Opitz trigonocephaly syndrome presenting with sudden unexplained death in the operating room: a case report

INTRODUCTION: Opitz trigonocephaly C syndrome (OTCS) is a rare malformation syndrome with the following features: synostosis of metopic suture, craniofacial abnormalities, severe mental retardation and a multitude of pathological findings affecting almost every organ system. OTCS is associated with a high mortality rate. CASE PRESENTATION: We describe the case of a Caucasian male baby who died at five months of age during surgical correction of the craniofacial anomaly. CONCLUSION: As previously reported, OTCS may have an increased mortality rate during craniofacial surgery. Careful evaluation of surgery risk-benefit ratio is warranted in such patients.