Introduction

Lymphangiomas are malformations of the lymphatic system that are fluid-filled and benign. They arise due to abnormal development of lymphatic vessels. The lymphatic vessels contain fluid called lymph which helps in protecting the body against infections and helps in removing toxins. Lymphangiomas most commonly appear in the neck and head region and are often present at birth or in early childhood.¹ When lymphangiomas appear in the neck region also called the cervical region they are called cervical lymphangiomas. 

Although cervical lymphangiomas are slow-growing and benign, they can become fatal if they cause obstruction of the upper airway, particularly in young children and infants.² Rapid expansion due to internal bleeding or any infection can lead to sudden airway obstruction which requires urgent medical intervention. Airway obstruction is clinically significant in cervical lymphangiomas due to their proximity to the airway structures. 

Causes and risk factors

Most of the time lymphangiomas are congenital which means they are present since birth. They primarily develop due to developmental anomalies in the lymphatic system during embryogenesis.¹ It's common to think about how these lymphangiomas arise, and the answer to this comes from the failure of the lymphatic system to connect effectively with the venous system which then leads to the accumulation of localised fluid and thereby leads to cyst formation. Though these malformations are non-cancerous they can cause trouble by obstructing the surrounding structures. 

Lymphangiomas most commonly occur in young children and infants. According to research about 90% of the cases are diagnosed by the age of two.² The neck region also called the cervical region is the most frequent site, thus an early diagnosis is important for intervention especially in infants that show symptoms of breathing difficulties.

Additionally, it is important to note that although lymphangiomas are slow growing they can be triggered and enlarge rapidly due to secondary triggers like infections, internal haemorrhage, or trauma.³ These may also cause swelling and inflammation which can further cause severe obstruction of the airway. 

Classification and location of lymphangiomas

Lymphangiomas are distinguished based on different sizes of the cyst : macrocystic, microcystic, and mixed. Macrocystic lymphangiomas have cysts bigger than 2 cm, smaller and clustered cysts are microcystic lymphangiomas. Mixed types of lymphangiomas include both the components.⁴ This classification aids in developing a treatment protocol for the treatment. The lymphangiomas are located in different locations; however, most commonly located in the neck and head, particularly in the posterior triangle of the neck. Studies show approximately 75% of cases occur in the neck region, which makes this may crucial to monitor airway safety in infants facing lymphangiomas.⁵

Symptoms

The symptoms of lymphangiomas may be present at birth or may develop in the later stage of growth. Discomfort in breathing can be the first sign of cervical lymphangiomas. As cervical lymphangiomas can cause complete or partial obstruction of the airway this may lead to discomfort during respiration. Common symptoms include noisy breathing, stridor which means breathing with a high pitch sound and in severe cases cyanosis that is bluish discoloration of the skin that is caused due to reduced oxygen supply. Apart from these signs cervical lymphangiomas show visible symptoms which include visible swelling in the neck, soft lump or cyst under the skin. Usually these lumps are painless; however, they can become tender or painful due to infection or any kind of trauma.¹ Moreover, these cysts can also compress the vocal cords which may lead to a change in the tone of the voice. 

Diagnostic approach

The diagnosis of cervical lymphangiomas includes a thorough subjective assessment, which includes the current medical history, past medical history, family and surgical history, followed by clinical examination, where the individual is assessed for visible swelling, feeding difficulties, and respiratory symptoms.⁶ Due to its effectiveness in identifying cysts and safety ultrasound is considered as the first line of imaging tool. To identify the further extent of the lesion, the possible airway obstruction, and its effect on the surrounding structures a CT scan or MRI may be advised. 

Airway management

Airway management is a critical step in treating individuals with cervical lymphangiomas, particularly when the lesion causes upper airway obstruction. In emergencies, where there is complete obstruction of breathing, endotracheal intubation or tracheostomy may be performed to ensure effective ventilation. 

Treatment options

Observation for stable, small lesions

When the lesions are small, stable and regressing then observation and monitoring of the condition is recommended. It is important to note that this is advised only when the lesion is not obstructing any surrounding vital structure or causing any secondary implications. Constant monitoring and regular imaging and clinical assessment is essential to detect any signs of progression or complications.³

Surgical excision for larger or symptomatic cases

In case of larger lymphangiomas and when they cause significant symptoms like airway obstruction, feeding difficulties, vocal issues, or cosmetic disfigurement total excision can cure the disease, but it can be difficult if the lesion has spread across important blood vessels or nerves.⁷ 

Sclerotherapy for cystic lesions

Another effective treatment tool utilised for the cystic lesion is sclerotherapy.This involves injecting a sclerosing agent into the cysts to induce shrinkage. This type of treatment is particularly useful in macrocystic lymphangiomas, and is often preferred over others due to its ability to preserve surrounding structure and low complications. The only drawback of this therapy is it may require multiple sessions to completely cure and heal the affected part.

Combined therapy when necessary

In many complex cases a combined treatment protocol is advised which includes a combination of sclerotherapy and surgery. This option yields better results as it reduces the complications of a surgery. For instance, before surgery sclerotherapy can be used to reduce the size of the lesion which will thereby make excision safer and easier.⁸ This is optimally used in complex cases or in conditions in which the lymphangiomas are especially located in an anatomical position where they are difficult to access. 

Medicines used for cervical lymphangiomas

In recent times, cervical lymphangiomas are treated by medicinal management where surgery is not an option. Sirolimus is a drug used that reduces inflammation and growth of the lymphatic vessel. But it is important to note that medicinal therapy is opted for complex cases or where other interventions are not possible and the case should be closely monitored to avoid further complications. 

Prognosis and Follow-Up

Often cervical lymphangiomas have a favourable prognosis rate, especially if they are detected early and treated promptly. Whether medicinal, surgical or sclero therapeutic timely intervention can help in achieving long-term management goals and cosmetic results. Moreover, it can significantly reduce the complication of airway blockage and thereby prevent breathing difficulties. Nevertheless, recurrence continues to be a risk, especially for micro lymphangiomas as the chances of them infiltrating the neighboring structures is high.⁹ Thus, these types of cervical lymphangiomas often require a combination of treatment options and also routine follow-up and monitoring. In all cases, long-term surveillance using ultrasound or MRI is important as it allows continuous assessment of structural development and airway in developing children and aids in early diagnosis of recurrence of the disease. 

Summary

In summary, efficient care of cervical lymphangiomas, particularly when there is potential airway obstruction as this can be fatal and may require immediate intervention. As airway obstruction can be life-threatening airway stabilisation and examination should be the first priority. Advanced treatment options like sclerotherapy and surgery have provided enhanced clinical outcomes. Individuals should seek doctor’s advice as soon as they notice signs and symptoms of lymphangiomas.