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

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Abstract

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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About the authors

Elena A. Titova

Filatov Children’s City Clinical Hospital

Author for correspondence.
Email: eletit@yandex.ru
ORCID iD: 0000-0001-9901-6093

MD, Cand. Sci. (Medicine)

Россия, Moscow

Angelika I. 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

Россия, Moscow; Moscow

Alexander Y. Razumovsky

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

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

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

Россия, Moscow; Moscow

Zorikto B. Mitupov

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

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

MD, Dr. Sci. (Medicine), Professor

Россия, Moscow; Moscow

Sergei R. 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-code: 6581-9484

Graduate Student

Россия, Moscow; Moscow

References

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  2. Razumovsky AY, Dronov AF, Rachkov VE, et al. Surgical treatment of portal hypertension in children. Moscow: Medical Information Agency; 2012. (In Russ.) EDN: UFFMPR
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Supplementary files

Supplementary Files
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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. 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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4. 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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5. 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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6. 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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7. 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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8. Fig. 8. Scheme of portal cavernoma formation [7, 8].

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Copyright (c) 2023 Titova E.A., Gurevich A.I., Razumovsky A.Y., Mitupov Z.B., Margaryan S.R.

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