Lymphangioma - Lymfangiom
Lymfangiom (Lymphangioma) är missbildningar i lymfsystemet som kännetecknas av lesioner som är tunnväggiga cystor. Dessa missbildningar kan uppstå i alla åldrar och kan involvera vilken del av kroppen som helst, men 90 % förekommer hos barn under 2 år och involverar huvud och nacke. Lymfaniom diagnostiseras vanligtvis före födseln med ultraljudsundersökning av foster. Förvärvade lymfangiom kan bero på trauma, inflammation eller lymfatisk obstruktion. Eftersom de inte har någon chans att bli maligna, behandlas lymfangiom vanligtvis endast av kosmetiska skäl.
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References
Lymphangioma är också känd som lymfatisk missbildning (LM) . Det är en kärlsjukdom som finns från födseln. Det kännetecknas av onormal tillväxt av lymfvävnad före och efter födseln. Lymphangioma påverkar ungefär 1 av 2000 till 4000 personer, utan någon signifikant skillnad mellan kön eller raser. De flesta fall (80–90 %) diagnostiseras före två års ålder. Symtomen varierar kraftigt, från lokal svullnad till omfattande abnormiteter i lymfkanalerna, vilket ibland leder till svår svullnad som kallas elefantiasis. Till exempel kan lymphangioma i nacken och ansiktet orsaka ansiktssvullnad och i svåra fall vanställdhet. När det påverkar tungan kan det leda till överväxt av käken och felinriktade tänder. I mun och hals kan det orsaka andningsproblem och livshotande nödsituationer. I ögonen kan det orsaka synförlust, begränsade ögonrörelser, hängande ögonlock och utbuktande ögon. Extremitetsinblandning kan orsaka svullnad och onormal tillväxt av vävnader och ben. Denna tumör växer vanligtvis långsamt, men infektioner, hormonella förändringar eller skador kan orsaka snabb tillväxt, vilket utgör livshotande risker som kräver akut behandling.
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.
I denna retrospektiva studie granskade vi 24 barn som hade lymphangioma och som behandlades med injektioner av bleomycinlösning från januari 1999 till december 2004. De flesta av lesionerna (63 %) försvann helt, 21 % fick ett bra svar och 16 % svarade inte bra. Två patienter fick tumören tillbaka senare och två andra fick bölder där de fick injektionerna. Som tur var såg vi inga andra stora problem eller biverkningar.
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.
Huvudtyperna av lymphangioma är följande - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Dessa utgör cirka 26 % av godartade vaskulära tumörer hos barn men är mindre vanliga hos vuxna. Lymphangioma circumscriptum , den vanligaste typen, visar lymfatiska kanaler som sticker ut genom huden och bildar klara vätskefyllda blåsor som liknar grodlek, tillsammans med vävnadssvullnad. Det uppträder vanligtvis på områden med ett rikt lymfatiskt nätverk som lemmar, bål och armhålor. En 71-årig kvinna kom till vår klinik med ihållande bensvullnad, rosa knölar på könsorganen, klåda och läckande lymfvätska. Vi tog kirurgiskt bort alla knölar med en procedur som kallas bilateral major labiectomy, och stannade på Colles fascianivå, samtidigt som klitoris och fourchette bevarades.
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.
