Abstract
Purpose
Portal hypertension in patients with biliary atresia (BA) is generally thought to result from portal vein (PV) narrowing secondary to hepatic fibrosis. To test the hypothesis, we morphometrically analyzed the PVs and hepatic arteries (HAs).
Methods
Morphometrical analyses of 25 BA and 26 non-BA liver biopsy specimens from patients treated from 2000 to 2014. The total specimen area, the fibrotic portal area, vessel diameter and medial thickness of the HAs were measured.
Results
The PV diameter in BA patients was significantly smaller than that in non-BA patients. In BA, the numbers of normal-sized PVs and capillaries were decreased and increased, respectively. The PV diameter was not significantly correlated with the degree of fibrosis. We newly found that medial hypertrophy and the HA diameter increased with the number of endothelial cells in BA. The PV diameter was not significantly correlated with the medial thickness and was positively correlated with the HA diameter in BA.
Conclusions
The narrowing of the PV is unlikely to occur secondarily to liver fibrosis. The medial hypertrophy of the HA is not correlated with the decrease in the PV blood flow. These findings seem to be unique to the primary vascular lesions of BA.
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Acknowledgements
We thank Prof. Atsuyuki Yamataka, M.D., Ph.D., F.A.A.P. (Hon) Department of Pediatric Surgery, Juntendo University, Tokyo, JAPAN for sponsoring submission of this manuscript. We also thank Mr. Brian Quinn for his comments and help with the manuscript. This study was supported by a Grant-in-Aid for Scientific Research from the Japan Society for the Promotion of Science (JSPS, Nos. 26670765, 16K10466, 16K10094, 16K10095, 16K10434, 16H07090, 17K10555, 17K11514, 17K10183, 17K11515), a research Grant from the President’s Discretionary Expenses of our university, a research Grant from The UBE Foundation, a research Grant from Kawano Masanori Memorial Public Interest Incorporated Foundation for Promotion of Pediatrics, a research Grant from Tateishi Science and Technology Foundation, a research Grant from Mitsui Life Social Welfare Foundation, a research Grant from The Kurata Grants of the Hitachi Global Foundation, a research Grant from the Japanese Society for Medical and Biological Engineering, a research Grant from the Japan Medical Education Foundation and a medical research Grant from Kagoshima Prefecture Medical Association.
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Masuya, R., Muraji, T., Ohtani, H. et al. Morphometric demonstration of portal vein stenosis and hepatic arterial medial hypertrophy in patients with biliary atresia. Pediatr Surg Int 35, 529–537 (2019). https://doi.org/10.1007/s00383-019-04459-4
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DOI: https://doi.org/10.1007/s00383-019-04459-4