Abstract
IntroductionOpitz 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 presentationWe describe the case of a Caucasian male baby who died at five months of age during surgical correction of the craniofacial anomaly.ConclusionAs previously reported, OTCS may have an increased mortality rate during craniofacial surgery. Careful evaluation of surgery risk-benefit ratio is warranted in such patients.
Highlights
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
Opitz trigonocephaly C syndrome (OTCS) is a rare and heterogeneous genetic disorder characterized by synostosis of metopic suture, dysmorphic facial features, variable mental retardation and other congenital somatic and cerebral anomalies
We describe a white male baby who died at five months of age during surgery performed to correct the craniofacial anomaly
Summary
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. Introduction Opitz trigonocephaly C syndrome (OTCS) is a rare and heterogeneous genetic disorder characterized by synostosis of metopic suture, dysmorphic facial features, variable mental retardation and other congenital somatic and cerebral anomalies. We describe a white male baby who died at five months of age during surgery performed to correct the craniofacial anomaly.
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