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. Clinical Relevance: 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.
[Show abstract][Hide abstract] ABSTRACT: Noonan syndrome (NS) is a relatively common condition characterized by chest deformation, congenital heart disease, short stature and distinctive facial features. Due to its genetic heterogeneity NS patients exhibit a range of clinical signs. Severe gingivitis and supernumerary teeth are rarely seen in connection with NS. In addition, there has not been a report on NS patients with atypical bilateral enlargement of the mental foramens and inferior-alveolar canals. This case report describes a NS patient who has undergone growth hormone (GH) therapy and is presenting with classical and rare NS phenotypes.
The Journal of clinical pediatric dentistry 12/2011; 36(2):197-202. DOI:10.17796/jcpd.36.2.81074271088334h2 · 0.35 Impact Factor
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