Lymphangioma natural history: Difference between revisions
(Mahshid) |
Badria Munir (talk | contribs) |
||
| Line 6: | Line 6: | ||
==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
Lymphangiomas have slower rate of progression. Thses malformations continue to grow as with the growth of patient. These are prent on birth and may not be evident until the age of 5 years. Initially they grow as soft, cystic, translucent swelling n head and neck regio n, but it is commonly found in extremities and other in the internal organs such as pancrease and stomach has well. The presence of lymphangiomas n soft tissues and bone is not unusual and this form of disease has been termed as lymphangiomatosis. | |||
If left untreated lymphangiomas can either progress into large swellings or they can resolve spontaneously. | |||
If the continue to progress, they cause overgrowth and swelling of tissue involved, such as tongue, jaws, cheeks, neck, arms, fingers and toes. | |||
Overgrowth can ultimately cause compressive effects on surrounding tissues, such as dyspnea due to compression on trachea, or dysphagia due to compression on esophagous and diplopia due if eye socket is involved. | |||
It is important to mention that all the patients may not have similar course of progression of disease. | |||
Clinical picture may vary from person to person because certain events such as puberty, infection, trauma and bleeding into lymphatic malformation can trigger rapid growth. | |||
Most of the patients with lymphangioma will develop a cervico-facial mass that often grows in proportion to the patient’s body growth rate. The mass usually recurs after [[surgical]] interventions. | Most of the patients with lymphangioma will develop a cervico-facial mass that often grows in proportion to the patient’s body growth rate. The mass usually recurs after [[surgical]] interventions. | ||
It is important to note that affected individuals may not have all of the symptoms discussed below and that one child’s experience can vary dramatically from another child’s experience. Parents of affected children should talk to their physician and medical team about their child’s specific case, associated symptoms and overall prognosis. The specific symptoms that develop depend upon the size and exact location of a lymphatic malformation. | |||
Radiographically lymphatic malformations have been categorized into three subtypes – macrocystic, microcystic, or mixed (a combination of the other two). Macrocystic and microcystic lymphatic malformations are differentiated by the size of the fluid-containing portion of the malformation. The macrocystic type is made up of large cysts, more than 2 centimeters in diameter; the microcystic type is made up of smaller cysts or soft tissue enlargement without cyst formation. Most lymphatic malformations have both macrocystic and microcystic portions and histologically there is no difference between these categories. Macrocystic lymphatic malformations are characterized by a single or multiple fluid-filled pockets or cysts that occur, most commonly in the nape of the neck. Macrocystic lymphatic malformations generally form soft, large translucent masses that can involve any area of the neck or chest. Overlying skin may have a bluish tinge. Although macrocystic lymphatic malformations typically appear on the back of the neck, they can, less frequently, originate near the armpits (axilla), in the groin, in the rear of the abdominal cavity, in the chest wall, or in the hip or tailbone region. Macrocystic lymphatic malformations can potentially be extremely large, even large enough to obstruct the airway at delivery. | |||
Microcystic lymphatic malformations may appear as several small, raised sacs (vesicles) on the skin that contain clear or bloody (hemorrhagic) fluid. They generally grow slowly usually in proportion with a growing child. Microcystic lymphatic malformations can thicken or swell causing enlargement of surrounding soft tissue and bones. They can be found on any area of skin or mucous membrane. | |||
Rarely do cutaneous lymphangiomas interfere with the well-being of patients. Patients are expected to live a full healthy life, and they usually seek medical intervention because of cosmetic reason. | |||
Lymphangiomas represent hamartomatous malformations with no risk of malignant transformation. In the case of cystic hygroma, total surgical excision is appropriate to prevent complications such as respiratory compromise, aspiration, and infections in critical areas, such as the neck. Lymphangiomas have a strong tendency for local recurrence unless they are completely excised. Recurrent episodes of cellulitis and minor bleeding are not uncommon. | |||
The signs and symptoms of a lymphatic malformation (or lymphangioma) vary depending on the type, size and location of the mass. There are different ways of classifying lymphatic malformations. The classification most frequently used divides these lesions into 2 major groups based on the depth and the size of these abnormal lymph vessels. The superficial vesicles are called lymphangioma circumscriptum. The more deep-seated group includes cavernous lymphangioma and cystic hygroma:[3][4] | |||
Cystic hygroma, which is also known as cystic lymphangioma or macrocystic lymphatic malformation, is a red or bluish swelling of the skin in the neck, armpit or groin region that consists of large, fluid-filled spaces. Some say it is a type of cavernous lymphangioma. | |||
Cavernous lymphangioma is also considered a macrocystic lesion. It is a a bluish or red rubbery swelling under the skin that can affect any area of the body, including the tongue. | |||
Lymphangioma circumscriptum is a microcystic lymphatic malformation. It appears as a cluster of small, firm blisters most commonly on the shoulders, neck, underarm area, limbs, and in the mouth, especially the tongue. It consists of tiny, fluid-filled spaces and ranges in color from clear to pink, dark red, brown or black. | |||
Other classification includes macrocystic lymphatic malformation, microcystic lymphatic malformation and mixed type. | |||
===Complications=== | ===Complications=== | ||
Common complications of | Common complications of lymphangiomas are primarily associated to the location of lesion. | ||
Growth of lesion causes compressive effects on surrounding tissues. | |||
which include following: | |||
Dyspnea: Trachea | |||
Dysphagia: Esophagous | |||
Constipation: Gastrointestinal tract | |||
Diplopia and proptosis: Retro-orbital tissue of eye | |||
Wheez: Lower Respiratory tract | |||
Chest Pain: Mediastinum | |||
Shortness of breadth: Lungs | |||
Bladder Obstruction: Urinary tract | |||
Bone loss or overgrowth: Skeletal tissue | |||
Inflammation or bleeding into the lesion | |||
Recuurent Cellulitis:Visible deformity | |||
===Prognosis=== | ===Prognosis=== | ||
Prognosis of lymphangioma is generally excellent. | Prognosis of lymphangioma is generally excellent after surgical intervention. | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
Revision as of 16:31, 11 October 2018
|
Lymphangioma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Lymphangioma natural history On the Web |
|
American Roentgen Ray Society Images of Lymphangioma natural history |
|
Risk calculators and risk factors for Lymphangioma natural history |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Most of the patients with lymphangioma will develop a cervico-facial mass that often grows in proportion to the patient’s body growth rate. The mass usually recurs after surgical interventions. Common complications of lymphangioma include esophageal obstruction, upper respiratory tract obstruction, infections, and compression of adjacent tissues such as nerves and blood vessels. Prognosis of lymphangioma is generally excellent.[1][2][3][4]
Natural History, Complications and Prognosis
Natural History
Lymphangiomas have slower rate of progression. Thses malformations continue to grow as with the growth of patient. These are prent on birth and may not be evident until the age of 5 years. Initially they grow as soft, cystic, translucent swelling n head and neck regio n, but it is commonly found in extremities and other in the internal organs such as pancrease and stomach has well. The presence of lymphangiomas n soft tissues and bone is not unusual and this form of disease has been termed as lymphangiomatosis. If left untreated lymphangiomas can either progress into large swellings or they can resolve spontaneously. If the continue to progress, they cause overgrowth and swelling of tissue involved, such as tongue, jaws, cheeks, neck, arms, fingers and toes. Overgrowth can ultimately cause compressive effects on surrounding tissues, such as dyspnea due to compression on trachea, or dysphagia due to compression on esophagous and diplopia due if eye socket is involved. It is important to mention that all the patients may not have similar course of progression of disease. Clinical picture may vary from person to person because certain events such as puberty, infection, trauma and bleeding into lymphatic malformation can trigger rapid growth.
Most of the patients with lymphangioma will develop a cervico-facial mass that often grows in proportion to the patient’s body growth rate. The mass usually recurs after surgical interventions.
It is important to note that affected individuals may not have all of the symptoms discussed below and that one child’s experience can vary dramatically from another child’s experience. Parents of affected children should talk to their physician and medical team about their child’s specific case, associated symptoms and overall prognosis. The specific symptoms that develop depend upon the size and exact location of a lymphatic malformation.
Radiographically lymphatic malformations have been categorized into three subtypes – macrocystic, microcystic, or mixed (a combination of the other two). Macrocystic and microcystic lymphatic malformations are differentiated by the size of the fluid-containing portion of the malformation. The macrocystic type is made up of large cysts, more than 2 centimeters in diameter; the microcystic type is made up of smaller cysts or soft tissue enlargement without cyst formation. Most lymphatic malformations have both macrocystic and microcystic portions and histologically there is no difference between these categories. Macrocystic lymphatic malformations are characterized by a single or multiple fluid-filled pockets or cysts that occur, most commonly in the nape of the neck. Macrocystic lymphatic malformations generally form soft, large translucent masses that can involve any area of the neck or chest. Overlying skin may have a bluish tinge. Although macrocystic lymphatic malformations typically appear on the back of the neck, they can, less frequently, originate near the armpits (axilla), in the groin, in the rear of the abdominal cavity, in the chest wall, or in the hip or tailbone region. Macrocystic lymphatic malformations can potentially be extremely large, even large enough to obstruct the airway at delivery.
Microcystic lymphatic malformations may appear as several small, raised sacs (vesicles) on the skin that contain clear or bloody (hemorrhagic) fluid. They generally grow slowly usually in proportion with a growing child. Microcystic lymphatic malformations can thicken or swell causing enlargement of surrounding soft tissue and bones. They can be found on any area of skin or mucous membrane.
Rarely do cutaneous lymphangiomas interfere with the well-being of patients. Patients are expected to live a full healthy life, and they usually seek medical intervention because of cosmetic reason.
Lymphangiomas represent hamartomatous malformations with no risk of malignant transformation. In the case of cystic hygroma, total surgical excision is appropriate to prevent complications such as respiratory compromise, aspiration, and infections in critical areas, such as the neck. Lymphangiomas have a strong tendency for local recurrence unless they are completely excised. Recurrent episodes of cellulitis and minor bleeding are not uncommon.
The signs and symptoms of a lymphatic malformation (or lymphangioma) vary depending on the type, size and location of the mass. There are different ways of classifying lymphatic malformations. The classification most frequently used divides these lesions into 2 major groups based on the depth and the size of these abnormal lymph vessels. The superficial vesicles are called lymphangioma circumscriptum. The more deep-seated group includes cavernous lymphangioma and cystic hygroma:[3][4] Cystic hygroma, which is also known as cystic lymphangioma or macrocystic lymphatic malformation, is a red or bluish swelling of the skin in the neck, armpit or groin region that consists of large, fluid-filled spaces. Some say it is a type of cavernous lymphangioma. Cavernous lymphangioma is also considered a macrocystic lesion. It is a a bluish or red rubbery swelling under the skin that can affect any area of the body, including the tongue. Lymphangioma circumscriptum is a microcystic lymphatic malformation. It appears as a cluster of small, firm blisters most commonly on the shoulders, neck, underarm area, limbs, and in the mouth, especially the tongue. It consists of tiny, fluid-filled spaces and ranges in color from clear to pink, dark red, brown or black. Other classification includes macrocystic lymphatic malformation, microcystic lymphatic malformation and mixed type.
Complications
Common complications of lymphangiomas are primarily associated to the location of lesion. Growth of lesion causes compressive effects on surrounding tissues. which include following: Dyspnea: Trachea Dysphagia: Esophagous Constipation: Gastrointestinal tract Diplopia and proptosis: Retro-orbital tissue of eye Wheez: Lower Respiratory tract Chest Pain: Mediastinum Shortness of breadth: Lungs Bladder Obstruction: Urinary tract Bone loss or overgrowth: Skeletal tissue Inflammation or bleeding into the lesion Recuurent Cellulitis:Visible deformity
Prognosis
Prognosis of lymphangioma is generally excellent after surgical intervention.
References
- ↑ Lymphangioma. Wikipedia (2016) https://en.wikipedia.org/wiki/Lymphangioma Accessed on March 3, 2015
- ↑ Lymphangioma. PathologyOutlines (2016) http://www.pathologyoutlines.com/topic/softtissuelymphangiomacystic.html Accessed on March 5, 2016
- ↑ Lymphangioma. Libre Pathology (2016) http://librepathology.org/wiki/Vascular_tumours#Lymphangioma Accessed on March 5, 2016
- ↑ Lymphangioma. Radiopeadia (2016) http://radiopaedia.org/articles/lymphangioma Accessed on March 5, 2016