Luis A L, López J C, Encinas J L, Suárez O, Burgos L, Diaz M, Soto C, Ros Z
Hospital Universitario La Paz, Departamento de Cirugía Pediátrica, Madrid.
Cir Pediatr. 2007 Apr;20(2):116-8.
Complex lymphatic malformations (CLM) consist of disturbances of lymphatic system development, most often with a genetic origin and with mixed vascular system involvement: lymphatic, venous and capillary. They affect a large corporal area or are associated to other syndromes or systemic diseases.
We reviewed 21 patients with CLM treated in our hospital during the last 15 years. We used D2-40 monoclonal antibody (by immunohistochemistry) as lymphatic marker to evaluate the level of lymphatic involvement. Furthermore we analysed surgical implications in this group of patients.
Twelve children had only lymphatic involvement and nine mixed lymphatic-capillary or lymphatic-venous one. Two died of: respiratory insufficiency (in the neonatal period) and refractory hypoproteinemia (at 8 years of age). The skin was affected between 10 and 35% of total body surface. Three patients suffered from visceral involvement (lungs and mediastinum) and eighteen musculoskeletal. Severe deformity (20), lymphorhagia (15), repeated lymphangitis and chronic pain (5) were the most common symptoms reported. The immunoreaction intensity with monoclonal antibody D2-40 was related to the severity of the local and systemic involvement as well as to the presence of associated malformations. Fifteen cases underwent sequential surgical treatment, seven were treated with sclerotherapy (OK-432) and four with CO2 laser vaporization. Residual lymphorhagia in patients with total extirpation of the lymphatic malformation stopped after repeated evacuator punctures and healing took place.
(1) D2-40 monoclonal antibody is a marker of bad prognosis in CLM. (2) The complete excision of the lymphatic malformation lead to healing and the associated lymphorragia should not be considered as a recurrence, which will stop with evacuator punctures in all cases. (3) A multidisciplinary team approach is essential for the proper care of CLM in order to minimize postoperative sequelae and late complications.
复杂淋巴管畸形（CLM）由淋巴系统发育紊乱组成，最常见的是具有遗传起源，并累及混合血管系统：淋巴管、静脉和毛细血管。它们累及大面积身体区域，或与其他综合征或全身性疾病相关。
我们回顾了过去15年在我院接受治疗的21例CLM患者。我们使用D2-40单克隆抗体（通过免疫组织化学）作为淋巴标记物来评估淋巴受累程度。此外，我们分析了该组患者的手术影响。
12名儿童仅累及淋巴管，9名累及混合性淋巴管-毛细血管或淋巴管-静脉。2例死亡，分别死于：呼吸功能不全（新生儿期）和难治性低蛋白血症（8岁时）。皮肤受累面积占全身表面积的10%至35%。3例患者有内脏受累（肺和纵隔），18例有肌肉骨骼受累。严重畸形（20例）、淋巴漏（15例）、反复淋巴管炎和慢性疼痛（5例）是最常见的报告症状。单克隆抗体D2-40的免疫反应强度与局部和全身受累的严重程度以及相关畸形的存在有关。15例患者接受了序贯手术治疗，7例接受了硬化治疗（OK-432），4例接受了二氧化碳激光汽化治疗。淋巴管畸形完全切除的患者在反复进行引流穿刺后，残留的淋巴漏停止，伤口愈合。
（1）D2-40单克隆抗体是CLM预后不良的标志物。（2）淋巴管畸形的完全切除可实现愈合，相关的淋巴漏不应被视为复发，在所有情况下通过引流穿刺均可停止。（3）多学科团队方法对于CLM的恰当治疗至关重要，以便将术后后遗症和晚期并发症降至最低。
