Lymphangioma - Limfangiom
Limfangiom (Lymphangioma) so malformacije limfnega sistema, za katere so značilne lezije v obliki tankostenskih cist. Te malformacije se lahko pojavijo v kateri koli starosti in lahko zajamejo kateri koli del telesa, vendar se 90 % pojavi pri otrocih, mlajših od 2 let, in pogosto zajame glavo ter vrat. Limfangiome običajno odkrijejo pred rojstvom z ultrazvokom ploda. Pridobljeni limfangiomi so lahko posledica travme, vnetja ali limfne obstrukcije. Ker ne morejo postati maligni, se limfangiomi običajno zdravijo le iz kozmetičnih razlogov.
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ReferencesLymphangioma je znana tudi kot limfna malformacija (LM). Gre za vaskularno motnjo, prisotno od rojstva. Zanj je značilna nenormalna rast limfnega tkiva pred in po rojstvu. Lymphangioma prizadene približno 1 od 2000 do 4000 ljudi, brez bistvenih razlik med spoloma ali rasami. Večina primerov (80–90 %) se diagnosticira pred dvema letoma. Simptomi se zelo razlikujejo – od lokalne otekline do obsežnih nepravilnosti v limfnih kanalih, ki včasih vodijo do hude otekline, znane kot elefantijaza. Na primer, lymphangioma na vratu in obrazu lahko povzroči otekanje obraza, v hujših primerih pa iznakaženost. Ko je prizadet jezik, lahko pride do zaraščanja čeljusti in nepravilno poravnanih zob. V ustih in vratu lahko povzroči težave z dihanjem ter življenjsko nevarne nujne primere. V očeh lahko povzroči izgubo vida, omejeno gibanje oči, povlečene veke in izbuljene oči. Prizadetost okončin lahko povzroči otekanje ter nenormalno rast tkiv in kosti. Ta tumor običajno raste počasi, vendar lahko okužbe, hormonske spremembe ali poškodbe povzročijo hitro rast, kar predstavlja življenjsko nevarno tveganje in zahteva nujno zdravljenje.
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 tej retrospektivni študiji smo pregledali 24 otrok z lymphangiomo, ki so bili zdravljeni z injekcijami raztopine bleomycin od januarja 1999 do decembra 2004. Večina lezij (63 %) je popolnoma izginila, 21 % je dosegla dober odziv, 16 % pa se ni dobro odzvalo. Tumor se je pozneje vrnil dvema bolnikoma, pri dveh drugih pa so se na mestu injekcij pojavili abscesi. Na srečo nismo opazili večjih zapletov ali stranskih učinkov.
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.
Glavne vrste limfangiom so naslednje – lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Te predstavljajo približno 26 % benignih žilnih tumorjev pri otrocih, vendar so manj pogoste pri odraslih. Lymphangioma circumscriptum, najpogostejši tip, prikazuje limfne kanale, ki štrlijo skozi kožo in tvorijo čiste vezikle, napolnjene s tekočino, podobne žabjemu mrestu, skupaj z oteklino tkiva. Običajno se pojavi na predelih z bogato limfno mrežo, kot so okončine, trup in pazduhe. V našo ambulanto je prišla 71‑letna ženska z nenehnim otekanjem nog, rožnatimi izboklinami na genitalijah, srbečico in iztekanjem limfne tekočine. Kirurško smo odstranili vse izbokline s postopkom, imenovanim dvostranska velika labiektomija, pri čemer smo se ustavili na nivoju Collesove fascije, pri tem pa ohranili klitoris in štirikolesnik.
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.
