Monitoring of fetus with osteogenesis imperfecta and severe anemia

Abstract

The patient was a 27-year-old woman, gravida 3, para 2, with two previous normal term deliveries. The blood was type 0, Rh positive, with no abnormal antibodies. The prenatal course was unremarkable until 26 weeks of gestation when she complained of spotting. Examination revealed the cervix to be 80 per cent effaced and 1 cm. dilated. A cerclage was placed on the cervix and she was treated with bed rest. At 36 weeks of gestation spontaneous contractions ensued and the cerclage was removed. Fetal monitoring revealed a base-line heart rate of 160 b.p.m. with a beat-to-beat variability of up to 12 b.p.m. Contractions registered every three and a half minutes and the peak of each contraction was followed by a typical late deceleration. The severity of bradycardia varied from 10 to 50 b.p.m. and its duration ranged from 80 to 200 seconds. In addition to the uniform patterns of late fetal bradycardia there were wide and erratic shifts of the base-line from 160 down to 65 b.p.m. for three to five minutes unassociated with contractions. To confirm the presence of a congenital malformation, an abdominal roentgenogram was obtained and it revealed the characteristic skeletal hypomineralization and the multiple intrauterine fractures of osteogenesis imperfecta. A female infant, weighing four pounds and nine ounces, was delivered by cesarean section. The newborn infant had severe deformities due to the fractures and marked hydrops and died 15 minutes after birth. Cord blood evaluation revealed: hemoglobin, 8.4 Gm.; hematocrit, 27.5 per cent: reticulocytes, 14.7 per cent; a negative direct Coombs test was 100 per cent hemoglobin F on electrophoresis.