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The twin-twin transfusion syndrome
1Centre for Fetal Care, Queen Charlotte's & Chelsea Hospital, Imperial College of Science, Technology and Medicine, Hammersmith Campus, Du Cane Road, London, W12 0NN, UK. l.wee@ic.ac.uk
Seminars in Neonatology : SN
|September 18, 2002
Summary
Twin-twin transfusion syndrome (TTTS) in monochorionic (MC) twins is poorly understood, often caused by specific placental vascular connections. Modern treatments improve survival but risks of neurological injury remain.
Area of Science:
- Perinatology
- Fetal Medicine
- Reproductive Biology
Background:
- Twin-twin transfusion syndrome (TTTS) affects 10-15% of monochorionic (MC) twin pregnancies.
- The precise etiology of TTTS, particularly the role of placental vascular anastomoses, is not fully elucidated.
- Understanding TTTS pathogenesis is crucial for improving fetal outcomes in high-risk pregnancies.
Purpose of the Study:
- To review the current understanding of TTTS etiology, clinical presentation, and management.
- To highlight recent advances in understanding the pathophysiology, including hormonal and vascular factors.
- To discuss the evolution of treatment strategies and their impact on survival and neurodevelopmental outcomes.
Main Methods:
- Review of ex vivo injection studies and in vivo Doppler assessments of chorionic plate vasculature.
- Analysis of clinical data on TTTS presentation, including diagnostic criteria and staging.
- Examination of recent research on the role of renin-angiotensin system and natriuretic peptides.
Main Results:
- TTTS is mediated by arterio-venous anastomoses (AVA) in conjunction with absent bi-directional arterio-arterial anastomoses (AAA).
- Clinical manifestations include oligo-polyhydramnios sequence, donor bladder abnormalities, and recipient cardiac issues.
- Survival rates have improved to 60-70% with modern interventions, but neurodevelopmental sequelae affect approximately 10% of survivors.
Conclusions:
- Effective management of TTTS relies on accurate staging to guide treatment selection, from less invasive procedures to more aggressive interventions.
- While survival has improved, long-term neurodevelopmental risks necessitate continued research and monitoring.
- Further understanding of TTTS pathophysiology may lead to even better therapeutic strategies and improved long-term outcomes for affected twins.