Pre-operative detailed coagulation tests are required in patients with Noonan syndrome

Publication date: Available online 29 December 2017
Source:Journal of Oral and Maxillofacial Surgery
Author(s): A. Morice, A. Harroche, P. Cairet, R.-H. Khonsari
PurposePatients with Noonan syndrome often require surgery at early ages, They are at high risk of peri-operative bleeding due to coagulation defects that may not have been detected by routine screening. These risks are rarely described in the specific field of oral and maxillofacial surgery (OMS). The aim of our study was to evaluate the peri-operative bleeding risks associated with Noonan syndrome and to propose pre-operative guidelines.MethodsHere we report a retrospective case series of patients with Noonan syndrome who underwent oral and maxillofacial procedures during a continuous observational period (2013-2016) in our center. Clinical data, blood screening tests and peri-operative bleeding were analyzed.ResultsFive patients (aged 4-20) with Noonan syndrome who underwent OMS procedures were included into our study. One patient presented spontaneous bleeding tendency (epistaxis requiring cauterization). Blood screening revealed clotting defects in three patients. One patient presented abnormal peri-operative bleeding due to a mild defect in factor XI.ConclusionPatients with Noonan syndrome must be referred to a haematologist for specific pre-operative investigations and for adapted peri-operative management.



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