Lymphangioma - Lymfangiom
Lymfangiom (Lymphangioma) er misdannelser af lymfesystemet karakteriseret ved læsioner, der er tyndvæggede cyster. Disse misdannelser kan forekomme i alle aldre og kan involvere en hvilken som helst del af kroppen, men 90 % forekommer hos børn under 2 år og involverer hoved og hals. Lymfangiomer diagnosticeres almindeligvis før fødslen ved hjælp af føtal ultralyd. Erhvervede lymfangiomer kan skyldes traumer, betændelse eller lymfatisk obstruktion. Da de ikke har nogen chance for at blive ondartede, behandles lymfangiomer normalt kun af kosmetiske årsager.
☆ I 2022 Stiftung Warentest-resultaterne fra Tyskland var forbrugernes tilfredshed med ModelDerm kun lidt lavere end med betalte telemedicinske konsultationer.
References
Lymphangioma er også kendt som lymfatisk misdannelse (LM) . Det er en vaskulær lidelse, der er til stede fra fødslen. Det er karakteriseret ved unormal vækst af lymfevæv før og efter fødslen. Lymphangioma påvirker omkring 1 ud af 2000 til 4000 mennesker, uden nogen signifikant forskel mellem køn eller racer. De fleste tilfælde (80-90%) diagnosticeres før 2 års alderen. Symptomerne varierer meget, fra lokal hævelse til omfattende abnormiteter i lymfekanalerne, som nogle gange fører til alvorlig hævelse kendt som elefantiasis. For eksempel kan lymphangioma i nakke og ansigt forårsage hævelse i ansigtet og i alvorlige tilfælde vansiring. Når det påvirker tungen, kan det føre til kæbeovervækst og skæve tænder. I mund og hals kan det give vejrtrækningsproblemer og livstruende nødsituationer. I øjnene kan det forårsage synstab, begrænset øjenbevægelse, hængende øjenlåg og svulmende øjne. Lemmer involvering kan forårsage hævelse og unormal vækst af væv og knogler. Denne tumor vokser normalt langsomt, men infektioner, hormonelle ændringer eller skader kan forårsage hurtig vækst, hvilket udgør livstruende risici, der kræver 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 denne retrospektive undersøgelse gennemgik vi 24 børn, der havde lymphangioma og blev behandlet med injektioner af bleomycinopløsning fra januar 1999 til december 2004. De fleste læsioner (63 %) forsvandt fuldstændigt, 21 % fik et godt svar, og 16 % svarede ikke godt. To patienter fik svulsten tilbage senere, og to andre fik bylder, hvor de fik injektionerne. Heldigvis så vi ikke andre store problemer eller bivirkninger.
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.
Hovedtyperne af lymphangioma er som følger - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Disse udgør omkring 26% af godartede vaskulære tumorer hos børn, men er mindre almindelige hos voksne. Lymphangioma circumscriptum , den hyppigste type, viser lymfekanaler, der stikker ud gennem huden og danner klare væskefyldte vesikler, der ligner frøgyder, sammen med hævelse af væv. Det optræder typisk på områder med et rigt lymfatisk netværk som lemmer, krop og armhuler. En 71-årig kvinde kom til vores klinik med vedvarende hævede ben, lyserøde knopper på kønsorganerne, kløe og utæt lymfevæske. Vi fjernede kirurgisk alle knopper med en procedure kaldet bilateral større labiectomy, stoppede ved Colles' fascia niveau, mens klitoris og fourchette bevares.
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.
