Dental Care and Oral Manifestations in a Child With Costello Syndrome: A 5‐Year Follow‐Up Case Report

INTRODUCTION: Costello Syndrome (CS) is a rare, autosomal dominant genetic disorder caused by mutations in the HRAS gene. Its systemic and orofacial manifestations are highly relevant to pediatric dentistry. This study reports the case of a Brazilian female child who began receiving dental care at 25 months of age at the Children's Specialties Clinic, located at the Baby Clinic of the State University of Londrina, with dental follow-up over five years. CASE REPORT: The patient presented systemic alterations (pulmonary valve stenosis, laryngomalacia, growth and developmental delay, among others), facial features (macrocephaly, hypertelorism, broad nasal base, wide mouth), and oral conditions (anterior open bite, posterior crossbite, enamel hypoplasia, diastemas, tooth gemination, bruxism, and oral hypersensitivity). The pediatric dental approach focused on individualized preventive strategies, including dental biofilm control, oral hygiene guidance for caregivers, dietary counseling, and topical application of silver diamine fluoride. At six years old, orthopedic treatment with the SN3 appliance was proposed, but adherence was low due to sensory issues. RESULTS: Over a 5-year follow-up period, there was progressive improvement in clinical behavior, oral hygiene, and adoption of healthier habits. CONCLUSION: The manifestations of CS are diverse and affect multiple tissues, with significant systemic, facial, and oral alterations. Early multidisciplinary follow-up is essential for timely diagnosis and therapeutic planning, with an emphasis on oral health promotion, functional rehabilitation of the stomatognathic system, and aesthetic interventions aimed at improving the child's quality of life and that of their family.

Authors

Institutions

Publication Details

Journal
Special Care in Dentistry
Published
2026-05-01
DOI
https://doi.org/10.1111/scd.70174
Primary Topic
Protein Tyrosine Phosphatases
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Dental Care and Oral Manifestations in a Child With Costello Syndrome: A 5‐Year Follow‐Up Case Report

Farlí Aparecida Carrilho Boer, Mariana Emi Nagata, Tatiane Garcia, Rodrigo Hayashi Sakuma et al.
Special Care in Dentistry
Protein Tyrosine Phosphatases
article

Dental Care and Oral Manifestations in a Child With Costello Syndrome: A 5‐Year Follow‐Up Case Report

Farlí Aparecida Carrilho Boer, Mariana Emi Nagata, Tatiane Garcia, Rodrigo Hayashi Sakuma, Fábio Anevan Ubiski Fagundes, Cássia Cilene Dezan, Lucas Fernando Oliveira Tomáz Ferraresso
article en

Abstract

INTRODUCTION: Costello Syndrome (CS) is a rare, autosomal dominant genetic disorder caused by mutations in the HRAS gene. Its systemic and orofacial manifestations are highly relevant to pediatric dentistry. This study reports the case of a Brazilian female child who began receiving dental care at 25 months of age at the Children's Specialties Clinic, located at the Baby Clinic of the State University of Londrina, with dental follow-up over five years. CASE REPORT: The patient presented systemic alterations (pulmonary valve stenosis, laryngomalacia, growth and developmental delay, among others), facial features (macrocephaly, hypertelorism, broad nasal base, wide mouth), and oral conditions (anterior open bite, posterior crossbite, enamel hypoplasia, diastemas, tooth gemination, bruxism, and oral hypersensitivity). The pediatric dental approach focused on individualized preventive strategies, including dental biofilm control, oral hygiene guidance for caregivers, dietary counseling, and topical application of silver diamine fluoride. At six years old, orthopedic treatment with the SN3 appliance was proposed, but adherence was low due to sensory issues. RESULTS: Over a 5-year follow-up period, there was progressive improvement in clinical behavior, oral hygiene, and adoption of healthier habits. CONCLUSION: The manifestations of CS are diverse and affect multiple tissues, with significant systemic, facial, and oral alterations. Early multidisciplinary follow-up is essential for timely diagnosis and therapeutic planning, with an emphasis on oral health promotion, functional rehabilitation of the stomatognathic system, and aesthetic interventions aimed at improving the child's quality of life and that of their family.

Special Care in DentistryVol. 46(3)
Universidade Federal do Rio de Janeiro (BR), Universidade Estadual de Londrina (BR), Universidade Estadual Paulista (Unesp) (BR)
Good health and well-being
Openalex Percentile: Top 8%
Protein Tyrosine Phosphatases
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.