Lymphangioma - Lymfangióm
Lymfangióm (Lymphangioma) sú malformácie lymfatického systému charakterizované léziami, ktoré sú tenkostennými cystami. Tieto malformácie sa môžu vyskytnúť v akomkoľvek veku a môžu zahŕňať akúkoľvek časť tela, ale 90 % sa vyskytuje u detí mladších ako 2 roky a zahŕňajú hlavu a krk. Lymfangiómy sa bežne diagnostikujú pred narodením pomocou ultrasonografie plodu. Získané lymfangiómy môžu byť výsledkom traumy, zápalu alebo lymfatickej obštrukcie. Keďže lymfangiómy nemajú šancu stať sa malígnymi, zvyčajne sa liečia len z kozmetických dôvodov.
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References
Lymphangioma je tiež známa ako lymfatická malformácia (LM) . Ide o vaskulárnu poruchu prítomnú od narodenia. Je charakterizovaný abnormálnym rastom lymfatického tkaniva pred a po pôrode. Lymphangioma postihuje približne 1 z 2 000 až 4 000 ľudí bez významného rozdielu medzi pohlaviami alebo rasami. Väčšina prípadov (80–90 %) je diagnostikovaná pred dosiahnutím veku dvoch rokov. Príznaky sa značne líšia, od lokalizovaného opuchu až po rozsiahle abnormality v lymfatických kanáloch, čo niekedy vedie k silnému opuchu známemu ako elefantiáza. Napríklad lymphangioma na krku a tvári môže spôsobiť opuch tváre a v závažných prípadoch aj znetvorenie. Keď postihne jazyk, môže to viesť k prerastaniu čeľuste a nesprávnemu zarovnaniu zubov. V ústach a na krku môže spôsobiť problémy s dýchaním a život ohrozujúce stavy. V očiach môže spôsobiť stratu zraku, obmedzený pohyb očí, ovisnuté viečka a vypuklé oči. Postihnutie končatín môže spôsobiť opuch a abnormálny rast tkanív a kostí. Tento nádor zvyčajne rastie pomaly, ale infekcie, hormonálne zmeny alebo zranenia môžu spôsobiť rýchly rast, čo predstavuje život ohrozujúce riziká, ktoré si vyžadujú urgentnú liečbu.
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.
V tejto retrospektívnej štúdii sme zhodnotili 24 detí, ktoré mali lymphangioma a boli liečené injekciami roztoku bleomycínu od januára 1999 do decembra 2004. Väčšina lézií (63 %) úplne vymizla, 21 % malo dobrú odpoveď a 16 % nereagoval dobre. Dvom pacientom sa nádor vrátil neskôr a dvaja ďalší dostali abscesy tam, kde dostali injekcie. Našťastie sme nezaznamenali žiadne ďalšie veľké problémy alebo vedľajšie účinky.
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.
Hlavné typy lymphangioma sú nasledovné - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Tieto tvoria približne 26 % benígnych vaskulárnych nádorov u detí, ale u dospelých sú menej časté. Lymphangioma circumscriptum , najčastejší typ, vykazuje lymfatické kanáliky vyčnievajúce cez kožu, tvoriace priehľadné vezikuly naplnené tekutinou podobné žabiemu poteru spolu s opuchom tkaniva. Typicky sa objavuje na miestach s bohatou lymfatickou sieťou, ako sú končatiny, trup a podpazušie. Na našu ambulanciu prišla 71-ročná žena s pretrvávajúcim opuchom nôh, ružovými hrbolčekmi na genitáliách, svrbením a vytekajúcou lymfatickou tekutinou. Chirurgicky sme odstránili všetky hrbole pomocou postupu nazývaného bilaterálna veľká labiektómia, pričom sme sa zastavili na úrovni Collesovej fascie, pričom sme zachovali klitoris a 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.
