Lymphangioma - 淋巴管瘤
https://en.wikipedia.org/wiki/Lymphangioma
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References
Recent Progress in Lymphangioma 34976885 NIH
Lymphangioma 也称为淋巴管畸形 (LM)。这是一种先天性血管疾病,特点是出生前后淋巴组织异常增生。Lymphangioma 的发病率约为 2000 至 4000 人中 1 人,性别和种族差异不显著。大多数病例(80‑90%)在两岁之前被诊断。临床表现多样,可从局部肿胀到广泛的淋巴管异常,有时出现严重水肿,称为象皮病。例如,颈部和面部的 lymphangioma 可导致面部肿胀,严重时甚至毁容。舌部受累时可能引起下颌过度生长和牙齿错位。口腔和颈部病变可导致呼吸困难,甚至危及生命的急症。眼部病变可能致视力丧失、眼球运动受限、眼睑下垂和眼球突出。四肢受累则出现组织和骨骼的肿胀及异常生长。该肿瘤通常生长缓慢,但感染、激素变化或外伤可引起快速增大,增加危及生命的风险,需要及时治疗。
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: Is intralesional bleomycin sclerotherapy effective? 22279495 NIH
在这项回顾性研究中,我们回顾了 1999 年 1 月至 2004 年 12 月期间,24 名患有 lymphangioma 并接受 bleomycin(博来霉素)溶液注射治疗的儿童。大多数病变(63%)完全消退,21% 取得良好反应,16% 反应不佳。两名患者的病灶随后复发,另外两名患者在注射时出现脓肿。幸运的是,我们未见其他严重问题或明显副作用。
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.
Surgical Resection of Acquired Vulvar Lymphangioma Circumscriptum - Case reports 24665431 NIH
Lymphangioma 的主要类型包括:lymphangioma circumscriptum、cavernous lymphangioma、cystic hygroma 和 lymphangioendothelioma。这类肿瘤约占儿童良性血管肿瘤的 26%,在成人中相对少见。最常见的类型是 lymphangioma circumscriptum,表现为淋巴管穿过皮肤向外突出,形成类似青蛙卵的透明充液囊泡,并伴有组织肿胀。它常见于淋巴网络丰富的部位,如四肢、躯干和腋窝。 一位 71 岁的女性来到我们诊所,主诉腿部持续肿胀,外阴出现粉红色肿块,伴有瘙痒和淋巴液渗漏。我们通过双侧大阴唇切除术切除所有肿块,切除至科尔斯筋膜水平,同时保留阴蒂。
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.