Lymphangioma - Lümfangioom
Lümfangioom (Lymphangioma) on lümfisüsteemi väärareng, mida iseloomustavad õhukeseseinalised tsüstid. Need väärarengud võivad ilmneda igas vanuses ja hõlmata mis tahes kehaosa, kuid 90 % esineb alla kahe‑aastastel lastel, peamiselt peas ja kaelas. Lümfangioomid diagnoositakse tavaliselt enne sündi loote ultraheliuuringu abil. Omandatud lümfangioomid võivad tuleneda traumast, põletikust või lümfisüsteemi obstruktsioonist. Kuna need ei muutu pahaloomulisteks, ravitakse lümfangioomi tavaliselt ainult kosmeetilistel põhjustel.
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ReferencesLymphangioma on tuntud ka kui lümfisüsteemi väärareng (LM). See on vaskulaarne haigus, mis esineb sünnist saati. Seda iseloomustab lümfikoele ebanormaalne kasv enne ja pärast sündi. Lymphangioma mõjutab umbes 1 inimest 2000–4000‑st, ilma märkimisväärse soo- või rassi‑erinevuseta. Enamik juhtudest (80–90 %) diagnoositakse enne kaheaastaseks saamist. Sümptomid on väga erinevad: alates lokaalsest tursest kuni ulatuslike kõrvalekalleteni lümfikanalites, mis mõnikord põhjustavad tõsist turset, mida nimetatakse elefantiasiseks. Näiteks kaela‑ ja näol paiknev lymphangioma võib põhjustada näo turset ning rasketel juhtudel moonutusi. Kui see mõjutab keelt, võib see põhjustada lõualuu ülekasvu ja hammaste valeasendust. Suus ja kaelas võib see põhjustada hingamisraskusi ning eluohtlikke hädaolukordi. Silmades võib see viia nägemise kaotuse, piiratud silmade liikumise, rippuvate silmalaugude ja punniste silmade tekkeni. Jäsemete haaratus võib põhjustada kudede ja luude turset ning ebanormaalset kasvu. See kasvaja kasvab tavaliselt aeglaselt, kuid infektsioonid, hormonaalsed muutused või vigastused võivad põhjustada kiiret kasvamist, tekitades eluohtlikke riske, mis nõuavad kiiret ravi.
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.
Selles retrospektiivses uuringus vaatasime läbi 24 last, kellel oli lymphangioma ja keda raviti bleomycinilahuse süstidega 1999. aasta jaanuarist kuni 2004. aasta detsembrini. Enamik kahjustustest (63 %) kadus täielikult, 21 % näitas hea vastust ja 16 % ei vastanud hästi. Kahel patsiendil tekkis kasvaja hiljem tagasi, ning kahel teisel tekkis süstimise kohas abstsess. Õnneks ei täheldatud muid suuri probleeme ega kõrvaltoimeid.
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.
Peamised lymphangioma tüübid on järgmised – lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Need moodustavad umbes 26 % laste healoomulistest vaskulaarsetest kasvajatest, kuid on harvemad täiskasvanutel. Lymphangioma circumscriptum, kõige sagedasem tüüp, ilmneb läbi naha väljaulatuvate lümfikoondadena, mis moodustavad konnakudedele sarnaseid selge vedelikuga täidetud vesikuid koos kudede tursega. Tavaliselt esineb see piirkondades, kus lümfisüsteem on rikkalik, näiteks jäsemed, kehatüvi ja kaenlaalused. Meie kliinikusse tuli 71‑aastane naine, kellel oli pidev jalgade turse, roosad punnid suguelunditel, sügelus ja lümfivedeliku lekkimine. Eemaldasime kirurgiliselt kõik punnid protseduuriga, mida nimetatakse kahepoolseks suureks labiektoomiaks, peatudes Colles'i fastsia tasemel, säilitades samal ajal kliitori ja fourchette'i.
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.
