Lymphangioma - Linfangioma
Linfangioma (Lymphangioma) são malformações do sistema linfático caracterizadas por cistos de paredes finas. Essas malformações podem ocorrer em qualquer idade e envolver qualquer parte do corpo; porém, 90 % dos casos ocorrem em crianças com menos de 2 anos e afetam principalmente a cabeça e o pescoço. Os linfangiomas são comumente diagnosticados antes do nascimento por meio de ultrassonografia fetal. Os linfangiomas adquiridos podem resultar de trauma, inflamação ou obstrução linfática. Como não apresentam risco de malignização, os linfangiomas geralmente são tratados apenas por motivos estéticos.
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Nos resultados do Stiftung Warentest de 2022 da Alemanha, a satisfação do consumidor com o ModelDerm foi apenas ligeiramente inferior do que com as consultas pagas de telemedicina.
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ReferencesLymphangioma, também conhecida como malformação linfática (LM), é um distúrbio vascular presente desde o nascimento. É caracterizada pelo crescimento anormal do tecido linfático antes e depois do nascimento. Lymphangioma afeta cerca de 1 em 2.000 a 4.000 pessoas, sem diferença significativa entre gêneros ou raças. A maioria dos casos (80–90 %) é diagnosticada antes dos dois anos de idade. Os sintomas variam amplamente, desde inchaço localizado até anomalias extensas nos canais linfáticos, podendo, em alguns casos, levar a um edema grave conhecido como elefantíase. Por exemplo, Lymphangioma no pescoço e no rosto pode causar inchaço facial e, em casos graves, desfiguração. Quando afeta a língua, pode provocar crescimento excessivo da mandíbula e dentes desalinhados. Na boca e no pescoço, pode gerar problemas respiratórios e emergências potencialmente fatais. Nos olhos, pode causar perda de visão, limitação do movimento ocular, ptose palpebral e proptose. O envolvimento dos membros pode resultar em inchaço e crescimento anormal de tecidos e ossos. Esse tumor geralmente cresce lentamente, mas infecções, alterações hormonais ou lesões podem acelerar seu crescimento, apresentando riscos fatais que requerem tratamento urgente.
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 crianças que apresentaram linfangioma (lymphangioma) e foram tratadas com injeções de solução de bleomicina (bleomycin) de janeiro de 1999 a dezembro de 2004. A maioria das lesões (63%) desapareceu completamente, 21% obtiveram boa resposta e 16% apresentaram resposta insatisfatória. Dois pacientes tiveram recidiva do tumor mais tarde e outros dois desenvolveram abscessos no local das injeções. Felizmente, não observamos nenhum outro problema grave ou efeito colateral.
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 lymphangioma são os seguintes: lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma e lymphangioendothelioma. Eles representam cerca de 26 % dos tumores vasculares benignos em crianças, mas são menos comuns em adultos. O lymphangioma circumscriptum, o tipo mais frequente, apresenta dutos linfáticos que se projetam através da pele, formando vesículas transparentes cheias de líquido, semelhantes a ovos de sapo, acompanhadas de edema dos tecidos. Normalmente aparece em áreas com rica rede linfática, como membros, tronco e axilas. Uma mulher de 71 anos compareceu à nossa clínica com edema persistente nas pernas, lesões rosadas nos órgãos genitais, prurido e vazamento de fluido linfático. Removemos cirurgicamente todas as protuberâncias por meio de um procedimento denominado labiectomia maior bilateral, parando no nível da fáscia de Colles, preservando o clitóris 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.
