Morice Anne, Harroche Annie, Cairet Pascale, Khonsari Roman H
Assistant Professor, Department of Maxillofacial and Plastic Surgery, APHP, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Sorbonne Paris Cité, APHP, Paris; Centre de références des malformations de la face et de la cavité buccale, Paris, France.
Consultant, Hemophilia Care Centre, Department of Hematology, APHP, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Sorbonne Paris Cité, APHP, Paris, France.
J Oral Maxillofac Surg. 2018 Jul;76(7):1553-1558. doi: 10.1016/j.joms.2017.12.012. Epub 2017 Dec 29.
Patients with Noonan syndrome often require surgery at young ages. They are at high risk of perioperative bleeding from coagulation defects that might not have been detected by routine screening. These risks are rarely described in the oral and maxillofacial surgery (OMS) literature. The aim of this study was to evaluate the perioperative bleeding risks associated with Noonan syndrome and to propose preoperative guidelines.
This report describes a retrospective case series of patients with Noonan syndrome who underwent OMS procedures during a continuous observational period (2013 through 2016) in the authors' center. Clinical data, blood screening test results, and perioperative bleeding were analyzed.
Five patients (age, 4 to 20 yr) with Noonan syndrome who underwent OMS procedures were included in this study. One patient presented a spontaneous bleeding tendency (epistaxis requiring cauterization). Blood screening showed clotting defects in 3 patients. One patient presented abnormal perioperative bleeding owing to a mild defect in factor XI.
Patients with Noonan syndrome must be referred to a hematologist for specific preoperative investigations and for adapted perioperative management.
努南综合征患者常在年轻时需要进行手术。他们因凝血缺陷而面临围手术期出血的高风险，而这种缺陷可能无法通过常规筛查检测出来。这些风险在口腔颌面外科（OMS）文献中很少被描述。本研究的目的是评估与努南综合征相关的围手术期出血风险，并提出术前指导方针。
本报告描述了一系列回顾性病例，这些患有努南综合征的患者在作者所在中心连续观察期（2013年至2016年）内接受了OMS手术。对临床数据、血液筛查测试结果和围手术期出血情况进行了分析。
本研究纳入了5例（年龄4至20岁）接受OMS手术的努南综合征患者。1例患者有自发出血倾向（鼻出血需烧灼止血）。血液筛查显示3例患者有凝血缺陷。1例患者因因子XI轻度缺陷出现围手术期异常出血。
必须将努南综合征患者转诊给血液科医生进行特定的术前检查和适当调整的围手术期管理。
