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Eur Radiol. 2022 Jan;32(1):112-121. doi: 10.1007/s00330-021-08098-z. Epub 2021 Jun 24.

Liver lymphatic anatomy and role in systemic lymphatic disease.

European radiology

Christopher L Smith, Mandi Liu, Madhumitha Saravanan, Aaron G Dewitt, David M Biko, Erin M Pinto, Fernando A Escobar, Ganesh Krishnamurthy, Jefferson N Brownell, Petar Mamula, Andrew C Glatz, Matthew J Gillespie, Michael L O'Byrne, Chitra Ravishankar, Jonathan J Rome, Yoav Dori

Affiliations

  1. Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, 19104, USA. [email protected].
  2. 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. [email protected].
  3. Jill and Mark Fishman Center for Lymphatic Disorders, The Children's Hospital of Philadelphia, Philadelphia, PA, 19104, USA.
  4. 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.
  5. Department of Radiology, The Children's Hospital of Philadelphia and Perelman School of Medicine at The University of Pennsylvania, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.
  6. Division of Gastroenterology, 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.

PMID: 34165621 DOI: 10.1007/s00330-021-08098-z

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.

© 2021. The Author(s).

Keywords: Ascites; Chylothorax; Liver; Lymphatic diseases; Lymphography

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