Opitz trigonocephaly syndrome presenting with sudden unexplained death in the operating room: a case report
نویسندگان
چکیده
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.
منابع مشابه
Opitz trigonocephaly syndrome died during surgery presenting with sudden unexplained death in the operating room: a case report
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Trigonocephaly and the Opitz C syndrome.
We present 12 cases of trigonocephaly of which six were associated with other malformations. On the basis of this experience we examine the diagnostic criteria for the presumed autosomal recessive trigonocephaly C syndrome.
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