Combined and complex vascular malformations

The correct diagnosis of vascular malformations is obtainable by clinical assessment and patient history in the majority of cases. Nonetheless, confusion in nomenclature, existence of multiple classifications and rarity of these lesions leads to misdiagnosis and related wrong treatment. This is especially the case in combined or complex vascular malformations or vascular malformations that are part of syndromes as these have overlapping clinical and imaging features. New entities in the field of vascular anomalies have been described recently like fibro-adipose vascular anomaly or central conducting lymphatic anomalies.

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DOZ047.40: Bronchopulmonary and vascular anomalies are frequent in children with esophageal atresia

Diseases of the Esophagus ◽

10.1093/dote/doz047.40 ◽

2019 ◽

Vol 32 (Supplement_1) ◽

Author(s):

S Lejeune ◽

A L Mee ◽

L Petyt ◽

C Mordacq ◽

R Sfeir ◽

...

Keyword(s):

Ct Scan ◽

Esophageal Atresia ◽

Respiratory Symptoms ◽

Vascular Malformations ◽

Vascular Anomaly ◽

Vascular Anomalies ◽

Ct Scans ◽

Congenital Cystic Adenomatoid Malformation ◽

Pediatric Radiologist ◽

Contrast Enhanced Ct

Abstract Introduction Esophageal atresia (EA) is frequently associated with other malformations although few data are available. Objective Describe tracheobronchial, pulmonary and/or vascular malformations in patients with EA using chest CT scans. Methods Monocentric retrospective study in children with EA, born between 1996 and 2013, who had a CT scan during their follow-up, reviewed by a pediatric radiologist. Results Among 234 children with EA, 48 patients underwent a CT scan available for interpretation, among which 69% were performed to explore persistent respiratory symptoms. Thirty-nine children (81%) were type III EA, 13 (27%) had a VACTERL association. Six patients (13%) had a pulmonary malformation: 4 lobar agenesis, 1 right pulmonary aplasia, and 1 congenital cystic adenomatoid malformation. All these patients presented with at least one associated malformation. Combined with the results of laryngotracheal endoscopy (n = 30), 43 patients (90%) had a tracheobronchial anomaly: tracheomalacia for 40 (83%), tracheal stenosis for 12 (25%), right tracheal bronchus for 2 (4%), communicating bronchopulmonary foregut malformation for 1 (2%). Combined with the results of echocardiography (n = 47), 7 patients (15%) had an isolated vascular anomaly, 8 (17%) had an isolated congenital heart disease and 7 (15%) had both. CT scans permitted the diagnosis of 6 pulmonary malformations (13%), 15 tracheobronchial anomalies (31%), and 2 vascular anomalies (4%). Only one patient (2%) in our study presented with an isolated EA. Conclusion Our study confirms the association of tracheobronchial, pulmonary, and vascular anomalies in patients with EA. Contrast-enhanced CT scan is complementary to echocardiography and laryngotracheal endoscopy in the exploration of persistent respiratory symptoms.

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Peripheral Vascular Anomalies – Essentials in Periinterventional Imaging

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren ◽

10.1055/a-0998-4300 ◽

2019 ◽

Vol 192 (02) ◽

pp. 150-162 ◽

Cited By ~ 1

Author(s):

Maliha Sadick ◽

Daniel Overhoff ◽

Bettina Baessler ◽

Naema von Spangenberg ◽

Lena Krebs ◽

...

Keyword(s):

Therapeutic Approach ◽

Vascular Malformations ◽

Vascular Anomaly ◽

Flow Dynamics ◽

Vascular Anomalies ◽

Vascular Tumors ◽

Slow Flow ◽

Peripheral Vascular ◽

Fast Flow ◽

Selection Of

Background Peripheral vascular anomalies represent a rare disease with an underlying congenital mesenchymal and angiogenetic disorder. Vascular anomalies are subdivided into vascular tumors and vascular malformations. Both entities include characteristic features and flow dynamics. Symptoms can occur in infancy and adulthood. Vascular anomalies may be accompanied by characteristic clinical findings which facilitate disease classification. The role of periinterventional imaging is to confirm the clinically suspected diagnosis, taking into account the extent and location of the vascular anomaly for the purpose of treatment planning. Method In accordance with the International Society for the Study of Vascular Anomalies (ISSVA), vascular anomalies are mainly categorized as slow-flow and fast-flow lesions. Based on the diagnosis and flow dynamics of the vascular anomaly, the recommended periinterventional imaging is described, ranging from ultrasonography and plain radiography to dedicated ultrafast CT and MRI protocols, percutaneous phlebography and transcatheter angiography. Each vascular anomaly requires dedicated imaging. Differentiation between slow-flow and fast-flow vascular anomalies facilitates selection of the appropriate imaging modality or a combination of diagnostic tools. Results Slow-flow congenital vascular anomalies mainly include venous and lymphatic or combined malformations. Ultrasound and MRI and especially MR-venography are essential for periinterventional imaging. Arteriovenous malformations are fast-flow vascular anomalies. They should be imaged with dedicated MR protocols, especially when extensive. CT with 4D perfusion imaging as well as time-resolved 3D MR-A allow multiplanar perfusion-based assessment of the multiple arterial inflow and venous drainage vessels of arterio-venous malformations. These imaging tools should be subject to intervention planning, as they can reduce procedure time significantly. Fast-flow vascular tumors like hemangiomas should be worked up with ultrasound, including color-coded duplex sonography, MRI and transcatheter angiography in case of a therapeutic approach. In combined malformation syndromes, radiological imaging has to be adapted according to the dominant underlying vessels and their flow dynamics. Conclusion Guide to evaluation of flow dynamics in peripheral vascular anomalies, involving vascular malformations and vascular tumors with the intention to facilitate selection of periinterventional imaging modalities and diagnostic and therapeutic approach to vascular anomalies. Key Points: Citation Format

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Vascular malformations: a hospital based study

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20193373 ◽

2019 ◽

Vol 7 (8) ◽

pp. 2936

Author(s):

Komanapalli Sunilkumar ◽

Karthik Aithal ◽

Unmesh Kumar Jena ◽

Uma Prasad ◽

Bhagyalakshmi Atla ◽

...

Keyword(s):

Vascular Malformations ◽

Tertiary Care ◽

Vascular Anomalies ◽

Imaging Features ◽

Venous Malformations ◽

Internal Elastic Lamina ◽

Tertiary Care Centre ◽

Vascular Neoplasms ◽

Diagnostic Difficulties ◽

Appropriate Therapy

Background: Vascular anomalies comprise a widely heterogeneous group of lesions. Diagnosis and management of these lesions present challenges to the surgeons, radiologists and histopathologists. Accurate classification of these lesions results in appropriate therapy. Aim of the study is to study the role of histopathology and histochemical stain in the diagnosis of vascular malformationMethods: The present study is a hospital based observational study on vascular malformations over a period of three years from 2016 to 2018 done in department of Pathology and Plastic Surgery at tertiary care centre, Visakhapatnam.Results: Out of 107 specimens of vascular anomalies received, 72 cases were vascular neoplasms, 35 were vascular malformations. Majority of the vascular malformations were seen in the cervicofacial region (43%) followed by extremities (37.1%). Most common malformation in the present study was arterio venous malformations (60%) followed by venous malformations (22.8%). Verhoeff’s VanGieson stain demonstrated discontinuity of internal elastic lamina in cases of arteriovenous malformation and helped in differentiating the lesions from capillary malformation and vascular tumors.Conclusions: Histopathology, special histochemical stains along with imaging features can be used to reduce diagnostic difficulties and in helping proper management of vascular malformations.

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COMPRESSION OF THE TRACHEA OR ESOPHAGUS VASCULAR ANOMALIES

PEDIATRICS ◽

10.1542/peds.7.1.69 ◽

1951 ◽

Vol 7 (1) ◽

pp. 69-88

Author(s):

ROBERT E. GROSS ◽

EDWARD B.D. NEUHAUSER

Keyword(s):

Aortic Arch ◽

Vascular System ◽

Vascular Malformations ◽

Pulmonary Ventilation ◽

Innominate Artery ◽

Vascular Anomaly ◽

Vascular Anomalies ◽

Ligamentum Arteriosum ◽

Superior Mediastinum ◽

Clear Idea

Description is given of five different types of anomalies of the great vessels in the superior mediastinum which can produce compression of the esophagus or trachea (or both). These malformations of the vascular system can give rise to difficulties in swallowing and to serious disturbances in pulmonary ventilation. These anomalies include double aortic arch, right aortic arch with a left ligamentum arteriosum, anomalous innominate artery, anomalous left common carotid artery, and an aberrant subclavian artery. The various symptom complexes which can be found with these anomalies are described. Endoscopic visualization of the interior of the esophagus or trachea gives valuable information in some cases. The investigation of these subjects should include fluoroscopic and film studies of the esophagus and trachea by contrast media. Data gathered from such roentgenologic examinations usually give a fairly clear idea of the type of vascular anomaly which is present. It is possible to attack surgically each of these vascular malformations. In general, the esophagus or trachea can be relieved by division of an anomalous vessel or by displacement of an artery in such a manner that it is carried away from the compressed structure. When necessary, these operative procedures can be undertaken in very young babies, even in the presence of a high degree of respiratory obstruction. All the operations have been through a left anterolateral, transpleural approach, under general anesthesia with a closed system. The results of operation in 40 patients are analyzed. It is evident that many vascular anomalies in the thorax which disturb the functions of the esophagus or trachea can now be treated with an excellent chance of relieving the obstructive symptoms.

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Imaging of congenital lung diseases presenting in the adulthood: a pictorial review

Insights into Imaging ◽

10.1186/s13244-021-01095-2 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Gamze Durhan ◽

Selin Ardali Duzgun ◽

Meltem Gülsün Akpınar ◽

Figen Demirkazık ◽

Orhan Macit Arıyürek

Keyword(s):

Lung Diseases ◽

Correct Diagnosis ◽

Tracheobronchial Tree ◽

Vascular Anomalies ◽

Dual Energy Ct ◽

Imaging Features ◽

Pictorial Review ◽

Short Echo Time ◽

Enhanced Computed Tomography ◽

New Applications

AbstractCongenital lung diseases in adults are rare diseases that can present with symptoms or be detected incidentally. Familiarity with the imaging features of different types of congenital lung diseases helps both in correct diagnosis and management of these diseases. Congenital lung diseases in adults are classified into three main categories as bronchopulmonary anomalies, vascular anomalies, and combined bronchopulmonary and vascular anomalies. Contrast-enhanced computed tomography, especially 3D reconstructions, CT, or MR angiography, can show vascular anomalies in detail. The tracheobronchial tree, parenchymal changes, and possible complications can also be defined on chest CT, and new applications such as quantitative 3D reconstruction CT images, dual-energy CT (DECT) can be helpful in imaging parenchymal changes. In addition to the morphological assessment of the lungs, novel MRI techniques such as ultra-short echo time (UTE), arterial spin labeling (ASL), and phase-resolved functional lung (PREFUL) can provide functional information. This pictorial review aims to comprehensively define the radiological characteristics of each congenital lung disease in adults and to highlight differential diagnoses and possible complications of these diseases.

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THE IMPORTANCE OF ACCURATE IMAGING DIAGNOSIS OF A VASCULAR ANOMALY PRIOR TO THE SURGICAL PROCEDURE

Romanian Medical Journal ◽

10.37897/rmj.2021.2.27 ◽

2021 ◽

Vol 68 (2) ◽

pp. 301-305

Author(s):

Maria Popescu ◽

◽

Gabriel Drăgan ◽

Alina Oprescu ◽

◽

...

Keyword(s):

Magnetic Resonance ◽

Treatment Plan ◽

Contrast Material ◽

Vascular Anomaly ◽

Clinical History ◽

Management Plan ◽

Vascular Anomalies ◽

Vascular Tumors ◽

Imaging Features ◽

Informed Treatment

The diagnosis of vascular tumors and malformations is usually achieved with clinical history and physical examination. The imaging of these patients has been sufficiently refined to answer pertinent questions when trying to make an accurate differential diagnosis in order to establish an informed treatment plan. The imaging modalities at hand include ultrasound, doppler sonography, magnetic resonance imaging and contrast material enhanced magnetic resonance angiography. We present two cases of different vascular anomalies that were managed according to the clinical aspect, natural history and imaging performed. Moreover, with better understanding of the imaging features, the radiologist has become a key player in the diagnosis and management plan of the vascular anomalies. This is crucial in avoiding misdiagnosis and improper treatment.

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How we approach localized vascular anomalies

10.22541/au.161746184.45717973/v1 ◽

2021 ◽

Author(s):

Kristy Pahl ◽

Waleska Pabon-Ramos ◽

Michael Jeng

Keyword(s):

Arteriovenous Malformations ◽

Vascular Malformations ◽

Management Strategies ◽

Vascular Anomalies ◽

Vascular Tumors ◽

Imaging Features ◽

Diagnosis And Management

Vascular anomalies are a group of disorders divided into two distinct subtypes: vascular tumors and vascular malformations. Vascular tumors are proliferative in nature, while malformations are non-proliferative. Simple, localized vascular malformations refer to a group of malformations that are localized to a single area of involvement. These simple malformations include capillary, lymphatic, venous, and arteriovenous malformations. The pediatric hematologist and oncologist is becoming increasingly involved in the diagnosis and management of these disorders. This review presents four cases as a means to discuss the diagnosis, clinical and imaging features, and management strategies of simple, localized vascular malformations.

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Magnetic Resonance Imaging of Uncommon Hepatic Mesenchymal Tumours: Haemangioendothelioma and Angiosarcoma

Current Medical Imaging Formerly Current Medical Imaging Reviews ◽

10.2174/1573405614666180628160809 ◽

2019 ◽

Vol 15 (4) ◽

pp. 362-368

Author(s):

Andrzej Cieszanowski ◽

Agnieszka Anysz-Grodzicka ◽

Joanna Podgorska ◽

Beata Jagielska ◽

Jakub Pałucki

Keyword(s):

Contrast Enhancement ◽

Correct Diagnosis ◽

Malignant Potential ◽

Hepatobiliary Phase ◽

Imaging Features ◽

Liver Tumours ◽

Aged Woman ◽

High Apparent Diffusion Coefficient ◽

Adc Values ◽

Mesenchymal Tumours

<P>Background: Primary Hepatic Epithelioid Haemangioendothelioma (HEHE) and Primary Hepatic Angiosarcoma (PHA) are rare mesenchymal tumours with different malignant potential. Whereas HEHE demonstrates low to intermediate malignant potential, PHA is an aggressive malignancy with poor prognosis. The knowledge of typical imaging features of these lesions may facilitate correct diagnosis; however, the ultimate diagnosis of HEHE and PHA is based on histopathologic examination. </P><P> Discussion: The most typical findings helpful in diagnosing HEHE are: Presence of multiple, confluent nodules located at the liver periphery (in young to middle-aged woman), retraction of the liver capsule, marked hyperintensity on T2-weighted images, “target-sign” appearance, progressive centripetal contrast enhancement, and relatively high Apparent Diffusion Coefficient (ADC) values. More than ≥50% of nodules are hyper- or isointense on Hepatobiliary Phase (HBP) images. Conclusion: The imaging features suggestive of PHA are: Occurrence of metastases (lungs, spleen) at the time of diagnosis, presence of a large dominant mass with smaller satellites, heterogeneity and areas of haemorrhage in a dominant mass, progressive contrast enhancement, slightly elevated ADC values as compared to other malignant liver tumours.</P>

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Peripheral Arteriovenous Malformations: Imaging and Endovascular Management Strategies

Journal of Clinical Interventional Radiology ISVIR ◽

10.1055/s-0041-1728986 ◽

2021 ◽

Author(s):

Virender Malik ◽

Harshith Kramadhari ◽

Jawahar Rathod ◽

Yadav W. Munde ◽

Uday Bhanu Kovilapu

Keyword(s):

Arteriovenous Malformation ◽

Vascular Malformation ◽

Management Strategies ◽

Treatment Strategies ◽

Vascular Anomaly ◽

Classification Systems ◽

Vascular Anomalies ◽

Final Decision ◽

Life Threatening

AbstractThe peripheral high-flow vascular malformation (HFVM) comprises arteriovenous malformation (AVM) and fistula (AVF), shows varied clinical presentation (ranging from subtle skin lesion to life-threatening congestive heart failure), and frequently poses diagnostic and therapeutic challenges. Importance of assigning a specific diagnosis to the vascular malformation cannot be overstated, as the treatment strategy is based on the type of vascular anomaly. Although the International Society for the Study of Vascular Anomalies (ISSVA) classification system is the most commonly accepted system for classifying congenital vascular anomalies in clinical practice, the Cho–Do et al classification is of utmost help in guiding optimal mode of treatment in peripheral AVM. Although transarterial approach remains the most commonly employed route for peripheral AVM embolization, the role of transvenous and direct percutaneous approach is ever increasing and the final decision on the approach depends on angioarchitecture of the AVM. In this article, we review various commonly employed classification systems for congenital vascular anomalies, and describe clinical features, imaging and treatment strategies for peripheral arteriovenous malformation (PAVM).

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