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

Travan L, Pecile V, Fertz M, Fabretto A, Brovedani P, Demarini S, Opitz JM - J Med Case Rep (2011)

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Neonatal Intensive Care Unit, Institute for Maternal and Child Health Burlo Garofolo, Via dell'Istria 65/1, 34100, Trieste, Italy. ltravan@yahoo.it.

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.

No MeSH data available.


Related in: MedlinePlus

Cerebral MRI (whitened T2 sequences), showing diffuse white matter periventricular hyperintensity (hypointensity in T1 sequences).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3141689&req=5

Figure 2: Cerebral MRI (whitened T2 sequences), showing diffuse white matter periventricular hyperintensity (hypointensity in T1 sequences).

Mentions: At 40 wks post-conceptional age brain MRI showed a small area of hyper-intensity under the posterior horn of the left ventricle (interpreted as calcification of a periventricular hemorrhage) and a diffused alteration of white periventricular matter (Figure 2).


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

Travan L, Pecile V, Fertz M, Fabretto A, Brovedani P, Demarini S, Opitz JM - J Med Case Rep (2011)

Cerebral MRI (whitened T2 sequences), showing diffuse white matter periventricular hyperintensity (hypointensity in T1 sequences).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3141689&req=5

Figure 2: Cerebral MRI (whitened T2 sequences), showing diffuse white matter periventricular hyperintensity (hypointensity in T1 sequences).
Mentions: At 40 wks post-conceptional age brain MRI showed a small area of hyper-intensity under the posterior horn of the left ventricle (interpreted as calcification of a periventricular hemorrhage) and a diffused alteration of white periventricular matter (Figure 2).

Bottom Line: 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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Neonatal Intensive Care Unit, Institute for Maternal and Child Health Burlo Garofolo, Via dell'Istria 65/1, 34100, Trieste, Italy. ltravan@yahoo.it.

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.

No MeSH data available.


Related in: MedlinePlus