Lymphangioma - 淋巴管瘤
淋巴管瘤 (Lymphangioma) 是以薄壁囊肿病变为特征的淋巴系统畸形。这些畸形可发生在任何年龄，并可能累及身体的任何部位，但 90% 发生于 2 岁以下的儿童，且多累及头颈部。淋巴管瘤通常在出生前通过胎儿超声检查发现。获得性淋巴管瘤可能由创伤、炎症或淋巴阻塞引起。由于淋巴管瘤几乎不发生恶变，治疗通常仅出于美观考虑。
relevance score : -100.0%
ReferencesLymphangioma 也称为淋巴管畸形 (LM)。这是一种从出生起就存在的血管疾病，其特点是出生前后淋巴组织异常生长。Lymphangioma 的发病率约为 2000–4000 人中 1 人，性别或种族之间没有显著差异。大多数病例（80–90%）在两岁之前被诊断出来。 症状多种多样，可能表现为局部肿胀或淋巴通道广泛异常，严重时可导致大面积水肿，称为象皮病。例如，颈部和面部的 lymphangioma 可引起面部肿胀，严重时甚至毁容。当病变累及舌头时，可能导致下颌过度生长和牙齿错位。口腔和颈部受累时，可能出现呼吸困难，甚至危及生命的紧急情况。眼部受累可导致视力下降、眼球运动受限、眼睑下垂和眼球突出。四肢受累则会出现组织和骨骼的肿胀及异常生长。 这种肿瘤通常生长缓慢，但感染、激素变化或外伤可能引起快速增大，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 (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.
在本回顾性研究中，我们回顾了1999年1月至2004年12月期间，24例患有lymphangioma并接受bleomycin(博来霉素)溶液注射治疗的儿童。大多数病变（63%）完全消失，21%获得良好反应，16%未见明显改善。两例患者的肿瘤随后复发，另有两例在注射时出现脓肿。值得庆幸的是，未观察到其他严重不良事件或副作用。
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.
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.
Lymphangioma 的主要类型有：lymphangioma circumscriptum、cavernous lymphangioma、cystic hygroma、lymphangioendothelioma。这些肿瘤约占儿童良性血管肿瘤的 26%，但在成人中较少见。lymphangioma circumscriptum 是最常见的类型，表现为淋巴管穿过皮肤突出，形成类似青蛙卵的透明充液囊泡，并伴有组织肿胀。它通常出现在淋巴网络丰富的区域，如四肢、躯干和腋窝。 一位 71 岁的女性来到本诊所就诊，她的腿部持续肿胀，外生殖器出现粉红色肿块，伴有瘙痒和淋巴液渗漏。我们采用双侧大阴唇切除术，切除所有肿块，止于科尔斯筋膜水平，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.
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.
