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.

Kun Zhang,Qingjun Wang,Huiyi Ye,Aitao Guo,Haiyi Wang,Weidong Duan

doi:10.1186/s40001-014-0063-7

Kun Zhang, Qingjun Wang + Show 4 more

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https://doi.org/10.1186/s40001-014-0063-7

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Abstract

BackgroundPatients with congenital absence of the portal vein (CAPV) often suffer from additional medical complications such as hepatic tumors and cardiac malformations.Case presentationCongenital 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.ConclusionsCAPV 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.

Highlights

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
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
Patients with CAPV often suffer from additional medical complications such as hepatic tumors and cardiac malformations

Summary

Conclusions

We describe a patient with CAPV, and typical and atypical (telangiectatic) FNH. This patient displayed slight liver dysfunction and cardiac anomaly. 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. Consent Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. Authors’ contributions All authors participated in the preparation of the manuscript. KZ collected the case information and drafted the manuscript. All authors read and approved the final manuscript

Background

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