Display options
Cite
Travan L, Pecile V, Fertz M, et al. Opitz trigonocephaly syndrome presenting with sudden unexplained death in the operating room: a case report. J Med Case Rep. 2011;5:222doi: 10.1186/1752-1947-5-222.
Travan, L., Pecile, V., Fertz, M., Fabretto, A., Brovedani, P., Demarini, S., & Opitz, J. M. (2011). Opitz trigonocephaly syndrome presenting with sudden unexplained death in the operating room: a case report. Journal of medical case reports, 5222. https://doi.org/10.1186/1752-1947-5-222
Travan, Laura, et al. "Opitz trigonocephaly syndrome presenting with sudden unexplained death in the operating room: a case report." Journal of medical case reports vol. 5 (2011): 222. doi: https://doi.org/10.1186/1752-1947-5-222
Travan L, Pecile V, Fertz M, Fabretto A, Brovedani P, Demarini S, Opitz JM. Opitz trigonocephaly syndrome presenting with sudden unexplained death in the operating room: a case report. J Med Case Rep. 2011 Jun 21;5:222. doi: 10.1186/1752-1947-5-222. PMID: 21689463; PMCID: PMC3141689.
Copy Download .nbib Format: NLM
Share it on

J Med Case Rep. 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.

Journal of medical case reports

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

Affiliations

  1. Neonatal Intensive Care Unit, Institute for Maternal and Child Health Burlo Garofolo, Via dell'Istria 65/1, 34100, Trieste, Italy. [email protected].

PMID: 21689463 PMCID: PMC3141689 DOI: 10.1186/1752-1947-5-222

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.

References

  1. Fetal Pediatr Pathol. 2006 Jul-Aug;25(4):211-31 - PubMed
  2. Am J Med Genet. 1981;9(2):147-63 - PubMed
  3. Pediatr Int. 2003 Dec;45(6):731-3 - PubMed
  4. Am J Med Genet. 1990 Dec;37(4):451-6 - PubMed
  5. Am J Med Genet A. 2003 Mar 1;117A(2):127-35 - PubMed
  6. Am J Hum Genet. 2007 Oct;81(4):835-41 - PubMed
  7. Am J Med Genet. 1997 Dec 31;73(4):484-8 - PubMed

Publication Types