The cerebrofacial metameric syndromes: An embryological review and proposal of a novel classification scheme

2021 ◽  
pp. 159101992110449
Author(s):  
Anthony S. Larson ◽  
Waleed Brinjikji ◽  
Timo Krings ◽  
Julie B. Guerin
Keyword(s):  
Cell Migration ◽  
Classification System ◽  
Classification Scheme ◽  
Vascular Malformations ◽  
High Flow ◽  
Low Flow ◽  
Pharyngeal Arches ◽  
Detailed Classification ◽  
Vascular Syndromes ◽  
The Brain

The cerebrofacial metameric syndromes are a group of congenital syndromes that result in vascular malformations throughout specific anatomical distributions of the brain, cranium and face. Multiple reports of patients with high-flow or low-flow vascular malformations following a metameric distribution have supported this idea. There has been much advancement in understanding of segmental organization and cell migration since the concept of metameric vascular syndromes was first proposed. We aim to give an updated review of these embryological considerations and then propose a more detailed classification system for these syndromes, predominately incorporating the contribution of neural crest cells and somitomeres to the pharyngeal arches.

scholarly journals 3T MRI of Peripheral Vascular Malformations: Characteristics and Comparison of Two Fat-Saturated sequences: Short Tau Inversion Recovery Versus Three-Dimensional High-Resolution Volume Interpolated Gradient Recalled Echo

2020 ◽  
Author(s):  
Florentine Höhn ◽  
Simone Hammer ◽  
Claudia Fellner ◽  
Florian Zeman ◽  
Wibke Uller ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
High Resolution ◽  
Vascular Malformations ◽  
Three Dimensional ◽  
High Flow ◽  
Low Flow ◽  
Peripheral Vascular ◽  
Contrast Enhanced ◽  
Hemodynamic Characteristics ◽  
Short Tau Inversion Recovery

Purpose To assess morphological and hemodynamic characteristics of peripheral vascular malformations on 3 T magnetic resonance imaging (MRI) including qualitative comparison of two fat-saturated sequences: short tau inversion recovery (STIR) and three-dimensional high-resolution volume interpolated gradient recalled echo (GRE). Materials and Methods During 9 months, 100 patients with suspected or known vascular malformations were prospectively assessed on a 3 T scanner using T2-weighted STIR and turbo spin echo (TSE), T1-weighted TSE, time-resolved contrast-enhanced magnetic resonance angiography (MRA) with interleaved stochastic trajectories (TWIST) and T1-weighted volume interpolated breath-hold examination (VIBE) after contrast enhancement. The analysis included signal behavior and morphologic and hemodynamic characteristics. Additionally, the image quality of the fat-saturated sequences was evaluated by 2 radiologists. Results 86 patients (14 dropouts; 57 female, 29 male; mean age 26.8 years, age range 1–56) were analyzed. 22 had high-flow and 64 low-flow malformations, including 14 with a lymphatic component. In 21 of 22 patients with high-flow malformations, typical characteristics (flow voids, hyperdynamic arteriovenous fistula, dilated main/feeder-arteries and draining veins) were documented. Patients with low-flow malformations had phleboliths in 35 cases, fluid-fluid levels in 47 and dilated draining veins in 23. Lymphatic malformations showed peripheral contrast enhancement of cyst walls in the volume interpolated GRE. The comparison of fat-saturated sequences showed significantly better results of the volume interpolated GRE in all categories except the presence of artifacts which were significantly reduced in the STIR (p < 0.05). Conclusion 3 T MRI with MRA provides detailed morphological and hemodynamic information of different types of peripheral vascular malformations. Contrast-enhanced high-resolution volume interpolated GRE proved superior to STIR in differentiating morphologic features and to be diagnostic in the differentiation of lymphatic parts and joint involvement. Key Points:  Citation Format

The Effect of Altered Cerebral Blood Flow on the Cerebral Kinetics of Thiopental and Propofol in Sheep

2000 ◽  
Vol 93 (4) ◽  
pp. 1085-1094 ◽  
Author(s):  
Richard N. Upton ◽  
Guy L. Ludbrook ◽  
Cliff Grant ◽  
David J. Doolette
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Random Order ◽  
High Flow ◽  
Low Flow ◽  
Flow State ◽  
Concentration Difference ◽  
Blood Flows ◽  
Kinetics Of ◽  
The Brain

Background Thiopental and propofol are highly lipid-soluble, and their entry into the brain often is assumed to be limited by cerebral blood flow rather than by a diffusion barrier. However, there is little direct experimental evidence for this assumption. Methods The cerebral kinetics of thiopental and propofol were examined over a range of cerebral blood flows using five and six chronically instrumented sheep, respectively. Using anesthesia (2.0% halothane), three steady state levels of cerebral blood flow (low, medium, and high) were achieved in random order by altering arterial carbon dioxide tension. For each flow state, 250 mg thiopental or 100 mg propofol was infused intravenously over 2 min. To quantify cerebral kinetics, arterial and sagittal sinus blood was sampled rapidly for 20 min from the start of the infusion, and 1.5 h was allowed between consecutive infusions. Various models of cerebral kinetics were examined for their ability to account for the data. Results The mean baseline cerebral blood flows for the "high" flow state were over threefold greater than those for the low. For the high-flow state the normalized arteriovenous concentration difference across the brain was smaller than for the low-flow state, for both drugs. The data were better described by a model with partial membrane limitation than those with only flow limitation or dispersion. Conclusions The cerebral kinetics of thiopental and propofol after bolus injection were dependent on cerebral blood flow, despite partial diffusion limitation. Higher flows produce higher peak cerebral concentrations.

scholarly journals Maxillomandibular Vascular Malformations: Report of Four Cases

2021 ◽  
Author(s):  
Krishnan Nagarajan ◽  
Mohan Amuthabarathi ◽  
Balasubramanian Krishnan ◽  
Sekar Sabarish ◽  
Rajendiran Saravanan
Keyword(s):  
Vascular Malformations ◽  
Pyogenic Granuloma ◽  
High Flow ◽  
Low Flow ◽  
Definitive Treatment ◽  
Embolic Agent ◽  
Arterial Phase ◽  
Cyanoacrylate Glue ◽  
Dental Arcade ◽  
Enhanced Computed Tomography

AbstractMaxillomandibular or dental arcade arteriovenous malformations (AVMs) are uncommon lesions with potential for life-threatening bleeding. We report three cases of vascular malformations and a case of pyogenic granuloma and propose a contrast-enhanced computed tomography (CT)-based workup of these lesions for definitive treatment. Four cases of orodental bleeding were diagnosed based on CT angiography (CTA) and treated using endovascular means. Three of them were subsequently operated and confirmed by histopathology. Two were low-flow AVMs, the third one a case of pyogenic granuloma, and one high-flow AVM. The first three lesions were embolized with polyvinyl alcohol particles and subsequently operated and the fourth high-flow one embolized using liquid embolic agent n-butyl cyanoacrylate (Glue). CTA can be used to show separate lesions of arterial vascularity and those of delayed/ venous enhancing lesions. Arterial phase-enhancing lesions are again subdivided in digital subtraction angiography into those of low-to-moderate vascularity and those with high flow, which are treated using particle embolization and liquid glue/onyx (± coil) embolization, respectively. The delayed venous phase enhancing lesions may mimic neoplasms and after tissue biopsy may be managed by sclerotherapy.

Vascular birthmarks

VASA ◽  
2008 ◽  
Vol 37 (1) ◽  
pp. 5-17 ◽  
Author(s):  
Willenberg ◽  
Baumgartner
Keyword(s):  
Vascular Malformations ◽  
Treatment Strategies ◽  
Multidisciplinary Care ◽  
High Flow ◽  
Low Flow ◽  
Management Options ◽  
Benign Neglect ◽  
Current Availability ◽  
History Of ◽  
Vascular Birthmarks

Vascular birthmarks can be classified into hemangioma and vascular malformations. Hemangioma are frequent tumours of early infancy demonstrating endothelial hyperplasia, a history of rapid neonatal growth and slow involution during later childhood. Treatment of hemangioma is dependent of stage and type of the lesion. Given the current availability of drugs, lasers, and other techniques to treat hemangioma safely, philosophy of "benign neglect" should not be considered anymore. Vascular malformations show a normal endothelial turnover, being present at birth and growing commensurately with the child. Exact diagnosis by employing modern diagnostic means, which are able to differentiate low-flow from high flow lesions is important for further therapeutic management. Beside conservative treatment strategies, use of laser, sclerotherapy, interventional embolization and surgical treatment are possible management options. Patients should receive multidisciplinary care in qualified vascular centres.

Balloon-assisted Onyx embolization of cerebral single-channel pial arteriovenous fistulas

2011 ◽  
Vol 7 (6) ◽  
pp. 637-642 ◽  
Author(s):  
C. Benjamin Newman ◽  
Yin C. Hu ◽  
Cameron G. McDougall ◽  
Felipe C. Albuquerque
Keyword(s):  
Treatment Strategy ◽  
Pediatric Patients ◽  
Single Channel ◽  
Vascular Malformations ◽  
High Flow ◽  
Flow Dynamics ◽  
Arteriovenous Fistulas ◽  
Male Patients ◽  
The Brain

Object Pial arteriovenous fistulas (AVFs) of the brain are rare vascular malformations associated with significant risks of hemorrhage and neurological deficit. Depending on their location and high-flow dynamics, these lesions can present treatment challenges for both endovascular and open cerebrovascular surgeons. The authors describe a novel endovascular treatment strategy that was used successfully to treat 2 pediatric patients with a pial AVF, and they discuss the technical nuances specific to their treatment strategy. Methods A single-channel high-flow pial AVF was diagnosed in 2 male patients (6 and 17 years of age). Both patients were treated with endovascular flow arrest using a highly conformable balloon followed by Onyx infusion for definitive closure of the fistula. Results Neither patient suffered a complication as a result of the procedure. At the 6-month follow-up in both cases, the simple discontinuation of blood flow had resulted in durable obliteration of the fistula and stable or improved neurological function. Conclusions Onyx can be delivered successfully into high-flow lesions after flow arrest to allow a minimally invasive and durable treatment for pial AVFs.

scholarly journals Multiple Venous Malformations with Phleboliths: Radiological-Pathological Correlation

2013 ◽  
Vol 3 ◽  
pp. 13 ◽  
Author(s):  
Venkateswara Rao Chava ◽  
Ashwini Naveen Shankar ◽  
Naveen Shankar Vemanna ◽  
Sudheer Kumar Cholleti
Keyword(s):  
Head And Neck ◽  
Clinical Symptoms ◽  
Vascular Malformations ◽  
High Flow ◽  
The Body ◽  
Low Flow ◽  
Venous Malformations ◽  
Congenital Lesions ◽  
The Face ◽  
Histopathologic Findings

Vascular malformations are congenital lesions that are present at birth and do not regress. However, they often present later in life. They are subdivided into two categories: (1) slow- or low-flow and (2) fast- or high-flow malformations. Low-flow malformations contain combinations of capillary, venous, and lymphatic components. Venous malformations can occur anywhere in the body, but are most frequently seen in the head and neck (40%). These lesions present in a variety of ways, from a vague blue patch to a soft blue mass, which may be single isolated or may occur in multiple areas. Treatment depends on the type of lesion, the location, degree of involvement, and the clinical symptoms. Here we are report the imaging and histopathologic findings in a patient with multiple venous malformations affecting the left side of the face and trunk.

scholarly journals High-Flow Vascular Malformations in Children

2020 ◽  
Vol 40 (03) ◽  
pp. 303-314
Author(s):  
Ramin A. Morshed ◽  
Ethan A. Winkler ◽  
Helen Kim ◽  
Steve Braunstein ◽  
Daniel L. Cooke ◽  
...  
Keyword(s):  
Vascular Malformations ◽  
Treatment Strategies ◽  
High Flow ◽  
Vascular Anomalies ◽  
Low Flow ◽  
Cavernous Malformations ◽  
Arteriovenous Fistulas ◽  
Dural Arteriovenous Fistulas ◽  
Clinical Consequences ◽  
The Central Nervous System

AbstractChildren can have a variety of intracranial vascular anomalies ranging from small and incidental with no clinical consequences to complex lesions that can cause substantial neurologic deficits, heart failure, or profoundly affect development. In contrast to high-flow lesions with direct arterial-to-venous shunts, low-flow lesions such as cavernous malformations are associated with a lower likelihood of substantial hemorrhage, and a more benign course. Management of vascular anomalies in children has to incorporate an understanding of how treatment strategies may affect the normal development of the central nervous system. In this review, we discuss the etiologies, epidemiology, natural history, and genetic risk factors of three high-flow vascular malformations seen in children: brain arteriovenous malformations, intracranial dural arteriovenous fistulas, and vein of Galen malformations.

Dynamic MRI for Distinguishing High-Flow from Low-Flow Peripheral Vascular Malformations

2005 ◽  
Vol 185 (5) ◽  
pp. 1131-1137 ◽  
Author(s):  
Yoshimitsu Ohgiya ◽  
Toshi Hashimoto ◽  
Takehiko Gokan ◽  
Shouji Watanabe ◽  
Masayoshi Kuroda ◽  
...  
Keyword(s):  
Vascular Malformations ◽  
Dynamic Mri ◽  
High Flow ◽  
Low Flow ◽  
Peripheral Vascular
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