Lymphangioma: Difference between revisions
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==Prognosis== |
==Prognosis== |
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==History== |
==History== |
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Lymphangioma circumscriptum |
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The typical history involves a small number of vesicles on the skin at birth or shortly after. In subsequent years, they tend to increase in number, and the area of skin involved continues to expand. Vesicles or other skin abnormalities may not be noticed until several years after birth. |
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Usually, lesions are asymptomatic, but, occasionally, patients may have spontaneous episodes of minor bleeding and copious drainage of clear fluid from ruptured vesicles. |
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Cavernous lymphangioma |
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Typically, during infancy, a solitary rubbery nodule with no skin changes becomes evident in a single location, such as the face, trunk, or extremity. These lesions often have a fast growth phase similar to that of raised hemangiomas. |
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No family history of prior lymphangiomas is described. |
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Cystic hygroma |
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Soon after birth, the infant is noted to have a deep subcutaneous cystic swelling, usually in the axilla, base of the neck, or groin. If these lesions are drained, they tend to fill up rapidly with lymph fluid. |
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The lesions tend to grow and increase to a large size if they are not completely excised at surgery. |
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==Society/Culture== |
==Society/Culture== |
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==References== |
==References== |
Revision as of 00:22, 15 October 2008
Lymphangioma,is a lymphatic malformation,a benign proliferation of lymph vessels, or a yellow-ish tumor on the skin, composed of a mass of dilated lymph vessels. It is often removed by surgery or a form of surgery using an electric current(electrocoagulation) for cosmetic reasons. It is rare, incidence estimated to be around 1 per 10 000 live births. They are fluid filled sacs that result from blockage of the lymphatic system. They are often associated with karyotype abnormalities such as Turner syndrome. These tumors may occur anywhere but usually occur in the head and neck (75%), with a predilection for the left side. Most (90%) are either evident at birth or become evident before age 2 years. Other types of Lymphangioma are Cystic lymphangioma, Lymphangioma cavernosum and Lymphangioma circumscriptum. The tumor is a hamartoma or vascular developmental abnormality arising from lymphatic vessels, manifesting as a raised, soft, shaggy, bubbly, pinkish-white lesion; cosmetic considerations may warrant attempted removal of lymphangiomas.
Types of Lymphangioma
Lymphangioma circumscriptum - Also referred to as cutaneous this is a "kind" of birthmark generally occurring in clusters. They resemble small blisters and range in color from pink to dark red. They are benign and usually require no medical treatment. For cosmetic reasons, some patients may choose to have them surgically removed. Lymphangiomas Simplex is often included in this type.This is also a type of tumor that is commonly seen in younger children. [1]
Cavernous lymphangioma - This is an uncommon form generally arising during infancy. These are deep seated underneath the dermis and the external skin is not involved. Because they are deep seated, they form a bulging mass, painless mass. This is also a tumor filled with lymph that is often mixed with blood. It usually affects the tongue and lips.[2]
Cystic Hygroma - These are soft lymph filled masses within a thin-walled "sac" generally appearing on the neck (75%), arm pit or groin areas. They usually present a swollen bulges underneath the skin. While they generally present at birth, children up to three years old may experience them. There is also disagreement on whether this type is distinctly different enough from cavernous lymphangioma to be classified separately.
Lymph Nodes
One of many small oval structures that filter the lymph, fight infection and in which there are formed white blood cells and blood plasma cells. They come in various sizes, they can range from being as small as a pin head or as big as a bean. Each node is enclosed in a fibrous capsule and consist of closely packed white cells(lymphocytes) connected tissue and lymph path ways.Most lymph nodes are clustered in certain areas, such as mouth, neck, lower armpit and groin.For example visceral lymph nodes filter lymph circulating in the lymphatic vessels of the viscera of the chest, lower body and pelvis. The lymphatic network and nodes of the breast are especially crucial in the diagnosis and treatment of breast cancer in women also called lymph gland.
- A Lymph
- is a clear, watery, sometimes faintly yellowish fluid derived from body tissues that contains white blood cells and circulates throughout the lymphatic system, returning to the venous bloodstream through the thoracic duct. Lymph acts to remove bacteria and certain proteins from the tissues, transport fat from the small intestine, and supply mature lymphocytes to the blood.[3]
- A Lymph Gland
- is any of the small bodies located along the lymphatic vessels, particularly at the neck, armpit, and groin, that filter bacteria and foreign particles from lymph fluid. During infection, lymph nodes may become swollen with activated lymphocytes.
- A Lymphatic Vessel
- is any of the vascular channels that transport lymph throughout the lymphatic system and freely anastomose with one another.[4]
Signs and Symptoms
Causes
There is no known cause for these lymphatic abnormalities. There are some suggested reasons but none are certain. Drinking alcohol during pregnancy is one of the most likely causes of this cosmetic deformity.Other hypotheses have been made but most likely are not true.[5]
Diagnosis
Prevention
Treatment
Lymphangioma Circumscription is usually successfully healed when treated with a flashlamp pulsed dye laser. This can cause port-wine stains and other vascular lesions.[6]
Pathophysiology
In 1976 Whimster studied the pathogenesis of lymphangioma circumscriptum. Whimster said, the basic pathologic process is the collection of lymphatic cisterns in the deep subcutaneous plane. These cisterns are separated from the normal network of lymph vessels, but they communicate with the superficial lymph vesicles through vertical, dilated lymph channels.
Whimster thought that these cisterns might arise from a primitive lymph sac that fails to connect with the rest of the lymphatic system during its embryonic development. A thick coat of muscle fibers that cause rhythmic contractions line these sequestered primitive sacs. Rhythmic contractions increase the intramural pressure, causing dilated channels to protrude from the walls of the cisterns toward the skin. He suggested that the vesicles seen in lymphangioma circumscriptum are outpouchings of these dilated projecting vessels.
Whimster's observations are supported by those of lymphangiographic and radiographic studies. These studies revealed that large multilobulated cisterns extend deep in the dermis and laterally beyond the obvious clinical lesions. These deep lymphangiomas show no evidence of communication with the adjacent normal lymphatics. The cause for the failure of these primitive lymph sacs to connect to the rest of the lymphatic system is not known.A thick coat of muscle fibers that cause rhythmic contractions line these sequestered primitive sacs. Rhythmic contractions increase the intramural pressure, causing dilated channels to protrude from the walls of the cisterns toward the skin. He suggested that the vesicles seen in lymphangioma circumscriptum are outpouchings of these dilated projecting vessels.[7]
Prognosis
History
Lymphangioma circumscriptum
The typical history involves a small number of vesicles on the skin at birth or shortly after. In subsequent years, they tend to increase in number, and the area of skin involved continues to expand. Vesicles or other skin abnormalities may not be noticed until several years after birth.
Usually, lesions are asymptomatic, but, occasionally, patients may have spontaneous episodes of minor bleeding and copious drainage of clear fluid from ruptured vesicles.
Cavernous lymphangioma
Typically, during infancy, a solitary rubbery nodule with no skin changes becomes evident in a single location, such as the face, trunk, or extremity. These lesions often have a fast growth phase similar to that of raised hemangiomas.
No family history of prior lymphangiomas is described.
Cystic hygroma
Soon after birth, the infant is noted to have a deep subcutaneous cystic swelling, usually in the axilla, base of the neck, or groin. If these lesions are drained, they tend to fill up rapidly with lymph fluid.
The lesions tend to grow and increase to a large size if they are not completely excised at surgery.
Society/Culture
References
- ^ "Acquired lymphangioma circumscriptum of the vulva". Retrieved 2008-10-03.
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(help) - ^ "Wrong Diagnosis". Adviware Pty Ltd. Retrieved 2008-10-02.
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(help) - ^ Weingold DH, White PF, Burton CS (1990). "Treatment of lymphangioma circumscriptum with tunable dye laser". Cutis; cutaneous medicine for the practitioner. 45 (5): 365–6. PMID 2357907.
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