Clinical Presentation

A neonate born to a diabetic mother presents with hypoglycemia and hypertension.

Figure 66A

Figure 66B (See Color Plate 66B.)

Radiologic Findings

Figure 66C Abdominal radiograph of a newborn showing the tip of the umbilical vein catheter projecting in the region of the right portal vein (arrow).

Figure 66D Axial image at the level of the porta hepatis demonstrates multiple anechoic areas of vascular origin on ultrasound representing cavernous transformation of the portal vein.

Diagnosis

Thrombosis of the left portal vein

Differential Diagnosis

• Acute portal vein thrombosis:
  
  
  
  • Umbilical vein catheterization
    
  • Underlying clotting anomalies including antithrombin III deficiency and protein C or S deficiency, antiphospholipid antibody syndrome
    
  • Pylephlebitis complicating an intra-abdominal inflammation (appendicitis, pancreatitis)
  
  
• Chronic portal vein thrombosis:
  
  
  
  • Idiopathic
    
  • Sequelae of omphalitis or umbilical vein catheterization in newborns
    
  • Portal hypertension (prehepatic)
  
  
• Different conditions:
  
  
  
  • Veno-occlusive disease (hepatic block)
    
  • Congenital absence of portal vein

Discussion

Background

Figure 66E CT demonstrating varices along the lesser curvature of the stomach in a child with splenomegaly.