Liver lymphatic anatomy and role in systemic lymphatic disease

Abstract

Objectives To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention. Methods 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. Results 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. Conclusion 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. Key Points • 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.

Serial T2-Weighted Thoracic and Abdominal Lymphatic Imaging in Fontan Patients—New Insights into Dynamics of Lymphatic Abnormalities after Total Cavopulmonary Connection

“…This, in addition to high lymphatic afterload, reduces the lymphatic drainage capacity. However, not all patients with a Fontan circulation develop severe lymphatic abnormalities and it is hypothesized that the physiologic changes in venous and lymphatic congestion, plus an anatomical variant of the lymphatic vasculature, might lead to lymphatic failure in some of those patients [ 14 ]. This would explain why some patients with high CVP do not develop lymphatic failure and some who seem to have favorable hemodynamics might show severe lymphatic dysfunction [ 2 , 3 , 15 , 16 ].…”

Plastic bronchitis and a novel management approach with streptokinase inhalation therapy: a case study

“…PB has also been observed in patients with variants of abnormal lymphatic anastomosis. 4 PB manifests clinically with the sudden onset of cough, wheezing, progressive dyspnea, refractory hypoxemia, chest or pleuritic pain, and occasionally fever. The most pertinent feature of PB is the formation of bronchial casts that can vary from rubbery structures filling the small airways to casts formed of amorphous material occupying the entire bronchial tree.…”

“…5 In addition, PB sometimes presents without any known risk factors, in these cases, the casts are acellular which are often suggesting of an undetected underlying abnormality of the lymphatic drainage system. 4,6 Upon physical examination wheezing, attenuated breath sounds, and dullness to percussion can be observed. Radiological examinations such as chest X-rays and CT scans typically show partial atelectasis of the segments involved with compensatory hyperinflation of the unaffected segments.…”

Lymphatic Disorders in Patients With Single Ventricle Heart Disease

“…Biko et al recently published on the development of intrahepatic DCMRL (IH-DCMRL) (15). In a recent manuscript, Smith et al showed the correlation between the different hepatic lymphatic flow patterns and systemic lymphatic diseases (16). In addition, Lemley et al demonstrated that all patients with PLE have duodenal involvement with IH-DCMRL (17).…”

“…Irrespective of clinical presentation in patients with thoracic lymphatic flow disorders, if central lymphatic imaging demonstrates a normal thoracic duct flow pattern without perfusion of the mediastinum or the lungs, then TDE or TDL should not be performed as it can lead to worsening of symptoms. In these cases, the etiology is most often hepatopulmonary connections that bypass the central lymphatic conduction system, including the thoracic duct (Figure 7) (16). Selective embolization of hepatopulmonary connections will lead to a resolution of symptoms in most cases while maintaining central lymphatic flow.…”