Lymphangioma - Limfangioma
Limfangioma (Lymphangioma) són malformacions del sistema limfàtic caracteritzades per lesions que són quists de parets primes. Aquestes malformacions poden ocórrer a qualsevol edat i poden afectar qualsevol part del cos, però el 90% es produeixen en nens menors de 2 anys i afecten el cap i el coll. Els limfangiomes es diagnostiquen habitualment abans del naixement mitjançant ecografia fetal. Els limfangiomes adquirits poden resultar de trauma, inflamació o obstrucció limfàtica. Com que no tenen possibilitats de convertir-se en malignes, els limfangiomes solen tractar-se només per motius estètics.
☆ En els resultats de la Stiftung Warentest d'Alemanya de 2022, la satisfacció dels consumidors amb ModelDerm va ser només lleugerament inferior a la de les consultes de telemedicina de pagament.
References
Lymphangioma també es coneix com a malformació limfàtica (LM) . És un trastorn vascular present des del naixement. Es caracteritza per un creixement anormal del teixit limfàtic abans i després del part. Lymphangioma afecta aproximadament 1 de cada 2000 a 4000 persones, sense cap diferència significativa entre gèneres o races. La majoria dels casos (80-90%) es diagnostiquen abans dels dos anys. Els símptomes varien àmpliament, des d'inflor localitzada fins a grans anomalies en els canals limfàtics, de vegades conduint a una inflor severa coneguda com elefantiasi. Per exemple, lymphangioma al coll i a la cara pot causar inflor facial i, en casos greus, desfiguració. Quan afecta la llengua, pot provocar un creixement excessiu de la mandíbula i dents desalineades. A la boca i al coll, pot causar problemes respiratoris i emergències que amenacen la vida. Als ulls, pot causar pèrdua de visió, moviment ocular limitat, parpelles caigudes i ulls saltats. La afectació de les extremitats pot causar inflor i creixement anormal de teixits i ossos. Aquest tumor acostuma a créixer lentament, però les infeccions, els canvis hormonals o les lesions poden provocar un creixement ràpid, suposant riscos mortals que requereixen tractament urgent.
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.
En aquest estudi retrospectiu, vam revisar 24 nens que tenien lymphangioma i van ser tractats amb injeccions de solució de bleomicina des de gener de 1999 fins a desembre de 2004. La majoria de les lesions (63%) van desaparèixer completament, el 21% va obtenir una bona resposta i el 16% no va respondre bé. Dos pacients van tornar el tumor més tard, i altres dos van tenir abscessos on van rebre les injeccions. Per sort, no vam veure cap altre gran problema o efectes secundaris.
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.
Els principals tipus de lymphangioma són els següents: lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Aquests representen al voltant del 26% dels tumors vasculars benignes en nens, però són menys freqüents en adults. Lymphangioma circumscriptum , el tipus més freqüent, mostra conductes limfàtics que sobresurten a través de la pell, formant vesícules clares plenes de líquid similars a la posta de granota, juntament amb inflor dels teixits. Normalment apareix en zones amb una rica xarxa limfàtica com les extremitats, el tronc i les aixelles. Una dona de 71 anys va venir a la nostra clínica amb una inflor persistent de les cames, cops rosats als genitals, picor i fuites de líquid limfàtic. Vam eliminar quirúrgicament tots els cops amb un procediment anomenat labiectomia major bilateral, aturant-nos a nivell de la fàscia de Colles, tot conservant el clítoris i la 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.
