Ad. Weeks et al., THE SEQUENTIAL IN-UTERO DEATH OF HETEROKARYOTIC MONOZYGOTIC TWINS - ACASE-REPORT AND LITERATURE-REVIEW, Prenatal diagnosis, 16(7), 1996, pp. 657-663
A case of monozygotic twins in a 19-year-old primigravida is presented
. Ultrasound examination at 15 weeks' gestation showed one twin to hav
e a cystic hygroma and hydrops fetalis. The other twin appeared normal
. The twins appeared to occupy the same amniotic cavity. Fluid was tak
en from the cystic hygroma under ultrasound guidance for karyotyping a
nd this showed 45,XO chromosomes. Conservative management was adopted.
Serial ultrasound examination showed deteriorating hydrops and at 26
weeks the first twin died. Intensive monitoring of the remaining twin
was undertaken with weekly ultrasound, cardiotocography (CTG), and clo
tting screens. At 29 weeks' gestation the CTG and clotting were normal
, but ultrasound revealed that multicystic encephalomalacia had develo
ped in the Second twin. A very thin dividing membrane was seen for the
first time between the twins. The parents decided to terminate the pr
egnancy. Prior to an intracardiac potassium chloride injection, a feta
l blood sample was taken which revealed 46,XX chromosomes and a normal
clotting screen including natural anticoagulant levels. Labour was th
en induced. Delivery took place 5 h later and the woman made an uneven
tful recovery. The mechanism for genetic differences between monozygot
ic twins is discussed and the literature reviewed. A non-disjunction e
vent around the time of splitting of the twins is proposed as the caus
e. The prognosis for the remaining twin is also discussed, as is the p
athogenesis of the cerebral damage.