Travan Laura, Pecile Vanna, Fertz Mariacristina, Fabretto Antonella, Brovedani Pierpaolo, Demarini Sergio, Opitz John M
Neonatal Intensive Care Unit, Institute for Maternal and Child Health Burlo Garofolo, Via dell'Istria 65/1, 34100, Trieste, Italy.
J Med Case Rep. 2011 Jun 21;5:222. doi: 10.1186/1752-1947-5-222.
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.
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.
奥皮茨三角头畸形C综合征（OTCS）是一种罕见的畸形综合征，具有以下特征：额缝早闭、颅面异常、严重智力迟钝以及影响几乎每个器官系统的众多病理表现。OTCS与高死亡率相关。
我们描述了一名白种男婴的病例，该男婴在颅面畸形手术矫正过程中于五个月大时死亡。
如先前报道，OTCS在颅面手术期间可能有更高的死亡率。对此类患者进行手术风险效益比的仔细评估是必要的。
