An 8-year-old Chinese boy visited our hospital and presented with emaciation without symptoms. He was previously healthy without known medical conditions.
Abdominal US examination revealed multiple hepatic nodules with a maximum size of 4.0 x 3.1 cm ( Figure 1A ), clear boundary, and non-uniform internal echo, leading to local inferior vena cava (IVC) compression. The following notable findings were reported: the left PV (LPV) directly communicated with IVC (1.2 cm in diameter); color Doppler US confirmed the PV flow toward IVC ( Figure 1B ), the flow velocity in the shunt was 98 cm/s ( Figure 1C ), the right PV (RPV) was not shown, and no abnormal echo was observed in IVC; and splenomegaly (2 cm below the ribs). The rest of the abdominal organs showed no abnormal findings, and the abdominal cavity had no effusion. The patient was believed to have ectopic PV drainage (PV communicating with IVC), with unknown nature of multiple nodules. We recommended that the patient should undergo an additional abdominal contrast-enhanced computed tomography (CT) and digital subtraction angiography (DSA) to better understand this anatomic anomaly and clarify the nature of the nodule.
Laboratory tests showed the total bilirubin (TB; 40.6 mumol/L; reference range: 1.7-25.0 mumol/L), direct bilirubin (DB; 13.2 mumol/L; reference range: 0.0-7.1 mumol/L), indirect bilirubin (27.4 mumol/L; reference range: 1.7-19.0 mumol/L), alanine aminotransferase (ALT) (68 U/L; reference range: 3-50 U/L), aspartate aminotransferase (59 U/L; reference range 8-40 U/L), alkaline phosphatase (296 U/L; reference range: 20-145 U/L), and glutamyl transpeptidase (233 U/L; reference range: 3-40 U/L) levels. Prothrombin time (PT) extended to 28.5 s (reference range: 12.0-14.8 s) and the prothrombin activity (PTA) was 28.0% (reference range: 80.0-120.0%). Furthermore, the blood ammonia level increased to 80 micromol/L (reference range: 10.0-47.0 micromol/L) without any evidence of HE.
Based on the abovementioned findings and personal preference, he was admitted to Children's Hospital, Zhejiang University School of Medicine to perform contrast-enhanced CT, which confirmed the presence of direct communication between PV and IVC ( Figure 1D ). Contrast-enhanced CT revealed the presence of hepatic nodules, which were misinterpreted as hemangiomas.
Subsequently, he was hospitalized in several hospitals in Shanghai, where lung CT, electrocardiogram, tumor marker (alpha fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 199) levels, hepatitis B antigen-antibody, and cranial magnetic resonance imaging (MRI) showed normal results. Hepatic nodule puncture was performed (three puncture tissues at the center and edge of hepatic nodules were collected), with a pathological diagnosis of focal nodular hyperplasia (FNH) or nodular regenerative hyperplasia (NRH). DSA revealed hepatic vascular malformation, LPV was closely associated with IVC, and RPV was not clearly visualized. He was finally diagnosed with intrahepatic portosystemic shunts (IPSS), and interventional therapy was used to occlude the shunt.
The patient visited our hospital 6 months after the shunt closure. Abdominal US revealed the complete disappearance of multiple FNH/NRH ( Figure 2A ), and the diameter of LPV was 1.3 cm, with a hyperechoic area of 2.1 x 1.3 cm between LPV and IVC ( Figure 2B ). Subsequent CT examination confirmed that this hyperechoic area was a metal occluder placed after the interventional therapy. Color Doppler US showed bidirectional venous waves in the intraportal spectrum ( Figure 2C ). Blood tests were repeated several times after the operation, which showed normal blood ammonia level and liver function gradually. No complication occurred during the 3-year follow-up after the shunt occlusion. Abdominal contrast-enhanced CT revealed normal liver size and shape, without local density abnormality and with annular metal density shadows in the hilum of the liver 3 years after the interventional therapy ( Figure 2D ). The process of disease discovery to patient diagnosis and treatment is shown in  Figure 3  as a flowchart.
Abdominal ultrasound and contrast-enhanced computed tomography (CT) reexamination were performed 3 years after the interventional therapy. contrast-enhanced CT on this reconstructed coronal view showed annular metal density shadows in the hilum of the liver (arrows).