Sindrome de williams
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Home > Volume 76, Issue 901 > Article
Postgrad Med J2000;76:712 doi:10.1136/pmj.76.901.712
Images in medicine
Dental anomalies in Williams syndrome
A S KASHYAP
Department of Medicine
Armed Forces Medical College
Pune 411040, India
Department of Dental Surgery
Armed Forces Medical College
Pune 411040, India
H S SHARMA,
P KUMAR
+ Author Affiliations
Department of Medicine
Armed Forces Medical College
Pune 411040, India
Department of Dental Surgery
Armed Forces Medical College
Pune 411040, India
A 2 year old boy with Williams syndrome was undergoing preoperative evaluation for supravalvular aortic stenosis. On examination his mouth was wide with full lower lip. He had small, irregular, widely spaced, peg shaped primary teeth with extensive caries (fig 1). These are the typical anomalies of primary teeth in Williams syndrome. In adulthood teeth tend to be crowded. His ionised serum calcium concentration was 1.6 mmol/l (normal 1.1–1.4 mmol/l), and 25-dihydroxycholecalciferol 454 pmol/l (36–144 pmol/l). The serum 25-hydroxycholecalciferol and phosphate levels were normal. He is on calcium, and a vitamin D restricted diet, and is undergoing treatment for dental caries. He is awaiting balloon dilatation for the supravalvular aortic stenosis.
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Figure 1
Dental anomalies in patient (photograph reproduced with permission).
Williams syndrome usually occurs as a sporadic new