Fetal hypokinesia syndrome in the monochorionic pair of a triplet pregnancy secondary to severe disruptive cerebral injury.
We report a set of triplets, two of whom were monochorionic diamnionic and demonstrated cerebellar hypoplasia and progressive arthrogryposis on an antenatal sonogram. At delivery the infants exhibited a Pena-Shokier phenotype. At autopsy, the twins were concordant for severe disruptive lesions of the cerebrum. The mechanism resulting in the devastating symmetric lesions may have been a transient cerebral vascular compromise associated with placenta vascular anastomoses characteristic of monochorionic twinning. This report accentuates the vulnerability of the monochorionic twin for ischemic cerebral injury.
Perlman
J M
JM
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas 75235-9063, USA.
Burns
D K
DK
Twickler
D M
DM
Weinberg
A G
AG
D000015:Abnormalities, Multiple; D001176:Arthrogryposis; D001921:Brain / Q000473:pathology*; D002540:Cerebral Cortex / Q000098:blood supply* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D005315:Fetal Diseases / Q000473:pathology*; D006801:Humans; D018476:Hypokinesia; D007511:Ischemia / Q000473:pathology*; D008297:Male; D013577:Syndrome; D014310:Triplets; D014430:Twins, Monozygotic