A lymphatic malformation of the face and neck is a soft, spongy swelling made from lymph vessels that grew in an abnormal way before birth. These vessels form small or large sacs filled with clear fluid, like tiny water balloons under the skin. The swelling is benign, which means it is not cancer, but it can still cause serious problems with breathing, swallowing, speaking, and appearance, especially when it is large or deep. Most lymphatic malformations appear in babies or small children, and about three-quarters occur in the head and neck area.
A lymphatic malformation of the face and neck is a birth problem where some lymph vessels (tiny tubes that carry clear fluid in the body) grow in a wrong way and form soft, fluid-filled cysts or swelling in the skin and deeper tissues. These swellings can be small or very large. They often appear in babies or young children and may slowly grow over time, especially during infections or after minor injury. They are benign (not cancer), but they can still cause serious problems such as trouble breathing, swallowing, speaking, or problems with appearance and self-confidence. Treatment usually needs a team of doctors. Main options are watching and waiting, sclerotherapy (medicine injected into the cysts), surgery, and sometimes medicines like sirolimus (an mTOR inhibitor) to shrink the abnormal vessels.
Other names
Doctors used many different names in the past for the same condition. Today, “lymphatic malformation” is the preferred name, but you may still see older names in books and older reports. These names can confuse patients and families, so it helps to know they are talking about the same kind of problem.
Common other names include:
Lymphangioma – an older term for these abnormal lymph vessel growths.
Cystic hygroma – usually used for large, fluid-filled malformations in the neck.
Cystic lymphangioma – another way to say a cystic lymphatic malformation.
Cervicofacial lymphatic malformation – means a lymphatic malformation in the neck (cervical) and face area.
Lymphangioma circumscriptum – older name mainly for small, surface (microcystic) lymphatic malformations that look like tiny blisters on the skin.
These older names are slowly being replaced, because the word “-oma” sounds like cancer, and these lesions are not cancerous. Using “lymphatic malformation” is clearer and less frightening for patients.
Types of lymphatic malformation of face and neck
Experts group lymphatic malformations mainly by the size of the fluid spaces (cysts) inside them. This helps doctors choose the best treatment, because some types respond better to injections, and others may need more complex surgery.
Macrocystic lymphatic malformation
In this type, the lesion is made mostly of large cysts (usually bigger than 1–2 cm). They often appear as a soft, doughy lump in the side of the neck or under the jaw. These cysts compress easily when pressed and often glow with a light (transilluminate). Macrocystic lesions often respond well to injection treatment (sclerotherapy).Microcystic lymphatic malformation
Here, the lesion is made of many tiny cysts spread through the skin, tongue, lips, or cheek. On the surface it may look like clusters of small, clear or red “bubbles” (vesicles) that may leak clear fluid or bleed. Microcystic lesions are harder to remove completely and usually need a combination of treatments.Mixed macrocystic–microcystic lymphatic malformation
Many lesions are mixed, with both large and small cysts together. They can spread widely through the cheek, jaw, tongue, floor of the mouth, or neck, and can distort the shape of the face and cause functional problems. Mixed lesions are often more complex to treat and may need several procedures over time.Generalized or complex lymphatic anomalies
In some patients, lymphatic malformations are part of a wider disease, with lymphatic overgrowth in many parts of the body (for example generalized lymphatic anomaly or Gorham–Stout disease). When these involve the face and neck, they may combine with bone, chest, or abdominal lesions and need care in a specialized center.
Causes and risk factors
Lymphatic malformations of the face and neck are mostly congenital, meaning they start during baby development in the womb. There is usually no single thing the parents did or did not do. Doctors talk about “causes and risk factors” to explain what is known, but in many children the exact reason is never found.
Developmental error of lymphatic vessels
The main cause is a mistake during early formation of the lymphatic system. Small lymph sacs fail to connect properly to the main lymph channels, leaving pockets of trapped lymph that slowly grow into cysts. This is a structural birth difference, not an infection or tumor.Somatic PIK3CA gene mutations
Many lymphatic malformations are linked to changes (mutations) in the PIK3CA gene that occur only in the affected tissue, not in every cell of the body. These mutations cause over-growth of lymphatic vessels and make them form abnormal cystic spaces.Other lymphatic-related gene changes
Some patients have changes in other genes linked to lymph vessel signaling and growth, such as TEK/TIE2 or related pathways. These changes disturb normal lymph vessel formation and can lead to focal malformations in areas like the neck or face.Family history of vascular anomalies
Most lymphatic malformations are not inherited, but in some families, several members have vascular malformations. This suggests an underlying genetic tendency that raises the chance of a child being born with a lymphatic malformation.Association with Turner syndrome
Turner syndrome, a condition where a girl has one missing or altered X chromosome, is often linked with large cystic hygromas in the neck on prenatal scans. These neck lymphatic malformations may be one of the first visible signs of Turner syndrome in the fetus.Association with Noonan syndrome
Children with Noonan syndrome can have lymphatic problems, including cystic hygromas and later limb or body swelling. Abnormal lymph development in this syndrome can affect the face and neck, leading to visible malformations.Association with trisomy 21 (Down syndrome)
Down syndrome is sometimes associated with neck cystic hygromas seen on prenatal ultrasound. These are thought to reflect disturbed lymphatic development when the chromosomes are abnormal.Association with trisomy 13 and 18
Some fetuses with trisomy 13 or trisomy 18 also show large cystic neck lesions. These cases support the idea that serious chromosome problems can interfere with normal lymph vessel development.Association with Klippel–Trénaunay and other overgrowth syndromes
Syndromes like Klippel–Trénaunay and other combined vascular anomalies may include lymphatic malformations as part of a larger pattern of abnormal vessels and tissue overgrowth, sometimes involving the face and neck.Generalized lymphatic anomaly and related disorders
In some children, lymphatic malformations in the face or neck are part of generalized lymphatic anomaly or kaposiform lymphangiomatosis, conditions with widespread abnormal lymph channels in bone, chest, and other organs.Acquired progressive lymphatic anomaly after local damage
Rarely, lymphatic malformations may develop or enlarge after local trauma, surgery, or infection. Damage may block or scar lymph channels, leading to dilation and new abnormal lymph spaces in the nearby face or neck area.Radiation exposure to neck in childhood
Radiation therapy can injure normal lymph vessels and cause long-term scarring and dilation. In rare cases, this may contribute to acquired lymphatic malformations or complex lymphatic problems in the treated area.Intrauterine infection and inflammation
Severe infections or inflammation during pregnancy may disturb normal tissue development, including lymph vessels, although exact proof is limited. Doctors consider this a possible but not well-proven risk factor in some complex cases.Maternal conditions that affect fetal development
Maternal diseases, severe diabetes, or uncontrolled thyroid and autoimmune problems may slightly raise the chance of developmental abnormalities in the baby, including vascular and lymphatic malformations, though this link is not strong in current studies.Combined lymphatic–venous malformations
Some lesions are mixed venous-lymphatic malformations. In these, abnormal development of both vein and lymph vessel systems leads to complex masses in the face or neck, sometimes called lymphovenous malformations.Hormonal and growth factor influences
Growth and sex hormones, and local growth factors, may influence how much a lymphatic malformation enlarges during childhood, puberty, or pregnancy. They do not cause the lesion, but they can make it grow faster at certain life stages.Repeated local infection or bleeding into the lesion
When a lymphatic malformation becomes infected or bleeds internally, the inflammation can make it larger, more fibrotic, and more complicated. This does not create the lesion but makes an existing small malformation much more noticeable.Rapid growth during respiratory infections
In children, common colds or throat infections can trigger sudden swelling of a neck lymphatic malformation, especially when the upper airway is inflamed. This can unmask a lesion that was previously small and hidden.Deep location delaying diagnosis
Some lesions start deeper in the tissues of the tongue, floor of mouth, or neck spaces. They may be present from birth but only cause visible swelling or symptoms when they grow larger, so they seem to “appear later” even though they were there earlier.Idiopathic cases (no clear cause)
In many children with a lymphatic malformation of the face or neck, no genetic syndrome, family history, or clear trigger is found. In these idiopathic cases, doctors believe a random developmental error in early lymph vessel formation is the most likely explanation.
Symptoms and signs
Symptoms depend on the size, depth, and exact location of the lymphatic malformation. Many are painless but can still cause serious problems, especially near the airway or inside the mouth.
Soft, painless swelling of face or neck
The most common sign is a soft, squishy lump in the cheek, jaw, neck, or under the chin. It often feels like a bag of jelly or small water balloons. It may be present at birth or appear gradually over months or years.Compressible mass that changes with pressure or position
The swelling usually flattens a bit when pressed and may get larger when the child cries, coughs, or lies flat, because more blood and fluid move into the area. This changing size helps doctors suspect a vascular or lymphatic lesion.Transillumination (glowing with light)
Large macrocystic lesions often glow when the doctor shines a bright light through the skin, because they are full of clear fluid. This is a classic sign that the mass is cystic and not solid, and it supports the diagnosis of lymphatic or similar malformation.Clusters of small clear or red “blisters” on the skin or tongue
Microcystic lesions on the lips, inside the cheeks, or on the tongue may look like grouped tiny bubbles or vesicles. They may be pink, red, or dark and can leak clear lymph fluid or sometimes blood if they are rubbed or bitten.Facial or neck asymmetry
A large malformation can change the contour of the face or neck, making one side look bigger or lower than the other. This can be very visible and may worsen as the child grows, leading to cosmetic concerns.Difficulty breathing or noisy breathing (stridor)
When the lesion lies close to the airway in the tongue, floor of mouth, or lower neck, it may narrow the breathing passage. The child may have noisy breathing, especially when asleep or during infections, and in severe cases breathing can become an emergency.Sleep apnea or snoring
Some children with large tongue or oropharyngeal lesions snore loudly or stop breathing for short moments during sleep (sleep apnea). This happens because the airway is crowded by the enlarged tissues when the child lies down and relaxes.Difficulty swallowing (dysphagia)
Malformations around the throat, base of the tongue, or esophagus entrance can interfere with swallowing. The child may choke on food, have prolonged mealtimes, or prefer softer foods. Babies may have poor feeding or trouble gaining weight.Voice changes or hoarseness
If the lesion presses on or involves the larynx (voice box) or nearby nerves, the voice may become hoarse, weak, or change in pitch. This is more likely when the malformation extends deeply into the lower neck or upper airway.Pain or tenderness during infection or bleeding
Most lymphatic malformations are painless, but when they become infected or when blood collects inside the cysts, they can suddenly become swollen, red, and painful. The child may have fever and feel unwell, and the area may be warm and tender.Recurrent local infections or cellulitis
The abnormal lymph channels do not drain properly, so bacteria can grow easily, leading to repeated infections of the overlying skin or deeper tissues. Each infection can cause temporary enlargement and further scarring.Bleeding or leaking lymph fluid from small surface vesicles
Microcystic lesions on the lips or tongue may leak clear fluid or bleed after minor trauma, such as biting the lip or brushing teeth. This can be distressing but usually stops with simple pressure.Limited movement of jaw, neck, or tongue
Very large lesions can restrict how far the jaw opens, how easily the neck turns, or how the tongue moves. This can affect eating, speaking, and daily activities, especially when the lesion is deep and fibrotic.Hearing or ear problems when near the ear region
When a lymphatic malformation is close to the ear or parotid region, it can press on the ear canal or middle ear structures. This may cause recurrent ear infections or mild hearing loss in some children.Psychological and social distress
Because the face is so visible, any deformity, swelling, or skin change can cause embarrassment, bullying, and anxiety for children and families. Studies show that vascular malformations often have serious effects on self-esteem and quality of life.
Diagnostic tests
Doctors use a combination of physical examination and tests to diagnose lymphatic malformations of the face and neck, to see how large they are, how deep they go, and whether they press on important structures like the airway. Not all tests are needed in every patient; the team chooses tests based on symptoms and treatment planning.
Physical exam tests
General head and neck inspection
The doctor looks carefully at the face and neck to see the size, shape, color, and position of the swelling. They check for asymmetry, skin changes, visible small blisters, and signs of infection. A detailed visual exam often gives the first strong clue that the lesion is a lymphatic malformation.Palpation of the mass
The doctor gently feels the swelling with the hands to judge whether it is soft, firm, or rubbery, and whether it is tender. Lymphatic malformations are usually soft and compressible, sometimes with a “doughy” feel, which helps distinguish them from solid tumors or enlarged lymph nodes.Transillumination test
In this test, a bright light is placed against the skin over the swelling in a dark room. If the lesion is filled with clear fluid, it often glows or lights up, showing that it is cystic. This simple bedside test supports a diagnosis of macrocystic lymphatic malformation.Oral cavity and tongue examination
The doctor examines the lips, cheeks, tongue, floor of mouth, and back of the throat. They look for small vesicles, tongue enlargement, and signs that the lesion is narrowing the airway or interfering with chewing or swallowing. This exam is key when the child has speech or feeding problems.Airway and breathing assessment
The clinician listens for noisy breathing, stridor, or wheezing and watches the chest and neck for signs of breathing difficulty. In more complex cases, an ENT specialist may perform flexible endoscopy to look directly at the larynx and lower airway to see how much is narrowed by the lesion.
Manual clinical tests
Compression test of the swelling
The doctor gently presses on the swelling to see whether it flattens and then slowly refills. Lymphatic malformations often compress easily but refill over seconds to minutes. This manual test helps separate them from hard tumors or solid cysts.Neck range-of-motion test
The clinician asks the patient to turn the head, bend it forward and backward, and tilt it to each side. Limited motion can show that a deep lesion is stiff or attached to muscles and surrounding tissues, which is important for planning surgery or injections.Swallowing assessment (bedside)
The patient is observed while swallowing water and soft food. The examiner watches for coughing, choking, or delayed swallowing. Problems may indicate that the lesion is pressing on the pharynx or esophagus and that further imaging or specialized swallow studies are needed.
Lab and pathological tests
Complete blood count (CBC)
A CBC checks for signs of infection (high white blood cells) or anemia (low red blood cells) that may result from repeated bleeding or infection in the lesion. While CBC does not diagnose the malformation, it guides safe timing of surgery and helps monitor complications.Inflammatory markers (CRP and ESR)
Blood tests such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) rise when there is active inflammation or infection in the body. Elevated levels can support the clinical impression of infected lymphatic malformation and guide treatment with antibiotics.Coagulation profile
Tests like PT, aPTT, and platelet count are sometimes checked before surgery or sclerotherapy to make sure the blood clots normally. This is important because large head and neck procedures can involve significant bleeding, and some vascular anomalies may be linked to clotting problems.Fine-needle aspiration cytology (FNAC)
In doubtful cases, a doctor may insert a thin needle into the cyst to withdraw fluid for analysis. The fluid from lymphatic malformations is usually clear or milky and contains lymphocytes. FNAC can help rule out other cystic neck masses when imaging is not typical.Histopathology (biopsy of the lesion)
When the lesion or part of it is surgically removed, a pathologist examines it under the microscope. They look for thin-walled lymphatic channels lined by endothelial cells, often filled with protein-rich fluid. This provides the final, definite diagnosis of lymphatic malformation.Molecular / genetic testing of lesion tissue
In some centers, tissue from the lesion is tested for PIK3CA and other gene mutations. Finding these mutations confirms that the malformation is driven by specific growth pathways and may help in choosing targeted drug therapies in difficult cases.
Electrodiagnostic tests
Nerve conduction studies (NCS) of facial and cervical nerves
Large lesions or repeated surgeries in the parotid and neck region can affect facial or other cranial nerves. When a child develops weakness of facial movement or other nerve symptoms, nerve conduction studies can measure how well the nerves carry electrical signals, helping to plan further treatment.Electromyography (EMG) of facial and neck muscles
EMG records electrical activity in muscles. It can show whether weakness is due to nerve damage or muscle changes caused by long-standing pressure from the lesion. Although not used in every patient, EMG can be useful when there are complex nerve or muscle problems in the face or neck.
Imaging tests
Ultrasound of face and neck
Ultrasound is usually the first imaging test. It is painless and uses sound waves, not radiation. It shows whether the lesion is cystic, how many cysts there are, and how deep they go. Doppler ultrasound can also show that blood flow is low, which supports a diagnosis of low-flow lymphatic malformation.Magnetic resonance imaging (MRI)
MRI gives very detailed pictures of the lesion and surrounding tissues. It shows the exact extent of the malformation, its relationship to muscles, nerves, and airway, and whether it is macrocystic, microcystic, or mixed. MRI is especially helpful before surgery or complex sclerotherapy.Computed tomography (CT) scan of head and neck
CT uses X-rays to create cross-sectional images. It is sometimes used when MRI is not available or when bone involvement needs to be seen clearly. CT helps define deep neck spaces and is useful for planning operations in large or unusual lesions, especially in children with airway compromise.Lymphatic imaging (lymphoscintigraphy or specialized MR lymphangiography)
In complex or generalized lymphatic diseases, special imaging tests can trace how lymph fluid flows through the vessels. Radioactive tracers or contrast agents are used to map abnormal channels and leaks. These techniques are not needed for every facial or neck lesion but can be very helpful in selected patients with widespread disease.
Non-pharmacological treatments
1. Watchful waiting with regular check-ups
Some lymphatic malformations stay small, soft, and do not cause pain or breathing or feeding problems. In these cases, doctors may simply “watch and wait.” You or your child will see the specialist regularly. The purpose is to avoid risky procedures when the lump is stable, and to act quickly if it grows or gets infected. This method works because many lesions grow very slowly or stay almost the same for years, especially macrocystic (large-cyst) types.
2. Good skin and wound care
The skin over the swelling can be thin and fragile. Gentle washing, using mild soap and soft towels, and applying moisturizer can protect the area. The purpose is to prevent cracks, bleeding, or infection on top of the malformation. The mechanism is simple: healthy skin acts as a strong barrier, stopping bacteria from entering and lowering the risk of cellulitis (skin infection) or abscess.
3. Avoiding pressure and trauma to the swelling
Patients are advised not to press, poke, or hit the lump, and to avoid tight collars, belts, or sports that can cause blows to the neck or face. The purpose is to prevent sudden bleeding inside the malformation and painful enlargement. The mechanism is that less mechanical stress means fewer episodes of internal bleeding or fluid leakage, which can otherwise make the lesion larger and harder to treat.
4. Positioning and sleep adjustments
Some children breathe worse when lying flat because the neck swelling presses on the airway. Sleeping with the head slightly raised on a pillow or keeping the baby’s head elevated can help. The purpose is to reduce airway obstruction and snoring. Gravity pulls fluid downwards and slightly away from the throat, which can ease breathing during sleep.
5. Physiotherapy and posture training
Large neck masses can affect posture, shoulder movement, or neck rotation. A physiotherapist can teach gentle stretches and exercises to keep the neck supple and strong. The purpose is to prevent muscle stiffness and long-term posture problems. Movement and stretching improve blood and lymph flow and stop muscles from shortening and tightening around the swelling.
6. Speech and swallowing therapy
When the malformation affects the tongue, floor of mouth, or throat, a speech-language therapist can help with swallowing techniques, food textures, and speech exercises. The purpose is to keep feeding safe and speech as clear as possible. Therapy works by teaching safer ways to chew and swallow and by strengthening muscles that can still work well around the lesion.
7. Breathing exercises and airway monitoring
For patients with partial airway narrowing, doctors may teach slow deep-breathing exercises and watch for signs of worsening like noisy breathing or pauses in breathing. The purpose is early detection of airway compromise. The mechanism is simple: regular observation and exercises help clinicians decide if more active treatment (like sclerotherapy or surgery) is needed before an emergency happens.
8. Lymphatic drainage massage by trained therapist
In some centers, specially trained therapists use very gentle massage techniques to encourage fluid to move away from the swollen area. The purpose is to reduce soft swelling and discomfort. The mechanism is that light rhythmic strokes can stimulate nearby healthy lymph vessels to pick up some of the extra fluid, although evidence is limited and this should only be done by experts on appropriate lesions.
9. Psychological and family counseling
Visible swellings on the face and neck can cause bullying, low self-esteem, and anxiety in children and parents. Counseling, support groups, and age-appropriate explanations help families cope. The purpose is to protect mental health and social development. Talking therapy works by giving emotional support, teaching coping skills, and reducing stress, which can also improve recovery from procedures.
10. School and social support planning
Teachers and caregivers can be informed about the condition, warning signs (like sudden swelling or breathing trouble), and activity limits. The purpose is to keep the child safe and understood in school. Mechanically nothing changes in the lesion, but better social planning lowers risk in emergencies and reduces emotional stress.
11. Infection prevention measures
Because infections can make the malformation swell, doctors often stress good hand hygiene, up-to-date vaccines, and quick treatment of throat or skin infections. The purpose is to prevent episodes of cellulitis and painful flare-ups. Mechanism: fewer infections mean less inflammatory fluid entering the abnormal lymph channels, so they are less likely to expand suddenly.
12. Oral hygiene and dental care
If the lymphatic malformation is inside the mouth, careful tooth-brushing, regular dental checks, and sometimes fluoride treatments are important. The purpose is to reduce mouth infections that could spread into the malformation. Healthy teeth and gums lower bacterial load, which cuts the risk of abscesses in or near the lesion.
13. Gentle cold or warm packs for pain
Short periods of cool or warm packs wrapped in cloth (never directly on skin) may ease mild pain or tightness after procedures, if the doctor agrees. The purpose is comfort without extra drugs. Temperature changes can briefly reduce nerve pain signals and muscle spasm, although they do not shrink the lesion itself.
14. Weight management and regular physical activity
Extra body weight can make neck and airway problems worse and may reduce mobility. Healthy eating and regular, safe activity help keep weight normal. The purpose is to lower breathing strain and support overall health. Good general health supports better healing after sclerotherapy or surgery and may reduce complications.
15. Sun protection for the affected area
Thin, stretched skin over big malformations can burn easily. Using hats and sunscreen protects from sun damage. The purpose is to avoid burns, pigment changes, and skin cancers later in life, especially in patients taking medicines like sirolimus that increase sun sensitivity. Reduced UV exposure may also limit skin aging and breakdown.
16. Written emergency plan
Families can carry a short written plan that explains the diagnosis and what to do if the child has sudden swelling, bleeding, or breathing trouble. The purpose is faster, safer care in emergency departments. The mechanism is organizational: clear information prevents delays and inappropriate treatment.
17. Multidisciplinary clinic follow-up
Seeing a team that includes a vascular anomalies specialist, pediatric surgeon, ENT surgeon, radiologist, and nurse helps coordinate care. The purpose is to choose the best combination of observation, sclerotherapy, medicines, and surgery. Studies show that combined approaches give better long-term results than single methods alone.
18. Laser therapy for small surface lesions
For very superficial lesions on the skin or mucosa, doctors may use laser to close abnormal vessels. The purpose is to reduce bleeding, oozing, or cosmetic problems. Laser light is absorbed by blood or lymph in tiny vessels and turns into heat, sealing them so they collapse over time.
19. Cryotherapy in selected cases
Cryotherapy uses extreme cold to freeze small patches of abnormal tissue. It may sometimes be used for very small or specific surface lesions. The purpose is local destruction of abnormal channels. Freezing causes ice crystals inside cells, which then die and are slowly removed by the body.
20. Education about warning signs
Teaching families to recognize red skin, fever, fast growth of the lump, noisy breathing, or trouble swallowing is crucial. The purpose is to get quick help before complications become life-threatening. The “mechanism” is early action: the sooner infection or airway problems are treated, the lower the risk of severe complications.
Drug treatments
Note: Many medicines below are off-label for lymphatic malformations. They are used only under specialist supervision, often in expert centers or clinical trials.
1. Sirolimus (oral mTOR inhibitor)
Sirolimus is now one of the most important drugs for complex lymphatic malformations of the face and neck. It blocks the mTOR pathway, which helps control cell growth and new vessel formation. By slowing this pathway, sirolimus can shrink lymphatic tissue, reduce pain, bleeding, and leakage, and improve quality of life. Dosing is weight-based and monitored with blood levels; only specialists should adjust it because side effects include infections, high cholesterol, mouth ulcers, delayed wound healing, and rarely lung problems.
2. Topical sirolimus (e.g., sirolimus gel)
Topical sirolimus gel has FDA approval for facial angiofibromas in tuberous sclerosis but is being explored off-label for very superficial lymphatic malformations. It is applied as a thin layer on the skin. The purpose is to reduce redness, leakage, and small papules without strong whole-body effects. Because absorption through skin is lower, systemic side effects are usually mild, but irritation, acne-like rash, or local burning can occur.
3. Everolimus (oral mTOR inhibitor)
Everolimus is another mTOR inhibitor used for some tumors and tuberous sclerosis. In complex vascular and lymphatic anomalies, it may be used when sirolimus is not tolerated. It works in a similar way by reducing abnormal vessel growth and inflammation. Dosing is individualized and monitored. Common side effects include mouth sores, increased infection risk, high lipids, and fatigue, so careful blood tests are needed.
4. Bleomycin (intralesional sclerosant)
Bleomycin injections directly into the cysts are a widely used sclerotherapy drug for lymphatic malformations. A specialist injects a carefully calculated dose into the lesion under ultrasound guidance. Bleomycin damages the endothelial cells lining the cysts, causing inflammation and scarring so the spaces close and shrink. It can give major size reduction, but total dose is limited because high cumulative doses can cause lung fibrosis; skin darkening and temporary fever are also possible.
5. Doxycycline (intralesional sclerosant and antibiotic)
Doxycycline is an antibiotic that also works as a sclerosant when injected into cysts. It irritates the cyst lining, leading to scarring and collapse. It is often used for macrocystic lesions. As a drug, it also fights bacterial infections. Side effects can include pain at the injection site, temporary swelling, and typical doxycycline effects like photosensitivity or stomach upset if given orally.
6. OK-432 (Picibanil) intralesional therapy
OK-432 is a preparation of killed Streptococcus pyogenes used as an immune-stimulating sclerosant in some countries. Injected into cysts, it triggers a strong local immune reaction that damages the lining of abnormal lymph spaces, leading to shrinkage. It can be very effective for macrocystic head and neck lesions but may cause fever, pain, and swelling after treatment, and access is limited in many regions.
7. Ethanol (absolute alcohol) sclerotherapy
Ethanol is a powerful sclerosant used carefully by experienced interventional radiologists. It removes water from cells and denatures proteins, causing vessel wall destruction and permanent closure of abnormal channels. It can be effective but carries higher risk of nerve injury, skin necrosis, or systemic toxicity, so it is reserved for selected cases.
8. Polidocanol or sodium tetradecyl sulfate (STS) foam
These detergents are sclerosant drugs used in foam form for some venous and lymphatic malformations. When injected into cysts, the foam spreads along the lining and irritates it, causing scarring and collapse. They are often chosen for lesions near nerves where alcohol might be too risky. Side effects include pain, inflammation, or rare allergic reactions.
9. Propranolol (oral beta-blocker)
Propranolol is famous for infantile hemangiomas and has been tried in some mixed vascular malformations, including lymphatic components. It blocks beta-adrenergic receptors, which may reduce vessel growth signals. It can help with pain or swelling in selected patients. Side effects include low blood pressure, slow heart rate, sleep problems, or low blood sugar, so dosing is weight-based and closely monitored.
10. Sildenafil (oral phosphodiesterase-5 inhibitor)
Sildenafil relaxes smooth muscle in blood vessels and is used for pulmonary hypertension. Small studies suggest it may improve symptoms in some complex lymphatic and venous malformations by improving abnormal circulation and lymph drainage. Side effects include headache, flushing, and rarely blood pressure changes. It is experimental in this setting and only used by specialized teams.
11. Systemic corticosteroids (e.g., prednisolone)
Short courses of steroids may be used during severe inflammation or airway compromise to quickly reduce swelling. These drugs damp down the immune system and reduce fluid leakage from vessels. They are not long-term solutions because of side effects such as weight gain, mood changes, high blood pressure, and bone thinning.
12. Antibiotics for secondary infection (e.g., amoxicillin-clavulanate)
If the malformation becomes infected, antibiotics are needed. Amoxicillin-clavulanate is a common broad-spectrum option for skin and soft tissue infections. It works by blocking bacterial cell wall building and beta-lactamase enzymes. Side effects include diarrhea, rash, or allergic reactions. Early treatment of infection helps avoid abscess formation and lesion enlargement.
13. Analgesics such as paracetamol (acetaminophen)
Paracetamol is used to relieve mild to moderate pain after procedures or during flare-ups. It reduces pain signals in the brain and helps with fever but does not reduce swelling of the lesion itself. It is usually safe at correct doses, but overdose can damage the liver, so dosing limits must be followed.
14. Non-steroidal anti-inflammatory drugs (NSAIDs, e.g., ibuprofen)
Ibuprofen can help with pain and inflammation when there is swelling or post-procedure discomfort. It blocks COX enzymes that make prostaglandins, which are chemicals that cause pain and swelling. Side effects include stomach upset, kidney strain, or increased bleeding risk, so doctors often use it carefully around surgery or sclerotherapy.
15. Antihistamines (e.g., cetirizine)
When the skin over the lesion becomes itchy after procedures or due to mild allergic reactions, antihistamines can help. They block histamine receptors, reducing itch and redness. Drowsiness, dry mouth, or headache can occur, depending on the drug. These medicines make life more comfortable but do not shrink the malformation.
16. Topical corticosteroid creams
Mild topical steroids may be used on inflamed skin over the lesion. They reduce local inflammation, redness, and irritation by calming immune cells in the skin. Side effects with long use include skin thinning and stretch marks, so they are used for short periods under medical advice.
17. Topical antibiotic creams (e.g., mupirocin)
When small skin breaks or crusted areas appear over the malformation, topical antibiotics can help prevent or treat localized infection. They work by killing or stopping the growth of bacteria on the skin surface, lowering the risk of deeper soft tissue infection. Skin irritation or rare allergy can occur.
18. Tranexamic acid (systemic anti-fibrinolytic, selected cases)
In some complex vascular anomalies with recurrent bleeding, tranexamic acid may be used. It stabilizes clots by blocking the breakdown of fibrin. For lymphatic malformations that bleed internally or from the mouth, it may reduce bleeding episodes, but must be used cautiously because of rare clotting risks.
19. Proton-pump inhibitors (PPIs) with systemic therapy
Drugs like omeprazole are sometimes added when patients take steroids or NSAIDs to protect the stomach. They reduce acid production by blocking the proton pumps in stomach cells. This lowers the risk of ulcers and bleeding, especially during longer treatment courses.
20. Vaccines and infection-prevention medicines
Although not specific to the malformation, up-to-date vaccines (e.g., influenza, pneumococcus) and prophylactic antibiotics may be recommended for patients on strong immunosuppressants such as sirolimus. They reduce infection risk, which is higher when the immune system is damped down by these drugs.
Dietary molecular supplements
Always discuss supplements with the treating team. Evidence in lymphatic malformations is limited; these are general supportive options for health and healing.
1. Omega-3 fatty acids (fish oil, algae oil)
Omega-3s support heart and vessel health and may gently reduce inflammation. In children and adults, they can be taken as capsules or liquid, with dose based on age and weight. They work by being built into cell membranes and changing the balance of inflammatory molecules. Main side effects are fishy aftertaste or mild stomach upset, and they may slightly increase bleeding tendency at high doses.
2. Vitamin D
Vitamin D supports bone health and immune function. Many children with chronic illness or little sun exposure are deficient. A daily supplement dose is chosen by the doctor after a blood test. Vitamin D works like a hormone, helping the gut absorb calcium and modulating immune cells. Too much can cause high calcium and kidney problems, so medical supervision is important.
3. Vitamin C
Vitamin C is an antioxidant that helps collagen formation and wound healing, which is important after surgery or sclerotherapy. It is usually taken in small daily doses through food (fruits and vegetables) or supplements. It neutralizes free radicals and supports white blood cells. Very high doses can cause stomach upset or kidney stones in susceptible people.
4. Zinc
Zinc is a trace mineral needed for immune function and skin repair. In children with repeated infections or poor healing, doctors may suggest a short course. Zinc supports enzyme systems in immune cells and skin cells. Too much zinc can cause nausea, metallic taste, or copper deficiency, so doses must be controlled.
5. Probiotics (selected strains)
Probiotics are “good bacteria” given as capsules or yogurt-type drinks. They aim to keep gut bacteria balanced, especially when patients need repeated antibiotics. By supporting a healthy microbiome, they may reduce diarrhea and improve general immune balance, though evidence for lymphatic malformations is indirect. Side effects are usually mild gas or bloating in otherwise healthy people.
6. Protein-rich medical nutrition drinks
When chewing or swallowing is difficult because of a large oral or neck malformation, protein-rich medical drinks help maintain weight and muscle. They provide balanced calories, protein, vitamins, and minerals. Adequate protein supports wound healing after operations or sclerotherapy. Side effects are mainly fullness or, rarely, lactose intolerance symptoms depending on the formula.
7. Multivitamin with minerals
A simple multivitamin can fill small gaps in diet when appetite is low. It supplies many vitamins and minerals at safe daily doses. This supports general health, immunity, and recovery from procedures, but it does not directly shrink the malformation. Large overdoses of single vitamins should be avoided.
8. Selenium
Selenium is a trace element with antioxidant roles and may help immune function. In carefully controlled doses, it supports enzymes that protect cells from oxidative damage. Overdosing can cause hair loss, nail problems, or nerve issues, so it must be supervised, especially in children.
9. Coenzyme Q10 (CoQ10)
CoQ10 is involved in energy production in cells and has antioxidant effects. Some clinicians use it as a general support during chronic illness or long-term medication. It works in mitochondria to help generate ATP. Side effects are usually mild, such as nausea or headache, and evidence is still limited and general, not specific to lymphatic malformations.
10. Curcumin (turmeric extract)
Curcumin is an anti-inflammatory compound from turmeric. In supplement form, it may gently reduce inflammatory signaling pathways like NF-κB. For children, only specialist-approved, safe doses should be used. It may cause stomach upset and can interact with blood-thinning medicines, so it must be used with caution.
Immunity-booster and regenerative / stem-cell-related drugs
There are no stem-cell drugs specifically approved for lymphatic malformations. The medicines below affect immune or growth pathways and are used only under expert supervision.
1. Sirolimus as an immune-modulating, vessel-regulating drug
Sirolimus not only shrinks abnormal lymph vessels but also strongly modulates the immune system by blocking mTOR in lymphocytes. This reduces abnormal inflammatory responses around the malformation and may support more orderly vessel repair. Because it suppresses immunity, it is never a “booster,” and infection risk actually increases, so careful monitoring and vaccines are needed.
2. Everolimus for mTOR pathway control
Everolimus has similar actions on the mTOR pathway and is sometimes used when sirolimus is not suitable. By changing how cells grow, divide, and respond to growth factors, it may help remodel abnormal vascular and lymphatic tissue in complex cases. It can cause low blood counts, infections, and high lipids, so it is considered a strong immunomodulatory drug, not a simple supplement.
3. Interferon-alpha (rare, specialized use)
In the past, interferon-alpha was used for severe vascular tumors and malformations. It stimulates certain immune cells and has anti-angiogenic effects, meaning it can reduce new vessel formation. Because it can cause serious side effects like mood changes, thyroid problems, and growth delay in children, it is now rarely used and reserved for life-threatening situations when safer options fail.
4. Bevacizumab (anti-VEGF monoclonal antibody, highly experimental)
Bevacizumab blocks VEGF, a key driver of new blood vessel formation. It is approved for several cancers but has been reported in some refractory vascular anomaly cases. By blocking VEGF, it may reduce abnormal vessel growth and leakage. However, it can impair wound healing, raise blood pressure, and increase bleeding risk. Its use for lymphatic malformation of the face and neck is limited and strictly experimental.
5. Mesenchymal stem cell (MSC) therapies (research only)
MSC infusions are being studied in many diseases to help tissue repair and modulate immunity. For lymphatic malformations, the idea is that MSCs may secrete growth factors that promote more normal vessel remodeling and reduce inflammation. At present, this is research only, not standard care, and may carry unknown long-term risks. It should only be done in formal clinical trials.
6. Growth factor-targeting agents in trials
Some clinical trials test drugs that target specific growth or signaling pathways involved in lymphangiogenesis, such as PI3K or AKT inhibitors. These drugs act “upstream” or “downstream” of mTOR to change how lymphatic endothelial cells grow. They are highly experimental and may have serious side effects, so they are only used in specialist centers as part of trials.
Surgeries
1. Complete surgical excision of the lesion
In some macrocystic malformations that are well-defined and not wrapped around vital nerves or vessels, surgeons can remove the whole lesion. The purpose is long-term cure or major size reduction. Surgery physically removes abnormal cysts and surrounding fibrous tissue. Risks include nerve injury, scarring, bleeding, and recurrence if tiny parts are left behind.
2. Partial debulking surgery
If the malformation surrounds nerves, airways, or blood vessels, complete removal may be impossible. Surgeons then “debulk” it, meaning they remove as much as they safely can to relieve pressure or improve appearance. This reduces symptoms like airway narrowing or deformity. Remaining tissue can later be treated with sclerotherapy or medicines like sirolimus.
3. Surgery combined with sclerotherapy (staged treatment)
A common modern approach is to first shrink the lesion with sclerotherapy and then perform a smaller, safer surgery. The purpose is to reduce operative time, blood loss, and nerve damage risk. Sclerotherapy scars many cysts, making the mass smaller; surgery then removes remaining solid parts or fibrotic tissue.
4. Airway surgery or tracheostomy in emergencies
In rare, very severe neck lesions, the airway can be so narrow that breathing is unsafe, especially in newborns. Surgeons may need to perform emergency airway procedures such as tracheostomy (making a safe breathing hole in the neck) or airway reconstruction. The purpose is life-saving respiration while other treatments (sclerotherapy, sirolimus) are planned.
5. Cosmetic and reconstructive surgery
After major size reduction or cure, some patients have extra skin, scarring, or facial asymmetry. Plastic and reconstructive surgeons can perform scar revision, skin tightening, or fat grafting to improve appearance and function. The purpose is better facial balance, speech, and social confidence. These procedures reshape normal tissues rather than treating active lymphatic channels.
Prevention strategies
Early specialist referral – See a vascular anomalies clinic as soon as a lesion is suspected, rather than waiting until severe symptoms appear. Early staging and planning reduce complication rates.
Regular follow-up visits – Keep all scheduled check-ups even if the swelling looks stable. Many problems can be caught early, such as small airway changes or early infection.
Prompt treatment of infections – Treat sore throats, skin infections, and dental problems quickly, especially on the same side as the malformation. This reduces sudden painful swelling and abscess formation.
Avoiding smoking exposure – For older patients and parents, avoiding smoking and second-hand smoke protects airway health, which is vital when neck structures are already under pressure.
Protecting the lesion from injury – Use soft protective padding in contact sports if allowed by the doctor, and avoid rough play that might strike the neck or face. Less trauma means fewer bleeding or swelling episodes.
Healthy body weight and fitness – Keeping weight in a normal range and staying active reduces strain on the airway and heart, helping the body cope better with treatments and anesthesia.
Sun and heat protection when on sirolimus or similar drugs – These medicines can increase sensitivity to sunlight and skin cancer risk, so hats, sunscreen, and shade are important.
Vaccination according to guidelines – Children and adults taking mTOR inhibitors or steroids should be fully vaccinated to reduce infection risk, following specialist advice about live vaccines.
Good mouth care and dental checks – Regular brushing, flossing, and dentist visits prevent dental infections that could spread to oral lesions and worsen swelling.
Education and emergency planning – Families, schools, and caregivers who understand warning signs can act quickly, which prevents minor problems from becoming major emergencies.
When to see doctors
You should see a doctor or return to the vascular anomalies clinic immediately if the face or neck swelling suddenly increases, becomes very firm and painful, turns very red or hot, or if there is fever, difficulty breathing, noisy breathing, trouble swallowing, drooling, or confusion. These can be signs of infection, bleeding inside the lesion, or airway obstruction that may need urgent hospital care.
You should also book a routine appointment if you notice small but steady growth over weeks, new problems with speech or chewing, changes in voice, sleep problems like loud snoring or pauses in breathing, or emotional difficulties such as anxiety or bullying at school. The team can adjust the treatment plan, consider sirolimus or new sclerotherapy sessions, or offer psychological support.
What to eat and what to avoid
Eat soft, easy-to-chew foods if swallowing is hard – Soft noodles, mashed potatoes, yogurt, smoothies, and soups reduce chewing effort and lower the risk of choking when tongue or throat are involved.
Choose protein-rich foods – Eggs, fish, lean meat, lentils, and dairy help repair tissues and support immune function, which is important after surgery or sclerotherapy.
Eat plenty of fruits and vegetables – These give vitamins, minerals, and antioxidants that support healing and general health. Soft fruits and cooked vegetables are easier to eat when mouth movement is limited.
Drink enough water – Good hydration keeps mucus thin and helps healing. It also reduces constipation, which can be a side effect of some pain medicines.
Avoid very hard, crunchy, or sharp foods – Hard chips, nuts, or crusty bread can scrape fragile mucosa or skin near oral malformations and increase bleeding or pain.
Limit very salty, spicy, or acidic foods if they cause pain – Hot spices, vinegar, or citrus can irritate mouth lesions and make eating uncomfortable, leading to poor intake.
Avoid sugary snacks and drinks – Lots of sugar raises the risk of tooth decay, especially when brushing is difficult because of pain or trismus (limited mouth opening).
Use medical nutrition drinks when weight is low – Dietitians may suggest specialized drinks to maintain weight and strength while safe swallowing is being improved.
Limit caffeine and energy drinks in older patients – These can worsen sleep and anxiety, which are already common in chronic illness, and may interact with some medicines.
Avoid alcohol and recreational drugs in adolescents and adults – These can impair judgment, increase injury risk to the lesion, and interact dangerously with immunosuppressant drugs like sirolimus.
Frequently asked questions (FAQs)
1. Is a lymphatic malformation a cancer?
No. Lymphatic malformations are benign, meaning they are not cancer. However, they can still cause serious problems such as breathing difficulty, infection, or disfigurement, so they must be monitored and sometimes treated actively.
2. Can a lymphatic malformation of the face and neck go away by itself?
Small macrocystic lesions sometimes shrink or become less obvious over time, especially after infections settle, but complete disappearance is uncommon. Many lesions remain stable or slowly enlarge, so doctors often combine watchful waiting with the option of sclerotherapy or surgery when needed.
3. What is the difference between macrocystic and microcystic malformations?
Macrocystic lesions have large fluid-filled spaces that are easier to drain and scar with sclerotherapy, while microcystic lesions have many tiny channels spread through tissues, which are harder to treat completely. Mixed lesions have both. This classification helps doctors choose the best treatment plan.
4. Is sirolimus safe for children?
Studies in children with complex vascular anomalies show that sirolimus can be effective and reasonably safe when used by experienced teams with close monitoring of blood levels and side effects. Infections, mouth ulcers, high lipids, and delayed wound healing are the main concerns, so regular blood tests and careful dosing are essential.
5. How many sclerotherapy sessions are usually needed?
Many children need several sessions, especially for large or microcystic lesions. The exact number depends on lesion size, type, and response to each injection. Treatment is spaced out to allow healing and to control total drug doses, especially for bleomycin.
6. Will surgery leave a scar on the face or neck?
Yes, surgery almost always leaves some scar, but surgeons plan incisions along natural skin lines and folds to make scars less visible. Over time, scars usually fade, and later cosmetic procedures can further improve appearance if needed.
7. Can my child play sports?
Many children can play non-contact sports safely, but contact sports or those with risk of blows to the face or neck may need to be avoided or modified. The specialist team will give individualized advice based on lesion size and position.
8. Does diet change the size of the malformation?
There is no special diet that cures or directly shrinks lymphatic malformations. However, a balanced diet supports overall health, healing after procedures, and normal growth, which makes treatment and recovery easier.
9. Can lymphatic malformations come back after treatment?
Yes. Even after surgery or multiple sclerotherapy sessions, some lesions can regrow or new cysts can appear, especially microcystic types. Long-term follow-up is therefore important, and newer medicines like sirolimus may help control relapses in some patients.
10. Are there new treatments being studied?
Yes. Research is ongoing on better sclerosing agents, improved imaging-guided techniques, and targeted drugs that act on specific signaling pathways in lymphatic endothelial cells, including new mTOR-related and PI3K pathway inhibitors. Many of these are still in clinical trials and not yet standard care.
11. Can pregnancy affect a lymphatic malformation?
Hormonal changes and increased blood volume during pregnancy can sometimes cause vascular and lymphatic malformations to enlarge or become more symptomatic. Women with known lesions should be followed by a team familiar with vascular anomalies during pregnancy to plan safe monitoring and delivery.
12. Is genetic testing needed?
Some complex vascular and lymphatic malformations are linked to mutations in genes involved in the PI3K–AKT–mTOR pathway or other signaling pathways. In difficult or atypical cases, doctors may suggest genetic testing to guide targeted therapy or to understand recurrence risk.
13. Can my child have normal school and social life?
Many children with well-managed lymphatic malformations lead active, normal lives. Extra support may be needed for appearance-related issues, speaking or swallowing problems, or time off for treatments. Open communication with school staff and classmates can greatly reduce stigma and bullying.
14. How long does sirolimus treatment usually last?
Treatment duration varies widely. Some patients need sirolimus for months; others may stay on it for several years, with dose adjustments based on response and side effects. Decisions to stop are made slowly and carefully by the specialist team, sometimes after imaging shows stable or reduced lesions.
15. What is the long-term outlook (prognosis)?
Most lymphatic malformations of the face and neck are long-term conditions rather than problems that disappear quickly. With modern combined care—sclerotherapy, surgery, systemic medicines like sirolimus, and psychosocial support—many patients achieve good function, acceptable appearance, and a full life. Early referral to experienced centers is key to the best outcomes.
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: January 27, 2025.
