Lymphangioma - Limfangioma
https://en.wikipedia.org/wiki/Lymphangioma
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References
Recent Progress in Lymphangioma 34976885 NIH
Lymphangioma je također poznata kao limfna malformacija (LM) . To je vaskularni poremećaj prisutan od rođenja. Karakterizira ga abnormalni rast limfnog tkiva prije i poslije rođenja. Lymphangioma pogađa oko 1 od 2000 do 4000 ljudi, bez značajne razlike između spolova ili rasa. Većina slučajeva (80-90%) dijagnosticira se prije druge godine života. Simptomi uvelike variraju, od lokalizirane otekline do opsežnih abnormalnosti u limfnim kanalima, što ponekad dovodi do ozbiljnog oticanja poznatog kao elefantijaza. Na primjer, lymphangioma na vratu i licu može uzrokovati oticanje lica, au teškim slučajevima i unakaženost. Kada utječe na jezik, može dovesti do prekomjernog rasta čeljusti i nepravilnog rasporeda zuba. U ustima i vratu može uzrokovati probleme s disanjem i hitne slučajeve opasne po život. U očima može uzrokovati gubitak vida, ograničeno kretanje očiju, spuštene kapke i izbočene oči. Zahvaćenost ekstremiteta može uzrokovati oticanje i abnormalni rast tkiva i kostiju. Ovaj tumor obično raste sporo, ali infekcije, hormonske promjene ili ozljede mogu uzrokovati brzi rast, predstavljajući rizike opasne po život koji zahtijevaju hitno liječenje.
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: Is intralesional bleomycin sclerotherapy effective? 22279495 NIH
U ovoj retrospektivnoj studiji pregledali smo 24 djece koja su imala lymphangioma i liječena injekcijama otopine bleomicina od siječnja 1999. Do prosinca 2004. Većina lezija (63%) potpuno je nestala, 21% imalo je dobar odgovor, a 16% nije dobro reagirao. Dvojici pacijenata tumor se kasnije vratio, a druga dva su dobila apscese na mjestima gdje su primili injekcije. Srećom, nismo vidjeli nikakve druge velike probleme ili nuspojave.
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.
Surgical Resection of Acquired Vulvar Lymphangioma Circumscriptum - Case reports 24665431 NIH
Glavni tipovi lymphangioma su sljedeći - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Oni čine oko 26% benignih vaskularnih tumora u djece, ali su rjeđi u odraslih. Lymphangioma circumscriptum , najčešći tip, pokazuje limfne kanale koji strše kroz kožu, tvoreći prozirne mjehuriće ispunjene tekućinom slične mrijestu žaba, zajedno s oteklinom tkiva. Obično se pojavljuje na područjima s bogatom limfnom mrežom poput udova, trupa i pazuha. U našu kliniku javila se 71-godišnja žena s stalnim oticanjem nogu, ružičastim izbočinama na genitalijama, svrbežom i curenjem limfne tekućine. Kirurški smo uklonili sve izbočine zahvatom koji se zove bilateralna velika labiektomija, zaustavljajući se na razini Collesove fascije, a pritom smo sačuvali klitoris i četvorku.
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.