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. 2011 Jun 21:5:222.
doi: 10.1186/1752-1947-5-222.

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

Laura Travan  1 , Vanna Pecile, Mariacristina Fertz, Antonella Fabretto, Pierpaolo Brovedani, Sergio Demarini, John M Opitz
Affiliations

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

Laura Travan et al. J Med Case Rep. .

Abstract

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.

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Figures

Figure 1
Figure 1
Three-dimensional computerized tomography. See the fusion of metopic suture.
Figure 2
Figure 2
Cerebral MRI (whitened T2 sequences), showing diffuse white matter periventricular hyperintensity (hypointensity in T1 sequences).
See this image and copyright information in PMC

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