Lymphangioma - Limfangioma
Limfangioma (Lymphangioma) adalah kecacatan sistem limfa yang dicirikan oleh lesi yang berdinding nipis sista. Kecacatan ini boleh berlaku pada mana-mana umur dan mungkin melibatkan mana-mana bahagian badan, tetapi 90% berlaku pada kanak-kanak berumur kurang daripada 2 tahun dan melibatkan kepala dan leher. Limfangioma biasanya didiagnosis sebelum kelahiran menggunakan ultrasonografi janin. Limfangioma yang diperoleh mungkin disebabkan oleh trauma, keradangan, atau halangan limfa. Oleh kerana mereka tidak mempunyai peluang untuk menjadi malignan, limfangioma biasanya dirawat atas sebab kosmetik sahaja.
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References
Lymphangioma juga dikenali sebagai malformasi limfa (LM) . Ia adalah gangguan vaskular yang wujud sejak lahir. Ia dicirikan oleh pertumbuhan abnormal tisu limfa sebelum dan selepas kelahiran. Lymphangioma menjejaskan kira-kira 1 dalam 2000 hingga 4000 orang, tanpa perbezaan ketara antara jantina atau kaum. Kebanyakan kes (80–90%) didiagnosis sebelum umur dua tahun. Gejala berbeza-beza secara meluas, daripada bengkak setempat kepada keabnormalan yang meluas dalam saluran limfa, kadangkala membawa kepada bengkak teruk yang dikenali sebagai elephantiasis. Contohnya, lymphangioma di leher dan muka boleh menyebabkan muka bengkak, dan dalam kes yang teruk, kecacatan. Apabila ia menjejaskan lidah, ia boleh menyebabkan rahang membesar dan gigi tidak sejajar. Di mulut dan leher, ia boleh menyebabkan masalah pernafasan dan kecemasan yang mengancam nyawa. Di mata, ia boleh menyebabkan kehilangan penglihatan, pergerakan mata terhad, kelopak mata terkulai dan mata membonjol. Penglibatan anggota badan boleh menyebabkan bengkak dan pertumbuhan abnormal tisu dan tulang. Tumor ini biasanya tumbuh dengan perlahan, tetapi jangkitan, perubahan hormon, atau kecederaan boleh menyebabkan pertumbuhan pesat, menimbulkan risiko yang mengancam nyawa yang memerlukan rawatan segera.
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.
Dalam kajian retrospektif ini, kami menyemak 24 kanak-kanak yang mempunyai lymphangioma dan dirawat dengan suntikan larutan bleomycin dari Januari 1999 hingga Disember 2004. Kebanyakan lesi (63%) hilang sepenuhnya, 21% mendapat tindak balas yang baik, dan 16% tidak bertindak balas dengan baik. Dua pesakit mempunyai tumor kembali kemudian, dan dua lagi mendapat abses di mana mereka mendapat suntikan. Nasib baik, kami tidak melihat sebarang masalah besar atau kesan sampingan.
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.
Jenis utama lymphangioma adalah seperti berikut - lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, lymphangioendothelioma. Ini membentuk kira-kira 26% daripada tumor vaskular benigna pada kanak-kanak tetapi kurang biasa pada orang dewasa. Lymphangioma circumscriptum , jenis yang paling kerap, menunjukkan saluran limfa yang terkeluar melalui kulit, membentuk vesikel berisi cecair yang jelas serupa dengan benih katak, bersama-sama dengan pembengkakan tisu. Ia biasanya muncul di kawasan yang mempunyai rangkaian limfa yang kaya seperti anggota badan, batang dan ketiak. Seorang wanita berusia 71 tahun datang ke klinik kami dengan bengkak kaki yang berterusan, benjolan merah jambu pada kemaluannya, gatal-gatal dan cecair limfa yang bocor. Kami mengeluarkan semua benjolan melalui pembedahan dengan prosedur yang dipanggil labiektomi major bilateral, berhenti pada tahap fascia Colles, sambil mengekalkan kelentit dan 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.
