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Computed tomography and magnetic resonance imaging of multiple focal nodular hyperplasias of the liver with congenital absence of the portal vein in a Chinese girl: case report and review of the literature.

Zhang K, Wang Q, Wang H, Ye H, Guo A, Duan W - Eur. J. Med. Res. (2014)

Bottom Line: Congenital absence of the portal vein (CAPV) is a rare malformation.We present a case of a 16-year-old Chinese girl with CAPV with multiple pathology-proven hepatic focal nodular hyperplasias (FNHs) and ventricular septal defect (VSD).Close follow-up, including laboratory testing and radiologic imaging, is recommended for all CAPV patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China. zkbeir@163.com.

ABSTRACT

Background: Patients with congenital absence of the portal vein (CAPV) often suffer from additional medical complications such as hepatic tumors and cardiac malformations.

Case presentation: Congenital absence of the portal vein (CAPV) is a rare malformation. We present a case of a 16-year-old Chinese girl with CAPV with multiple pathology-proven hepatic focal nodular hyperplasias (FNHs) and ventricular septal defect (VSD). The CT and MRI features of this case are described, and previously reported cases are reviewed.

Conclusions: CAPV is a rare congenital anomaly and in such patients, clarifying the site of portosystemic shunts, liver disease, and other anomalies is critical for appropriate treatment selection and accurate prognosis determination. Close follow-up, including laboratory testing and radiologic imaging, is recommended for all CAPV patients.

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Related in: MedlinePlus

Abdominal precontrast computed tomography (CT) and magnetic resonance imaging (MRI). The portal trunk drains directly into the inferior vena cava (IVC) just below the level of the right atrium, shown on both postcontrast computed tomography (CT) (A) and T2-weighted magnetic resonance imaging (MRI) (B). White arrowhead = IVC; white arrow = portal trunk.
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Fig3: Abdominal precontrast computed tomography (CT) and magnetic resonance imaging (MRI). The portal trunk drains directly into the inferior vena cava (IVC) just below the level of the right atrium, shown on both postcontrast computed tomography (CT) (A) and T2-weighted magnetic resonance imaging (MRI) (B). White arrowhead = IVC; white arrow = portal trunk.

Mentions: During the radiologic workup of the patient, including both CT and MRI, no intrahepatic portal vein was seen. After joining of the middle and left hepatic veins, the portal trunk drained directly into the inferior vena cava (IVC) just below the level of the right atrium (Figure 3A,B). These vascular abnormalities were not identified prior to the hepatic surgery.Figure 3


Computed tomography and magnetic resonance imaging of multiple focal nodular hyperplasias of the liver with congenital absence of the portal vein in a Chinese girl: case report and review of the literature.

Zhang K, Wang Q, Wang H, Ye H, Guo A, Duan W - Eur. J. Med. Res. (2014)

Abdominal precontrast computed tomography (CT) and magnetic resonance imaging (MRI). The portal trunk drains directly into the inferior vena cava (IVC) just below the level of the right atrium, shown on both postcontrast computed tomography (CT) (A) and T2-weighted magnetic resonance imaging (MRI) (B). White arrowhead = IVC; white arrow = portal trunk.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4260376&req=5

Fig3: Abdominal precontrast computed tomography (CT) and magnetic resonance imaging (MRI). The portal trunk drains directly into the inferior vena cava (IVC) just below the level of the right atrium, shown on both postcontrast computed tomography (CT) (A) and T2-weighted magnetic resonance imaging (MRI) (B). White arrowhead = IVC; white arrow = portal trunk.
Mentions: During the radiologic workup of the patient, including both CT and MRI, no intrahepatic portal vein was seen. After joining of the middle and left hepatic veins, the portal trunk drained directly into the inferior vena cava (IVC) just below the level of the right atrium (Figure 3A,B). These vascular abnormalities were not identified prior to the hepatic surgery.Figure 3

Bottom Line: Congenital absence of the portal vein (CAPV) is a rare malformation.We present a case of a 16-year-old Chinese girl with CAPV with multiple pathology-proven hepatic focal nodular hyperplasias (FNHs) and ventricular septal defect (VSD).Close follow-up, including laboratory testing and radiologic imaging, is recommended for all CAPV patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, PLA General Hospital, #28 Fuxing Road, Beijing, 100853, China. zkbeir@163.com.

ABSTRACT

Background: Patients with congenital absence of the portal vein (CAPV) often suffer from additional medical complications such as hepatic tumors and cardiac malformations.

Case presentation: Congenital absence of the portal vein (CAPV) is a rare malformation. We present a case of a 16-year-old Chinese girl with CAPV with multiple pathology-proven hepatic focal nodular hyperplasias (FNHs) and ventricular septal defect (VSD). The CT and MRI features of this case are described, and previously reported cases are reviewed.

Conclusions: CAPV is a rare congenital anomaly and in such patients, clarifying the site of portosystemic shunts, liver disease, and other anomalies is critical for appropriate treatment selection and accurate prognosis determination. Close follow-up, including laboratory testing and radiologic imaging, is recommended for all CAPV patients.

Show MeSH
Related in: MedlinePlus