Rapid Onset of Severe Twin--Twin Transfusion Syndrome Caused by Placental Venous Thrombosis

Byline: Peter G.J. Nikkels (1), Martin J.C. van Gemert (2), Krystyna M. Sollie-Szarynska (3), Harry Molendijk (4), Bert Timmer (5), Geoffrey A. Machin (6) Keywords: Key words: monochorionic twin pregnancy, placental anastomoses, twin--twin transfusion syndrome, venous thrombosis Abstract: We report a case of rapid onset of severe twin--twin transfusion syndrome (TTTS) at 25 weeks gestation in a monochorionic twin pregnancy that was uneventful before that time. Thrombosis of a main venous branch draining several arteriovenous (AV) anastomoses to the donor changed the previous hemodynamic balance that existed between multiple bidirectional AV anastomoses. The opposing AVs became hemodynamically uncompensated and, despite amnioreductions, severe TTTS developed. At 27 weeks a cesarean section was performed because of worsening cardiotocography parameters of both fetuses. Birth weights were 750 and 1840 g, and initial hemoglobin concentrations were 9.2 and 13.4 mmol/liter for donor and recipient, respectively. The recipient twin died 5 months later of an ischemic, necrotic, and perforated small intestine due to a thrombosed superior mesenteric artery. The donor is well at 2.5 years. No abnormalities in several factors associated with thrombophilia, including factor V Leiden mutations, were found in the parents. Author Affiliation: (1) Department of Pathology, University Medical Center Utrecht, PO Box 85500, 3508 GA The Netherlands , NL (2) Laser Center and Department of Obstetrics and Gynecology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands , NL (3) Department of Obstetrics and Gynecology, University Hospital Groningen, Groningen, The Netherlands , NL (4) Department of Neonatology, University Hospital Groningen, Groningen, The Netherlands , NL (5) Department of Pathology, University Hospital Groningen, Groningen, The Netherlands , NL (6) Department of Pathology, Kaiser Permanente, Oakland, CA, USA , US Article note: Received September 25, 2001 accepted January 22, 2002.