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23 - PROGERIA
from PART II: - HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS
- By E. Steve Roach, Irena Anselm, E. Paul Rosman, Louis R. Caplan
Edited by Louis R. Caplan, Julien Bogousslavsky
Book:

Uncommon Causes of Stroke

Published online:

06 January 2010

Print publication:

09 October 2008, pp 145-148
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Summary

Pulmonary arteriovenous malformations (PAVMs) are rare occurrences among the population. These malformations can be acquired in various clinical settings or be congenital. Single PAVMs <2 cm in diameter may be asymptomatic. The severity of symptoms is believed to be proportionally related to the size (diameter) of the PAVMs. The specific anatomy of the vascular malformation must then be analyzed either with helical computed tomography (CT) or magnetic resonance angiography (MRA). Digital subtraction angiography is eventually performed. The aims of treatment are to prevent neurological complications and pulmonary hemorrhage and to improve hypoxemia. Lobectomy or pneumonectomy is performed only in rare situations such as in patients in whom the feeding artery is so short that there is a risk of coil migration. Embolization therapy is applied for PAVMs, and is carried out by transcatheter embolotherapy, which consists in obliterating the feeding artery.

7 - MR angiography of the head and neck: basic principles and clinical applications
- By Robert R. Edelman, Joel Meyer
Edited by Stephen Davis, Marc Fisher, National Institute of Mental Health, Bethesda, Maryland, Steven Warach, National Institutes of Health, Baltimore
Book:

Magnetic Resonance Imaging in Stroke

Published online:

26 August 2009

Print publication:

20 March 2003, pp 85-102
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Summary

This chapter opens with a discussion on the basic principles of Magnetic Resonance Angiogram (MRA), including time of flight and phase contrast techniques. It introduces the use of paramagnetic contrast agents for MRA. The advantages and pitfalls of MRA as compared with duplex sonography (DUS) and X-ray angiography (XRA) are discussed. MR uses a combination of magnetic fields and radiofrequency energy to produce images. MRI is highly accurate at measuring the carotid wall area and T2-weighted images can discriminate between the fibrous cap and lipid core of a plaque. The combination of MRI and MRA is an accurate non-invasive means for the detection of carotid and vertebral dissections. MRI depicts the nidus of a vascular malformation. MRA is reliable for detecting stenoses and occlusions involving the vertebrobasilar system. For the intracranial circulation, MRA is well accepted for imaging of the dural sinuses and larger intracranial veins.

Unusual Vascular Malformation of the Kidney in Both Twins of a Monozygotic Pair: Short Case Report
G. Mastroberardino, A. Sciarra, R. Gualtieri, C. Di Fusco, P. Grammatico, G. Del Porto
Journal:

Acta geneticae medicae et gemellologiae: twin research / Volume 36 / Issue 4 / October 1987

Published online by Cambridge University Press:

01 August 2014, pp. 567-570

Print publication:

October 1987
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A case of double bilateral renal vessels in both twins of a MZ pair is reported for the first time. Even in non-twins, the anomalies reported so far involved the inferior polar arteries in agreement with the embryological development. The two male twins, examined at the age of 14 years, had simultaneously developed a marked hypertension at the age of 7 years. Zygosity was determined by blood group and HLA analysis and various clinical tests were carried out to diagnose the condition. It is suggested that the anomaly is the result of a genetically induced early block in embryonic development.