Preoperative Detailed Coagulation Tests Are Required in Patients With Noonan Syndrome

Publication date: July 2018

Source: Journal of Oral and Maxillofacial Surgery, Volume 76, Issue 7

Author(s): Anne Morice, Annie Harroche, Pascale Cairet, Roman H. Khonsari

Purpose

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.

Materials and Methods

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.

Results

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.

Conclusion

Patients with Noonan syndrome must be referred to a hematologist for specific preoperative investigations and for adapted perioperative management.

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