Lymphangioma: Symptoms, Types, Causes and Treatment

Lymphangioma is a rare and fascinating condition involving the lymphatic system, most often presenting in childhood but also occurring in adults. It can affect various parts of the body, causing a range of symptoms depending on its location and size. Understanding the intricacies of lymphangioma—its symptoms, different forms, underlying causes, and available treatments—is crucial for patients, families, and healthcare providers alike. This article presents a comprehensive, evidence-based overview, synthesizing recent research to offer a clear and practical guide.

Symptoms of Lymphangioma

Lymphangioma symptoms can be subtle or dramatic, depending on the lesion's size, depth, and location. Many cases are discovered in children, but adults can also be affected, sometimes with different symptom profiles. Early recognition of these symptoms is key to prompt diagnosis and management.

Symptom	Prevalence/Occurrence	Notes/Location	Source(s)
Swelling	Common	Head, neck, skin, extremities	3 4 7 10
Pain	Variable; common in adults	Abdomen, extremities, deep lesions	1 5 7
Palpable mass	Frequent	Abdomen, trunk, neck, subcutaneous	1 2 7 10
Bleeding	Occasional	Small bowel, skin, orbital lesions	1 3 5
Disfigurement	Common in visible areas	Face, neck, skin	4 5 7 10
Obstruction	If large or near airway/GI tract	Head/neck (airway), bowel, mediastinum	1 4 6 7
Recurrence	Not a symptom, but common outcome	Especially after incomplete excision	2 10

Table 1: Key Symptoms

Swelling and Palpable Masses

Swelling is the most prominent symptom, especially when lymphangioma is located in superficial tissues like the skin, head, or neck. In these regions, lymphangioma often appears as a soft, compressible mass that may gradually enlarge. When deeper tissues are involved, as in the abdomen or thorax, the swelling may not be visible but can manifest as a palpable mass or cause pressure-related symptoms 1 4 7 10.

Pain and Discomfort

Pain is variable and more commonly reported in adults, particularly with abdominal or deep-seated lesions. In children, pain may occur when the lesion becomes infected, bleeds, or grows rapidly 1 5 7.

Bleeding and Infection

Some forms, especially those involving mucous membranes or the gastrointestinal tract, can bleed. In rare instances, bleeding may be significant, as seen in small bowel lymphangiomas or orbital lesions 1 3 5. Infection within the lymphangioma can lead to redness, tenderness, and systemic symptoms like fever.

Disfigurement and Functional Impairment

Large lymphangiomas, particularly in the head and neck, can cause significant facial or neck disfigurement, impacting a child's psychosocial development or causing functional impairment (e.g., airway compression) 4 7 10.

Obstructive Symptoms

Obstruction depends on the lesion's location. For example, lymphangiomas in the neck may compress the airway, causing breathing difficulties. Lesions in the abdomen or mediastinum can cause gastrointestinal or respiratory symptoms 1 4 6 7.

Recurrence

Even after treatment, recurrence is a notable issue, particularly when lesions cannot be completely removed or are treated with aspiration or sclerotherapy alone 2 10. Recurrence is not a symptom per se, but impacts the long-term outcomes and quality of life.

Types of Lymphangioma

Lymphangiomas are classified based on their histological and clinical features. Each type presents unique challenges and implications for diagnosis and treatment.

Type	Main Features	Common Locations	Source(s)
Simplex (Capillary)	Thin-walled, capillary-sized channels	Skin, subcutaneous	2 3 6
Cystic (Hygroma)	Large, cystic spaces (macrocystic)	Neck, axilla, head	3 4 6 7
Cavernous	Dilated, sponge-like lymphatic channels	Thorax, abdomen, bones	1 5 6
Mixed	Combination of cystic and cavernous	Varies	6 11
Special forms	Lymphangioma circumscriptum, polyposis	Skin, bowel	1 2 3

Table 2: Classification of Lymphangioma Types

Simplex (Capillary) Lymphangioma

These are made up of small, capillary-sized lymphatic channels. They most often affect the skin and subcutaneous tissue, manifesting as small vesicle-like lesions (sometimes described as "frog spawn") 2 3 6. Lymphangioma circumscriptum is the classic example, often congenital and present at birth.

Cystic Lymphangioma (Cystic Hygroma)

Cystic lymphangiomas, or cystic hygromas, are characterized by large, fluid-filled spaces. They are most commonly seen in the neck and axilla of infants and young children, but can also occur in the mediastinum, face, or oral cavity. These lesions are often macrocystic and can become very large 3 4 6 7.

Cavernous Lymphangioma

This type features dilated lymphatic channels that form sponge-like or cavernous spaces. Cavernous lymphangiomas are more likely to involve deeper tissues, such as the thorax, abdomen, and bones. They can cause significant symptoms due to their size and location 1 5 6.

Mixed and Special Forms

Mixed lymphangiomas display both cystic and cavernous features, and may occur anywhere. Unique forms include lymphangioma circumscriptum (localized to skin) and polyposis-type lymphangioma (seen in the bowel) 1 2 6 11.

Causes of Lymphangioma

Understanding what leads to lymphangioma formation is crucial for both prevention and management. While the exact causes remain elusive, several key factors have been identified.

Cause/Factor	Description	Evidence/Examples	Source(s)
Congenital Malformation	Developmental defect in lymphatic system	Most common cause	3 6 7 12
Failure of Vessel Connections	Lymph sacs not connecting to lymphatic/venous	Embryological error	7 12
Localized Lymphatic Obstruction	Lymph flow blocked in a specific area	Less common	7
Radiation/Acquired	Rare, post-radiation or trauma	Documented cases	6
Unknown	No clear cause in some patients	Idiopathic cases	6 12

Table 3: Proposed Causes of Lymphangioma

Congenital Malformation

The overwhelming majority of lymphangiomas are congenital malformations, meaning they arise due to abnormal development of the lymphatic system during embryogenesis. This may involve the "pinching off" of lymph sacs that fail to connect with the main lymphatic or venous channels 3 6 7 12. As a result, lymphatic fluid collects in these isolated sacs, forming cystic or cavernous structures.

Failure of Vessel Connections

Developmental errors where the lymph sacs either do not connect properly to the main lymphatic or venous system, or become isolated, are central to the formation of these lesions. These errors can occur in utero and present at birth or in early childhood 7 12.

Localized Lymphatic Obstruction

In rare cases, localized obstruction of lymphatic flow (due to trauma, infection, or inflammation) may lead to lymphangioma formation. However, this mechanism is less common compared to congenital origins 7.

Acquired and Unknown Causes

Acquired forms are very rare but have been observed following radiation therapy or trauma, particularly in adults 6. In a minority of cases, the exact cause cannot be determined, and these are labeled as idiopathic.

Treatment of Lymphangioma

The management of lymphangioma has evolved considerably, with both surgical and non-surgical options available. The choice of treatment depends on lesion type, location, size, symptoms, and patient age.

Treatment	Indications/Effectiveness	Recurrence/Side Effects	Source(s)
Surgical Excision	Symptomatic, accessible lesions	Lowest recurrence if complete	2 4 7 10
Sclerotherapy (OK-432)	Macrocystic lesions, children	Effective, mild side effects	4 8 9 11
Observation	Small, asymptomatic lesions	Some may regress; monitor	7 10
Aspiration	Temporary relief (rarely curative)	High recurrence	10
Laser/Other Modalities	Selected cases	Varies	10 12

Table 4: Main Treatment Approaches

Surgical Excision

Surgery remains the gold standard for accessible and symptomatic lymphangiomas, particularly when complete removal is possible. Complete excision offers the lowest risk of recurrence, but this isn't always feasible—especially in complex, infiltrative, or head and neck lesions where vital structures are involved 2 4 7 10. Incomplete excision carries a higher recurrence risk.

Sclerotherapy (OK-432)

Sclerotherapy has transformed the treatment of certain lymphangiomas, especially macrocystic types. The most widely studied agent is OK-432 (Picibanil), a lyophilized preparation from a strain of Streptococcus pyogenes. OK-432 induces an inflammatory reaction, resulting in the shrinkage of cystic spaces 4 8 9 11. Studies confirm high efficacy and low complication rates, particularly for macrocystic lesions. Side effects are generally mild, limited to local inflammation and transient fever 8 9 11.

Observation

For small, asymptomatic lymphangiomas, a period of watchful waiting may be suitable, since some lesions (especially recurrent ones) can regress spontaneously 7 10. However, spontaneous regression is not common, so careful monitoring is needed.

Aspiration and Other Treatments

Simple aspiration provides only temporary relief and is associated with high recurrence rates. Laser excision and other modalities may be considered in selected cases, but recurrence rates vary and depend on the lesion's characteristics 10 12.

Multidisciplinary Approach

Given the complexity of many lymphangiomas, especially those involving the head, neck, or vital organs, a multidisciplinary team—including surgeons, radiologists, and pediatricians—is often required for optimal management 7 12.

Conclusion

Lymphangioma is a rare but significant condition with variable symptoms, types, and outcomes. Prompt recognition and tailored treatment can prevent complications and improve quality of life. Here's a concise summary:

• Symptoms: Swelling, pain, disfigurement, bleeding, and obstruction, varying by location and patient age 1 3 4 5 7 10.
• Types: Classified as simplex (capillary), cystic (hygroma), cavernous, and mixed, each with distinct features and locations 2 3 4 5 6 7.
• Causes: Primarily congenital malformations; rarely acquired or idiopathic 3 6 7 12.
• Treatment: Surgical excision (best for complete removal), sclerotherapy (OK-432 for macrocystic lesions), observation (selected cases), and a multidisciplinary approach for complex situations 2 4 7 8 9 10 11 12.

Awareness and multidisciplinary collaboration are essential for ensuring the best outcomes for patients with lymphangioma.