Year: 2026 | Month: September | Volume: 13 | Issue: 9 | Pages: 239-243
DOI: https://doi.org/10.52403/ijrr.20260924
Orofacial Manifestations and Comprehensive Dental Management in a Pediatric Patient with Williams-Beuren syndrome: A Case Report
Merin Mathew1, Supriya S2, Veronica Rose Puthenpurackal3, Aparna Krishnan4, Danu Dayakar P S5
1,2,3,4,5Department of Pediatric & Preventive Dentistry, Govt. Dental College, Alappuzha, Kerala
Corresponding Author: Merin Mathew
ABSTRACT
Williams–Beuren syndrome (WBS) is a rare multisystem genetic disorder caused by microdeletion at chromosome 7q11.23. This case report describes the orofacial manifestations and management of a 11year old male child with WBS. The medical history revealed global developmental delay, mild intellectual disability, impaired gait due to limb weakness, limited verbal communication, hypothyroidism, and a history of seizure managed with antiepileptic medication. The child was also receiving iron and folic acid supplementation for hematinic deficiency. Premature greying of the scalp hair in addition to characteristic craniofacial features were observed. Intraoral examination revealed poor oral hygiene, generalized marginal gingivitis, black extrinsic staining of the teeth, proclination of maxillary incisors, multiple retained primary teeth with extensive dental caries and delayed eruption of the permanent dentition. Following medical evaluation, the retained primary teeth were extracted. Oral hygiene instructions, dietary counselling, and periodic follow-up were advised to the guardians. This case highlights the importance of comprehensive medical assessment, individualized treatment planning, preventive dental care, and multidisciplinary management to achieve favorable oral health outcomes in children with Williams Beuren syndrome.
Keywords: Williams-Beuren syndrome; William syndrome; Delayed tooth eruption; Dental caries in syndrome; premature greying of hair.
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