The birth of a baby is something that parents eagerly look forward to with great joy and anticipation. Their tiny finger curl, their first cry, the suckling of the breast and the debate on which parent the baby took after in looks and other features.

However, in some cases, the parents are met with the burden of a sick child battling with the possible chances to make their baby more healthy.

This burden is usually associated with an unusual, soft swelling on the neck, face, or chest of the baby, and this is caused by a condition called lymphangioma.

This condition is not so common, but its manifestation in children results in serious health issues and worsens when the growth (swelling) increases rapidly or obstructs the baby’s airway.

This article explains the incidence of lymphangioma, the aetiology, the challenges associated and the treatment options, especially in emergencies.

What is lymphangioma?

Lymphangioma is an uncommon benign malformation of the lymphatic system occurring on any part of the skin or mucous membrane.¹ This condition is classified into deep or superficial lymphangioma depending on the depth and size of the abnormal lymphatic vessels, as well as whether it is congenital or acquired.¹

Superficial lymphangioma consists of the lymphangioma circumscriptum and lymphangiectasia, while the deep lymphangioma consists of two congenital entities: the cavernous lymphangioma and the cystic hygroma.²

Lymphangioma circumscriptum is a type of lymphangioma where the lymphatic channel is dilated from birth due to malformation.³ While lymphangiectasia simply means acquired lymphangioma, this occurs when a healthy lymphatic channel gets altered due to obstruction or secondary issues.³

The cystic hygroma is a translucent swelling usually found in the neck, armpit and groin. They are usually large and can result in complications if they obstruct vital organs.  Cavernous lymphangioma is usually painless. They are bigger and can be localised in any part of the body, like the tongue. It is soft and spongy in texture, and the colour is usually red or blue.  

This lymphatic malformation is noncancerous. The lymphatic system is responsible for holding fluids and preventing infections. In babies with lymphangioma, the lymphatic vessels accumulate fluid. This fluid collection causes swelling or lumps in the affected area, which become visible and palpable. It occurs on different parts of the body but commonly manifests around the head 

and neck, and in some situations, the chest, armpit or the mouth and tongue. Depending on the amount of fluid accumulation, the swellings may vary in size, leading to several complications from compressing important organs like the windpipe or foodpipe in babies, resulting in compromised airways and breathing difficulties, to infections, which further increase the size of the swelling, causing pain and redness.

The incidence rate of lymphangioma is about 0.8 to 18 per 10,000 fetuses. This condition is rare in the United States. However, it represents 4% of all vascular tumours and 25% of all benign pediatric vascular tumours with no gender preference.⁴ This happens at birth or in the first few years of life, with the acquired version manifesting at adulthood.

What causes lymphangioma in newborns?

Lymphangioma in newborns occurs in the second and third trimesters of embryonic development, resulting from an isolation of the original lymphatic sac from the lymphatic system due to impaired lymphatic and venous flow, abnormal hyperplasia of the lymphatic epithelium or lymphatic obstruction.⁵ The sizes of lymphangioma varies in sizes between a few centimetres to greater than 10cm in diameter.⁶
Most occurrences of lymphangiomas are usually congenital. Though the main cause is unknown, it is linked to genetic conditions like Turner, Down, and Noonan syndrome.
While it is common knowledge that some behavioural patterns and changes that happen during pregnancy affect the baby, it is important to know that they have no relationship to occurrences that happen during pregnancy, diet, medications, stress or infections.

Though recent studies showed that monitoring the fetuses can help determine lesions, best delivery and treatment plans, lymphangioma cannot be predicted.

How to recognise lymphangioma in a baby

Lymphangiomas are usually asymptomatic until they affect a vital organ and impede breathing or eating in the baby. They vary in size and can pass unnoticed, while in some situations they are large and can vary in size. Some of the signs include:  

• A tender, squishy lump under the skin
• The lump is either blue or translucent in colour on its surface
• The lump gets larger over time
• A painless swelling that is usually uncomfortable

It is important to note that in severe cases, this swelling can interfere with the baby’s ability to eat or breathe, especially when the growth is around the throat or the mouth. It results in symptoms like:

• Fast or noisy breathing
• Trouble breastfeeding or bottle feeding
• Coughing, gagging, or choking
• Signs of distress when lying down

The occurrence of any of these symptoms calls for a medical emergency.

Challenges in diagnosing and managing lymphangioma

The most challenging situation when diagnosing lymphangioma is the differentials, which include: common swelling in newborns (birth trauma, bruises or benign cyst). This makes diagnosis difficult without proper medical imaging. Another challenge is how unpredictable the swelling can be, maintaining the same size for months and then getting bigger unexpectedly due to infection, bleeding or pressure. There are various scans to be done to enable an accurate diagnosis. These scans are as follows:

An ultrasound Scan: This enables practitioners to estimate the exact size and texture of the growth.

An MRI or CT scan helps to estimate how deep it goes and what organs are affected.

Prenatal ultrasound: This is a routine pregnancy scan that helps detect the disease condition before the baby is born.
Lymphangioma is associated with emotional challenges, especially from the parents. Having a newborn with a large lump can be frightening and exhausting when complicated, and can result in feeding or breathing problems.

When lymphangioma becomes an emergency

Lymphangioma often does not lead to life-threatening scenarios unless it is impeding breathing or eating. There are emergency signs to watch out for in babies, and they include the following: Difficulty breathing or noisy breathing, rapid swelling, high fever (indicative of an infection inside the mass), redness, warmth, or pus from the area, bleeding or bruising in or around the lump.

These symptoms pose a threat to newborns, who have limited breathing capacity and rely on feeding for survival. In cases like this, it is advised to rush the baby to the neonatal intensive care unit (NICU) for immediate intervention.

There are various emergency treatment options, and the goal of emergency care is to keep the baby as stable as possible and enable breathing by relieving pressure on the airway, as the case may be. The treatment choice is often dependent on the size, location, urgency and baby’s response to the condition.

Treatment may include:

1. Airway Support

If the baby is struggling to breathe, doctors may use oxygen or insert a breathing tube to ensure air gets to the lungs.

• Steroid Medication

This helps to reduce swelling and inflammation quickly.

• Antibiotics

This is used when an infection is noticed inside the lymphangioma.

• Sclerotherapy

This procedure involves injecting a sclerosing agent into the affected area. It enables obliteration of the vessels, reducing the flow of lymph fluid in the affected area as well as reducing the size of the swelling. This procedure is minimally invasive, effective with minimal side effects.

• Surgical Intervention

In severe cases, when non-surgical options have been exhausted, surgery is done to remove part or all of the growth when it compromises vital organs.

What happens after emergency care?

It is important to follow up with appointments. This is because there is a chance of lymphangioma recurring, especially if it was not entirely removed. The baby still needs repeated imaging to monitor regrowth, speech and feeding therapy if those functions were affected, emotional support and counselling for parents and guardians.

Prognosis: Most babies recover well

The prognosis is usually good, especially with early diagnosis and immediate intervention. In some cases, most children do not require surgery and respond well to treatments, as well as live healthy and happy lives.  Parents are advised to report any lump, swelling, change in colour or breathing and eating difficulties to the nearest hospital.

Tips for parents and caregivers

• Don’t panic. Lymphangiomas are not cancerous
• Seek medical interventions if any of the above symptoms manifest
• Strict adherence to follow-up appointments and medications
• Be open to seeking emotional support or counselling. Connect with parents in similar situations or who have been in similar situations, that way you don’t feel alone

Summary

Lymphangioma in newborns can be exhausting, but the trick is early diagnosis and intervention. Parents and guardians should always be on the lookout for anything unusual and report it to the nearest health facilities. Children with lymphangioma can live healthily and happily, just like any other normal kid. The prognosis is usually very good, even though it is important to keep up with follow-up appointments