venous malformation is defined as malformations comprised of slow - flowing , abnormal dilated veins , and venous network .
clinically venous malformations ( vm ) are present at birth and tend to grow steadily in proportion to the somatic growth of the child .
venous malformations are congenital lesions that affect boys and girls equally with a reported risk of developing other conditions within a specified period of time .
the occurrence rate is 1 - 2 per 10,000 births and 0.1 - 1% of a population are found to have this condition .
histologic and histochemical studies show that abnormalities are formed by small and large dysplastic post - capillary , thin - walled vascular channels with patchy deficiency of mural smooth muscle .
a 28-year - old male was referred to the oral and maxillofacial clinic for evaluation of multiple swellings on the left side of the face and inside his mouth .
the patient first noticed the swelling over the temple when he was 11-years - old . as the boy grew , the swelling also proportionately increased .
multiple poorly defined swellings were noted on the left side of the head and neck region around the temple , cheek , and submandibular region [ figure 1a and b ] .
similar lesion was noted in the supraclavicular and scapular region [ figure 2a and b ] .
the swelling was pulsatile and increased in size the when patient was standing ( dependent position ) .
intraorally lobulated swelling was noted on the floor of the mouth in the left sublingual region .
the lesion measured 3  4 cm , filling the entire floor of mouth , and purplish discoloration was also noted over the swelling [ figure 3a and b ] .
( a ) photograph ( front view ) shows swelling over the temple region and ( b ) photograph ( side view ) reveals well defined swelling over temple ( large arrow ) diffused swelling in the cheek , and submandibular region ( small arrow ) .
( a ) photograph shows a well - defined swelling measuring 1  1 cm in the left supraclavicular region and ( b ) photograph of the scapular region shows a diffused swelling .
( a ) intraoral photograph shows swelling in the floor of mouth filling the entire floor of mouth ( arrow ) and a small swelling in the buccal mucosa that has a purplish hue ( small arrow ) .
( b ) intraoral photograph of the labial sulcus reveals a purplish swelling ( arrow ) .
ultrasonography of the submandibular region showed a well - defined hypoechoic lesion having anechoic areas with septations , showing flow inside the lesion .
ultrasonography of the submandibular region with 7 mhz small parts transducer shows heterogeneous hypoechoic lesion with slow flow and hyperechoic foci suggestive of calcifications ( arrow ) . magnetic resonance imaging ( mri)-t2-weighted fat suppression , post - contrast axial images showed hyperintense lesions , which are well - defined with no flow voids .
all the lesions in the submandibular , cheek , and temple region had similar findings [ figure 5 ] .
direct puncture phlebogram of the left frontal scalp region revealed pooling of contrast into cavernous spaces draining into external jugular vein [ figure 6 ] .
( a ) axial section at the submandibular region shows well - defined hyperintense lesion ( arrow ) in the floor of mouth .
( b ) axial section at the level of cheek shows well - defined hyperintense lesion in the cheek region ( arrow ) .
phlebography of the lesion in the temple region shows filling of the entire lesion and draining to the regional area ( arrow ) .
histopathological examination revealed several thin - walled venous channels lined by flattened endothelium , supported by a dense uninflamed fibrous connective tissue stroma .
hematoxylin and eosin stained specimen ( 40 ) shows irregular venous channels ( large arrow ) lined by thin endothelium .
venous malformation ( vm ) is the second most common vascular anomaly of the head and neck after hemangioma .
venous malformations can occur anywhere in the body but are most frequently seen in the head and neck ( 40% ) , extremities ( 40% ) , and trunk ( 20% ) .
vms are slow - flow vascular anomalies composed of ectatic venous channels that will continue to grow throughout the patient 's lifetime .
they grow slowly in size with age , but their growth may be exacerbated following trauma , sepsis , or hormonal changes and they do not regress spontaneously . both men and women are equally affected .
vascular malformations are believed to be the result of a congenital error of vascular morphogenesis that occurs between the 4 and 10 weeks of intrauterine life .
vascular malformations have a quiescent endothelium and are considered to be localized defects of vascular morphogenesis , likely to be caused by dysfunction in pathways regulating embryogenesis and vasculogenesis .
these lesions vary in color depending on depth of involvement and range from mild detectable color change to deep purple color .
these lesions fill when the patient is standing and are compressible , which helps to distinguish them from lymphatic malformations on physical examination .
areas frequently involved in the head and neck region are masseter , temporalis , tongue musculature , as well as oral and airway mucosa .
soft tissue lesions are most frequently facial in location , with the buccal region being the most common site followed by the mandibular space , sublingual space , tongue , and orbit .
intraosseous calvarial involvement is most frequent in the frontoparietal region and the mandible is the most frequent location within the facial skeleton .
there may , however , be no visible manifestations with deeper lesions . in our case ,
the first anatomopathologic classification of vascular lesions based on the microscopic appearance was developed by virchow and wegner .
mulliken and glowacki ( 1982 ) developed a biological classification of vascular anomalies that included physical findings , clinical behavior , and cellular kinetics and classified them as hemangiomas and vms .
a more recently updated classification of vascular anomalies by international society for the study of vascular anomalies is now widely used [ table 1 ] .
classification of vascular anomalies ( issva 2007 ) plain radiographs have limited role in the investigations as they can identify only phleboliths .
ultrasound ( us ) is often the initial investigation to evaluate vascular malformations and it may characterize and define the extent of more superficial lesions . on gray - scale imaging , venous malformations can appear as hypoechoic or heterogenous lesions with anechoic structures visible in 50% of cases .
in addition , the doppler flow is generally monophasic low velocity flow , and in some cases flow is only discernible with compression and release of the lesion .
they typically appear as isointense or hypointense lesions on t1-weighted images , but could be hyperintense depending on the presence of intralesional fat .
lesions are typically lobulated , which gives them the characteristic  bunch of grapes  configuration . septations and rounded signal voids corresponding to phleboliths are additional distinguishing features . in t2-weighted or inversion recovery sequences ,
vms demonstrate high - signal intensity.this imaging modality is used to determine the full extent of the lesion and its relationship to adjacent vital structures .
gradient echo sequences reveal areas of low signal corresponding to calcification or hemosiderin or thrombosis .
t1-weighted post contrast imaging demonstrates homogenous or heterogeneous enhancement , and dynamic contrast - enhanced mri has increased the specificity of venous malformation diagnosis .
vms are best demonstrated by direct phlebography , which fills the sinusoidal spaces and any anomalous veins , allowing assessment of the size and extent of the lesion . in the present study , phleboliths were seen on plain radiography and us features were suggestive of vascular anomaly with slow flow rate . whereas , the mri features were suggestive of venous malformation , which was confirmed by direct phlebography where the lesion was seen draining the regional vein .
venous malformations usually are associated with syndromes like proteus syndrome and blue rubber bleb nevus ( bean ) syndrome .
multiple treatment options exist for venous malformations , including conservative measures such as head of bed elevation and compression , laser therapy , sclerotherapy , and surgery .
conservative management of venous malformations is usually reserved for smaller isolated asymptomatic lesions and is also important in controlling the growth and symptoms .
elevation of the head of the bed is important as it decrease hydrostatic pressure in the malformation , which can lead to expansion and can also decrease symptoms of airway obstruction , swelling , and pain that are experienced .
sclerotherapy remains a good option for the treatment of venous malformations in the head and neck .
sclerotherapy involves percutaneous injection of a substance to induce inflammation and thrombosis of the lesion , which then will lead to more long - term fibrosis and hopefully decrease or eliminate the expansion of the lesion .
sotradecol foam or ethibloc ( glue ) , or the sclerosant is mixed with fibrin glue or ethyl cellulose , bleomycin ( pingyangmycin ) and picibanil ( ok-432 ) have recently been used as sclerosants in asia with promising results .
large cervicofacial venous malformations present a much greater challenge , and one must be prepared to use multimodal therapy to keep the lesion under control .
these lesions generally can not be cured as doing so would leave devastating functional and cosmetic results .
therefore , therapy is used to control growth , maintain cosmesis , and decrease symptoms .
venous malformations are either superficial or deep veins that are abnormally formed and dilated . a thorough examination and investigation of the condition is needed to establish the exact extension of the condition and plan proper treatment .
 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 . 