Lymphangioma - Limfangioma
Limfangioma (Lymphangioma) su malformacije limfnog sustava, karakterizirane lezijama koje su ciste tankih stijenki. Ove malformacije mogu se pojaviti u bilo kojoj dobi i zahvatiti bilo koji dio tijela, ali 90 % slučajeva javlja se kod djece mlađe od 2 godine i najčešće zahvaća glavu i vrat. Limfangiomi se obično dijagnosticiraju prenatalno ultrazvučnim pregledom fetusa. Stečeni limfangiomi mogu biti posljedica traume, upale ili limfne opstrukcije. Budući da ne mogu postati maligni, limfangiomi se obično liječe samo iz kozmetičkih razloga.
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ReferencesLymphangioma je također poznata kao limfna malformacija (LM). To je vaskularni poremećaj prisutan od rođenja. Karakterizira ga abnormalni rast limfnog tkiva prije i nakon rođenja. Lymphangioma pogađa oko 1 od 2000–4000 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, a u teškim slučajevima i deformitet. Kada zahvati jezik, može dovesti do prekomjernog rasta čeljusti i nepravilnog položaja zuba. U ustima i vratu može uzrokovati probleme s disanjem te hitne slučajeve, opasne po život. U očima može uzrokovati gubitak vida, ograničeno kretanje očiju, spuštene kapke i izbočene oči. Zahvaćenost ekstremiteta može uzrokovati oticanje te abnormalni rast tkiva i kostiju. Ovaj tumor obično raste sporo, ali infekcije, hormonske promjene ili ozljede mogu potaknuti brzi rast, što predstavlja rizik po život i zahtijeva 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 (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.
U ovoj retrospektivnoj studiji pregledali smo 24 dijete koja su imala lymphangioma i liječena injekcijama otopine bleomycin od siječnja 1999. do prosinca 2004. Većina lezija (63 %) potpuno je nestala, 21 % je imalo dobar odgovor, a 16 % nije dobro reagiralo. Kod dvojice pacijenata tumor se kasnije vratio, a kod druga dva su se razvili apscesi na mjestima gdje su primili injekcije. Srećom, nismo primijetili druge ozbiljne 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.
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.
Glavni tipovi limfangioma su sljedeći – lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma i lymphangioendothelioma. Oni čine oko 26 % benignih vaskularnih tumora u djece, ali su rjeđi kod odraslih. Lymphangioma circumscriptum, najčešći tip, pokazuje limfne kanale koji se protežu kroz kožu, tvoreći prozirne mjehuriće ispunjene tekućinom slične mjestu žaba, zajedno s oteklinom tkiva. Obično se pojavljuje na područjima s bogatom limfnom mrežom, poput udova, trupa i pazuha. U našu kliniku je došla 71‑godina žena s kroničnim oticanjem nogu, ružičastim izbočinama na genitalijama, svrbežom i curenjem limfne tekućine. Kirurški smo uklonili sve izbočine zahvatom koji se naziva bilateralna velika labiektomija, zaustavljajući se na razini Colles' fascije, a pritom smo sačuvali klitoris i labija.
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.
