Lymphangioma - Limfangioma
Limfangioma (Lymphangioma) ir limfātiskās sistēmas anomālijas, ko raksturo bojājumi, kas ir plānsienu cistas. Šīs malformācijas var rasties jebkurā vecumā un var skart jebkuru ķermeņa daļu, bet 90% gadījumu rodas bērniem, kas jaunāki par 2 gadiem, un tie skar galvu un kaklu. Limfangiomas parasti diagnosticē pirms dzimšanas, izmantojot augļa ultrasonogrāfiju. Iegūtās limfangiomas var rasties traumas, iekaisuma vai limfas obstrukcijas rezultātā. Tā kā limfangiomas nav izredzes kļūt par ļaundabīgām, tās parasti ārstē tikai kosmētisku iemeslu dēļ.
☆ 2022. gada Stiftung Warentest rezultātos no Vācijas patērētāju apmierinātība ar ModelDerm bija tikai nedaudz zemāka nekā ar maksas telemedicīnas konsultācijām.
References
Lymphangioma ir pazīstams arī kā limfātiskās malformācijas (LM) . Tā ir asinsvadu slimība, kas pastāv kopš dzimšanas. To raksturo patoloģiska limfātisko audu augšana pirms un pēc dzimšanas. Lymphangioma skar aptuveni 1 no 2000 līdz 4000 cilvēkiem, bez būtiskām atšķirībām starp dzimumiem vai rasēm. Lielākā daļa gadījumu (80–90%) tiek diagnosticēti pirms divu gadu vecuma. Simptomi ir ļoti dažādi, sākot no lokāla pietūkuma līdz plašām limfātisko kanālu anomālijām, kas dažkārt izraisa smagu pietūkumu, ko sauc par ziloni. Piemēram, lymphangioma kaklā un sejā var izraisīt sejas pietūkumu un smagos gadījumos izkropļojumus. Kad tas skar mēli, tas var izraisīt žokļa aizaugšanu un nepareizi novietotus zobus. Mutē un kaklā tas var izraisīt elpošanas problēmas un dzīvībai bīstamas ārkārtas situācijas. Acīs tas var izraisīt redzes zudumu, ierobežotas acu kustības, nokarenus plakstiņus un izspiedušās acis. Ekstremitāšu iesaistīšanās var izraisīt audu un kaulu pietūkumu un patoloģisku augšanu. Šis audzējs parasti aug lēni, bet infekcijas, hormonālās izmaiņas vai traumas var izraisīt strauju augšanu, radot dzīvībai bīstamus riskus, kam nepieciešama steidzama ārstēšana.
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.
Šajā retrospektīvajā pētījumā mēs apskatījām 24 bērnus, kuriem bija lymphangioma un kuri tika ārstēti ar bleomicīna šķīduma injekcijām no 1999. Gada janvāra līdz 2004. Gada decembrim. Lielākā daļa bojājumu (63%) pilnībā izzuda, 21% saņēma labu reakciju un 16% labi neatbildēja. Diviem pacientiem audzējs atgriezās vēlāk, bet diviem citiem, kur viņi saņēma injekcijas, dabūja abscesus. Par laimi, mēs neredzējām nekādas citas lielas problēmas vai blakusparādības.
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.
Galvenie lymphangioma veidi ir šādi - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Tie veido aptuveni 26% no labdabīgiem asinsvadu audzējiem bērniem, bet retāk sastopami pieaugušajiem. Lymphangioma circumscriptum , kas ir visizplatītākais veids, parāda limfas kanālus, kas izvirzīti caur ādu, veidojot dzidrus, ar šķidrumu pildītus pūslīšus, kas līdzīgi varžu nārstam, kopā ar audu pietūkumu. Tas parasti parādās vietās ar bagātīgu limfas tīklu, piemēram, ekstremitātēs, stumbrā un padusēs. Mūsu klīnikā ieradās 71 gadu veca sieviete ar pastāvīgu kāju pietūkumu, rozā pumpiņām uz dzimumorgāniem, niezi un limfas šķidruma noplūdi. Mēs ķirurģiski noņēmām visus izciļņus ar procedūru, ko sauc par divpusēju lielo labiektomiju, apstājoties Kolesa fascijas līmenī, vienlaikus saglabājot klitoru un furšetu.
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.
