INTRAUTERINE GROWTH AND ULTRASOUND FINDINGS IN FETUSES WITH BECKWITH-WIEDEMANN SYNDROME

Objective: To assess intrauterine growth in a series of nine fetuses d iagnosed with Beckwith-Wiedemann syndrome. Methods: Infants confirmed postnatally to have Beckwith-Wiedemann syndrome were identified from r ecords maintained in the Division of Clinical Genetics. Antenatal ultr asound and birth records were evaluated. Head circumference (HC), abdo minal circumference (AC), and estimated fetal weight (EFW) were assign ed percentiles based on gestational age. Newborn HC and birth weight w ere also assigned percentiles. Polyhydramnios was diagnosed using eith er amniotic fluid index or documented subjective assessment. Results: Nine infants with Beckwith-Wiedemann syndrome had antenatal ultrasound examinations. Seven of these had more than one examination. Two infan ts were suspected to have Beckwith-Wiedemann syndrome in utero. Import ant ultrasound findings included omphalocele (four), enlarged liver an d kidneys (one), and enlarged liver (one). Fetal tongue protrusion on ultrasound was not identified in any fetus. Six of nine fetuses (66%) with ultrasound examinations after 25 weeks' gestation had polyhydramn ios. Evaluation of the fetal HC, AC, and EFW percentiles demonstrated that fetuses with Beckwith-Wiedemann syndrome may exhibit accelerated growth as early as 25-30 weeks' gestation, but may exceed the 90th per centile only after 36 weeks' gestation. Conclusions: Fetuses with omph alocele, polyhydramnios, and an AC less than the 90th percentile may h ave Beckwith-Wiedemann syndrome. Polyhydramnios and accelerated growth beginning between 25 and 36 weeks' gestation, even without omphalocel e, should alert the physician to the possibility of: Beckwith-Wiedeman n syndrome. (C) 1947 by The American College of Obstetricians and Gyne cologists.