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

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.

We describe the case of a Caucasian male baby who died at five months of age during surgical correction of the craniofacial anomaly.

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.