Lymphangioma - Linfangioma
Linfangioma (Lymphangioma) son malformacións do sistema linfático caracterizadas por lesións que consisten en quistes de paredes finas. Estas malformacións poden ocorrer a calquera idade e afectar a calquera parte do corpo, pero o 90 % ocorre en nenos menores de 2 anos e afecta a cabeza e ao pescozo. Os linfangiomas son comunmente diagnosticados antes do nacemento mediante ecografía fetal. Os linfangiomas adquiridos poden resultar de trauma, inflamación ou obstrución linfática. Dado que non teñen posibilidades de converterse en malignos, os linfangiomas adoitan tratarse só por motivos estéticos.
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ReferencesLymphangioma tamén se coñece como malformación linfática (LM). É un trastorno vascular presente dende o nacemento. Caracterízase por un crecemento anormal do tecido linfático antes e despois do nacemento. Lymphangioma afecta aproximadamente a 1 de cada 2000‑4000 persoas, sen diferenzas significativas entre xéneros ou razas. A maioría dos casos (80‑90 %) diagnostícanse antes dos dous anos. Os síntomas varían moito, dende inchazo localizado ata anomalías extensas nas canles linfáticas, que ás veces provocan inchazo grave coñecido como elefantiasis. Por exemplo, lymphangioma no pescozo e na cara pode causar inchazo facial e, en casos graves, desfiguración. Cando afecta a lingua, pode provocar un crecemento excesivo da mandíbula e dentes desalineados. Na boca e no pescozo, pode causar problemas respiratorios e emerxencias que ameazan a vida. Nos ollos, pode causar perda de visión, movemento ocular limitado, pálpebras caídas e ollos saltones. A afectación das extremidades pode causar inchazo e crecemento anormal de tecidos e ósos. Este tumor adoita crecer lentamente, pero as infeccións, os cambios hormonais ou as lesións poden provocar un crecemento rápido, poñendo en perigo a vida e requirindo tratamento urxente.
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.
Neste estudo retrospectivo revisamos 24 nenos que tiñan lymphangioma e foron tratados con inxeccións de bleomycin desde xaneiro de 1999 ata decembro de 2004. A maioría das lesións (63 %) desapareceron por completo, o 21 % obtivo unha boa resposta e o 16 % non respondeu adecuadamente. En dous pacientes o tumor reapareceu máis tarde, e en outros dous se produciron abscesos nas áreas onde se administraron as inxeccións. Afortunadamente, non observamos ningún outro problema grave nin efectos secundarios.
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.
Os principais tipos de linfangioma son os seguintes: linfangioma circunscripto, linfangioma cavernoso, higroma quístico, linfangiolendoteliooma. Representan aproximadamente o 26 % dos tumores vasculares benignos nos nenos, pero son menos frecuentes nos adultos. O linfangioma circunscripto, o tipo máis frecuente, presenta conductos linfáticos que sobresalen na pel, formando vesículas claras cheas de líquido semellantes a pequenas ampollas, xunto con inflamación dos tecidos. Normalmente aparece en áreas con abundante rede linfática, como os membros, o tronco e as axilas. Unha muller de 71 anos acudiu á nosa clínica con edema persistente nas pernas, protuberancias rosadas nos xenitais, prurito e fuga de líquido linfático. Elimináronse cirurxicamente todas as protuberancias mediante un procedemento chamado labiectomía maior bilateral, ata o nivel da fascia de Colles, conservando o clítoris e o 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.
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.
