The twin-twin transfusion syndrome.

The aetiology of twin-twin transfusion syndrome (TTTS), which affects 10-15% of monochorionic (MC) twin pregnancies, remains poorly understood. Although all MC twins have placental vascular anastomoses, unbalanced intertwin transfusion has been shown by ex vivo injection and in vivo Doppler studies of chorionic plate vasculature to be mediated by > or =1 arterio-venous anastomoses (AVA) in association with absent bi-directional arterio-arterial anastomoses (AAA). TTTS presents in the mid trimester with the oligo-polyhydramnios sequence, the donor may have a small or non-visible bladder and abnormal umbilical artery Doppler, while the recipient has a large bladder and may develop cardiac hypertrophy, triscupid regurgitation, and eventually hydrops. Recently, discordant renal renin angiotensin expression, endothelin and atrial natriuretic peptide have been implicated in the pathogenesis. Survival has increased from <20% to <60-70% with modern treatments, although survivors remain at increased risk of antenatally acquired cerebral white matter injury, and neurodevelopmental sequelae are documented in c.10% (range 5-23%). The recent introduction of a staging system for TTTS facilitates selection of therapy with less invasive amnioreduction and septostomy preferred for early stage disease, and more aggressive modalities such as laser ablation and cord occlusion with their attendant risk of procedure related fetal loss, reserved for advanced stage disease.