Sachin Sarcoma Society
FAVA

FAVA

Fibroadipose Vascular Anomaly (FAVA) is a rare and complex vascular condition that affects the blood vessels and surrounding tissues. Below are answers to the questions you might consider asking your doctor if diagnosed with FAVA:

What is Fibroadipose Vascular Anomaly (FAVA), and how does it develop?

FAVA is a rare vascular condition characterized by the presence of abnormal blood vessels, fatty tissue, and fibrous tissue, usually found in deep soft tissues such as muscles or fat. It can lead to pain, swelling, and sometimes functional impairment. The exact cause is not fully understood, but it may be related to developmental issues or abnormal blood vessel growth.

How common is FAVA, and what causes it?

FAVA is very rare, with only a limited number of cases reported in the medical literature. Its exact cause remains unknown, though it may be associated with genetic mutations, vascular malformations, or trauma. Most of the lesions are sporadic without a clear family history.

What are the key symptoms of FAVA, and how does it progress over time?

Symptoms of FAVA include pain, swelling, limited mobility, and soft tissue masses. The condition may progress slowly, with worsening symptoms such as increasing pain and swelling, or it may remain stable for years. The impact on mobility can vary depending on the location and severity of the anomaly.

Is FAVA a genetic condition? Should my family members be screened?

FAVA is typically not inherited but may have a genetic component in some cases. If there is any family history of similar vascular issues, it might be useful for family members to consult with a healthcare provider for screening, though most cases are sporadic.

How was my FAVA diagnosed, and what tests confirmed it?

FAVA is typically diagnosed through imaging studies such as MRI, ultrasound, and sometimes CT scans. A biopsy may also be done in some cases to rule out other conditions. The diagnosis is often confirmed when imaging reveals a combination of abnormal blood vessels, fat, and fibrous tissue.

What imaging tests (MRI, ultrasound, etc.) are needed to monitor my condition?

MRI is the most common imaging modality used to assess FAVA due to its ability to provide detailed images of soft tissues. Ultrasound may also be used to evaluate blood flow and detect swelling or changes in tissue composition. Periodic follow-ups are recommended to monitor disease progression.

How severe is my FAVA, and what areas of my body are affected?

The severity of FAVA varies from person to person. It can affect different parts of the body, including the limbs, trunk, or face. Your doctor will assess the extent of the condition using imaging tests and physical examination.

What treatment options are available for FAVA?

Treatment options include surgery, pain management, compression therapy, and physical therapy. Surgery is often used to remove or reduce abnormal tissue if it is causing significant discomfort or functional impairment. Non-surgical treatments may help alleviate symptoms.

What are the goals of treatment—pain management, improved mobility, or other outcomes?

The primary goals of treatment are to manage pain, reduce swelling, improve mobility, and address any functional limitations caused by the condition. In some cases, the goal may also include preventing further complications or progression.

Is surgery necessary for my condition, and what does the procedure involve?

Surgery may be necessary if FAVA causes significant pain, swelling, or disability. The procedure typically involves removing the abnormal tissue (fibroadipose tissue), which can help alleviate symptoms. The specific approach depends on the location and extent of the anomaly.

Are there non-surgical options, such as physical therapy or medications?

Yes, non-surgical options can include physical therapy to maintain or improve mobility, pain management with medications, and the use of compression garments to reduce swelling. These options may be recommended in conjunction with or as alternatives to surgery.

What are the risks and benefits of each treatment option?

Surgical risks include infection, scarring, and complications related to anaesthesia. The benefits include improved mobility and pain reduction. Non-surgical options generally have fewer risks but may not fully address symptoms or prevent further progression of the condition.

Can embolization or sclerotherapy be used to treat FAVA in my case?

Embolization and sclerotherapy are procedures used to treat vascular malformations by blocking blood vessels. These techniques may be considered if there is a significant vascular component to FAVA, though they are not always effective and are less commonly used for FAVA.

How can I manage pain and swelling caused by FAVA?

Pain and swelling can often be managed with non-steroidal anti-inflammatory drugs (NSAIDs), compression garments, physical therapy, and elevation. In severe cases, corticosteroid injections or other pain management strategies may be used.

Are there specific exercises or therapies that can help maintain mobility?

Yes, physical therapy can help improve joint mobility, muscle strength, and overall function. Stretching and strengthening exercises are often tailored to your specific needs and symptoms.

Should I avoid certain activities or movements?

You should avoid activities that could aggravate swelling or pain, such as heavy lifting or high-impact exercises, depending on the affected areas. Your doctor or physical therapist can guide you on safe movements and activities.

Can compression garments or other supportive devices help reduce symptoms?

Compression garments, like sleeves or stockings, can help reduce swelling and provide support to affected areas. These devices are particularly helpful in cases where FAVA causes significant swelling.

What is the long-term outlook for someone with FAVA?

The long-term outlook for individuals with FAVA varies. In many cases, symptoms can be well-managed with treatment. However, FAVA can sometimes progress, leading to more significant pain or mobility challenges. Regular monitoring is important to track changes over time.

How often should I follow up with you to monitor my condition?

Follow-up appointments will depend on the severity of your FAVA and how well your condition is being managed. Typically, monitoring may be needed every 6 to 12 months, but more frequent visits may be necessary if symptoms worsen.

Are there risks of FAVA worsening or recurring after treatment?

Yes, there is a potential for FAVA to recur or worsen, particularly if not completely removed or if the underlying vascular malformation is not fully addressed. Regular monitoring is necessary to detect any changes early.

How does FAVA impact my daily life, and can I expect improvements with treatment?

FAVA can impact daily life by causing pain, swelling, and difficulty moving. Many people experience improvement with appropriate treatment, including pain relief, reduced swelling, and improved mobility. However, the level of improvement varies.

Should I see a specialist, such as a vascular surgeon, pain management expert, or physical therapist?

Yes, seeing a vascular surgeon can be helpful for surgical options or advanced imaging. A pain management expert can assist with symptom control, and a physical therapist can provide guidance on maintaining mobility.

Are there specific centres or hospitals with expertise in treating FAVA?

There are specialized vascular centres and academic hospitals like AIIMS with experience in treating rare conditions like FAVA. Your doctor may refer you to a centre with expertise in vascular anomalies.

Are there any support groups or resources for people living with FAVA?

Support groups for vascular anomalies exist, and they can provide emotional support and valuable information. Organizations like the Vascular Birthmarks Foundation may offer resources for patients with FAVA and other related conditions.

These answers provide an overview of what to expect when diagnosed with FAVA. Always work closely with your healthcare provider to tailor a treatment plan specific to your needs.