Medical considerations in dental treatment of children with Williams syndrome

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Medical considerations in dental treatment of children with Williams syndrome
Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology
Volume 99, Issue 5, May 2005, Pages 573-580

Moti Moskovitz DMD, Ph, a, Dan Brener DMD, MSc, b, Sarit Faibis DMD, a and Benjamin Peretz DMD, c
a Instructor, Israel
b Postgraduate student, Israel
c Clinical Associate Professor, Israel
Received 5 June 2003; Revised 10 December 2003; accepted 15 March 2004 Jerusalem, Israel
HEBREW UNIVERSITYÒHADASSAH SCHOOL OF DENTAL MEDICINE DEPARTMENT OF PEDIATRIC DENTISTRY Available online 12 April 2005.

Abstract
Williams syndrome is a rare disorder that was first described in 1961. It is thought to be caused by a microdeletion in the long arm of chromosome 7 at 7q11.23 and is a multisystem, congenital, and panethnic disorder characterized by a number of developmental and physical abnormalities, including congenital cardiovascular abnormalities, mental retardation and neurological features, growth deficiency, genitourinary manifestation, gastrointestinal and musculoskeletal problems, behavioral characteristics, craniofacial features, ophthalmologic features, and dental problems. We describe cases of children with Williams syndrome treated in the department of Pediatric Dentistry of the Hadassah School of Dental Medicine, Jerusalem, Israel. The different treatments rendered to these children are discussed followed by general remarks drawn from those treatments and from a literature review. We conclude that sedation can be helpful in the younger age group to reduce anxiety and uncooperative behavior during minimal dental treatments. Treatment under general anesthesia seems more appropriate for older children and adolescents. Special attention should be given to initial evaluation of these patients, especially because with age aortic stenosis tends to intensify, which together with the progressive renal impairment can escalate blood pressure elevation.

Corresponding author. Reprint requests: Moti Moskovitz, DMD, PhD, Department of Pediatric Dentistry, The Hebrew University- Hadassah School of Dental Medicine, P.O.B. 12272, Jerusalem 91120 ISRAEL

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