Starry sky pattern of fetal liver sonogram as first sign of twin-twin transfusion syndrome

'Starry sky' liver is one of the most common sonographic patterns in diffuse liver disease. It is characterized by clearly identified portal venules due to diminished parenchymal echogenicity. In advanced cases of twin-to-twin transfusion syndrome (TTTS), volume overload is considered the key factor in the pathogenesis of cardiac dysfunction of the recipient twin. When right-sided failure occurs, the liver might show signs of edema and, as in acute hepatitis, the appearance of starry sky might develop. We present a case in which the sonographic appearance of starry sky liver, along with right-sided cardiac failure (tricuspid regurgitation), were the first signs of TTTS in monochorionic twins at 20 weeks. A short time later, at 21 weeks, other signs of overload and signs of worsening heart failure were noted, as the typical triphasic waves in the inferior vena cava were replaced by a biphasic flow profile. Twin 2 at that time had relative oligohydramnios. A few days later, relative polyhydramnios and edema of the placental domain of the recipient twin were also noted. To the best of our knowledge, this is the first case report describing this hepatic sonographic pattern as an early sonographic sign of TTTS.