Lymphangioma (patient information): Difference between revisions
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{{Lymphangioma (patient information)}} | {{Lymphangioma (patient information)}} | ||
{{CMG}} | {{CMG}}{{Badria}} | ||
==Overview== | ==Overview== | ||
[[Lymphangioma]] is abnormality of [[lymph]] vessels. | [[Lymphangioma]] is abnormality of [[lymph]] vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream. The exact pathogenesis of lymphangioma is not fully understood. It is thought that lymphangioma is caused by either sequestration of lymph tissue, abnormal budding of lymph vessels, lack of fusion with the venous system, or obstruction of lymph vessels. Lymphangiomas most commonly develop at the head and neck regions. | ||
==What are the symptoms of Lymphangioma?== | ==What are the symptoms of Lymphangioma?== | ||
| Line 49: | Line 49: | ||
==Treatment options== | ==Treatment options== | ||
Medical Therapy | |||
There is no medical treatment for lymphangioma; the mainstay of therapy is surgery. | |||
Surgery | |||
The mainstay of therapy for lymphangioma is surgery. Treatment for lymphangioma involves the removal of the abnormal tissue; however complete removal may be impossible without removing other normal areas. Most patients need at least two procedures done for the removal process to be achieved. | |||
==Where to find medical care for Lymphangioma?== | ==Where to find medical care for Lymphangioma?== | ||
| Line 54: | Line 59: | ||
==Prevention of Lymphangioma== | ==Prevention of Lymphangioma== | ||
There are no established measures for the primary prevention of lymphangioma. | |||
==What to expect (Outlook/Prognosis)?== | ==What to expect (Outlook/Prognosis)?== | ||
Prognosis of lymphangioma is generally excellent after surgical intervention. | |||
==Possible complications== | ==Possible complications== | ||
*Common [[complications]] of [[lymphangiomas]] are primarily associated to the location of lesion.<ref name="pmid10712360">{{cite journal |vauthors=Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, Raffin TA |title=Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome |journal=Am. J. Respir. Crit. Care Med. |volume=161 |issue=3 Pt 1 |pages=1037–46 |date=March 2000 |pmid=10712360 |doi=10.1164/ajrccm.161.3.9904056 |url=}}</ref> | |||
*Growth of lesion causes compressive effects on surrounding tissues. Which include following:<ref name="pmid4537032">{{cite journal |vauthors=Runyon BA, Forker EL, Sopko JA |title=Pleural-fluid kinetics in a patient with primary lymphedema, pleural effusions, and yellow nails |journal=Am. Rev. Respir. Dis. |volume=119 |issue=5 |pages=821–5 |date=May 1979 |pmid=453703 |doi=10.1164/arrd.1979.119.5.821 |url=}}</ref> | |||
**[[Dyspnea]]: Trachea<ref name="pmid8444562" /> | |||
**[[Dysphagia]]: Esophagous<ref name="pmid40657002">{{cite journal |vauthors=Duhra PM, Quigley EM, Marsh MN |title=Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome |journal=Gut |volume=26 |issue=11 |pages=1266–9 |date=November 1985 |pmid=4065700 |pmc=1432907 |doi= |url=}}</ref> | |||
**[[Constipation]]: Gastrointestinal tract<ref name="pmid4065700">{{cite journal |vauthors=Duhra PM, Quigley EM, Marsh MN |title=Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome |journal=Gut |volume=26 |issue=11 |pages=1266–9 |date=November 1985 |pmid=4065700 |pmc=1432907 |doi= |url=}}</ref> | |||
**[[Diplopia]] and [[proptosis]]: Retro-orbital tissue of [[eye]]<ref name="pmid453703">{{cite journal |vauthors=Runyon BA, Forker EL, Sopko JA |title=Pleural-fluid kinetics in a patient with primary lymphedema, pleural effusions, and yellow nails |journal=Am. Rev. Respir. Dis. |volume=119 |issue=5 |pages=821–5 |date=May 1979 |pmid=453703 |doi=10.1164/arrd.1979.119.5.821 |url=}}</ref> | |||
**[[Wheeze]]: Lower Respiratory tract<ref name="pmid8977864">{{cite journal |vauthors=Papsin BC, Evans JN |title=Isolated laryngeal lymphangioma: a rare cause of airway obstruction in infants |journal=J Laryngol Otol |volume=110 |issue=10 |pages=969–72 |date=October 1996 |pmid=8977864 |doi= |url=}}</ref> | |||
**[[Chest pain|Chest Pain]]: Mediastinum | |||
**[[Shortness of breath|Shortness of breadth]]: [[Lung|Lungs]]<ref name="pmid8444562">{{cite journal |vauthors=Williams WT, Cole RR |title=Lymphangioma presenting as congenital stridor |journal=Int. J. Pediatr. Otorhinolaryngol. |volume=26 |issue=2 |pages=185–91 |date=March 1993 |pmid=8444562 |doi= |url=}}</ref> | |||
**Bladder Obstruction: [[Urinary tract]] | |||
**Bone loss or overgrowth: Skeletal tissue<ref name="pmid9473098">{{cite journal |vauthors=Ogita S, Deguchi E, Tokiwa K, Iwata J, Kubota Y, Iwai N |title=Ongoing osteolysis in patients with lymphangioma |journal=J. Pediatr. Surg. |volume=33 |issue=1 |pages=45–8 |date=January 1998 |pmid=9473098 |doi= |url=}}</ref> | |||
**[[Inflammation]] or [[bleeding]] into the lesion | |||
**Recurrent [[Cellulitis]]:Visible deformity<ref name="pmid14395215">{{cite journal |vauthors=MANN TP |title=Hemihypertrophy left side of body; congenital lymphatic oedema of left arm; radiological enlargement of heart shadow |journal=Proc. R. Soc. Med. |volume=48 |issue=5 |pages=330–1 |date=May 1955 |pmid=14395215 |pmc=1918895 |doi= |url=}}</ref> | |||
==Source== | ==Source== | ||
Revision as of 14:09, 1 November 2018
For the WikiDoc page on this topic, click here
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Lymphangioma |
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Lymphangioma On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Badria Munir M.B.B.S.[2]
Overview
Lymphangioma is abnormality of lymph vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream. The exact pathogenesis of lymphangioma is not fully understood. It is thought that lymphangioma is caused by either sequestration of lymph tissue, abnormal budding of lymph vessels, lack of fusion with the venous system, or obstruction of lymph vessels. Lymphangiomas most commonly develop at the head and neck regions.
What are the symptoms of Lymphangioma?
- The majority of patients with lymphangioma are asymptomatic.
- As the mass increases in size, lymphangioma patients may develop site-specific symptoms, such as:
- Dysphagia
- Difficulty breathing
- Constipation
- Failure to thrive
What causes Lymphangioma?
- There are no known direct causes for lymphangioma.
- Cystic hygroma usually presents in syndromes such as Turner syndrome and Down syndrome.
- Therefore it is thought that the lymphatic malformations can arise from gene mutations, occuring as missing or deleted part of chromosomes.
- Acquired lymphangioma circumscriptum occur as a sequele of chronic lymphatic obstruction such as after surgery, infections or radiotherapy
Who is at highest risk?
The most potent risk factor in the development of lymphangioma is the presence of genetic disorders such as Turner syndrome, Down syndrome, and Noonan syndrome.[1]
Diagnosis
Biopsy:
- Biopsy is used to analyze the structure of tissue.
- It shows:[1]
- Dilated lymph channels ultimately causes the papillary dermis to expand.
- These channels extend to subcutis.
- Deeper vessels have larger lumen and muscular coat.
- The lumen has lymphatic fluid, but it often contains red blood cells, lymphocytes, macrophages, and neutrophils.
- These channels are lined by endothelial cells.
- Numerous lymphoid cells are also seen.
Histochemical staining:
- Histochemical staining is recommended to document lymphangioma.[2]
MRI:
- MRI is primarily used for the diagnosis of lymphangioma.
- MRI can mar the degree of involvement and extent of lesion.
- MRI can prevent extensive, incomplete surgical resection, because poorly removed lesion can lead to recurrence.
When to seek urgent medical care?
In cases of severe complications of disease, such as
- Difficulty breathing
- Difficulty swallowing
Treatment options
Medical Therapy
There is no medical treatment for lymphangioma; the mainstay of therapy is surgery. Surgery The mainstay of therapy for lymphangioma is surgery. Treatment for lymphangioma involves the removal of the abnormal tissue; however complete removal may be impossible without removing other normal areas. Most patients need at least two procedures done for the removal process to be achieved.
Where to find medical care for Lymphangioma?
Directions to Hospitals Treating Lymphangioma
Prevention of Lymphangioma
There are no established measures for the primary prevention of lymphangioma.
What to expect (Outlook/Prognosis)?
Prognosis of lymphangioma is generally excellent after surgical intervention.
Possible complications
- Common complications of lymphangiomas are primarily associated to the location of lesion.[3]
- Growth of lesion causes compressive effects on surrounding tissues. Which include following:[4]
- Dyspnea: Trachea[5]
- Dysphagia: Esophagous[6]
- Constipation: Gastrointestinal tract[7]
- Diplopia and proptosis: Retro-orbital tissue of eye[8]
- Wheeze: Lower Respiratory tract[9]
- Chest Pain: Mediastinum
- Shortness of breadth: Lungs[5]
- Bladder Obstruction: Urinary tract
- Bone loss or overgrowth: Skeletal tissue[10]
- Inflammation or bleeding into the lesion
- Recurrent Cellulitis:Visible deformity[11]
Source
- ↑ Invalid
<ref>tag; no text was provided for refs namedpmid21938186 - ↑ Sauter, Birthe; Foedinger, Dagmar; Sterniczky, Barbara; Wolff, Klaus; Rappersberger, Klemens (2016). "Immunoelectron Microscopic Characterization of Human Dermal Lymphatic Microvascular Endothelial Cells: Differential Expression of CD31, CD34, and Type IV Collagen with Lymphatic Endothelial Cells vs Blood Capillary Endothelial Cells in Normal Human Skin, Lymphangioma, and Hemangioma In Situ". Journal of Histochemistry & Cytochemistry. 46 (2): 165–176. doi:10.1177/002215549804600205. ISSN 0022-1554.
- ↑ Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, Raffin TA (March 2000). "Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome". Am. J. Respir. Crit. Care Med. 161 (3 Pt 1): 1037–46. doi:10.1164/ajrccm.161.3.9904056. PMID 10712360.
- ↑ Runyon BA, Forker EL, Sopko JA (May 1979). "Pleural-fluid kinetics in a patient with primary lymphedema, pleural effusions, and yellow nails". Am. Rev. Respir. Dis. 119 (5): 821–5. doi:10.1164/arrd.1979.119.5.821. PMID 453703.
- ↑ 5.0 5.1 Williams WT, Cole RR (March 1993). "Lymphangioma presenting as congenital stridor". Int. J. Pediatr. Otorhinolaryngol. 26 (2): 185–91. PMID 8444562.
- ↑ Duhra PM, Quigley EM, Marsh MN (November 1985). "Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome". Gut. 26 (11): 1266–9. PMC 1432907. PMID 4065700.
- ↑ Duhra PM, Quigley EM, Marsh MN (November 1985). "Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome". Gut. 26 (11): 1266–9. PMC 1432907. PMID 4065700.
- ↑ Runyon BA, Forker EL, Sopko JA (May 1979). "Pleural-fluid kinetics in a patient with primary lymphedema, pleural effusions, and yellow nails". Am. Rev. Respir. Dis. 119 (5): 821–5. doi:10.1164/arrd.1979.119.5.821. PMID 453703.
- ↑ Papsin BC, Evans JN (October 1996). "Isolated laryngeal lymphangioma: a rare cause of airway obstruction in infants". J Laryngol Otol. 110 (10): 969–72. PMID 8977864.
- ↑ Ogita S, Deguchi E, Tokiwa K, Iwata J, Kubota Y, Iwai N (January 1998). "Ongoing osteolysis in patients with lymphangioma". J. Pediatr. Surg. 33 (1): 45–8. PMID 9473098.
- ↑ MANN TP (May 1955). "Hemihypertrophy left side of body; congenital lymphatic oedema of left arm; radiological enlargement of heart shadow". Proc. R. Soc. Med. 48 (5): 330–1. PMC 1918895. PMID 14395215.