Lymphangioma - Linfangioma
Linfangioma (Lymphangioma) sistema linfatikoaren malformazioak dira, horma meheko kisteak diren lesioen ezaugarriak. Malformazio hauek edozein adinetan gerta daitezke eta gorputzeko edozein atal izan ditzakete, baina % 90 2 urte baino gutxiagoko haurrengan gertatzen da eta burua eta lepoa hartzen dituzte. Linfangiomak jaio aurretik diagnostikatzen dira normalean fetuaren ultrasonografia erabiliz. Lortutako linfangiomak traumatismo, hantura edo oztopo linfatikoen ondorioz izan daitezke. Gaizto bihurtzeko aukerarik ez dutenez, linfangiomak arrazoi kosmetikoengatik soilik tratatzen dira normalean.
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References
Lymphangioma malformazio linfatikoa (LM) bezala ere ezagutzen da. Jaiotzetik agertzen den nahaste baskular bat da. Ehun linfatikoaren hazkunde anormala da jaio aurretik eta ondoren. Lymphangioma 2000 eta 4000 pertsonatik 1 ingururi eragiten dio, genero edo arrazen arteko alde handirik gabe. Kasu gehienak (%80-90) bi urte baino lehen diagnostikatzen dira. Sintomak asko aldatzen dira, hantura lokalizatuetatik kanal linfatikoetan anomalia zabaletaraino, batzuetan elefantiasia bezala ezagutzen den hantura larria eragiten du. Adibidez, lymphangioma lepoan eta aurpegian aurpegiko hantura eragin dezakete, eta kasu larrietan, itxuragabetzea. Mihiari eragiten dionean, masailezurren hazkuntza eta hortzak gaizki lerrokatuta sor ditzake. Ahoan eta lepoan, arnasketa arazoak eta bizitza arriskuan jartzen dituzten larrialdiak sor ditzake. Begietan, ikusmen-galera, begien mugimendu mugatua, betazalak erortzea eta begiak handitzea eragin dezake. Gorputz-adarraren inplikazioak ehunen eta hezurren hantura eta hazkunde anormala eragin dezake. Tumore hau poliki hazten da normalean, baina infekzioek, aldaketa hormonalek edo lesioek hazkuntza azkarra eragin dezakete, eta bizitza arriskuan jartzen dute premiazko tratamendua eskatzen dutenak.
Lymphangioma (lymphatic malformation, LM), a congenital vascular disease, is a low-flow vascular abnormality in lymphatic diseases that is characterized by excessive growth of lymphatic tissue during prenatal and postpartum development. The incidence rate of LM is ~1:2000–4000, with no variation between genders and races. Most patients (80–90%) are diagnosed before the age of two. The clinical manifestations of lymphangioma are quite different among patients, varying from local swelling leading to superficial mass to a large area of diffuse infiltrating lymphatic channel abnormalities resulting in elephantiasis. Cervicofacial LM can cause facial elephantiasis, and in some severe cases, it can lead to serious disfigurement of the face. Tongue LM can lead to mandibular overgrowth and occlusal asymmetry, and oral and cervical LM can cause obstructive acute respiratory distress and life-threatening situations. Orbital LM may lead to decreased vision, decreased extraocular muscle movement, ptosis and exophthalmos. LM of the extremities can trigger swelling or gigantism, accompanied by overgrowth of soft tissue and bones. LM usually grows slowly and steadily, but under certain conditions, such as infection, hormonal changes or trauma, it can grow explosively and become a life-threatening disease requiring immediate treatment.
Lymphangioma (lymphatic malformation, LM), a congenital vascular disease, is a low-flow vascular abnormality in lymphatic diseases that is characterized by excessive growth of lymphatic tissue during prenatal and postpartum development. The incidence rate of LM is ~1:2000–4000, with no variation between genders and races. Most patients (80–90%) are diagnosed before the age of two. The clinical manifestations of lymphangioma are quite different among patients, varying from local swelling leading to superficial mass to a large area of diffuse infiltrating lymphatic channel abnormalities resulting in elephantiasis. Cervicofacial LM can cause facial elephantiasis, and in some severe cases, it can lead to serious disfigurement of the face. Tongue LM can lead to mandibular overgrowth and occlusal asymmetry, and oral and cervical LM can cause obstructive acute respiratory distress and life-threatening situations. Orbital LM may lead to decreased vision, decreased extraocular muscle movement, ptosis and exophthalmos. LM of the extremities can trigger swelling or gigantism, accompanied by overgrowth of soft tissue and bones. LM usually grows slowly and steadily, but under certain conditions, such as infection, hormonal changes or trauma, it can grow explosively and become a life-threatening disease requiring immediate treatment.
Atzera begirako ikerketa honetan, lymphangioma zuten eta 1999ko urtarriletik 2004ko abendura bleomizina soluzio injekzioak tratatu zituzten 24 ume aztertu genituen. Lesio gehienak (%63) erabat desagertu ziren, %21ek erantzun ona izan zuten eta %16k. Ez zuen ondo erantzun. Bi pazientek tumorea itzuli zuten geroago, eta beste bik injekzioak jaso zituzten lekuan abszeoak izan zituzten. Zorionez, ez genuen beste arazo edo albo-ondorio handirik ikusi.
This is a retrospective study of 24 children diagnosed with lymphangioma and treated with intralesional injection of bleomycin aqueous solution from January 1999 to December 2004. Complete resolution was seen in 63% (15/24) of lesions, 21% (5/24) had good response and 16% (4/24) had poor response. The tumour recurred in 2 patients. Two other patients had abscess formation at the site of injection. No other serious complications or side effects were observed.
This is a retrospective study of 24 children diagnosed with lymphangioma and treated with intralesional injection of bleomycin aqueous solution from January 1999 to December 2004. Complete resolution was seen in 63% (15/24) of lesions, 21% (5/24) had good response and 16% (4/24) had poor response. The tumour recurred in 2 patients. Two other patients had abscess formation at the site of injection. No other serious complications or side effects were observed.
Lymphangioma mota nagusiak hauek dira - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Hauek haurren tumore baskular onberen % 26 inguru dira, baina ez dira hain ohikoak helduengan. Lymphangioma circumscriptum , ohikoena denak, hodi linfatikoak azaletik irteten dira, igelaren kumearen antzeko likidoz betetako besikula argiak eratuz, ehunen hanturarekin batera. Normalean, gorputz-adarrak, enborra eta besapeak bezalako sare linfatiko aberatsa duten eremuetan agertzen da. 71 urteko emakume bat etorri zen gure klinikara hanketako hantura iraunkorra, genitaleetan kolpe arrosak, azkura eta likido linfatiko isuriarekin. Kolpe guztiak kirurgikoki kendu genituen aldebiko labiektomia handia izeneko prozedura batekin, Collesen faszia mailan geldituz, klitorisa eta fourchette mantenduz.
The predominant types of lymphangioma are lymphangioma circumscriptum (LC), cavernous lymphangioma, cystic hygroma, and lymphangioendothelioma. These entities account for approximately 26% of benign vascular tumors in children but are rarer in adults. LC is the most common form of cutaneous lymphangioma and is characterized by superficial lymphatic ducts protruding through the epidermis. This condition results in clusters of clear fluid-filled vesicles resembling frog spawn and associated tissue edema. It is usually found on the proximal extremity, trunk, and axilla, which has an abundant lymphatic system. A 71-year-old female presented to our outpatient clinic with persistent edema of both lower limbs, clusters of pink labial papules, pruritus, and watery lymph oozing. We removed all the papules by performing bilateral major labiectomy down to the level of Colles' fascia, sparing the clitoris and fourchette.
The predominant types of lymphangioma are lymphangioma circumscriptum (LC), cavernous lymphangioma, cystic hygroma, and lymphangioendothelioma. These entities account for approximately 26% of benign vascular tumors in children but are rarer in adults. LC is the most common form of cutaneous lymphangioma and is characterized by superficial lymphatic ducts protruding through the epidermis. This condition results in clusters of clear fluid-filled vesicles resembling frog spawn and associated tissue edema. It is usually found on the proximal extremity, trunk, and axilla, which has an abundant lymphatic system. A 71-year-old female presented to our outpatient clinic with persistent edema of both lower limbs, clusters of pink labial papules, pruritus, and watery lymph oozing. We removed all the papules by performing bilateral major labiectomy down to the level of Colles' fascia, sparing the clitoris and fourchette.
