Center for Lymphatic Imaging and Interventions, Children's Hospital of Philadelphia/Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania.
Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
J Am Coll Cardiol. 2017 Jun 20;69(24):2929-2937. doi: 10.1016/j.jacc.2017.04.023.
Protein-losing enteropathy (PLE), characterized by loss of proteins in the intestine, is a devastating complication in patients with congenital heart disease. The cause of PLE is unknown, but lymphatic involvement has been suspected.
The authors evaluated the use of lymphangiographic imaging and liver lymphatic embolization as a treatment for PLE.
This was a single-center, retrospective review of imaging and interventions used in 8 consecutive patients with liver lymphatic embolization and congenital heart disease with elevated central venous pressure complicated by PLE.
Liver lymphangiography was performed in 8 patients (5 males, 3 females; median age, 21 years), 7 of whom demonstrated leakage of liver lymph into the duodenum through abnormal hepatoduodenal lymphatic communications. This was confirmed by duodenoscopy with simultaneous injection of isosulfan blue dye into the liver lymphatics in 6 of 7 patients. Liver lymphatic embolization with ethiodized oil in 2 patients resulted in a temporary increase in albumin blood level and symptom improvement in 1 patient, but was complicated by duodenal bleeding in both patients. Of the remaining 6 patients, liver lymphatic embolization with n-butyl cyanoacrylate glue resulted in sustained improvement of the serum albumin level and symptoms in 3 patients, temporary improvement in 2 patients, and no change in 1 patient with median follow-up of 135 days (range, 84 to 1,005 days).
The authors demonstrated liver lymph leakage as a cause of PLE in patients with congenital heart disease and elevated central venous pressure. Lymphatic embolization led to improved albumin levels and relief of symptoms. Further experience with the technique is needed to determine long-term outcome of this procedure.
以肠道蛋白质丢失为特征的蛋白丢失性肠病（PLE）是先天性心脏病患者的一种严重并发症。PLE 的病因不明，但怀疑与淋巴系统受累有关。
作者评估了淋巴管造影成像和肝淋巴管栓塞术作为 PLE 治疗方法的应用。
这是一项针对 8 例连续接受肝淋巴管栓塞术和先天性心脏病伴升高的中心静脉压合并 PLE 的患者的影像学和介入治疗的单中心回顾性研究。
8 例患者（5 例男性，3 例女性；中位年龄 21 岁）进行了肝淋巴管造影，其中 7 例患者通过异常的肝十二指肠淋巴交通显示肝脏淋巴漏入十二指肠。6 例患者同时向肝淋巴管内注射异硫蓝染料进行十二指肠镜检查证实了这一点。2 例患者采用乙基碘油进行肝淋巴管栓塞术，其中 1 例患者白蛋白血水平暂时升高，症状改善，但 2 例患者均出现十二指肠出血。其余 6 例患者中，3 例采用 n-丁基氰基丙烯酸酯胶进行肝淋巴管栓塞术，3 例患者的血清白蛋白水平和症状持续改善，2 例患者暂时改善，1 例患者无变化，中位随访时间为 135 天（范围 84 至 1005 天）。
作者证明了先天性心脏病和升高的中心静脉压患者中肝淋巴漏是 PLE 的病因。淋巴管栓塞术可导致白蛋白水平升高和症状缓解。需要进一步的经验来确定该手术的长期效果。
