Editorial Type:
Article Category: Other
 | 
Online Publication Date: Jan 01, 2014

Anesthetic and Dental Management of a Child With IMAGe Syndrome

DMD,
DMD,
DMD, and
MD
Page Range: 165 – 168
DOI: 10.2344/0003-3006-61.4.165
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Abstract

IMAGe syndrome (OMIM 300290) is a rare multisystem disorder that has a broad phenotypic presentation. Though variable, this disorder mainly consists of Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital abnormalities. Patients with IMAGe syndrome present as an uncommon yet important challenge for dentists and anesthesiologists due to their wide range of dysmorphic facial features, adrenal insufficiency, electrolyte imbalances, and need for steroid replacement. The purpose of this case report is to describe the successful anesthetic management of a pediatric patient diagnosed with IMAGe syndrome who presented for full mouth dental rehabilitation.

Keywords: IMAGe syndrome; Adrenal hypoplasia; General anesthesia; Dental rehabilitation
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Figure 1.
Figure 1.

Dysmorphic facial features of patient with IMAGe syndrome.

Figure 2.
Figure 2.

High, narrow, and constricted palate with multiple carious nonrestorable teeth and evidence of dental abscesses.

Figure 3.
Figure 3.

Full radiographic series.

Contributor Notes

Address correspondence to Dr Rochelle G. Lindemeyer, Department of Pediatric Dentistry, University of Pennsylvania School of Dental Medicine, 240 S. 40th Street, Philadelphia, PA 19104; lindemey@dental.upenn.edu.
Received: Jan 09, 2014
Accepted: Aug 23, 2014