Dental management of a child patient with Noonan's syndrome.
This case report describes a 9-year-old Caucasian girl who required comprehensive dental treatment under general anaesthesia but gave a history of Noonan's syndrome. The patient was extremely needle phobic. Because of the association between Noonan's syndrome and underlying coagulopathies, for which no previous investigations were evident, dental treatment had to be postponed pending further investigation. The patient was referred to a haematologist and underwent coagulation screening, which revealed the presence of von Willebrand's disease. The patient was prescribed Desmopressin to raise plasma levels of factor VIII: C and von Willebrand's factor (VWF) in order that dental treatment, including extractions, could be carried out under an in-patient general anaesthetic.


CONCLUSIONS
Congenital heart defects and bleeding diatheses are regarded as a common association of Noonan's syndrome. Witt et al estimated that around one-third of the patients have an associated bleeding disorder, although a later report suggested that as many as 74% of the coagulation profiles could be abnormal. Most of the bleeding problems are reported to be mild, and resolve with age in some patients, but, clearly, they may cause problems during dental treatment, necessitating haematological investigations and a multidisciplinary approach.
Sharma
Paul R
PR
Aberdeen Dental Institute, Cornhill Road, Aberdeen AB25 2ZR, K.
MacFadyen
Una M
UM
Fung
Diane E
DE
D000768:Anesthesia, General; D002648:Child; D003190:Comprehensive Dental Care; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D017742:Dental Care for Chronically Ill; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D009634:Noonan Syndrome / Q000150:complications; D014842:von Willebrand Diseases / Q000150:complications / Q000188:drug therapy
D006490:Hemostatics; D003894:Deamino Arginine Vasopressin