Last updated date: 16-Jun-2023
Originally Written in English
Lymphangiomas are uncommon, accounting for just 4% of all vascular tumors (tumors formed from blood or lymph artery cells) and 25% of all noncancerous vascular tumors in children in the United States.
A lymphangioma is a rare noncancerous fluid-filled cyst beneath the skin produced by enlarged lymph veins. Lymph veins (or channels) carry lymphatic fluid and white blood cells throughout your tissues and circulation. If your child has a blockage in their lymph vessels, fluid gathers and forms cysts.
The majority of lymphangiomas arise shortly after your child is delivered. They most commonly appear on the head and neck, although they can appear anywhere on the body.
A clinical diagnosis may usually be obtained based on the history and examination results. Dermoscopy and biopsy can be done to confirm the diagnosis if needed, and imaging may be required to determine the depth and breadth of a lesion.
Destructive treatments with carbon dioxide (CO2) laser, long-pulsed Nd-YAG laser, and electrosurgery have been reported to improve symptoms. Cryotherapy, superficial radiotherapy, and sclerotherapy with 23.4% hypertonic saline are less commonly used modalities. Direct injection of a sclerosing agent, including 1% or 3% sodium tetradecyl sulfate, doxycycline or ethanol, can be made into lymphatic malformations. Compression may reduce swelling caused by lymphedema. Infection prevention is crucial.
What are the Symptoms of Lymphangioma?
Symptoms of lymphangioma are unique to each person diagnosed with the condition and vary based on the size (depth) and location of the cyst including:
- Cystic hygroma (cystic lymphangioma): A red to blue swollen, fluid-filled mass often found on the neck, groin or armpit.
- Cavernous lymphangioma: A red to blue swollen, rubbery mass often found on the tongue but can form anywhere on the body.
- Lymphangioma circumscriptum: A small group of clear to pink to red to brown or black pimple-sized, fluid-filled blisters found on the mouth, shoulders, neck, arms and legs.
All cysts, regardless of size, contain fluid. Clear fluid will spill out if an injury ruptures a cyst.
Lymphangiomas do not usually cause discomfort and are not irritating. These cysts are virtually invariably benign (noncancerous) and seldom dangerous. Life-threatening situations are determined by the size and position of the cyst, particularly if the cyst obstructs a crucial organ such as your child's eyes, mouth, or lungs and stops them from utilizing their body as they should.
What Causes Lymphangioma?
Lymphangiomas have no known cause, but they form when your child's lymphatic system fails to mature properly during prenatal development.
Lymphatic fluid, which includes white blood cells, is transported across your circulation and tissues by a network of veins, tissues, and organs in your body. The lymphatic system controls the quantity of fluid in your body, allowing your immune system to function properly. Your lymphatic system is always moving, much like a hose conveying water.
The fluid in the lymph vessel can sometimes back up, comparable to a kink in a hose. This kink causes a pool of lymph fluid to form in front of the obstruction. As a result, the pool of fluid appears on the skin as a liquid-filled bump (cyst).
How Long Does a Lymphangioma Last?
Lymphangiomas have no treatment and can grow more visible with age. Lymphangiomas seldom go away on their own, therefore your kid may require treatment if the cyst is big or placed in a portion of their body that is dangerous to their health. Small lymphangiomas do not require treatment, and the cyst is part of what differentiates your child.
Preparations Before the Surgery According to Age
The specialists will explain the surgical process to your kid and give emotional and educational support throughout your child's hospital stay, including pre-surgery tours, medical preparation, and support during a procedure. Doctors will handle the details of getting your kid ready for surgery so that you can focus on your child.
Children frequently catch up on their parents' emotional cues. Your child will be more relaxed if you are calm and confident. Here are some suggestions to help you talk to your child about surgery and make their experience less stressful:
- Infants & Toddlers (ages 0-2): Toddlers have a different time perception than adults. As a result, we recommend informing your child about surgery only a few days before it occurs. We also suggest having a pacifier, favorite blanket, doll, or stuffed animal to make your kid feel more comfortable.
- Preschoolers (ages 3-5): Preschoolers are old enough to be curious about their environment and what's going on, so telling your child about surgery a few days to a week in advance might be helpful. During your conversation, use words like "repair" or "improve" and address any questions your child may have.
- School-age (ages 6-10): If your child is in grade school, it can be helpful to tell them about the surgery at least a week in advance and explain what will happen the day of. You can tell your child about the pediatric specialists who will make sure they will stay asleep during the surgery and help manage pain after. Let your child know the nurses and doctors want to answer any questions they have during pre-surgery visits.
- Teens: Talk with and include your teen in the decision-making process from the beginning. It’s important for kids this age to maintain their independence and sense of control. We encourage them to ask their surgical team any questions they may have during the process.
What to Do Before Surgery?
We’ll help you take care of the details necessary to prepare for your child’s procedure and make it as quick and easy as possible.
Registration: To save time on the day of surgery, register your kid online ahead of time and give the relevant insurance and contact information. Please register your child as soon as possible, preferably at least two days before the operation. Also, make sure to check with your insurance company to see if your child's operation is covered.
Pre-Admission Interview: A nurse will contact you a few days before your kid's operation to address any questions, go over your child's medical history, and inquire about any medications your child is currently taking. Please notify the nurse if your kid has a cold, cough, fever, pink eye (conjunctivitis), chickenpox, measles, COVID-19, or has recently been exposed to chickenpox, measles, or COVID-19 in order to protect other children from extremely infectious diseases. The nurse will also need to be aware of any guardianship arrangements, such as foster care or custody concerns, in order for the relevant legal guardian to sign the permission form.
Pre-Surgery Tests: Before surgery, your child's pediatrician may request blood tests or x-rays. Tests must be performed 72 hours before surgery, so plan ahead of time and finish them on time. If the test is performed by a primary care physician rather than your pediatric surgeon, please request that a copy of the results be given to the surgeon or bring one with you on the day of surgery.
Pre-Surgery Checklist: Use this checklist to help make your child’s surgery day less hectic and make it easier for you to focus on your child.
One Day Before the Surgery
- Complete the pre-surgery admission interview. If a nurse hasn’t called you within 48 hours of your child’s surgery, call the hospital.
- If your child takes medications, talk to your pediatric surgeon about how they should be taken before surgery.
- Arrange childcare for other children while you’re at the hospital or surgery center.
- Check with your insurance provider if you have questions about your copayment or deductible.
- Call your pediatric surgeon if your child has symptoms of a cold, the flu, a rash or any other illness or infection.
- Your child shouldn’t bring jewelry or use make-up, nail polish or deodorant the day of surgery.
Your child’s pediatric surgeon will also give you guidelines for what your child can eat or drink before surgery. Generally, your child should not have:
- Food 8 hours before.
- Full liquids (soups, smoothies and juices) and milk 6 hours before.
- Breast milk (infants) 4 hours before.
- Clear liquids 2 hours before.
Complete surgical excision is the primary therapy for lymphangiomas. The Whimster theory suggests that the big subcutaneous cisterns be eliminated to prevent the lesion from returning.
Lymphangiomas frequently recur locally. Adequate lymphangioma excision can be difficult and, at times, impossible. This is the primary cause of the high recurrence rate. Tumors restricted to the superficial dermis are more susceptible to surgical removal, which has a high success rate.
Other therapeutic techniques, such as cryotherapy, sclerotherapy, cautery, and radiofrequency therapy, have been promoted. For head and neck lymphangiomas, bleomycin sclerotherapy has been proposed.
Simple electrodessication can be used to treat Lymphangioma circumscriptum. It does not always responsive to pulsed-dye laser usage.
Vaporization using a carbon dioxide laser has been tested and found to be effective. Other lasers may be used as well. A fractional carbon dioxide laser can be used to treat intra-oral lymphangioma.
Sclerotherapy with 23.4% hypertonic saline is another treatment option for lymphangioma circumscriptum.
The use of intralesional OK432 (Picibanil) is a new and effective treatment for macrocystic lesions, but the response of microcystic or cavernous lesions to OK-432 has been disappointing and surgery remains the most effective treatment for these microcystic and cavernous lesions.
Postoperative vacuum-assisted closure devices may decrease the risks of recurrence and infection.
What Happens After?
After recovering from the anesthesia, your kid will be admitted to the ward. Following a general anesthesia, some children become ill and vomit. Your kid may get a headache, sore throat, or dizziness, although these side effects are generally brief and minor. When your kid is ready, they can resume typical eating and drinking habits.
Because bruising and swelling last for a few days following the treatment, the lymphatic malformation will almost probably seem worse. To prepare your child, show them some 'before and after' images, such as those in this information leaflet. The treated region will feel painful afterward, but children's pain relief medication is generally sufficient to alleviate any discomfort.
You will be allowed to return home if the doctors have determined that your kid is recovering properly.
Over the first week or two, the drug injected into the lymphatic malformation may cause swelling. The affected region may also be red, bruised, and tender. These side effects are typical and indicate that the medication is working. Most children require just one or two doses of children's pain medication, such as paracetamol or ibuprofen. Any swelling should begin to subside within a week, but the treatment's benefits, such as a reduction in discomfort or size, may not be apparent for several months.
Lymphatic abnormalities are also prone to the production of blood clots within the cysts. Your child's lymphatic malformation may have previously displayed symptoms such as rapid swelling and bruising. The therapy of the lymphatic malformation may result in another bleed in the week after the procedure. If this happens, give your kid one or two doses of children's pain reliever. If the hemorrhage or clot resolves without treatment, it will have no effect on how well the sclerotherapy works.
If the clot remains large and hard for more than two weeks, the treatment may need to be repeated.
Following therapy for the lymphatic malformation, an infection may occur. An infection, like an abscess, causes the region to become bloated, heated, and painful. Your child will have a fever and will be generally ill. If this happens, your kid may require a brief course of oral antibiotics prescribed by your family doctor (GP).
You should call the hospital if:
- Your child is in a lot of pain and pain relief does not seem to help.
- The treated area is unusually hot, red and painful and your child is general unwell with a high temperature or not eating or drinking as usual.
If you have any questions, please telephone the ward from which your child was discharged or one of the numbers below.
The physicians will tell you if and when your kid need more sclerotherapy operations. Before making this decision, your kid may need to have a standard outpatient checkup a few weeks later. You will be sent a letter confirming the outpatient appointment or next treatment session date.
To decrease the deformity, your kid may require many sclerotherapy operations. Because sclerotherapy produces swelling and discomfort, it is preferable to do a series of smaller treatments that reduce the consequences each time. Sclerotherapy will not 'cure' the deformity, but it will greatly decrease it. As children get older, they may require more sclerotherapy, and some may require surgery to remove any loose skin.
Are there Any Complications?
Because it is difficult to detect and remove all affected lymphatic cells that caused the cyst to form, there is a significant possibility that the cyst may return following therapy. Cysts near the skin's surface have a lesser likelihood of recurring since most cells are easily removed. Other cysts are huge and deep beneath the tissue of the skin. These cysts are difficult to remove and frequently reappear following therapy.
Following surgery, there is a considerable chance of infection, which might impair the healing process. Taking precautions to avoid infection by cleaning and caring for the surgery site yields the greatest results.
Lymphangiomas are noncancerous fluid-filled cysts that commonly occur on the head and neck of children. When lymph fluid accumulates and does not move correctly across tissues, these cysts develop. Most lymphangiomas do not require treatment, however if necessary, your physician can remove lymphangiomas surgically. Cysts return at a significant incidence following surgery.
If your child's symptoms are mild or non-existent, his or her doctor may advise against treatment. If the lymphangioma is big, painful, or affecting your child's well-being in any way, your doctor will most likely recommend one of many treatments. The kind, size, and location of the lymphangioma will all influence treatment.
Treatment may include:
- Surgery: This involves cutting out the cyst, but it can be difficult if the mass is close to organs and nerves.
- Sclerotherapy: This therapy involves injecting a chemical directly into the mass to shrink and collapse it, and it’s becoming more commonly used. Sclerotherapy has about the same success rate as surgery in treating lymphangiomas, but with fewer complications.
- Laser therapy or radiofrequency ablation: A laser or needle delivers a current to destroy the mass.
Because it is practically hard to remove or destroy every lymphangioma cell, they tend to regrow. Multiple procedures or therapies may be required to completely control this problem over time.
Recurrence rates differ depending on the nature and location of the lymphangioma, as well as whether it existed in one or numerous locations across the body.