2015 HSC Section 1 Book of Articles

A. Tekes et al. / Clinical Radiology 69 (2014) 443 e 457

mitotically quiescent. VaMs are subclassifed based on fl ow dynamics, as slow- fl ow, and fast- fl ow VaMs. 30

Table 4 Syndromes associated with vascular malformations (VMs). Syndromes associated with VM Klippel e Trenaunay Blue rubber bleb nevus Maffucci syndrome Syndromes associated with CM Klippel e Trenaunay Sturge e Weber Syndromes associated with LM Gorham syndrome Syndromes associated with AVM Parkes e Weber Rendu e Osler e Weber Bannayan e Riley e Ruvalcaba syndrome VM, Venous malformation; LM, Lymphatic malformation; CM, Capillary malformation; AVM, Arteriovenous malformation.

Slow- fl ow vascular malformations

Venous malformation Venous malformations (VMs) are the most common of all types of VaM. They present as soft, compressible lesions that typically in fi ltrate multiple tissue planes. Physical ex- amination generally reveals bluish lesions ( Figs 1 and 6 ) that may enlarge with Valsalva manoeuvre or gravity. There may be overlying skin involvement. They usually present during mid to late childhood and become more symptom- atic as time passes. The lesions vary in size from very small to extensive involving multiple body parts. The can appear as sacs fi lled with venous blood or as dilated venous channels with or without communication of systemic veins. Generally, even when large, VMs tend to be continuous in nature. They tend to extend within the muscle groups of extremities, along the nerves and major arteries or veins.

Figure 12 (a) A 7-year-old female patient with multiple dark, slightly raised, fi rm skin lesions on both knees, and over entire body as well. These are the skin lesions of BRBNS. She has multiple deep VMs on the shoulder and right arm that have been previously percutaneously sclerosed. She recently reported severe pain around both knees and thighs that prompted MRI. (b e c) Coronal T2-weighted images demonstrate multiple small, lobular T2-bright lesions in the muscle groups and medulla of the bones representing VMs. (d) Ultrasound showing intramuscular VM with needle accessing it for percutaneous sclerotherapy. (e) Percutaneous venogram of right leg intramuscular VM demonstrating type II drainage into normal veins (white arrow). (f) Percutaneous venogram of left leg intramuscular VM and infrapatellar VM. Note again the type II VM with drainage into normal veins (white arrow), and additional type I VM without a draining vein (black arrow).

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