Plausible predicament in emergency endodontic rehabilitation of a child with sturge-weber syndrome: A case report

Case Report

Contemp Pediatr Dent 2022:3(2):86-91


Plausible predicament in emergency endodontic rehabilitation of a child with Sturge-Weber syndrome: A case report

OrcidAmit Khatri1, Orcid Namita Kalra2, Orcid Rishi Tyagi3 , Orcid Khadeeja Kulood M4 , Orcid Mayank Sharma5✉ , Orcid Drishti Kaushal6

Affiliations

1.Professor, Department of Paedodontics and Preventive Dentistry, University College of Medical Sciences, India

2. Professor, Department of Paedodontics and Preventive Dentistry, University College of Medical Sciences, India

3. Professor, Department of Paedodontics and Preventive Dentistry, University College of Medical Sciences, India

4. Post graduate student, Department of Paedodontics and Preventive Dentistry, University College of Medical Sciences, India

5. Senior Resident, Department of Paedodontics and Preventive Dentistry, University College of Medical Sciences, India ( Correspondence: mayank0993@gmail.com )

6.Senior Resident, Department of Paedodontics and Preventive Dentistry, University College of Medical Sciences, India


Highlights

Sturge–Weber syndrome (SWS) is a developmental disorder portrayed by a triad of unilateral capillary malformation, leptomeningeal hemangioma, and secondary glaucoma.

This case report observes SWS in a child with acute periapical abscess requiring emergency intervention and observation of typical port wine lesion intraorally.

The patient had a full mouth rehabilitation preceded by emergency endodontic intervention.


Abstract

Sturge–Weber Syndrome (SWS) is a neuro-oculo-cutaneous vascular disorder that includes leptomeningeal hemangioma and port wine stains, usually ipsilaterally, with ocular manifestations and extended lesions over the oral cavity. It is an embryonic developmental disorder affecting both mesodermal and ectodermal germ layers, associated with somatic mutation of gene GNAQ. It has a sporadic occurrence of 1:50,000 and no gender predilection. This case report added to the knowledge and understanding of systematic rehabilitation of a child patient with SWS and its associated risk factors in an emergency scenario. A 3.5-year-old girl child with a known diagnosis of SWS presented abscess over the right lower mandibular deciduous teeth and distinct oral features of SWS, requiring emergency endodontic intervention, followed by full mouth rehabilitation. Thus, an in-depth knowledge of the clinical state that helps in the expert assessment of dental interventions, including emotional co-regulation in those children, is essential.

Keywords: Angiomatosis; Endodontics; Port Wine Stain; Syndrome


Copyright © 2022 Contemporary Pediatric Dentistry

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Full Text


DOI

10.51463/cpd.2022.97


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27 views

Article Info

Received:

29 January 2022

Accepted:

18 July 2022

Online First:

05 August 2022