In Utero Platelets Transfusion in Fetuses with TAR Syndrome: Long-Term Follow Up

  • Professor Boris Petrikovsky, Department of Obstetrics and Gynecology, New York Downtown Hospital, New York, USA, United States
  • Dr L Wyse, Division of Maternal Fetal Medicine, North Shore University Hospital, Manhasset, USA., United States
  • Dr M Weinstein, Department of Obstetrics and Gynecology, Winthrop University Hospital, Mineola, USA, United States
  • Dr Emil Gurshumov, Department of Obstetrics and Gynecology, New York Downtown Hospital, New York, USA, United States
  • Objectives: Thrombocytopenia with absent radius (TAR syndrome) is an autosomal recessive disorder, which includes bilateral absence of radius with hypoplastic or absent ulna, occasional micrognatia, cardiac and renal anomalies and thrombocytopenia. We report our experience with three long-term follow up cases of in utero platelets transfusions in TAR fetuses.
    Methods and materials: Three fetuses underwent for in utero platelet transfusion between 36 and 39 weeks of gestation.
    Results: Initial platelet count was below 20,000 in 2 cases and 40,000 in one.
    Case # 1 transfused at 37 weeks, delivered at 39 weeks via cesarean delivery. Platelet count at birth - 39,000.
    Case # 2 transfused at 38 weeks, delivered at 40 weeks via cesarean delivery. Platelet count at birth - 27,000.
    Case # 3 transfused at 40 weeks, delivered at 40 weeks via NSVD. Platelet count at birth - 92,000
    During first year of life, all infants required between two and four transfusions of single-donor irradiated platelets. Two are alive. One is doing well 14 years later, active in baseball after corrective hand surgery. One lost to follow up after 3 years.
    Conclusions: In utero platelets transfusion should be considered in TAR syndrome fetuses allowing for a chance of vaginal birth.
    References: 1) Weinblatt M, Petrikovsky B, Bialer M, et al, Prenatal evaluation and in utero platelet transfusion for thrombocytopenia absent radii syndrome. Prenat Diagn. 1994 Sep;14(9):892-6.