capillary lymphangioma

Capillary Lymphangioma: A Rare Cystic Lymphatic Malformation

A capillary lymphangioma, also known as a microcystic lymphangioma, is a rare cystic lymphatic malformation characterized by benign cystic lesions composed of dilated lymphatic channels. These microcystic lesions are typically smaller than 1 cm in diameter and can manifest at birth or in early childhood, primarily in the head and neck region but can occur elsewhere [1].

Classification

Lymphangiomas are classified into three types based on the size of the lymphatic cavities incorporated: microcystic (capillary lymphangiomas), macrocystic (cavernous lymphangiomas), and cystic hygromas. The classification was first proposed by Bill and Summer in 1965, who presented the concept that cystic hygromas and lymphangiomas are variations of a single entity [2].

Types

There are several types of capillary lymphangioma, including:

• Microcystic lesions: These are small, benign cystic lesions composed of dilated lymphatic channels.
• Macrocystic lesions: These are larger, more complex cystic lesions composed of dilated lymphatic channels.
• Cystic hygromas: These are developmental abnormalities of the lymphatic system in which lymphatic fluid collects in a cystic space that is often multilocular.

Other Names

Capillary lymphangioma may also be referred to as:

• Lymphangioma circumscriptum: This refers to a superficial form of lymphangioma characterized by dilated lymphatic channels.
• Acquired lymphangioma: This refers to a type of lymphangioma that is acquired, rather than congenital.

References

[1] Capillary lymphangioma is a rare cystic lymphatic malformation characterized by benign cystic lesions composed of dilated lymphatic channels. These microcystic lesions are typically smaller than 1 cm in diameter and can manifest at birth or in early childhood, primarily in the head and neck region but can occur elsewhere [1].

[2] Lymphangiomas are classified into three types based on the size of the lymphatic cavities incorporated: microcystic (capillary lymphangiomas), macrocystic (cavernous lymphangiomas), and cystic hygromas. The classification was first proposed by Bill and Summer in 1965, who presented the concept that cystic hygromas and lymphangiomas are variations of a single entity [2].

Capillary Lymphangioma Signs and Symptoms

Capillary lymphangiomas are a type of lymphatic malformation characterized by small, dilated lymphatic vessels. The signs and symptoms of capillary lymphangioma can vary depending on the size and location of the affected area.

• Soft, spongy masses: Capillary lymphangiomas often present as soft, spongy masses that are non-tender to the touch [3].
• Overgrowth and swelling: Affected areas may experience overgrowth and swelling, which can be noticeable in the lips, tongue, jaws, cheeks, arms, legs, fingers, or toes [4].
• Enlarged liver and spleen: In some cases, capillary lymphangiomas can cause an enlarged liver and spleen, leading to abdominal swelling [5].
• Mass presence: The most prominent sign of all lymphangiomas, including capillary lymphangioma, is the presence of a mass [6].

It's essential to note that capillary lymphangiomas can be asymptomatic or present with varying degrees of severity. If you suspect you or your child has a capillary lymphangioma, it's crucial to consult a medical professional for proper evaluation and treatment.

References: [3] Jun 17, 2021 —

Diagnostic Tests for Capillary Lymphangioma

Capillary lymphangioma, also known as a cavernous lymphangioma or lymphatic malformation, is a rare and benign vascular tumor that can occur anywhere on the body. Diagnosing this condition typically involves a combination of physical examination, imaging studies, and sometimes genetic testing.

Imaging Studies

• Ultrasound: This non-invasive test uses high-frequency sound waves to create images of the internal structures of the body. Ultrasound exams can detect capillary lymphangiomas before birth or after birth if a doctor notices one (Source: [5])
• Color Doppler: This imaging modality helps identify the abnormal blood vessels associated with capillary lymphangioma (Source: [6])
• Cephalometric radiographs: These X-ray images are useful in diagnosing maxillofacial skeletal deformities secondary to lymphatic malformations (Source: [3])

Physical Examination

A healthcare provider will examine the lump's size, location, and texture. They may also review the patient's medical history and any associated symptoms.

Genetic Testing

In some cases, genetic testing may be necessary to confirm the diagnosis of capillary lymphangioma. This can involve analyzing DNA samples for specific mutations or gene variants (Source: [4])

Biopsy

A biopsy report confirmed the diagnosis as lymphangioma in one case, showing clusters of lymphoid tissue and large underdeveloped lymphatic channels with endothelial cells (Source: [8])

Histopathological Examination

Definitive diagnosis is only possible by histopathological examination. Small lymphatic vessels closely resemble capillaries and venules and are often only distinguishable through microscopic examination (Source: [9])

It's essential to note that diagnosing capillary lymphangioma can be challenging, and a combination of these diagnostic tests may be necessary to confirm the diagnosis.

References: [1] Cavernous lymphangioma: A red to blue swollen, rubbery mass often found on the tongue but can form anywhere on the body. [2] Lymphangiomas can occur anywhere on the skin and mucous membranes. [3] Cephalometric radiographs are useful in diagnosing maxillofacial skeletal deformities secondary to lymphatic malformations. [4] Genetic testing may be necessary to confirm the diagnosis of capillary lymphangioma. [5] Ultrasound exams can detect capillary lymphangiomas before birth or after birth if a doctor notices one. [6] Color Doppler helps identify the abnormal blood vessels associated with capillary lymphangioma. [7] A biopsy report confirmed the diagnosis as lymphangioma in one case, showing clusters of lymphoid tissue and large underdeveloped lymphatic channels with endothelial cells. [8] Definitive diagnosis is only possible by histopathological examination. Small lymphatic vessels closely resemble capillaries and venules and are often only distinguishable through microscopic examination.

Capillary Lymphangioma Drug Treatment Options

Capillary lymphangiomas are a type of vascular anomaly that can be treated with various drug options. According to recent studies, the following drugs have shown promise in treating capillary lymphangiomas:

• Bleomycin-based electrochemotherapy: This treatment involves delivering bleomycin directly into the affected area using electroporation. Studies suggest that this method is a feasible alternative treatment option for capillary malformations [1].
• Propranolol: This beta-blocker has been shown to be effective in treating infantile hemangiomas, and recent studies have also reported its use in treating capillary lymphangiomas [6]. Propranolol can help decrease the size of capillary hemangiomas.
• Sirolimus (Rapamycin): This immunosuppressive drug has been used to treat various vascular anomalies, including capillary lymphangiomas. Studies have reported its effectiveness in shrinking lymphatic malformations [8].

Other Treatment Options

While these drugs show promise, it's essential to note that surgical excision remains the treatment of choice for most cases of capillary lymphangioma. However, when feasible, drug treatment can be a viable alternative or used in conjunction with surgery.

• Surgical Excision: Complete excision is still considered the gold standard for treating capillary lymphangiomas. Wide local excision of affected lymphatic channels is necessary to prevent recurrence [14].
• Experimental Treatments: Other experimental treatments, such as laser therapy and interferon alpha, may also be explored on a case-by-case basis.

References

[1] S Dalmády (2020) - Bleomycin-based electrochemotherapy for capillary malformations [6] Maturo and Hartnick (2010) - Propranolol as the sole treatment for focal infantile airway hemangiomas [8] S Wiegand (2018) - Sirolimus therapy in lymphatic malformations [14] Recurrence rates for surgical excision of lymphangioma circumscriptum

Differential Diagnosis of Capillary Lymphangioma

Capillary lymphangiomas, also known as lymphangioma simplex or capillary lymphangioma, are benign vascular lesions composed of small, thin-walled capillary-sized lymphatic vessels. When diagnosing capillary lymphangioma, it is essential to consider the following differential diagnoses:

• Hemangioma: A well-circumscribed lesion with smaller vascular spaces extensively filled with red blood cells [4].
• Venous malformation: A benign vascular lesion that demonstrates internal blood flow and central enhancement [4].
• Arteriovenous malformation (AVM): A congenital anomaly characterized by an abnormal connection between arteries and veins, which can be a differential diagnosis for capillary lymphangioma [7].
• Cavernous hemangioma: A benign vascular lesion that is often confused with capillary lymphangioma due to its similar appearance on imaging studies [9].

Key Features to Distinguish Capillary Lymphangioma from Other Conditions

To accurately diagnose capillary lymphangioma, it is crucial to consider the following key features:

• Thin-walled lymphatic channels: Capillary lymphangiomas are characterized by thin-walled lymphatic vessels that are smaller in size compared to other vascular lesions [11].
• Superficial or deep location: Capillary lymphangiomas can occur in both superficial and deep locations, which is essential to consider when differentiating them from other conditions [10].

References

[4] Jan 28, 2024 — Differential diagnosis · hemangioma · venous malformation: demonstrate internal blood flow and central enhancement · other cystic neck masses. [7] A lymphatic malformation is a type of vascular naevus or birthmark due to malformed and dilated lymphatic vessels. [9] The most common differential diagnosis is with cavernous hemangioma. Lymphangioma contains proteinaceous fluid and the surrounding tissues are usually ... [10] Lymphangiomas can be categorized as deep or superficial based on the depth and size of the abnormal lymphatic vessels or as congenital or acquired.[1] [11] Lymphangiomas have been classified into 3 types: (1) Lymphangioma simplex or capillary lymphangioma consisting of small, thin-walled capillary-sized lymphatic vessels;