Dental management of a child patient with Noonan's syndrome.

Aberdeen Dental Institute, Cornhill Road, Aberdeen AB25 2ZR, K.
Dental update 04/2007; 34(2):117-8, 120.
Source: PubMed


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.

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    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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    ABSTRACT: Noonan syndrome (NS) was described by Noonan and Ehmke as a multi-system disorder, which is typically evident at birth. The incidence of this syndrome is estimated to be one per 2500 to one per 1000 and affects both genders. While the clinical manifestations of NS have been well documented, the oral manifestations have not been extensively discussed. The purpose of the present article is to (a) review the oral manifestations of NS reported in the literature, and (b) describe four cases (three females and one male) of NS, who presented with short stature, cardiac problems and various oral findings. Based on these cases, we conclude that many oral anomalies may have possible relationships with NS, which require multidisciplinary treatment planning and timely management. The importance of oral findings in NS has largely gone unnoticed and it is essential to consider oral manifestations as scoring criteria in the diagnosis of NS.
    Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie 12/2014; 55(4):1503. · 0.66 Impact Factor

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