissection was diagnosed in 19 patients, 14 men (75%) and five women, between July 2008 and August 2014. The average age at
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the time of diagnosis was 54 6 10.4 years (range, 39-76 years). In all patients a computed tomography angiogram (CTA) confirmed the diagnosis of isolated celiac artery dissection (Fig). None of the patients had evidence of median arcuate ligament compression of the celiac artery on CTA. Seven patients (37%) presented with abdominal pain that led to the diagnosis, whereas the celiac dissection in 12 (63%) was discovered incidentally on imaging studies done for other reasons. No patients presented with signs or symptoms of visceral ischemia. Patient characteristics, including smoking status, hypertension, and antiplatelet therapy, without intervention are summarized in Table I. No patients were specifically initiated on antiplatelet therapy solely for treatment of their dissection. Any antiplatelet drugs that the patient was previously taking were continued. One patient had a marfanoid body habitus, but Marfan syndrome was not confirmed. One patient had type IV Ehlers-Danlos syndrome. One patient, who had a concomitant dissection of the superior mesenteric artery (SMA), presented with abdominal pain leading to the diagnosis but did not have any evidence of threatened end organs. His pain improved without intervention. Three patients were initially seen at other institutions and were prescribed anticoagulation before referral because of their dissection. This was discontinued after our initial evaluation in two patients. One patient had pre-existing atrial fibrillation and remained on anticoagulation with warfarin for that indication. Three patients (16%) continued to have abdominal pain, presumably related to their dissection, and underwent endovascular stenting of the celiac artery to treat the dissection. These three patients were prescribed antiplatelet therapy with aspirin and clopidogrel after stent placement. The abdominal pain improved after stenting in all three, and at last follow-up, none of these patients reported any abdominal pain. One patient who did not have any comorbidities and had quit smoking 30 years before her diagnosis of celiac artery dissection developed aneurysmal degeneration of the dissected celiac artery measuring 5 cm in diameter and underwent open repair of this aneurysm. This was an incidental finding on a CT scan done for another purpose. However, she presented to our institution with this finding so the timing between the initial dissection and aneurysm development cannot be ascertained. She was prescribed aspirin after her repair. Twelve patients were seen in clinic #1 year of data collection, and an additional six were contacted by telephone. The average time from diagnosis to last follow-up for the entire cohort was 46 6 35 months (range, 1-104 months). The average time from the initial imaging to last follow-up imaging was 35 6 30 months for the entire cohort. For those patients contacted by telephone for follow-up, the average time from the last imaging to the telephone call was 28 months. One patient died of other causes and was found to have a patent celiac artery at the time of autopsy. Of the remaining 18 living patients, none had abdominal or back pain
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Fig. Computed tomography angiogram (CTA) demonstrates a celiac artery dissection extending into the common hepatic artery. The false lumen is thrombosed.
Table I. Patient characteristics Characteristic Sex Male Female Symptomatic Endovascular stent placement Asymptomatic Aspirin (w/o intervention) Plavix (w/o intervention)a Smoking Current smoker Former smoker Never smoker Hypertension
No. (%) (N ¼ 19) 14 5 7 3 12 5 2
(74) (26) (37) (16) (63) (26) (11)
3 4 12 7
(16) (21) (63) (37)
a
Sanofi-Aventis, Bridgewater, NJ.
related to their dissection at last contact. None have had to change eating habits or have unintentionally lost weight since being diagnosed with their dissection. One patient who had undergone stent placement for symptomatic dissection had evidence of a nonflow-limiting in-stent stenosis on follow-up imaging. No significant change from the initial imaging to the last follow-up imaging was seen in any of the other patients. The false lumen was stable or smaller in size compared with the initial examination in all patients. DISCUSSION Isolated celiac artery dissection without concomitant aortic dissection is a rare diagnosis. Initial reports on the topic came from autopsy series, making natural history studies impossible. As CT computed tomography has become more prevalent and resolution has improved, the diagnosis of celiac artery dissection and monitoring patients
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Table II. Literature review Celiac dissection First author
Sx, No.
Asx, No.
SMA dissection Sx, No.
Asx, No.
Treatment
Outcome
20
1 open repair, 1 anticoagulation Anticoagulation Observation 1 open repair for SMA ischemia, no medication in rest Antiplatelet 2 anticoagulation, 1 antiplatelet
Asymptomatic at 6 months Asymptomatic at 6 months Stable imaging at 3 months 31 stable imaging, 3 shrinking false lumen, 2 expanding false lumen Shrinking false lumen at 4 months Resolution of symptoms, stable imaging All asymptomatic at 13 months
Fenoglio2 Poylin3 McGuinness4 Tokue5
2 1 1 6
Wang6 Zhang7
1 2
Takach8
7
4 (1 with celiac)
Choi9
3
11
2
Takayama10
3
5 (1 with celiac)
8 (1 with celiac)
Kang11
2 12
Neychev
1
Batt13
1
Glehen14
5
Amabile15
6
Jung16
4
17
Oh
8
4
7 1
5
4 (2 with celiac) 8
17
11
3 (all with celiac)
2 open repair (1 celiac rupture, 1 SMA ischemia), 6 anticoagulation 2 endovascular stent placement (both SMA), 12 anticoagulation and antiplatelet, 3 observation 1 hepatic artery aneurysm repair, 1 anticoagulation and antiplatelet, 17 no medication Stent graft placement in both for ruptured dissecting aneurysm Stent graft placement and embolization of dissecting aneurysm Embolization of dissecting aneurysm 3 open repair, 2 medical management 2 open repair (1 celiac rupture, 1 SMA ischemia), 6 anticoagulation 3 open repair, 6 endovascular stent, 19 no anticoagulation 1 endovascular stent placement, 7 anticoagulation
Stable or improved dissection on follow-up imaging No progression on 20 month follow up Resolution of symptoms, thrombosis of false lumen Asymptomatic with patent stent graft at 3 months Thrombosis of aneurysm at 9 months All symptom free at 18 months Thrombosis of false lumen in 4 managed conservatively 2 progression of dissection, 14 stable, 12 improved All asymptomatic, stable false lumen in 3, shrinking in 5 at 16 months follow-up
Asx, Asymptomatic; SMA, superior mesenteric artery; Sx, symptomatic.
with dissections has become easier. However, this remains a rare clinical condition with relatively little data surrounding its management. Many published reports mix celiac and SMA dissection, making the data confusing. The case reports and small series that have been published suggest systemic anticoagulation and nonsurgical management of symptomatic and asymptomatic patients presenting with celiac dissection without acute complication.2-10 In cases of bleeding, malperfusion, or aneurysm formation, endovascular intervention or open repair has been described.11-15 Table II summarizes these data. Several of these reports were included in a recent review of spontaneous mesenteric dissection by Garrett,1 who reviewed the course of 88 patients with celiac dissection from across the literature. Treatment data were only available for 62 patients, with 51 being treated initially with medical therapy. The remaining 11 patients required intervention, six endovascular and five open. Only six of those initially managed medically went on to require invasive therapy, four with open surgery and two with endovascular stenting. The author proposed an algorithm for the treatment of spontaneous mesenteric artery
dissection (both celiac artery and SMA) that initially calls for medical management with anticoagulation and antihypertensive therapy with follow-up CTA, unless there is persistent pain or compromised distal perfusion, in which case endovascular or open surgical intervention is recommended.7-9,14,15 Two studies focused specifically on the initial and late radiologic changes seen after splanchnic artery dissection. Jung et al16 reviewed the CT scans of 34 patients with mesenteric artery dissection; of these, 21 had dissections of the SMA, six in the celiac, and multiple dissections in seven. Intimal flap was the most common finding on CT scan in 88% of the patients, a thrombosed false lumen was found in 64%, and aneurysm in 52%. Symptoms were present in 19 patients, and 15 were asymptomatic. Five cases of bowel ischemia were seen on CT scan, all in patients with isolated SMA dissections and none in patients with celiac artery dissections. Only one of the five patients had actual bowel infarction requiring resection. Median time to follow-up imaging was w2 months in the symptomatic group and 1 year in the asymptomatic group. Eight symptomatic patients were treated without anticoagulation
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therapy, one of whom had progression of disease on follow-up imaging manifest by enlargement of the false lumen, whereas four had improvement and three had stable disease. This compares with the 11 patients in the asymptomatic group treated conservatively. Ten had stable disease, and one showed improvement with reduction in the size of the false lumen. Of the six patients requiring endovascular stent placement, all of whom were symptomatic, only one had an isolated celiac artery dissection. Another group reported eight patients with symptomatic spontaneous celiac dissection, three of whom also had concomitant SMA dissections.17 Seven patients were treated with anticoagulation alone, and one required endovascular stent placement for ongoing abdominal pain. The seven treated with anticoagulation remained asymptomatic once their initial symptoms resolved. All patients remained asymptomatic, with a stable false lumen in three and a shrinking false lumen in five at the 16-month follow-up. None of the patients developed aneurysmal degeneration. In summary, our experience is larger compared with previous individual reports of spontaneous isolated celiac dissection. In our series, like most others, most patients were men with an average age in their mid-50s. Nearly two-thirds of the patients were asymptomatic at diagnosis, and of those who were symptomatic, less than half required intervention for relief of symptoms. No patients were treated with longterm systemic anticoagulation for their dissection. All living patients remain asymptomatic with stable or smaller false lumen diameter on follow-up imaging. On the basis of our experience, extension of the dissection into the branches of the celiac artery does not change the outcome or management of these patients. None have had late aneurysmal degeneration other than the patient who initially presented with a 5-cm celiac aneurysm and underwent open repair. There are no consistent recommendations regarding follow-up imaging of these patients. We recommend a repeat CTA and clinical examination at 6 months and 12 months after diagnosis for asymptomatic patients. If the dissection is stable, without aneurysm formation, and the patient remains asymptomatic, then annual duplex ultrasound surveillance is recommended to monitor for aneurysmal degeneration. We recommend continuing annual surveillance for at least 5 years. If the patient remains asymptomatic and the dissection is stable without aneurysmal degeneration, then further follow-up is only needed if the patient becomes symptomatic. If the patient is symptomatic, we recommend CTA and clinical examination every 6 months until symptoms improve or the patient undergoes intervention to treat the dissection. If the patient undergoes endovascular stent placement, we recommend a clinical examination and duplex ultrasound imaging at 6 and 12 months after stent placement, then annually thereafter. There is a lack of data regarding when to repair an aneurysm of the celiac artery, particularly in the setting of a dissection. However, extrapolating from the other visceral artery aneurysm literature, we recommend repair once the aneurysm reaches 3 cm in diameter.
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CONCLUSIONS These findings lead us to conclude that spontaneous celiac artery dissections without evidence of end organ malperfusion or aneurysm formation can be safely managed with an initial strategy of observation and do not require long-term anticoagulation. In the acute, symptomatic setting, however, a course of oral anticoagulation appears reasonable if there is evidence of significant luminal obstruction. Patients who remain symptomatic with abdominal pain after an initial observation period can be treated with endovascular stenting, although the effectiveness of this treatment is impossible to be determined at this time. Open surgical intervention should be reserved for aneurysmal degeneration or arterial occlusion not amenable to endovascular intervention. AUTHOR CONTRIBUTIONS Conception and design: PD, EC Analysis and interpretation: PD, MO, EC Data collection: PD, MO, EC Writing the article: PD, EC Critical revision of the article: PD, MO, EC Final approval of the article: PD, MO, EC Statistical analysis: Not applicable Obtained funding: Not applicable Overall responsibility: EC REFERENCES 1. Garrett HE. Options for treatment of spontaneous mesenteric artery dissection. J Vasc Surg 2014;59:1433-9. 2. Fenoglio L, Allione A, Scalabrino E, Alberto G, Benedetti V, Pomero F, et al. Spontaneous dissection of the celiac artery: a pitfall in the diagnosis of acute abdominal pain. Presentation of two cases. Dig Dis Sci 2004;49:1223-7. 3. Poylin V, Hile C, Campbell D. Medical management of spontaneous celiac artery dissection: case report and literature review. Vasc Endovascular Surg 2008;42:62-4. 4. McGuinness B, Kennedy C, Holden A. Spontaneous coeliac artery dissection. Australas Radiol 2006;50:400-1. 5. Tokue H, Tsushima Y, Endo K. Imaging findings and management of isolated dissection of the visceral arteries. Jpn J Radiol 2009;27: 430-7. 6. Wang HC, Chen JH, Hsiao CC, Jeng CM, Chen WL. Spontaneous dissection of the celiac artery: a case report and literature review. Am J Emerg Med 2013;31:1000. e3-5. 7. Zhang W, Killeen JD, Chiriano J, Bianchi C, Teruya TH, AbouZamzam AM. Management of symptomatic spontaneous isolated visceral artery dissection: is emergent intervention mandatory? Ann Vasc Surg 2009;23:90-4. 8. Takach TJ, Madjarov JM, Holleman JH, Robicsek F, Roush TS. Spontaneous splanchnic dissection: application and timing of therapeutic options. J Vasc Surg 2009;50:557-63. 9. Choi JY, Kwon OJ. Approaches to the management of spontaneous isolated visceral artery dissection. Ann Vasc Surg 2013;27:750-7. 10. Takayama T, Miyata T, Shirakawa M, Nagawa H. Isolated spontaneous dissection of the splanchnic arteries. J Vasc Surg 2008;48: 329-33. 11. Kang UR, Kim YH, Lee YH. Endovascular stent graft for treatment of complicated spontaneous dissection of the celiac artery: report of two cases. Korean J Radiol 2013;14:460-4. 12. Neychev V, Krol E, Dietzek A. Unusual presentation and treatment of spontaneous celiac artery dissection. J Vasc Surg 2013;58:491-5.
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13. Batt M, Baque J. Successful percutaneous embolization of a symptomatic celiac artery dissection with aneurysmal dilation with detachable vascular plugs. J Vasc Surg 2011;54:1812-5. 14. Glehen O, Feugier P, Aleksic Y, Delannoy P, Chevalier JM. Spontaneous dissection of the celiac artery. Ann Vasc Surg 2001;15:687-92. 15. Amabile P, Ouaissi M, Cohen S, Piquet P. Conservative treatment of spontaneous and isolated dissection of the mesenteric arteries. Ann Vasc Surg 2009;23:738-44.
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16. Jung SC, Lee W, Park EA, Jae HJ, Chung JW, Park JH. Spontaneous dissection of the splanchnic arteries: CT findings, treatment and outcome. AJR Am J Roentgenol 2013;200:219-25. 17. Oh S, Cho YP, Kim JH, Shin S, Kwon TW, Ko GY. Symptomatic spontaneous celiac artery dissection treated by conservative management: serial imaging findings. Abdom Imaging 2011;36:79-82. Submitted Sep 15, 2014; accepted Oct 30, 2014.
REQUEST FOR SUBMISSION OF SURGICAL ETHICS CHALLENGES ARTICLES The Editors invite submission of original articles for the Surgical Ethics Challenges section, following the general format established by Dr. James Jones in 2001. Readers have benefitted greatly from Dr. Jones’ monthly ethics contributions for more than 6 years. In order to encourage contributions, Dr. Jones will assist in editing them and will submit his own articles every other month, to provide opportunity for others. Please submit articles under the heading of “Ethics” using Editorial Manager, and follow the format established in previous issues.
Isolated celiac artery dissection Paul D. DiMusto, MD, Molly M. Oberdoerster, BS, and Enrique Criado, MD, Ann Arbor, Mich Objective: Spontaneous celiac artery dissection is rare, and its natural history is not well studied. The objective of this study was to review our experience with the evaluation and management of this condition. Methods: During the last 8 years, 19 patients (14 men, five women) presented with the diagnosis of spontaneous celiac artery dissection. Each patient’s clinical course was retrospectively reviewed, and patients were contacted for assessment of current symptoms. Results: All patients had computed tomography scans documenting a celiac artery dissection without concomitant aortic dissection. Ages ranged from 39 to 76 years. Seven patients presented with abdominal pain, and 12 were diagnosed incidentally. All patients were initially treated with observation because none had threatened end organs. Patients presenting with aspirin or clopidogrel therapy were continued on these medications, but no patients were prescribed any medications due to their dissection. Three patients continued to have abdominal pain and eventually underwent celiac artery stenting. Pain improved after the intervention in all three. One patient with aneurysmal degeneration of the celiac artery underwent surgical repair. No other patients required intervention. Eighteen patients had follow-up within a year of data collection in the clinic or over the phone. The average time from the initial diagnosis to follow-up for the entire cohort was 46 months. None had abdominal or back pain related to the celiac dissection, had lost weight, or had to change their eating habits. Conclusions: Celiac artery dissection can be safely managed initially with observation. If abdominal pain is persistent, endovascular stenting may stabilize or improve the pain, and surgical reconstruction can be done for aneurysmal degeneration or occlusion, both unusual events. Long-term anticoagulation does not appear necessary in these patients. (J Vasc Surg 2015;61:972-6.)
Spontaneous isolated celiac artery dissection without associated aortic dissection is a rare clinical condition. Fewer than 100 cases have been reported in the literature in the last 12 years, most as isolated case reports.1 The long-term course of celiac dissections is uncertain, and there is no consensus on the optimal strategy of management for this rare problem. Some authors recommend operative or endovascular intervention, whereas others recommend oral anticoagulation. However, whether there is significant benefit from any of these strategies is unclear. We have used a strategy of initial conservative management for patients presenting with spontaneous celiac artery dissection without end-organ malperfusion. Patients were observed clinically, and none were immediately prescribed anticoagulation or taken for operative intervention. We report the results of our experience with 19 patients treated with this approach. METHODS Patients with celiac artery dissection presenting to the senior author (E.C.) through inpatient consultation or From the Section of Vascular Surgery, University of Michigan. Author conflict of interest: none. Presented at the Rapid Fire session of the Thirty-eighth Annual Meeting of the Midwestern Vascular Surgical Society, Coralville, Iowa, September 4-6, 2014. Reprint requests: Enrique Criado, MD, 1500 E Medical Center Dr, 5463 Cardiovascular Center, Ann Arbor, MI 48109-5867 (e-mail: ecriado@ umich.edu). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2015 by the Society for Vascular Surgery. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jvs.2014.10.108
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outpatient referral during the last eight years were prospectively entered into a database that was retrospectively reviewed. Data on patients presenting with threatened end organs needing emergency intervention are not available. Demographic information, presenting complaint, comorbidities, and initial management strategy for each patient were gathered. The clinical course of each patient was also reviewed, and any interventions, including medication changes, endovascular interventions, or open surgical repair, were documented. Patients were enrolled in annual surveillance with celiac duplex ultrasound imaging; however, those who had not been seen in the clinic within the last year were contacted by phone to obtain long-term follow-up. They were asked about any symptoms of abdominal or back pain at rest, pain after eating, weight loss, or change in eating habits since the diagnosis of the celiac artery dissection, any current anticoagulant use, and treatment that may have been received at another institution. Similar data points were abstracted from the medical record of patients who had been seen in the clinic within the last year. The data were organized into a database using Excel software (Microsoft Corp, Redmond, Wash). Given the relatively small number of patients, no statistical analysis was conducted. This study was given exempt status by the University of Michigan Medical Institutional Review Board, including a waiver of informed consent. RESULTS Isolated spontaneous celiac artery dissection was diagnosed in 19 patients, 14 men (75%) and five women, between July 2008 and August 2014. The average age at
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the time of diagnosis was 54 6 10.4 years (range, 39-76 years). In all patients a computed tomography angiogram (CTA) confirmed the diagnosis of isolated celiac artery dissection (Fig). None of the patients had evidence of median arcuate ligament compression of the celiac artery on CTA. Seven patients (37%) presented with abdominal pain that led to the diagnosis, whereas the celiac dissection in 12 (63%) was discovered incidentally on imaging studies done for other reasons. No patients presented with signs or symptoms of visceral ischemia. Patient characteristics, including smoking status, hypertension, and antiplatelet therapy, without intervention are summarized in Table I. No patients were specifically initiated on antiplatelet therapy solely for treatment of their dissection. Any antiplatelet drugs that the patient was previously taking were continued. One patient had a marfanoid body habitus, but Marfan syndrome was not confirmed. One patient had type IV Ehlers-Danlos syndrome. One patient, who had a concomitant dissection of the superior mesenteric artery (SMA), presented with abdominal pain leading to the diagnosis but did not have any evidence of threatened end organs. His pain improved without intervention. Three patients were initially seen at other institutions and were prescribed anticoagulation before referral because of their dissection. This was discontinued after our initial evaluation in two patients. One patient had pre-existing atrial fibrillation and remained on anticoagulation with warfarin for that indication. Three patients (16%) continued to have abdominal pain, presumably related to their dissection, and underwent endovascular stenting of the celiac artery to treat the dissection. These three patients were prescribed antiplatelet therapy with aspirin and clopidogrel after stent placement. The abdominal pain improved after stenting in all three, and at last follow-up, none of these patients reported any abdominal pain. One patient who did not have any comorbidities and had quit smoking 30 years before her diagnosis of celiac artery dissection developed aneurysmal degeneration of the dissected celiac artery measuring 5 cm in diameter and underwent open repair of this aneurysm. This was an incidental finding on a CT scan done for another purpose. However, she presented to our institution with this finding so the timing between the initial dissection and aneurysm development cannot be ascertained. She was prescribed aspirin after her repair. Twelve patients were seen in clinic #1 year of data collection, and an additional six were contacted by telephone. The average time from diagnosis to last follow-up for the entire cohort was 46 6 35 months (range, 1-104 months). The average time from the initial imaging to last follow-up imaging was 35 6 30 months for the entire cohort. For those patients contacted by telephone for follow-up, the average time from the last imaging to the telephone call was 28 months. One patient died of other causes and was found to have a patent celiac artery at the time of autopsy. Of the remaining 18 living patients, none had abdominal or back pain
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Fig. Computed tomography angiogram (CTA) demonstrates a celiac artery dissection extending into the common hepatic artery. The false lumen is thrombosed.
Table I. Patient characteristics Characteristic Sex Male Female Symptomatic Endovascular stent placement Asymptomatic Aspirin (w/o intervention) Plavix (w/o intervention)a Smoking Current smoker Former smoker Never smoker Hypertension
No. (%) (N ¼ 19) 14 5 7 3 12 5 2
(74) (26) (37) (16) (63) (26) (11)
3 4 12 7
(16) (21) (63) (37)
a
Sanofi-Aventis, Bridgewater, NJ.
related to their dissection at last contact. None have had to change eating habits or have unintentionally lost weight since being diagnosed with their dissection. One patient who had undergone stent placement for symptomatic dissection had evidence of a nonflow-limiting in-stent stenosis on follow-up imaging. No significant change from the initial imaging to the last follow-up imaging was seen in any of the other patients. The false lumen was stable or smaller in size compared with the initial examination in all patients. DISCUSSION Isolated celiac artery dissection without concomitant aortic dissection is a rare diagnosis. Initial reports on the topic came from autopsy series, making natural history studies impossible. As CT computed tomography has become more prevalent and resolution has improved, the diagnosis of celiac artery dissection and monitoring patients
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Table II. Literature review Celiac dissection First author
Sx, No.
Asx, No.
SMA dissection Sx, No.
Asx, No.
Treatment
Outcome
20
1 open repair, 1 anticoagulation Anticoagulation Observation 1 open repair for SMA ischemia, no medication in rest Antiplatelet 2 anticoagulation, 1 antiplatelet
Asymptomatic at 6 months Asymptomatic at 6 months Stable imaging at 3 months 31 stable imaging, 3 shrinking false lumen, 2 expanding false lumen Shrinking false lumen at 4 months Resolution of symptoms, stable imaging All asymptomatic at 13 months
Fenoglio2 Poylin3 McGuinness4 Tokue5
2 1 1 6
Wang6 Zhang7
1 2
Takach8
7
4 (1 with celiac)
Choi9
3
11
2
Takayama10
3
5 (1 with celiac)
8 (1 with celiac)
Kang11
2 12
Neychev
1
Batt13
1
Glehen14
5
Amabile15
6
Jung16
4
17
Oh
8
4
7 1
5
4 (2 with celiac) 8
17
11
3 (all with celiac)
2 open repair (1 celiac rupture, 1 SMA ischemia), 6 anticoagulation 2 endovascular stent placement (both SMA), 12 anticoagulation and antiplatelet, 3 observation 1 hepatic artery aneurysm repair, 1 anticoagulation and antiplatelet, 17 no medication Stent graft placement in both for ruptured dissecting aneurysm Stent graft placement and embolization of dissecting aneurysm Embolization of dissecting aneurysm 3 open repair, 2 medical management 2 open repair (1 celiac rupture, 1 SMA ischemia), 6 anticoagulation 3 open repair, 6 endovascular stent, 19 no anticoagulation 1 endovascular stent placement, 7 anticoagulation
Stable or improved dissection on follow-up imaging No progression on 20 month follow up Resolution of symptoms, thrombosis of false lumen Asymptomatic with patent stent graft at 3 months Thrombosis of aneurysm at 9 months All symptom free at 18 months Thrombosis of false lumen in 4 managed conservatively 2 progression of dissection, 14 stable, 12 improved All asymptomatic, stable false lumen in 3, shrinking in 5 at 16 months follow-up
Asx, Asymptomatic; SMA, superior mesenteric artery; Sx, symptomatic.
with dissections has become easier. However, this remains a rare clinical condition with relatively little data surrounding its management. Many published reports mix celiac and SMA dissection, making the data confusing. The case reports and small series that have been published suggest systemic anticoagulation and nonsurgical management of symptomatic and asymptomatic patients presenting with celiac dissection without acute complication.2-10 In cases of bleeding, malperfusion, or aneurysm formation, endovascular intervention or open repair has been described.11-15 Table II summarizes these data. Several of these reports were included in a recent review of spontaneous mesenteric dissection by Garrett,1 who reviewed the course of 88 patients with celiac dissection from across the literature. Treatment data were only available for 62 patients, with 51 being treated initially with medical therapy. The remaining 11 patients required intervention, six endovascular and five open. Only six of those initially managed medically went on to require invasive therapy, four with open surgery and two with endovascular stenting. The author proposed an algorithm for the treatment of spontaneous mesenteric artery
dissection (both celiac artery and SMA) that initially calls for medical management with anticoagulation and antihypertensive therapy with follow-up CTA, unless there is persistent pain or compromised distal perfusion, in which case endovascular or open surgical intervention is recommended.7-9,14,15 Two studies focused specifically on the initial and late radiologic changes seen after splanchnic artery dissection. Jung et al16 reviewed the CT scans of 34 patients with mesenteric artery dissection; of these, 21 had dissections of the SMA, six in the celiac, and multiple dissections in seven. Intimal flap was the most common finding on CT scan in 88% of the patients, a thrombosed false lumen was found in 64%, and aneurysm in 52%. Symptoms were present in 19 patients, and 15 were asymptomatic. Five cases of bowel ischemia were seen on CT scan, all in patients with isolated SMA dissections and none in patients with celiac artery dissections. Only one of the five patients had actual bowel infarction requiring resection. Median time to follow-up imaging was w2 months in the symptomatic group and 1 year in the asymptomatic group. Eight symptomatic patients were treated without anticoagulation
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therapy, one of whom had progression of disease on follow-up imaging manifest by enlargement of the false lumen, whereas four had improvement and three had stable disease. This compares with the 11 patients in the asymptomatic group treated conservatively. Ten had stable disease, and one showed improvement with reduction in the size of the false lumen. Of the six patients requiring endovascular stent placement, all of whom were symptomatic, only one had an isolated celiac artery dissection. Another group reported eight patients with symptomatic spontaneous celiac dissection, three of whom also had concomitant SMA dissections.17 Seven patients were treated with anticoagulation alone, and one required endovascular stent placement for ongoing abdominal pain. The seven treated with anticoagulation remained asymptomatic once their initial symptoms resolved. All patients remained asymptomatic, with a stable false lumen in three and a shrinking false lumen in five at the 16-month follow-up. None of the patients developed aneurysmal degeneration. In summary, our experience is larger compared with previous individual reports of spontaneous isolated celiac dissection. In our series, like most others, most patients were men with an average age in their mid-50s. Nearly two-thirds of the patients were asymptomatic at diagnosis, and of those who were symptomatic, less than half required intervention for relief of symptoms. No patients were treated with longterm systemic anticoagulation for their dissection. All living patients remain asymptomatic with stable or smaller false lumen diameter on follow-up imaging. On the basis of our experience, extension of the dissection into the branches of the celiac artery does not change the outcome or management of these patients. None have had late aneurysmal degeneration other than the patient who initially presented with a 5-cm celiac aneurysm and underwent open repair. There are no consistent recommendations regarding follow-up imaging of these patients. We recommend a repeat CTA and clinical examination at 6 months and 12 months after diagnosis for asymptomatic patients. If the dissection is stable, without aneurysm formation, and the patient remains asymptomatic, then annual duplex ultrasound surveillance is recommended to monitor for aneurysmal degeneration. We recommend continuing annual surveillance for at least 5 years. If the patient remains asymptomatic and the dissection is stable without aneurysmal degeneration, then further follow-up is only needed if the patient becomes symptomatic. If the patient is symptomatic, we recommend CTA and clinical examination every 6 months until symptoms improve or the patient undergoes intervention to treat the dissection. If the patient undergoes endovascular stent placement, we recommend a clinical examination and duplex ultrasound imaging at 6 and 12 months after stent placement, then annually thereafter. There is a lack of data regarding when to repair an aneurysm of the celiac artery, particularly in the setting of a dissection. However, extrapolating from the other visceral artery aneurysm literature, we recommend repair once the aneurysm reaches 3 cm in diameter.
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CONCLUSIONS These findings lead us to conclude that spontaneous celiac artery dissections without evidence of end organ malperfusion or aneurysm formation can be safely managed with an initial strategy of observation and do not require long-term anticoagulation. In the acute, symptomatic setting, however, a course of oral anticoagulation appears reasonable if there is evidence of significant luminal obstruction. Patients who remain symptomatic with abdominal pain after an initial observation period can be treated with endovascular stenting, although the effectiveness of this treatment is impossible to be determined at this time. Open surgical intervention should be reserved for aneurysmal degeneration or arterial occlusion not amenable to endovascular intervention. AUTHOR CONTRIBUTIONS Conception and design: PD, EC Analysis and interpretation: PD, MO, EC Data collection: PD, MO, EC Writing the article: PD, EC Critical revision of the article: PD, MO, EC Final approval of the article: PD, MO, EC Statistical analysis: Not applicable Obtained funding: Not applicable Overall responsibility: EC REFERENCES 1. Garrett HE. Options for treatment of spontaneous mesenteric artery dissection. J Vasc Surg 2014;59:1433-9. 2. Fenoglio L, Allione A, Scalabrino E, Alberto G, Benedetti V, Pomero F, et al. Spontaneous dissection of the celiac artery: a pitfall in the diagnosis of acute abdominal pain. Presentation of two cases. Dig Dis Sci 2004;49:1223-7. 3. Poylin V, Hile C, Campbell D. Medical management of spontaneous celiac artery dissection: case report and literature review. Vasc Endovascular Surg 2008;42:62-4. 4. McGuinness B, Kennedy C, Holden A. Spontaneous coeliac artery dissection. Australas Radiol 2006;50:400-1. 5. Tokue H, Tsushima Y, Endo K. Imaging findings and management of isolated dissection of the visceral arteries. Jpn J Radiol 2009;27: 430-7. 6. Wang HC, Chen JH, Hsiao CC, Jeng CM, Chen WL. Spontaneous dissection of the celiac artery: a case report and literature review. Am J Emerg Med 2013;31:1000. e3-5. 7. Zhang W, Killeen JD, Chiriano J, Bianchi C, Teruya TH, AbouZamzam AM. Management of symptomatic spontaneous isolated visceral artery dissection: is emergent intervention mandatory? Ann Vasc Surg 2009;23:90-4. 8. Takach TJ, Madjarov JM, Holleman JH, Robicsek F, Roush TS. Spontaneous splanchnic dissection: application and timing of therapeutic options. J Vasc Surg 2009;50:557-63. 9. Choi JY, Kwon OJ. Approaches to the management of spontaneous isolated visceral artery dissection. Ann Vasc Surg 2013;27:750-7. 10. Takayama T, Miyata T, Shirakawa M, Nagawa H. Isolated spontaneous dissection of the splanchnic arteries. J Vasc Surg 2008;48: 329-33. 11. Kang UR, Kim YH, Lee YH. Endovascular stent graft for treatment of complicated spontaneous dissection of the celiac artery: report of two cases. Korean J Radiol 2013;14:460-4. 12. Neychev V, Krol E, Dietzek A. Unusual presentation and treatment of spontaneous celiac artery dissection. J Vasc Surg 2013;58:491-5.
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13. Batt M, Baque J. Successful percutaneous embolization of a symptomatic celiac artery dissection with aneurysmal dilation with detachable vascular plugs. J Vasc Surg 2011;54:1812-5. 14. Glehen O, Feugier P, Aleksic Y, Delannoy P, Chevalier JM. Spontaneous dissection of the celiac artery. Ann Vasc Surg 2001;15:687-92. 15. Amabile P, Ouaissi M, Cohen S, Piquet P. Conservative treatment of spontaneous and isolated dissection of the mesenteric arteries. Ann Vasc Surg 2009;23:738-44.
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16. Jung SC, Lee W, Park EA, Jae HJ, Chung JW, Park JH. Spontaneous dissection of the splanchnic arteries: CT findings, treatment and outcome. AJR Am J Roentgenol 2013;200:219-25. 17. Oh S, Cho YP, Kim JH, Shin S, Kwon TW, Ko GY. Symptomatic spontaneous celiac artery dissection treated by conservative management: serial imaging findings. Abdom Imaging 2011;36:79-82. Submitted Sep 15, 2014; accepted Oct 30, 2014.
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