|Year : 2014 | Volume
| Issue : 2 | Page : 129-130
Fissured and geographic tongue in Williams-Beuren syndrome
Neeta Sharma1, Reet Kamal2
1 Departments of Oral Medicine and Radiology, H.P. Government Dental College and Hospital, Shimla, Himachal Pradesh, India
2 Department of Oral Pathology, H.P. Government Dental College and Hospital, Shimla, Himachal Pradesh, India
|Date of Web Publication||16-Oct-2014|
Department of Oral Medicine and Radiology, H.P. Government Dental College and Hospital, Shimla - 171 009, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Williams-Beuren Syndrome (WBS) is a rare, most often sporadic, genetic disease caused by a chromosomal microdeletion at locus 7q11.23 involving 28 genes. It is characterized by congenital heart defects, neonatal hypercalcemia, skeletal and renal abnormalities, cognitive disorder, social personality disorder, and dysmorphic facies. A number of clinical findings has been reported, but none of the studies evaluated this syndrome considering oral cavity. We here report a fissured and geographic tongue in association with WBS.
Keywords: Fissured tongue, geographic tongue, Williams syndrome
|How to cite this article:|
Sharma N, Kamal R. Fissured and geographic tongue in Williams-Beuren syndrome
. J Orofac Sci 2014;6:129-30
| Introduction|| |
Williams-Beuren Syndrome (WBS) is a rare familial multisystem disorder that occurs in 1/20,000 live births.  It is described officially in 1961 by Williams, Barrot-Boyes and Lowe. It affects male and female equally and can occur in all ethnic groups.  Patients with WBS show a hemizygous submicroscopic deletion of 7q11.23 detected by fluorescent in situ hybridization.  The detected portion of the chromosome corresponds to the Elastin gene. The elastin gene encode for the essential component of the arterial extracellular matrix.  The most frequent specific features are elf-like face, alteration of cognitive functions, and cardiovascular diseases including isolated supra valvular aortic stenosis.  Other features are moderate mental retardation, relatively well-preserved language skills, visuospatial defects and hypersociability. The rare features such as neonatal hypercalcemia, the nutritional problem in infancy, opthalmological anomalies, hypothyroidism, growth retardation, joint disturbances, dental anomalies, and hypertension arising in adolescence or adulthood. 
| Case report|| |
A 23-year-old female patient of WBS was referred to Department of Oral Diagnosis from Cardiology Department for Oro-Dental evaluation. The patient had a history of recurrent febrile illness since childhood. The patient had moderate aortic stenosis, hypertension of 170/70 mm of Hg, pulse rate 80/min, serum alk.phosphates-69 KAV, Cl− -109 mEq/L, Ca ++ -10.5 mg%, mild pallor, anemia with Hb-9.6 g%, typical elf-like face, wide mouth, thick lips, long philtrum, short stature, grey hairs, telecanthus, poor in studies, hypersociable nature [Figure 1]. Oral features present were Angle's class I bimax malocclusion, deep palate, spacing between teeth, and poor oral hygiene [Figure 2]. The tongue showed fissures on central and posterior dorsum of the tongue and depapillated irregular area with a white border on left lateral aspect of the dorsum of the tongue without any complaint of burning sensation, diagnosed as fissured and geographic tongue [Figure 3]. The patient was advised oral prophylaxis and orthodontic consultation.
|Figure 1: Facial profile of the patient with exopthalmos, broad nasal bridge, protruded lips, graying of hairs|
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| Discussion|| |
Williams-Beuren syndrome is quite a rare syndrome and much literature about oro-dental features is not available. The most frequent specific features are elf like face, alteration of cognitive functions and cardiovascular disease including isolated supravalvular aortic stenosis. A number of clinical findings has been reported, but none of the studies evaluating this syndrome considering oral cavity. 
Kashyap et al. reported typical anomalies in primary teeth are small, irregular, widely spaced peg-shaped primary teeth.  A dentist can help in diagnosing this syndrome early. Tarjan et al. reported wide mouth, thick lips, long philtrum, and 90% fan-shaped positioning of anterior teeth.  We also found similar features in our case. Some cases reported cleft palate in WBS. Hertzberq et al. reported a higher than normal prevalence of class II and class III occlusions, open and deep bites and anterior crossbites, microdontia, tongue thrusting, and excessive interdental spacing.  Sedation during dental treatment in younger age group and treatment under GA seems more appropriate for older children and adolescents.  Fissured and geographic tongue was associated with this syndrome in our case, in the literature, none of the case reported fissured and geographic tongue with association with this syndrome. Early diagnosis of the syndrome is important since many of its features require treatment, and the prognosis can be dramatically improved by timely management. 
| Conclusion|| |
Williams-Beuren syndrome is a rare syndrome. Geographic and fissured tongue has never been referred to the part of WBS due to lack of medical literature.
| References|| |
Zamani H, Babazadeh K, Fattahi S, Mokhtari-Esbuie F. Williams-beuren's syndrome: A Case Report. Case Rep Med 2012;2012:585726.
Domenico S, Orlando C, Graziana FF, Papi P, Giulia A. Cleft palate in Williams syndrome. Ann Maxillofac Surg 2013;3:84-6.
Teo SH, Chan DK, Yong MH, Ng IS, Wong KY, Knight L, et al.
Williams syndrome - The Singapore General Hospital experience. Ann Acad Med Singapore 1997;26:360-4.
Lacroix A, Pezet M, Capel A, Bonnet D, Hennequin M, Jacob MP, et al.
Williams-Beuren syndrome: A multidisciplinary approach. Arch Pediatr 2009;16:273-82.
Joseph C, Landru MM, Bdeoui F, Gogly B, Dridi SM. Periodontal conditions in Williams Beuren syndrome: A series of 8 cases. Eur Arch Paediatr Dent 2008;9:142-7.
Kashyap AS, Sharma HS, Kumar P. Dental anomalies in Williams syndrome. Postgrad Med J 2000;76:712.
Tarjan I, Balaton G, Balaton P, Varbiro S, Vajo Z. Facial and dental appearance of Williams syndrome. Postgrad Med J 2003;79:241.
Hertzberg J, Nakisbendi L, Needleman HL, Pober B. Williams syndrome - Oral presentation of 45 cases. Pediatr Dent 1994;16:262-7.
Moskovitz M, Brener D, Faibis S, Peretz B. Medical considerations in dental treatment of children with Williams syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:573-80.
[Figure 1], [Figure 2], [Figure 3]