Lymphangioma - Limfangioma
Limfangioma (Lymphangioma) sò malformazioni di u sistema linfaticu caratterizati da lesioni chì sò cisti di pareti sottili. Sti malformazioni ponu accadenu à ogni età è ponu involucrà ogni parte di u corpu, ma 90 % si trovanu in i zitelli di menu di 2 anni è implicanu a testa è u collu. I linfangiomi sò comunemente diagnosticati prima di nascita cù ultrasonografia fetale. I linfangiomi acquistati ponu esse dovuti à traumi, inflammazioni o ostruzzione linfatica. Siccome ùn anu micca a possibilità di diventà maligni, i linfangiomi sò generalmente trattati solu per ragioni cosmetichi.
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ReferencesLymphangioma hè ancu cunnisciutu cum’è malformazione linfatica (LM). Hè un disordine vascolariu presente da a nascita. Hè caratterizatu da una crescita anormale di u tissutu linfaticu prima è dopu a nascita. Lymphangioma affetta circa 1 persona su 2000 à 4000, senza differenza significativa trà i sessi o e razze. A maiò parte di i casi (80‑90 %) sò diagnosticati prima di dui anni. I sintomi varianu assai, da l'infiammazione localizata à l'anormalità estensiva in i canali linfatici, à volte chì portanu à una forma severa di gonfiore cunnisciuta cum’è elefantiasis. Per esempiu, un lymphangioma in u collu è a faccia pò causà gonfiore faciale è, in casi severi, deformazione. Quandu affetta a lingua, pò purtà à una crescita di a mandibula è denti mal allineati. In a bocca è u collu, pò causà prublemi di respirazione è emergenze vitale. In l'ochji, pò causà perdita di vista, muvimentu limitatu di l'ochji, palpebre cadenti è occhi sbulicati. L'implicazione di i membri pò causà gonfiore è crescita anormale di tessuti è ossa. Stu tumore di solitu cresce lentamente, ma infezioni, cambiamenti hormonali o ferite ponu causà una rapida crescita, chì ponenu risichi per a vita è richiedenu trattamentu 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.
In questu studiu retrospettivu, avemu rivisu 24 zitelli chì avianu lymphangioma è sò stati trattati cù iniezioni di soluzione di bleomicina da ghjennaghju 1999 à dicembre 2004. A maiò parte di e ferite (63 %) si n’andò cumpletamente, u 21 % hà avutu una bona risposta, è u 16 % ùn hà micca rispostu bè. Dui pazienti anu avutu una recidiva di tumore dopu, è dui altri anu sviluppatu asscessi in i siti d’iniezione. Fortunatamente, ùn avemu micca vistu altri grandi prublemi o effetti secundarii.
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.
I tipi principali di lymphangioma sò i seguenti: lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Questi custituiscenu circa u 26 % di i tumori vascolari benigni in i zitelli, ma sò menu cumuni in l'adulti. Lymphangioma circumscriptum, u tipu più frequente, mostra i dotti linfatici chì sporgenu da a pelle, furmendu vesiculi chjaru pieni di fluidu simile à a spuma di rana, inseme cù gonfiore di i tessuti. Tipicamenti, appare in e zone cù una ricca reta linfatica, cum'è i membri, u troncu è l'ascelle. Una donna di 71 anni hè ghjunta à a nostra clinica cù gonfiore persistente di a gamba, bumps rosati nantu à i genitali, pruritu è perdita di fluidu linfaticu. On a supprimé chirurgicalement toutes les bosses grâce à une procédure appelée labiectomie majeure bilatérale, en s'arrêtant à l'échelle du fascia de Colles, tout en conservant le clitoris et la 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.
