Abstract
We reported three cases of trigonocephaly associated with trisomy 13 in neonates. They cvere transferred to our hospital for many dysmorphic features and respiratory disturbance. CT scan revealed the abnormal findings characteristic of trigonocephaly, including keel shaped forehead, hypertrophic bone ridge at the metopic suture and hypotelorism. In any of these cases, no abnormalities of central nervous system were demonstrated except microencephaly. However, at autopsy one patient was found to have arhinencephaly (aplasia of olfactory bulbs and tracts) and agenesis of the corpus callosum. We reviewed the literature and discussed the clinical and neuroradiological features in trigonocephaly as well as chromosomal abnormality and hereditary aspects.