Lymphangioma
Lymphangioma eru vansköpun á sogæðakerfinu sem einkennist af þunnum, blöðrum. Þessar vansköpun geta komið fram á hvaða aldri sem er og á hvaða líkamshluta sem er, en 90 % koma fram hjá börnum yngri en 2 ár og tengjast höfði og hálsi. Eitilfrumuæxli eru almennt greind fyrir fæðingu með ómskoðun fósturs. Áunnin eitilfrumuæxli geta stafað af áverka, bólgu eða sogæðastíflu. Þar sem þau geta ekki orðið illkynja, eru eitilfrumuæxli venjulega aðeins meðhöndluð af snyrtifræðilegum ástæðum.
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ReferencesLymphangioma er einnig þekkt sem sogæðavandamál (LM). Það er æðasjúkdómur sem er til staðar frá fæðingu. Hann einkennist af óeðlilegum vexti sogæðavefs fyrir og eftir fæðingu. Lymphangioma hefur áhrif á um það bil 1 af hverjum 2000 til 4000 manns, án marktæks munar milli kynja eða kynþátta. Flest tilfelli (80–90 %) greina má áður en barnið náð tveggja ára aldri. Einkennin eru mjög breytileg, allt frá staðbundinni þrota til víðtækra frávika í sogæðagöngum, sem stundum leiða til alvarlegrar bólgu sem kallast fílasjúkdómur. Til dæmis getur lymphangioma í hálsi og andliti valdið bólgu í andliti og í alvarlegum tilfellum myndað útfellingar. Þegar það hefur áhrif á tunguna getur það leitt til ofvaxtar í kjálka og skekktra tanna. Í munni og hálsi getur það valdið öndunarerfiðleikum og lífshættulegum neyðartilvikum. Í augum getur það valdið sjónskerðingu, takmörkuðum augnhreyfingum, horandi augnlokum og bólgnum augum. Þátttaka í útlínum getur valdið bólgu og óeðlilegum vexti í vefjum og beinum. Þetta æxli vex venjulega hægt, en sýkingar, hormónabreytingar eða meiðsli geta valdið hröðum vexti, sem veldur lífshættulegri áhættu og krefst bráðrar meðferðar.
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.
Í þessari afturvirku rannsókn skoðuðum við 24 krakka sem voru með lymphangioma og fengu meðferð með bleomycin‑lausn frá janúar 1999 til desember 2004. Flestar skemmdirnar (63 %) náðu fullkomnum bata, 21 % sýna góða svörun og 16 % svaraði ekki vel. Tveir sjúklingar fengu æxlið aftur síðar og tveir aðrir þurftu ígerð þar sem þeir fengu sprauturnar. Sem betur fer sáum við engin önnur alvarleg vandamál eða aukaverkanir.
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.
Helstu tegundir lymphangioma eru sem hér segir – lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Þetta eru um 26 % góðkynja æðaæxla hjá börnum, en eru sjaldgæfari hjá fullorðnum. Lymphangioma circumscriptum, algengasta tegundin, sýnir sogæðar sem standa út í gegnum húðina og mynda tærar, vökvafylltar blöðrur svipaðar froskahrogn, ásamt vefjabólgu. Það birtist venjulega á svæðum með ríkt sogæðakerfi, eins og útlimum, bol og handarkrika. 71 ára gömul kona kom á heilsugæslustöðina okkar með viðvarandi bólgu í fótleggjum, bleika hnúða á kynfærum, kláða og lekandi eitlavökva. Við fjarlægðum alla högg með skurðaðgerð sem kallast tvíhliða meiriháttar labiectomy, stöðvuðum á Colles' fascia stigi, en varðveittum snípinn og 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.
