Venous Malformations ("Hemangioma")

 

Facial Venous Malformations (cavernous hemangioma)
Extremity Venous Malformations (cavernous hemangioma)

Venous malformations (cavernous hemangiomas) are the most common symptomatic vascular malformations (birthmark). These common birthmarks usually become symptomatic in older children or young adults, with bluish skin discoloration, local swelling, and pain. 

The characteristic physical finding of this vascular birthmark is a soft and easily compressible soft-tissue mass (or swelling) that is associated with bluish skin discoloration. Increasing engorgement with dependency is typical. These birthmarks can be small and localized or extensive and involve the entire extremity or body part. 

Do venous malformations cause any health problems ?. Although venous malformations are considered benign entities, some extensive venous malformations can result in significant morbidity, particularly those in the head and neck (eg, airway involvement). Some of large head and neck venous malformations are associated with sinus pericranii and developmental intracranial venous anomalies. Large pharyngeal, laryngeal, and deep cervical-oropharyngeal venous malformations can expand to compress the airway and cause significant deviation that requires tracheostomy in most cases. Extremity venous malformations may be associated with a limb-length discrepancy, particularly if the malformation is large. Bone venous malformations can cause structural weakening of the osseous shaft and pathologic fractures. Involvement of a joint by a venous malformation may result in hemosiderin-arthropathy due to repeated intraarticular bleeding, which is typically seen in the knee. Venous malformations  of the gastrointestinal tract most commonly cause chronic bleeding and anemia. Lesions that involve the foregut can be associated with portal venous anomalies (absence of the portal vein, portal hypertension, etc).

Are venous malformations familial Conditions? Most venous malformations are not familial conditions. However, a rare form of venous malformation (birthmark) called "Glomangioma" or "Glomovenous malformation" is an autosomal dominant disorder that is characterized by multiple, often tender, blue nodular skin venous malformations.  Familial cutaneous mucosal venous malformation is another familial but rarely seen condition which is also associated with multiple venous malformations.

Glomovenous malformation (arm)

How can venous malformations be diagnosed?. The diagnosis of a venous malformation (vascular birthmark) is usually straightforward at clinical examination, although indeterminate lesions are usually confirmed with imaging studies. Imaging is also used to assess the extent of the abnormality, as well as possible associated abnormalities. The most typical radiographic finding is a soft-tissue mass or prominence containing phleboliths (small calcifications). Bone changes are seen in some cases. Ideal diagnostic test is MRI. Aside from a small percentage of venous malformations, high-flow vascular signal voids and flow-related enhancement are not features of venous malformations. Fluid-fluid levels may rarely be present, although these are more suggestive of a low-flow vascular anomaly with a lymphatic origin (eg, lymphatic malformation). Venous malformations are high-signal intensity lesions on T2-weighted images and low-signal intensity lesions on T1-weighted MRI; they have lobulated margins and multiple, rounded, signal voids that represent phleboliths. Phleboliths are usually better demonstrated on plain radiographs or CT scans. 

Venous malformations may involve only the skin, or they can extend into the muscles, joints, and bone (similar to any other birthmark). Prominent or dysplastic draining veins may be identified with either MRI or direct intralesional contrast agent injections. Enhancement of the abnormal vascular channels is typically seen on MRI after the intravenous administration of gadolinium based contrast material. The contrast enhancement pattern allows the differentiation of venous malformations from other low-flow anomalies, particularly lymphaticmalformations (enhancement is more patchy and central in venous malformations, whereas no enhancement or minimal peripheral enhancement is seen in lymphatic malformations).

 

                

Magnetic Resonance Angiography (MRA) of Venous Malformation (cavernous hemangioma) (Images#1 &2). Initial image (image#1) is early phase of contrast enhancement. The malformation is not opacified on this image. However, the lesion enhances significantly on the delayed image (image#2).This enhancement pattern is characteristic for venous malformations.  Image #3 Sarcoma Mimicking Venous Malformation. Although this lesion appears to be a similar lesion to venous malformations, biopsy result was sarcoma.

In the head and neck, venous malformations should also be differentiated from brachial cleft cysts, foregut duplication cysts, and thyroglossal duct cysts. If a venous malformation (cavernous hemangioma) involves a joint, the joint is best assessed with MRI, in terms of articular cartilage damage. The compartment syndrome is a particular concern after sclerotherapy in the extremity venous malformations. Therefore, the determination of the precise anatomic relationship between the venous malformation and the neurovascular bundles is mandatory on MRI. If a cervicofacial venous malformation (cavernous hemangioma) is found, the patient should be assessed for the possibility of sinus pericranii, which may be best performed with carotid arteriography and/or direct intralesional injections of the contrast agent. Although the demonstration of such a sinus has been reported with MRA, conventional angiography (digital subtraction angiography [DSA]) is usually required for complete evaluation of the intracranial circulation.

 
    
1st MR image shows an extensive venous malformation ("cavernous hemangioma" in the leg (bright signals). Post-contrast MR angiography (2nd image) of the lower extremities after IV administration of gadolinium demonstrates a large area of contrast opacification in the left thigh with patent appearing normal deep veins. It is important to image in multiple phases after intravenous contrast injection to see in various opacification phases in these patients to evaluate these lesions fully. Conventional arteriograpies usually do not show the malformation or demonstrate a subtle late contrast filling in most patients. 

The diagnosis of a gastrointestinal venous malformation (gastrointestinal vascular birthmark) is problematic. Because MRI is not sensitive enough to depict small gastrointestinal venous malformations, endoscopic examination of the gastrointestinal system is usually required. Patients with large intraabdominal venous malformations should also be examined to rule out portal hypertension. When a patient presents with gastrointestinal bleeding and when BRBNS is suspected, the presence of characteristic cutaneous vascular lesions (soft, blue, sometimes nodular lesions) should be assessed. Angiography may be necessary if the diagnosis remains in doubt or when associated anomalies, such as sinus pericranii or small arteriovenous fistulas (AVFs), are suspected. The venous channels are usually faintly filled-in on venous-phase angiograms.

 
         Varicoid form venous malformation (low flow vascular birthmark) in the calf. T2 weighted coronal image (image#1) demonstrates bright T2 signal abnormalities representing venous varicose veins extending into the muscles. Contrast enhanced MR angiography was performed in this case in multiple phases. 1st phase image (image#2) shows leg runoff arteries (tibial arteries) and relatively early opacification of the venous birthmark. Delayed phase MRA shows progressive filling of the malformation with contrast material and also draining tibial veins.  

 
    

     Do Venous Malformations cause bone deformities?

Venous malformations may cause changes in the bones depending on the size of the lesion, as well as the degree of direct contact with the bony structures. This radiographic image (1st image) shows multiple small calcifications (phleboliths), associated with osseous deformities involving the radius and ulna. The ulna is deformed and shortened. Increased soft tissue densities are seen representing soft tissue low-flow malformations. 

Sagittal MRI of the knee (2nd image) demonstrates significant joint surface irregularity and increased joint effusion in a patient with a venous malformation (slow-flow vascular birthmark) in the knee. Chronic bleeding into the joint may cause significant joint damage in some patients and may require aggressive approach to eliminate this sequela. 

 

 

 

Treatment?

Treatment options for patients with venous malformation or "cavernous hemangioma" include:  

1- Sclerotherapy Sclerotherapy is generally considered less invasive and more effective treatment modality. Most patients (approximately 60-80%) benefit from sclerotherapy. Please click the subtitle to read more about sclerotherapy procedure.

2- Surgery. Surgical excision should only be considered for superficial small lesions.  Attempts to excise large venous malformations usually cause significant morbidity and also cause significant scar formation (see the photo, venous malformation of the chest wall following failed surgical attempts). Therefore, surgical excision should be used for lesions that do not involve significant muscle groups or bones and should only be performed by an experienced surgeon. On the other hand, despite successful removal in many cases, most lesions recur after surgery. 

3- Radiofrequency ablation: Radiofrequency ablation for treatment of symptomatic low-flow vascular malformations is recently published by van der Linden et al (JVIR 2005;16:747-750): In this report, total 3 patients (all reportedly diagnosed with capillary venous malformation, all in the lower extremities) were treated with radiofrequency ablation. The paper reported no complications. All patients were reported to be treated successfully, although one patient became symptomatic after a year. Radiofrequency ablation is commonly used for cancers of the liver, kidneys, lung and bone. However, I believe that radiofrequency ablation may also be a therapeutic option for patients who are not good surgical or sclerotherapy candidates or for patients with failed sclerotherapy and/or surgery. 

 

 
 

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