Ultrasound diagnostics of extrahepatic portal hypertension in children: a review of clinical cases

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BACKGROUND: Ultrasound is a method of choice which is recommended in patients with portal hypertension for diagnosing and monitoring the disease, since it is informative, non-invasive, available and can be performed at patient’s bedside. The problem of portal hypertension in adults, methods of its diagnostics and treatment are widely covered in the medical literature. In clinical practice, portal hypertension in adults in most cases has an intrahepatic form and is associated with cirrhosis as the consequence of chronic liver diseases. Unlike in adults, portal hypertension in children is more often extrahepatic and is associated with some block to the blood flow through the portal vein. Portal vein impaired patency may be caused by thrombosis due to catheterization of umbilical vessels, omphalitis, peritonitis, developmental malformation of the portal system. A considerable difference in the frequency of portal hypertension forms in children and adults impacts the strategy of ultrasound diagnostic examination.

CLINICAL CASE DESCRIPTION: The article presents two clinical observations of extrahepatic form of portal hypertension in children aged one year one month and seven years. The authors demonstrate potentials and main aspects of ultrasound diagnostics in this pathology. They also underline the priority of assessing the portal system patency so as to detect cavernous transformation in the portal vein. Echographic signs such as changes in echogenicity and liver structure, presence of uneven bumpy contour, dilation of portal vessels, recanalization of the umbilical vein, ascites are not typical for the extrahepatic form of portal hypertension in children.

CONCLUSION: In children with extrahepatic portal hypertension, clinical manifestations such as enlarged abdomen, changed blood tests and ecchymoses on the body may be unnoticed for a long time. Bleeding from varicose veins of the esophagus and stomach is met as a frequent sign in children. Therefore, it is of particular importance to allocate newborn children from the risk group (with umbilical vascular catheterization, omphalitis, etc. in the anamnesis) to the special diagnostic group with detailed ultrasound examination of the liver, Dopplerographic assessment of portal vein patency so as to identify possible signs of portal hypertension as early as possible.

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作者简介

Elena Titova

Filatov Children’s City Clinical Hospital

编辑信件的主要联系方式.
Email: eletit@yandex.ru
ORCID iD: 0000-0001-9901-6093

MD, Cand. Sci. (Medicine)

俄罗斯联邦, Moscow

Angelika Gurevich

Filatov Children’s City Clinical Hospital; Russian Medical Academy of Continuous Professional Education

Email: gurevichai@yandex.ru
ORCID iD: 0000-0001-8187-214X

MD, Dr. Sci. (Medicine), Professor

俄罗斯联邦, Moscow; Moscow

Alexander Razumovsky

Filatov Children’s City Clinical Hospital; Pirogov Russian National Research Medical University

Email: 1595105@mail.ru
ORCID iD: 0000-0003-3511-0456
SPIN 代码: 3600-4701

MD, Dr. Sci. (Medicine), Professor, Corresponding Member of the Russian Academy of Sciences

俄罗斯联邦, Moscow; Moscow

Zorikto Mitupov

Filatov Children’s City Clinical Hospital; Pirogov Russian National Research Medical University

Email: zmitupov@mail.ru
ORCID iD: 0000-0002-0016-6444
SPIN 代码: 5182-1748

MD, Dr. Sci. (Medicine), Professor

俄罗斯联邦, Moscow; Moscow

Sergei Margaryan

Filatov Children’s City Clinical Hospital; Pirogov Russian National Research Medical University

Email: sergey.margaryan27@mail.ru
ORCID iD: 0000-0002-9968-2931
SPIN 代码: 6581-9484

Graduate Student

俄罗斯联邦, Moscow; Moscow

参考

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  10. De Gaetano AM, Lafortune M, Patriquin H, et al. Cavernous transformation of the portal vein: Patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR Am J Roentgenol. 1995;165(5):1151–1155. doi: 10.2214/ajr.165.5.7572494
  11. Pargewar SS, Desai SN, Rajesh S, et al. Imaging and radiological interventions in extra-hepatic portal vein obstruction. World J Radiol. 2016;8(6):556–570. doi: 10.4329/wjr.v8.i6.556
  12. Sherlock S, Dooley J. Diseases of the liver and bile ducts. Trans. from English. Z.G. Aprosina, N.A. Mukhina, editors. Moscow: GEOTAR-Media; 1999. (In Russ.)
  13. Sacerdoti D, Serianni G, Gaiani S, et al. Thrombosis of the portal venous system. J Ultrasound. 2007;10(1):12–21. doi: 10.1016/j.jus.2007.02.007
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1. JATS XML
2. Fig. 1. Liver ultrasound: а — B-mode. Linear dimensions are not increased, parenchyma is homogeneous, of medium echogenicity; b — B-mode. The cavernous transformation of the portal vein is determined in the gates of the liver. The trunk and lobar branches of the portal vein are absent; c — B-mode, linear sensor, magnification. The cavernous transformation of the portal vein is determined in the gates of the liver; d — Doppler color flow mode. A conglomerate of convoluted vessels with multidirectional blood flow (cavernous transformation of the portal vein) is determined in the gates of the liver.

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3. Fig. 2. Ultrasound of the healthy child's liver: а — B-mode. Normal architectonics of the portal vein in its bifurcation zone; b — Doppler color flow mode. The bifurcation zone of the portal vein. The left lobe branch is colored red (blood flow is directed to the probe), the right lobe branch is colored blue (blood flow is directed from the probe).

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4. Fig. 3. Ultrasound in the Doppler color flow mode: а — the gallbladder. Venous collaterals in the gallbladder wall; b — the spleen. Convoluted collateral vessels in the spleen gate.

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5. Fig. 4. Pulse-wave Dopplerometry mode. Study of blood flow in the splenic vein. Reversed blood flow (blood flow towards the spleen) in the splenic vein is determined.

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6. Fig. 5. Liver ultrasound examination. B-mode. The cavernous transformation of the portal vein is determined in the gates of the liver. The lobar branches of the portal vein are not determined.

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7. Fig. 6. Ultrasound examination of the pancreas. Multiple convoluted collaterals are determined in the parenchyma: а — C-mode; b — Doppler color flow mode.

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8. Fig. 7. Ultrasound examination: a — epigastric region; b — pelvic cavity. Doppler color flow mode. In the projection of the small omentum and the small pelvis, convoluted collaterals are determined. Increased echogenicity of the small omentum.

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9. Fig. 8. Scheme of portal cavernoma formation [7, 8].

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版权所有 © Titova E.A., Gurevich A.I., Razumovsky A.Y., Mitupov Z.B., Margaryan S.R., 2023

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