Smith Christopher L, Liu Mandi, Saravanan Madhumitha, Dewitt Aaron G, Biko David M, Pinto Erin M, Escobar Fernando A, Krishnamurthy Ganesh, Brownell Jefferson N, Mamula Petar, Glatz Andrew C, Gillespie Matthew J, O'Byrne Michael L, Ravishankar Chitra, Rome Jonathan J, Dori Yoav
Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, 19104, USA.
Division of Cardiology, The Children's Hospital of Philadelphia and Department of Pediatrics Perelman School of Medicine at The University of Pennsylvania, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.
Eur Radiol. 2022 Jan;32(1):112-121. doi: 10.1007/s00330-021-08098-z. Epub 2021 Jun 24.
To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention.
In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed.
During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics.
We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal.
• We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). • Abnormal imaging patterns correlated with increased morbidity.
利用肝内淋巴管造影术描述系统性淋巴疾病患者肝与全身淋巴管的连接情况，并比较淋巴介入治疗后的结果。
在这项回顾性研究中，纳入了2014年5月至2019年4月在我院接受肝内淋巴管造影术的患者。进行影像学检查并描述肝淋巴管连接和血流模式。回顾临床资料，对接受或未接受异常肝淋巴管介入治疗的患者进行比较。
在研究期间，105例患者接受了肝内淋巴管造影术。主要临床表现包括腹水（19/105）、乳糜胸（27/105）、塑形支气管炎（PB）（17/105）和蛋白丢失性肠病（PLE）（42/105）。确定了五类肝淋巴管连接和血流模式（%）：正常（25%，26/105）、肝-腹膜（12%，13/105）、肝-肺（10.5%，11/105）、肝-肠系膜（7.5%，8/105）和肝-十二指肠（41%，43/105），4例患者有不止一种异常模式。临床表现与影像学分类之间的比较显示，肝-腹膜连接时出现腹水的可能性增加（p<0.0001），肝-肺连接时出现乳糜胸/PB的可能性增加（p = 0.01），肝-十二指肠连接时出现PLE的可能性增加（p<0.001）。76例患者接受了淋巴介入治疗，24%肝淋巴管正常，76%肝淋巴管异常。两组患者的住院时间或死亡率无差异，但肝淋巴管异常组症状缓解时间延长（p = 0.006），6个月后仍有持续症状（5%对44%，p = 0.002）。
我们确定了五种肝脏淋巴管成像类别，与所呈现的淋巴疾病有显著相关性。异常成像模式与发病率增加相关。如果中央淋巴管成像正常，对于系统性淋巴疾病患者应考虑评估肝脏淋巴管。
• 我们确定了五种肝脏淋巴管成像模式：正常、肝-腹膜、肝-肠系膜、肝-肺和肝-十二指肠。• 成像模式与疾病表现相关（正常-乳糜胸/PB，肝-腹膜-腹水/乳糜胸，肝-肺-乳糜胸/PB，肝-十二指肠-PLE）。• 异常成像模式与发病率增加相关。
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