Taghinia Amir H, Upton Joseph, Trenor Cameron C, Alomari Ahmad I, Lillis Anna P, Shaikh Raja, Burrows Patricia E, Fishman Steven J
Department of Plastic and Oral Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115.
Department of Plastic and Oral Surgery, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115; Vascular Anomalies Center, Boston Children's Hospital, Boston, MA 02115.
J Pediatr Surg. 2019 Mar;54(3):562-568. doi: 10.1016/j.jpedsurg.2018.08.056. Epub 2018 Sep 9.
Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement.
We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography.
Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well.
Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options.
IV.
中央传导性淋巴管异常（CCLA）可能由于中央淋巴通道功能障碍而导致乳糜漏和蛋白丢失性肠病（PLE）。这些病症的大多数治疗策略都是姑息性的，只能提供短暂的改善。
我们采用一种新型技术，即胸导管末端淋巴管静脉旁路术，治疗了14例难治性乳糜漏和/或PLE患者。乳糜漏发生在多个不同的解剖部位。所有患者均患有CCLA，术前淋巴结内淋巴管造影显示胸导管排空失败。
5例患者症状完全缓解，2例患者部分改善。无重大并发症。在5例PLE患者中，只有1例在淋巴管静脉旁路术后有所改善。对4例未改善的患者进行了重复传统淋巴管造影，结果显示在所有病例中旁路均通畅，但引流持续缓慢。1例患者进行了重复磁共振淋巴管造影，未清晰显示胸导管。
胸导管末端旁路术是一种新型手术，为一些缺乏其他有效治疗选择、具有CCLA严重表现的患者提供了改善和治愈的机会。
IV级。
