Imaging Pearls ❯ Vascular ❯ AVMs and Vasc Malformations
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- Dieulafoy's lesion (or Dieulofoy lesion) is a medical condition characterized by a large tortuous arteriole most commonly in the stomach wall (submucosal) that erodes and bleeds. It can present in any part of the gastrointestinal tract.[2] It can cause gastric hemorrhage[3] but is relatively uncommon. It is thought to cause less than 5% of all gastrointestinal bleeds in adults. It was named after French surgeon Paul Georges Dieulafoy, who described this condition in his paper "Exulceratio simplex: Leçons 1-3" in 1898.
- “A Dieulafoy lesion describes a tortuous, submucosal artery in the gastrointestinal tract—most commonly the posterior stomach—that penetrates through the mucosa over time, eventually perforating to cause severe gastrointestinal bleeding. Due to its insidious onset, tendency to cause intermittent but severe bleeding, and difficulty of endoscopic diagnosis, Dieulafoy lesion has a very high mortality rate. Although originally thought not to be a radiologically diagnosable entity, Dieulafoy lesions can be seen at enhanced CT of the abdomen.”
Dieulafoy lesion: CT diagnosis of this lesser-known cause of gastrointestinal bleeding
A. Batouli et al.
Clinical Radiology,Volume 70, Issue 6,2015,Pages 661-666 - “A Dieulafoy lesion describes a tortuous, submucosal artery in the gastrointestinal tract—most commonly the posterior stomach—that penetrates through the mucosa over time, eventually perforating to cause severe gastrointestinal bleeding. The lesion is not associated with surrounding ulcer or inflammation. Due to its insidious onset, tendency to cause intermittent but severe bleeding, and difficulty of diagnosis, a Dieulafoy lesion has up to an 80% mortality rate. Due to intermittent and heavy bleeding, initial endoscopic evaluation often does not provide accurate diagnosis of Dieulafoy lesions.”
Dieulafoy lesion: CT diagnosis of this lesser-known cause of gastrointestinal bleeding
A. Batouli et al.
Clinical Radiology,Volume 70, Issue 6,2015,Pages 661-666 - "Dieulafoy lesions are twice as common in men as they are in women and can occur at any age, most commonly presenting in the fifth decade.Patients usually present with massive bleeding, most often without an associated prodrome of gastric complaints, which can be seen with ulcer disease. Interestingly, a large portion of presentations occur in patients that are already hospitalized for a different condition, pointing towards physiological stress as an inciting factor for arterial perforation.”
Dieulafoy lesion: CT diagnosis of this lesser-known cause of gastrointestinal bleeding
A. Batouli et al.
Clinical Radiology,Volume 70, Issue 6,2015,Pages 661-666 - "The CT findings of a Dieulafoy lesion include an abnormally enlarged submucosal vessel, which may appear serpentine, linear, or as a non-specific blush of apparent mucosal/submucosal contrast medium. Contrast medium within the bowel or stomach lumen makes the lesion more conspicuous and suggests active extravasation. It is important to view the gastric mucosa and bowel in as many imaging planes as possible, preferably at least two, as sagittal or coronal imaging may make the abnormally large submucosal vessel more easily identifiable.”
Dieulafoy lesion: CT diagnosis of this lesser-known cause of gastrointestinal bleeding
A. Batouli et al.
Clinical Radiology,Volume 70, Issue 6,2015,Pages 661-666 - "Near-definitive diagnosis of a Dieulafoy lesion can be obtained with enhanced CT of the abdomen. Optimal studies are performed in the arterial phase of intravenous contrast enhancement without administration of oral contrast material. CTA shows an enlarged submucosal arteriole in the gastrointestinal submucosal layer with or without active contrast medium extravasation into the lumen. Familiarity with the lesion and its manifestations can help guide radiologists to make the correct diagnosis in cases where endoscopy or standard angiography have failed. CT diagnosis can then help guide endoscopic and endovascular localization and treatment, ultimately improving patient outcomes.”
Dieulafoy lesion: CT diagnosis of this lesser-known cause of gastrointestinal bleeding
A. Batouli et al.
Clinical Radiology,Volume 70, Issue 6,2015,Pages 661-666
- “Hereditary hemorrhagic telangiectasia(HHT),also known as Osler- Weber-Rendu disease, is an auto- somal dominant genetic disorder affecting 1 in 8000 people. HHT is known to be caused by at least three mutations: ENG (type I HHT) encoding endoglin; ACVRL1 (type II HHT) encoding activin receptorlike kinase 1 (ALK-1); and SMAD4, associated with juvenile polyposis. Clinically, HHT is diagnosed according to the Curaçao criteria, which include multiple mucocutaneous telangiectases, epistaxis, visceral arteriovenous malformations (AVMs), and a family history of HHT in a first-degree relative .”
CT Angiography Findings of Pulmonary Arteriovenous Malformations in Children and Young Adults With Hereditary Hemorrhagic Telangiectasia
Shin SM et al.
AJR 2020; 214:1369–1376 - "Pulmonary AVMs (PAVMs) are seen in as many as 49% of patients with HHT. Although serious complications are rare, PAVMs can lead to life-threatening events including massive hemorrhage from PAVM rupture and complications of right-to-left cardiac shunt, including pulmonary hypertension, paradoxic embolism with stroke, and cerebral abscess. PAVMs tend to be progressive with age. To prevent progression in size and the serious complications associated with PAVMs, early detection and intervention are critical.”
CT Angiography Findings of Pulmonary Arteriovenous Malformations in Children and Young Adults With Hereditary Hemorrhagic Telangiectasia
Shin SM et al.
AJR 2020; 214:1369–1376 - "The imaging findings of PAVM have been well described in adult chest CT studies and include presence of a feeding artery, a draining vein or veins, and an aneurysmal sac or nidus. A feeding artery larger than 3 mm is currently accepted as an indication for prophylactic embolization in an effort to reduce the risk of cerebral complications, although smaller lesions can be embolized if symptomatic.”
CT Angiography Findings of Pulmonary Arteriovenous Malformations in Children and Young Adults With Hereditary Hemorrhagic Telangiectasia
Shin SM et al.
AJR 2020; 214:1369–1376 - "The most common PAVMs in children and young adults with HHT were less ma- ture than those seen in adult populations. Use of MIP enabled more precise detec- tion of the afferent and efferent vessels. The highest grade in each patient had the strongest correlation with the severity of pulmonary right-to-left shunt. Unlike the situation in older adults, age was not an important factor in determining the severity of PAVMs in children and young adults. Even in patients without symptoms, PAVMs are noted in most patients with abnormal CE- TTE findings."
CT Angiography Findings of Pulmonary Arteriovenous Malformations in Children and Young Adults With Hereditary Hemorrhagic Telangiectasia
Shin SM et al.
AJR 2020; 214:1369–1376 - "Routine screening of all patients with HHT for PAVM is recom- mended, and contrast-enhanced transthoracic echocardiography (CE-TTE) and chest CT have been the primary imaging tools.”
CT Angiography Findings of Pulmonary Arteriovenous Malformations in Children and Young Adults With Hereditary Hemorrhagic Telangiectasia
Shin SM et al.
AJR 2020; 214:1369–1376 - "Accessory renal arteries are present in 30% of the individuals and can be found bilaterally in about 10% of the individuals. Accessory renal arteries are much more common in individuals with renal fusion or positional anomalies of the kidneys, including horseshoe kidney or crossed fused renal ectopia. Accessory renal arteries usually arise more inferior to the main renal arteries. However, they can arise anywhere between T11 and L4 vertebral bodies. Accessory renal arteries cross anterior to the ureter and can cause obstruction renal arteries), in contrast to accessory renal arteries that enter the kidney through the renal hilum, considered a relative contraindication for living of the ureter.”
Recent Innovations in Renal Vascular Imaging
Arash Bedayat et al
Radiol Clin N Am 58 (2020) 781–796
- “ CTA can be used as part of the preoperative evaluation prior to laparoscopic nephrectomy to provide anatomical information about the presence of multiple renal arteries in the affected kidney of patients with RCC. This could help with planning the surgery and reducing surgical complications.”
Multiple renal arteries with renal cell carcinoma: preoperative evaluation using computed tomography angiography prior to laparoscopic nephrectomy.
Guan WH et al.
J Int Med Res. 2013 Oct;41(5):1705-15. - “ Congenital renal arteriovenous malformations (AVMs) are abnormal communications between the intrarenal venous and arterial system. Such anomalies in the renal vasculature are rare causes of gross hematuria. A significant number of patients with renal AVMs are hypertensive, and its clinical presentation is variable, ranging from incidental finding in an asymptomatic patient to congestive cardiac failure due to high output from a giant AVM. Its classical presentation is that of gross hematuria, occasionally associated with the passage of blood clots and flank pain.”
Congenital renal arteriovenous malformation presenting with gross hematuria after a routine jog: a case report
Erin L Dames et al.
Journal of Medical Case Reports 2014, 8:65 - “Renal AVM can be idiopathic, congenital or acquired, and are usually differentiated by their angiographic configuration. Acquired renal AVM (more frequently referred to as arteriovenous fistulas) are the most common type (up to 75% of all cases), frequently due to iatrogenic trauma such as renal biopsy or surgery. Other causes include blunt or penetrating trauma, pyelonephritis and renal cell carcinoma. Such cases are characterized in imaging studies as a solitary communication between an artery and a vein. Congenital renal AVM make up about 25% of all cases, and usually present in the third to fourth decade of life.”
Congenital renal arteriovenous malformation presenting with gross hematuria after a routine jog: a case report
Erin L Dames et al.
Journal of Medical Case Reports 2014, 8:65 - “Minimally invasive percutaneous transarterial embolization therapy is the first line in the treatment of renal AVM. Embolization can be performed using different materials such as glue, the Onyx® liquid embolic system, alcohol, gelatin sponges and foams, and PVA particles. Disadvantages of embolization therapy include the risk of nephropathy secondary to the contrast used during radiographic evaluation as well as the possible need for repeat sessions.
Surgical treatment of renal AVM is reserved for cases of large cirsoid malformations, cases intractable to medical and/or endovascular therapy and cases related to malignancy; there have been documented cases of renal AVM requiring urgent nephrectomy.”
Congenital renal arteriovenous malformation presenting with gross hematuria after a routine jog: a case report
Erin L Dames et al.
Journal of Medical Case Reports 2014, 8:65
- “Acute arterial injuries are often complicated by the development of an arteriovenous fistula (AVF). In the acute setting, an AVF may present at the same time as the arterial injury. A case of traumatic AVF in the thigh that presented with normal neurovascular examination findings is reported. AVF was diagnosed by duplex scan and managed promptly. The authors suggest that duplex imaging together with arteriography, where appropriate, should be performed routinely when penetrating wounds are in close proximity to major vessels despite a normal clinical neurovascular examination.”
Diagnosis and management of acute traumatic arteriovenous fistula
Nagpal K et al.
Int J Angiol 2008;17(4):214-216 - “Ectasia at the proximal arterial structures is a phenomenon secondary to distal arteriovenous (AV) fistulae and proximal arterial structures must be evaluated carefully prior to an intervention to the lesion especially in long lasting AV fistulae. In this report we present the images of a 58-year-old male patient with a 25-year history of gun shot injury and arteriovenous fistula formation at the femoral region.”
Arteriovenous fistula leading to severe tortuosity andaneurysm formation
Ugurlucan M et al.
J Card Surg 2009 Mar-Apr 24(2):109
- Vascular Malformations and Hemangiomatosis Syndromes
- Blue rubber bleb nevus syndrome
- Proteus syndrome
- Klippel-Trenauney Sydrome
- Kasabach-Merritt syndrome
- Blue rubber bleb nevus syndrome: facts
- Multiple subcutaneous as well as GI tract small tumors
- Vascular GI lesions will commonly bleed
- Can involve entire GI tract but most commonly small bowel
- Associated with increased incidence of medulloblastoma, CLL, RCC and squammous cell carcinoma
- Proteus Syndrome: facts
- Hemihypertrophy, partial gigantism
- Vascular tumors and AVMs
- Lipomas and fatty tumor proliferation
- splenomegaly
- Klippel-Trenaunay Syndrome: Facts
- Three key findings
–Port wine stain
–Abnormal vascular structures
–Osseous and soft tissue hypertrophy
- Klippel-Trenaunay Syndrome: Facts
- Patients have GI tract hemangiomas including bowel involvement with calcifications
- Kasabach-Merritt Syndrome: facts
- Consumptive thrombocytopenia and coagulopathy
- Can lead to DIC
- May have large hepatic or splenic hemangioma
- Association with angiosarcoma and AVMs
- Pulmonary Arteriovenous Malformations: Facts
- Congenital in origin
- 60-90% of patients have hereditary hemorrhagic telangiectasia (aka Rendu- Osler-Weber syndrome)