A Rare Cause of Abdominal Pain After Transjugular Intrahepatic Portosystemic Shunt

Question: A 77-year-old woman presented with progressive dyspnea for 4 months. The patient was diagnosed with primary biliary cirrhosis more than 1 years ago and was ever since on ursodeoxycholic acid medication. Recurrent ascites occurred at least 6 months before diagnosis and was responsive to diuretics. Beyond these, she did not experience any onset of gastrointestinal bleeding or hepatic encephalopathy. Laboratory examination indicated the decreased hemoglobin (10.7 g/dL, normal value [nv]: 11.5–15 g/dL), leukocyte (2.85 × 103/μL, nv: [3.5–9.5] × 103/μL), and platelet counts (53 × 103/μL, nv: [100–300] × 103 /μL), increased alkaline phosphatase (195 IU/L, nv: 50–135 IU/L) and glutamyl transpeptidase (149 IU/L, nv: <45 IU/L), and normal aminotransferase and total bilirubin levels. Chest computerized tomography showed a large amount of right-sided pleural effusion. Pleural fluid tests revealed an uncomplicated hepatic hydrothorax with serum-pleural fluid albumin gradient >1.1 g/dL and polymorphonuclear cell count <500 cells/μL. However, the patient did not show significant improvement of symptoms with dietary sodium restriction and adequate-dose diuretics. Considering the existence of hepatic hydrothorax refractory, transjugular intrahepatic portosystemic shunt (TIPS) was performed thereafter. The portal pressure gradient decreased from 18 mm Hg–7 mm Hg after stent implantation. The interventional procedure was uneventful and no complication occurred during the processes. Postoperatively, the patient experienced slight dull pain in the right upper abdomen, and the symptom intensified gradually despite giving analgesic medication in the following 36 hours. Abdominal examination showed tenderness in the right upper quadrant and percussion pain in the liver area. Liver function tests revealed obviously elevated alanine aminotransferase (1147 IU/L, nv: <50 IU/L) and aspartate aminotransferase (1582 IU/L, nv: <40 IU/L), mildly elevated total bilirubin (1.73 mg/dL, nv: 0.29–1.64 mg/dL). Blood routine showed the change of hemoglobin (11.4 g/dL) and leukocyte (6.67 × 103 /μL). Abdominal computerized tomography showed multiple strips of low-density shadows along the blood vessels and scattered low-density nodules in the liver parenchyma (Figure A, white arrowheads). What is the most likely diagnosis? See the Gastroenterology website (www.gastrojournal.org) for more information on submitting to Gastro Curbside Consult. Abdominal contrast-enhanced computerized tomography (CECT) showed diffuse linear hypodensity around portal vein branches and multiple low-density nodules in the liver, but no obvious enhancement was observed (Figures B and C, white arrowheads). These imaging findings were absent from hepatic vein and intrahepatic inferior vena cava (Figure D). According to the history of hepatic invasive procedure, laboratory findings, and CECT presentation, intrahepatic lymphangiectasia was suspected. After a short course of nonsteroidal anti-inflammatory drug therapy, her symptoms completely resolved and transaminases gradually returned to normal levels. Follow-up CECT scans at 30 days showed complete resolution of the periportal low-density areas (Figures E and F). Intrahepatic lymphangiectasia (IHL), manifested as dilated hepatic lymphatics and related lymphedema, is caused by either increased lymph flow or lymphatic obstruction.1Pupulim L.F. Vilgrain V. Ronot M. et al.Hepatic lymphatics: anatomy and related diseases.Abdom Imaging. 2015; 40: 1997-2011Crossref PubMed Scopus (29) Google Scholar Liver transplantation, blunt abdominal trauma, hepatic malignant neoplasms, and congestive hepatomegaly due to cardiac decompensation are the most common underlying etiology of IHL.2Koslin D.B. Stanley R.J. Berland L.L. et al.Hepatic perivascular lymphedema: CT appearance.AJR Am J Roentgenol. 1988; 150: 111-113Crossref PubMed Scopus (45) Google Scholar “Periportal halo,” characterized by hypodense appearance on computed tomography and hyperintense appearance on T2-weighted magnetic resonance images, has important clinical significance in the diagnosis of IHL.3Karcaaltincaba M. Haliloglu M. Akpinar E. et al.Multidetector CT and MRI findings in periportal space pathologies.Eur J Radiol. 2007; 61: 3-10Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar Presence of hypodense appearance on both sides of the portal venous system can allow differentiation from biliary dilatation.3Karcaaltincaba M. Haliloglu M. Akpinar E. et al.Multidetector CT and MRI findings in periportal space pathologies.Eur J Radiol. 2007; 61: 3-10Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar In fact, IHL could be an overlooked complication of TIPS that has never been previously reported. Iatrogenic injury during a TIPS procedure may interrupt lymphatic vessels and initiate coagulation at the injury site, and thrombus/microthrombus provisionally compresses and blocks the hepatic lymphatic system. Once the thrombus/microthrombus dissolves, the occluded lymphatics are recanalized and IHL could be spontaneously resolved. In conclusion, IHL is a procedure-related complication of TIPS that should not be neglected. Periportal halo by CECT provides valuable evidence for both diagnosis and differential diagnosis. Further studies with a larger sample size are necessary to investigate the morbidity and natural course of IHL after TIPS.