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

Abstract

'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-totwin transfusion syndrome (TTTS), CASE REPORTA 39-year-old woman, gravida 9 para 3, was referred for a fetal anatomy scan at 15 weeks' gestation. The patient had had two Cesarean deliveries near term due to pregnancyinduced hypertension. She had had three terminations of pregnancy due to fetal molar pregnancy, chromosomal abnormality and severe early oligohydramnios in the second trimester. She had also had two early spontaneous miscarriages. Blood tests revealed the patient was positive for lupus anticoagulant, and she was also found to be homozygotic for methyltetrahydrofolate reductase mutation. In the current pregnancy, because of her history of miscarriage and thrombophilia, the patient was placed on prophylactic treatment with low molecular weight heparin and aspirin in addition to folate. The current pregnancy was induced by a follicle-stimulating hormone/human chorionic gonadotropin combination for ovulation induction. The pregnancy was recognized to be monochorionic-diamniotic twins at 11 weeks' gestation. At that time, the twins were found to be concordant for nuchal translucency thickness (NT), which measured 1.94 and 1.81 mm. A targeted anatomic scan was performed in the 15 th week of pregnancy and showed both twins to be appropriate in size for gestational age, with normal and concordant amounts of amniotic fluid and normal anatomy scan. In the 20 th week, however, signs of tricuspid regurgitation were seen on examination of Twin 1. The liver was hypoechoic and edematous, with increased brightness of the portal venule walls, which suggested the appearance of 'starry sky' (Figure 1). The fetal weight was estimated to be 288 g for Twin 1 and 264 g for Twin 2. Acute viral infection was ruled out by serologic analysis.In week 21, signs of worsening heart failure were noticed while the typical triphasic waveform in the inferior vena cava (IVC) was replaced by a biphasic flow profile that consisted of only the ventricular component and the atrial contraction (Figure 2 and Videoclips S1 and S2). Twin 2 at that time had relative oligohydramnios. A few days later, we also noted in the recipient twin relative polyhydramnios and edema of the recipient's placental domain, defined as twin-to-twin transfusion syndrome (TTTS) Quintero stage I-II.Repeat examination in the 22 nd and 23 rd weeks showed worsening signs of liver edema, tricuspid regurgitation (Videoclip S3), right-sided heart failure and severe

Sonographic Detection of Starry Sky Liver in a Pregnancy With Twin-Twin Transfusion Syndrome

“…8 The appearance of a starry sky liver was originally synonymous with acute hepatitis but over the years has been identified in patients with leukemia, toxic shock syndrome, Burkett's lymphoma, and right-sided heart failure. 9 It has recently been reported in a case of TTTS 10 and a less common variant of TTTS, anemia-polycythemia sequence. 11 The case reported by Segev et al 10 described a starry sky liver in conjunction with TTTS that closely resembled our case.…”

Twin Anemia-Polycythemia Sequence (Zwillings-Anämie-Polyzythämie-Sequenz)

“…Zusätzlich kann der polyzythämische Rezipient eine hypoechogene Hepatomegalie mit echoreichen Portalvenenwänden aufweisen ("starry sky liver"). Diese Ultraschallmarker können bereits vor den dopplersonografischen Veränderungen in der ACM auftreten [14,15].…”