Lymphangioma - Limfangioma
Limfangioma (Lymphangioma) adalah malformasi sistem limfatik yang ditandai dengan lesi berupa kista berdinding tipis. Malformasi ini dapat terjadi pada semua usia dan dapat mengenai bagian tubuh mana pun, namun 90 % kasus terjadi pada anak‑anak berusia kurang dari 2 tahun, terutama pada kepala dan leher. Limfangioma biasanya didiagnosis sebelum lahir menggunakan ultrasonografi janin. Limfangioma yang muncul mungkin disebabkan oleh trauma, peradangan, atau obstruksi limfatik. Karena tidak bersifat ganas, limfangioma biasanya diobati hanya untuk alasan kosmetik.
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ReferencesLymphangioma, yang juga dikenal sebagai malformasi limfatik (LM), adalah kelainan pembuluh darah yang hadir sejak lahir. Penyakit ini ditandai oleh pertumbuhan jaringan limfatik yang tidak normal sebelum dan sesudah kelahiran. Lymphangioma mempengaruhi sekitar 1 dari 2.000 hingga 4.000 orang, tanpa perbedaan signifikan antara jenis kelamin atau ras. Sebagian besar kasus (80‑90 %) didiagnosis sebelum usia dua tahun. Gejalanya sangat beragam, mulai dari pembengkakan lokal hingga kelainan luas pada saluran limfatik, yang terkadang menyebabkan pembengkakan parah yang dikenal sebagai penyakit kaki gajah. Misalnya, lymphangioma pada leher dan wajah dapat menimbulkan pembengkakan pada wajah, bahkan menyebabkan cacat pada kasus yang parah. Jika menyerang lidah, kondisi ini dapat menyebabkan pertumbuhan rahang berlebih dan gigi tidak sejajar. Pada mulut dan leher, lymphangioma dapat mengganggu pernapasan dan menimbulkan keadaan darurat yang mengancam jiwa. Pada mata, dapat menyebabkan kehilangan penglihatan, pergerakan mata terbatas, kelopak mata terkulai, dan mata melotot. Keterlibatan anggota tubuh dapat menghasilkan pembengkakan serta pertumbuhan jaringan dan tulang yang tidak normal. Tumor ini biasanya tumbuh lambat, namun infeksi, perubahan hormonal, atau cedera dapat memicu pertumbuhan cepat yang berisiko mengancam jiwa, sehingga memerlukan penanganan 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 penelitian retrospektif ini, kami meninjau 24 anak yang menderita lymphangioma dan diobati dengan suntikan larutan bleomycin dari Januari 1999 hingga Desember 2004. Sebagian besar lesi (63%) menghilang sepenuhnya, 21% menunjukkan respons baik, dan 16% tidak merespons dengan baik. Dua pasien mengalami kekambuhan tumor, dan dua pasien lainnya mengalami abses setelah menerima suntikan. Untungnya, tidak ada masalah atau efek samping serius yang kami temukan.
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 sebagai berikut: lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, dan lymphangioendothelioma. Penyakit ini mencakup sekitar 26 % tumor vaskular jinak pada anak‑anak, namun lebih jarang terjadi pada orang dewasa. Lymphangioma circumscriptum, jenis yang paling sering, menampilkan saluran limfatik yang menonjol melalui kulit, membentuk vesikel bening berisi cairan yang menyerupai bibit katak, disertai pembengkakan jaringan. Biasanya muncul pada area dengan jaringan limfatik yang kaya, seperti anggota badan, batang tubuh, dan ketiak. Seorang wanita berusia 71 tahun datang ke klinik kami dengan keluhan kaki bengkak terus‑menerus, benjolan berwarna merah muda pada alat kelamin, gatal‑gatal, dan keluarnya cairan getah bening. Kami melakukan pembedahan untuk menghilangkan semua benjolan dengan prosedur yang disebut labiektomi mayor bilateral, berhenti pada tingkat fasia Colles, sambil mempertahankan klitoris 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.
