221 lines
11 KiB
Markdown
221 lines
11 KiB
Markdown
---
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title: "Intimointimal Intussusception"
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docid: "a7004bbb-3699-4ed4-af21-78f51a3685d9"
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authors:
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- key: "b00d2bdb-66e1-41ed-90b4-c52904f4d598"
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value: "Seth Kligerman, MD, MS"
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breadcrumbs:
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-
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name: "Cardiac"
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slug: "cardiac"
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treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
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-
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name: "Aorta"
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slug: "aorta"
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treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
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-
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name: "Intimointimal Intussusception"
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slug: "intimointimal-intussusception"
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treeNodeId: null
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category: "Cardiac"
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documentVersionId: "178f7921-c162-43ee-b7ae-313021635390"
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imageCount: 4
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lastUpdated: "01/28/25"
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pageDescription: "Intimointimal Intussusception"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Intimointimal Intussusception"
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pageTitle: "Intimointimal Intussusception | STATdx"
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enhancedTitle: "Intimointimal Intussusception"
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type: "DX"
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references: true
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breadcrumbs:
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- "Cardiac"
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- "Diagnosis"
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- "Aorta"
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- "Intimointimal Intussusception"
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---
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# KEY FACTS
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- ## Terminology
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- Complete circumferential intimal tear in type A > type B aortic dissection
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- Delaminated intima then rolls upon itself
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- ## Imaging
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- Best visualized with ECG-gated CTA
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- Circumferential intimal tear → unidirectional or bidirectional intimointimal intussusception (IIIS)
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- Anterograde (43%): Intima superior circumferential intimal tear delaminates upward
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- Intimal often folded upon itself in aortic arch
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- Retrograde (56%): Intima inferior to circumferential tear delaminates inferiorly
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- Intimal may prolapse into LV during diastole
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- Bidirectional (1%): IIIS both anterograde and retrograde
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- Between delaminated intima that has intussuscepted superiorly &/or inferiorly, portion of aorta appears "normal" but actually lacks intima
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- Missing flap or naked aorta sign
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- ## Clinical Issues
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- Diagnosis not prospectively made before surgical intervention in 49% of anterograde IIIS and 16% of retrograde IIIS
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- Anterograde
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- Chest pain
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- Neurologic symptoms (62%)
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- Asymmetric blood pressure (57%)
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- Retrograde
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- Myocardial infarction (26.5%)
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- Severe aortic regurgitation
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- Mean age: 54 years
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- M > F
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- Overall mortality: 24%
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- Treatment is surgical repair
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# TERMINOLOGY
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- ## Abbreviations
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- Intimointimal intussusception (IIIS)
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- ## Definitions
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- Complete circumferential intimal tear in type A aortic dissection; delaminated intima then rolls upon itself like tube sock; given this is circumferential tear, intima can intussuscept anterograde (upward) into aortic arch (AA)/descending thoracic aorta (DTA) &/or retrograde (downward) into aortic root and left ventricle (LV)
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Type A dissection where layers of intimal are folded in on itself within ascending &/or DTA
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- ### Location
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- Ascending aorta or AA (98%)
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- DTA (2%)
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- ## Radiographic Findings
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- No specific radiographic findings are visible other than findings associated with type A dissection
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- ## CT Findings
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- Best visualized with ECG-gated CTA
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- Findings of type A dissection in most cases
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- Circumferential intimal tear leads to either unidirectional or bidirectional IIIS
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- Anterograde (43%): Intima superior to level of circumferential intimal tear delaminates upward into AA and DTA
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- Intimal is often seen folded upon itself in AA or DTA
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- Intima may obstruct arch vessels leading to neurologic sequela
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- Retrograde (56%): Intima inferior to level of circumferential tear delaminates inferiorly into aortic root and may prolapse into LV during diastole
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- Bidirectional (1%): IIIS both anterograde and retrograde
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- Between delaminated intima that has intussuscepted superiorly &/or inferiorly, there is portion of aorta that appears normal, i.e., free of dissection flap
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- This portion of aorta lacks intima
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- Sometimes called missing flap or naked aorta sign
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- Anterograde IIIS can involve entire thoracic aorta and extend into abdominal aorta or into iliac arteries
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- IIIS occluding superior mesenteric artery (SMA) can lead to mesenteric ischemia
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- ## MR Findings
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- Few case reports of IIIS visualized with MR
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- Best seen with bright-blood imaging, such as gated SSFP imaging or contrast-enhanced MRA
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- Similar findings to CT with unidirectional or bidirectional intima that has rolled upon itself superiorly &/or inferiorly
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- ## Echocardiographic Findings
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- May see portion of intimal flap prolapsing into LV
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- 71% in retrograde IIIS
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- Intimal flap in aortic root
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- Aortic regurgitation; often severe
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- 84% in retrograde IIIS
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- ## Imaging Recommendations
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- ### Best imaging tool
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- ECG-gated CTA
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# DIFFERENTIAL DIAGNOSIS
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- ## Type A Dissection Without Intimointimal Intussusception
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- Will not see delaminated intimal folded on itself in aortic lumen
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Anterograde
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- Chest pain
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- Neurologic symptoms (62%)
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- Cerebrovascular accident
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- Syncope
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- Asymmetric blood pressure (57%)
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- Likely due to subclavian artery occlusion
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- Retrograde
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- Chest and back pain
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- Dyspnea
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- Myocardial infarction (26.5%)
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- Aortic regurgitation (84%); often severe
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- ## Demographics
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- Mean age: 54 years
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- M > F
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- ## Natural History & Prognosis
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- Overall mortality: 24%
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- Preoperative and postoperative mortality of 14% and 9%, respectively
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- Similar mortality for anterograde and retrograde IIIS
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- ## Treatment
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- Diagnosis not prospectively made before surgical intervention in 49% of anterograde IIIS and 16% of retrograde IIIS
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- Aortotomy and surgical repair
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- Various techniques, including Bentall repair, ascending aortic and arch replacement
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- Intussuscepted intima may be retracted upward &/or downward and tacked down to aorta
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588886d4-5705-455d-83be-af8b469012b8
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## References
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# Selected References
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1. [Dokollari A et al: Aortic intimo-intimal intussusception in Stanford type A acute aortic dissection. Eur Heart J. 42(34):3410, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33085750%5Bpmid%5D)
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1. [Wu ZY et al: Aortic Intimo-intimal intussusception: a pooled analysis of published reports. Ann Vasc Surg. 75:471-8, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=33831523%5Bpmid%5D)
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1. [Thunberg CA et al: Echocardiographic detection of intimo-intimal intussusception in a patient with acute Stanford type A aortic dissection. Ann Card Anaesth. 18(2):227-30, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25849697%5Bpmid%5D)
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1. [Sanders LH et al: Radiological diagnosis and classification of antegrade and retrograde Stanford type A intimal intussusception. Int J Cardiovasc Imaging. 23(5):659-65, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17160426%5Bpmid%5D)
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1. [Fan ZM et al: Acute aortic dissection with intimal intussusception: MRI appearances. AJR Am J Roentgenol. 186(3):841-3, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16498118%5Bpmid%5D)
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## Images
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### Selected Images
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*Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde <img src='img/arrows/CS.png'/> into the aortic arch and retrograde <img src='img/arrows/CC.png'/> into the left ventricle.*
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*Type A aortic dissection with bidirectional intimointimal intussusception (IIIS) is shown. At the point of circumferential intimal tear, the delaminated intima rolls anterograde <img src='img/arrows/CS.png'/> into the aortic arch and retrograde <img src='img/arrows/CC.png'/> into the left ventricle.*
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*Sagittal oblique CECT shows the anterograde IIIS folded upon itself in the proximal descending thoracic aorta <img src='img/arrows/CS.png'/>. Intimal calcifications can be seen.*
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*Echo image during systole (left) shows a portion of the delaminated intima in the ascending aorta <img src='img/arrows/CO.png'/>. During diastole (right), there is retrograde IIIS into the left ventricle <img src='img/arrows/CS.png'/>.*
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*CTA in a 31-year-old man with chest and arm pain shows a type A dissection with a circumferential intimal tear and flap <img src='img/arrows/CS.png'/> in the aortic root. The anterograde IIIS has lodged into and occluded the left subclavian artery <img src='img/arrows/CO.png'/>. Only a small portion of intima is in the aortic arch <img src='img/arrows/CC.png'/>.*
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