Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy

Hillaire, Sophie; Cazals–Hatem, Dominique; Bruno, Onorina; Miranda, S. De; Grenet, Dominique; Poté, Nicolas; Soubrane, O.; Erlinger, S; Lacaille, Florence; Mellot, François; Vilgrain, Valérie; Paradis, Valérie

doi:10.1002/lt.24842

Cited by 47 publications

(45 citation statements)

References 6 publications

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“…These patients present with extreme portal hypertensive manifestations characterized by an increased portal vein caliper measurement, large portosystemic collaterals, and severe splenomegaly. (27) Before LT, our case closely matched this clinical picture, and it is noteworthy that the histopathological revision of the explanted liver showed some findings consistent with obstructive portal venopathy (Fig. 4B), although in the presence of significant fibrosis.…”

Section: Discussionsupporting

confidence: 82%

“…Although some patients develop portal hypertension as a result of increased bile viscosity leading to bile duct obstruction, inflammation, proliferation, focal biliary fibrosis, and, ultimately, biliary cirrhosis, recent series have shown that a significant proportion of patients develop noncirrhotic portal hypertension because of obstructive portal venopathy, which is characterized by narrowing, thrombosis, or nonvisibility of the intrahepatic portal vein branches. These patients present with extreme portal hypertensive manifestations characterized by an increased portal vein caliper measurement, large portosystemic collaterals, and severe splenomegaly . Before LT, our case closely matched this clinical picture, and it is noteworthy that the histopathological revision of the explanted liver showed some findings consistent with obstructive portal venopathy (Fig.…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, liver disease arising in patients with CF is a rare indication for LT. (26) The pathogenesis of portal hypertension in CF liver disease has recently been reappraised. Although some patients develop portal hypertension as a result of increased bile viscosity leading to bile duct obstruction, inflammation, proliferation, focal biliary fibrosis, and, ultimately, biliary cirrhosis, recent series (27)(28)(29) have shown that a significant proportion of patients develop noncirrhotic portal hypertension because of obstructive portal venopathy, which is characterized by narrowing, thrombosis, or nonvisibility of the intrahepatic portal vein branches. These patients present with extreme portal hypertensive manifestations characterized by an increased portal vein caliper measurement, large portosystemic collaterals, and severe splenomegaly.…”

Section: Discussionmentioning

confidence: 99%

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Vascular Remodeling of Visceral Arteries Following Interruption of the Splenic Artery During Liver Transplantation

et al. 2019

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Splenic artery (SA) ligation can be performed during liver transplantation (LT) to avoid portal hyperperfusion, which is involved in the pathogenesis of both small‐for‐size and SA syndrome. The SA can also be used as an inflow for arterial reconstruction. Exceptionally, SA interruption or agenesis has been associated with positive remodeling of collateral arteries supplying the spleen via the left gastric artery (LGA), short gastric vessels, and the gastroepiploic arcade (GEA), with subsequent severe upper gastrointestinal (GI) bleeding. To determine incidence, magnitude, predictors, and clinical implications of vascular remodeling after SA interruption during LT, we identified 465 patients transplanted in the period 2007‐2017 who had the SA ligated or interrupted at LT. Among them, 88 had a computed tomography angiography suitable for evaluation of vascular remodeling after LT. The presence of prominent gastric arterial collaterals and the increase in LGA and GEA diameter were evaluated on 2‐dimensional axial images and multiplanar reconstructions. Of the 88 patients, 28 (31.8%), 32 (36.4%), and 22 (25.0%) developed gastric collateralization graded as mild, moderate, or severe. Of the patients for whom comparison with pre‐LT imaging was possible (n = 54), 51 (94.4%) presented a median 37% and 55% increase in LGA and GEA diameter, respectively. Severe gastric collateralization was associated with lower body mass index (odds ratio, 0.84; 95% confidence interval [CI], 0.71‐0.98; P = 0.03), whereas a GEA caliper measurement increase was positively correlated with Model for End‐Stage Liver Disease score (r2 = 0.12; 95% CI, 0.65‐4.15; P = 0.008). Out of 465 patients, 2 (0.43%) had severe episodes of arterial upper GI bleeding, possibly exacerbated by vascular remodeling. In conclusion, vascular remodeling after SA interruption during LT is frequent and can aggravate GI bleeding during follow‐up.

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Section: Discussionsupporting

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Vascular Remodeling of Visceral Arteries Following Interruption of the Splenic Artery During Liver Transplantation

et al. 2019

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“…Although such tests may be useful, it should be kept in mind that the extent of fibrosis is not correlated with the degree of portal hypertension, as we recently reported in a series of 10 patients with CFLD who had whole-liver histological examination after liver transplantation. (2) In our series, 8 patients had idiopathic noncirrhotic portal hypertension (INCPH), as the only patient with a liver biopsy in the Koh et al study. The high frequency of INCPH in patients with CFLD also explains why liver biopsy has been described as inconsistent for the accurate diagnosis of liver disease in cystic fibrosis, unless a dual pass is performed.…”

Section: To the Editormentioning

confidence: 66%

“…This is why hepatic transplantation was performed in our patients who underwent lung transplantation. (2) It would be extremely useful to have predictive markers of liver decompensation to better select the patients to whom liver transplantation should be proposed.…”

Section: To the Editormentioning

confidence: 99%