Dental management and oral manifestations in child with Robinow syndrome: case report

Robinow syndrome (RS) is a rare genetic disease that may involve cardiovascular, skeletal and urogenital systems and manifest through short limb dwarfism, defects in vertebral segmentation, hypoplastic genitalia and cranial and facial abnormalities. In this article, we hereby present a patient of a...

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Main Authors: Lucas Fernando Oliveira Tomáz Ferraresso, Beatriz Cruz Lopes, Mariella Padovese, Tatiane Garcia, Farli Aparecida Carrilho Boer, Mariana Emi Nagata, Cássia Cilene Dezan
Format: Article
Language:English
Published: Asociación Latinoamericana de Odontopediatría 2025-08-01
Series:Revista de Odontopediatria Latinoamericana
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Online Access:https://www.revistaodontopediatria.org/index.php/alop/article/view/762
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author Lucas Fernando Oliveira Tomáz Ferraresso
Beatriz Cruz Lopes
Mariella Padovese
Tatiane Garcia
Farli Aparecida Carrilho Boer
Mariana Emi Nagata
Cássia Cilene Dezan
author_facet Lucas Fernando Oliveira Tomáz Ferraresso
Beatriz Cruz Lopes
Mariella Padovese
Tatiane Garcia
Farli Aparecida Carrilho Boer
Mariana Emi Nagata
Cássia Cilene Dezan
author_sort Lucas Fernando Oliveira Tomáz Ferraresso
collection DOAJ
description Robinow syndrome (RS) is a rare genetic disease that may involve cardiovascular, skeletal and urogenital systems and manifest through short limb dwarfism, defects in vertebral segmentation, hypoplastic genitalia and cranial and facial abnormalities. In this article, we hereby present a patient of a 10-year-old Brazilian boy with RS who presented with a remarkable number of classical general and dental features. Physical examination revealed disproportionate short stature with shortening limbs, syndromic face with normal hair appearance, low-set ears, macrocephaly, prominent forehead, hypertelorism and ocular prominence, nose anomalies, triangular mouth with long/short philtrum, incompetent lip seal, midface hypoplasia, retrognathia, deficient malar prominence, among others. Clinical examination revealed gingival hyperplasia, malocclusion, crowding dental, diastemas, microdontia, enamel hypoplasia, prolonged retention, dental caries, delay of dental eruption v-shaped palate, bifid tongue and ankyloglossia history. Radiographically, dental agenesis, hypertaurodontism and thick roof pulp chambers and delayed tooth eruption were observed. Preventive, restorative and rehabilitative dental procedures are being carried out. Robinow syndrome presents clinical and radiographic findings that may be present from birth and require pediatric dentistry follow-up. Furthermore, the pediatric dentist plays an essential role in developing effective planning and treatment in terms of health promotion, aesthetic rehabilitation and functional interventions in the stomatognathic system.
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spelling doaj-art-7b733b86b9d7404ba8cb4cbf8ac0d7052025-08-23T03:25:29ZengAsociación Latinoamericana de OdontopediatríaRevista de Odontopediatria Latinoamericana2174-07982025-08-011510.47990/vh7bd212Dental management and oral manifestations in child with Robinow syndrome: case reportLucas Fernando Oliveira Tomáz Ferraresso0https://orcid.org/0000-0002-2225-9262Beatriz Cruz Lopes1https://orcid.org/0000-0003-4830-7696Mariella Padovese2https://orcid.org/0000-0001-6549-293XTatiane Garcia3https://orcid.org/0000-0002-3387-1035Farli Aparecida Carrilho Boer4https://orcid.org/0000-0003-1911-8247Mariana Emi Nagata5https://orcid.org/0000-0003-3754-4303Cássia Cilene Dezan6https://orcid.org/0000-0002-8660-0677Department of Preventive and Restorative Dentistry, São Paulo State University, School of Dentistry, Araçatuba, Brasil.Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, Brazil.Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, Brazil.Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil.Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, Brazil.Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, PR, Brazil.Department of Oral Medicine and Dentistry for Children, State University of Londrina, Londrina, Brazil. Robinow syndrome (RS) is a rare genetic disease that may involve cardiovascular, skeletal and urogenital systems and manifest through short limb dwarfism, defects in vertebral segmentation, hypoplastic genitalia and cranial and facial abnormalities. In this article, we hereby present a patient of a 10-year-old Brazilian boy with RS who presented with a remarkable number of classical general and dental features. Physical examination revealed disproportionate short stature with shortening limbs, syndromic face with normal hair appearance, low-set ears, macrocephaly, prominent forehead, hypertelorism and ocular prominence, nose anomalies, triangular mouth with long/short philtrum, incompetent lip seal, midface hypoplasia, retrognathia, deficient malar prominence, among others. Clinical examination revealed gingival hyperplasia, malocclusion, crowding dental, diastemas, microdontia, enamel hypoplasia, prolonged retention, dental caries, delay of dental eruption v-shaped palate, bifid tongue and ankyloglossia history. Radiographically, dental agenesis, hypertaurodontism and thick roof pulp chambers and delayed tooth eruption were observed. Preventive, restorative and rehabilitative dental procedures are being carried out. Robinow syndrome presents clinical and radiographic findings that may be present from birth and require pediatric dentistry follow-up. Furthermore, the pediatric dentist plays an essential role in developing effective planning and treatment in terms of health promotion, aesthetic rehabilitation and functional interventions in the stomatognathic system. https://www.revistaodontopediatria.org/index.php/alop/article/view/762Craniofacial anomaliesOral manifestationsPediatric dentistry.
spellingShingle Lucas Fernando Oliveira Tomáz Ferraresso
Beatriz Cruz Lopes
Mariella Padovese
Tatiane Garcia
Farli Aparecida Carrilho Boer
Mariana Emi Nagata
Cássia Cilene Dezan
Dental management and oral manifestations in child with Robinow syndrome: case report
Revista de Odontopediatria Latinoamericana
Craniofacial anomalies
Oral manifestations
Pediatric dentistry.
title Dental management and oral manifestations in child with Robinow syndrome: case report
title_full Dental management and oral manifestations in child with Robinow syndrome: case report
title_fullStr Dental management and oral manifestations in child with Robinow syndrome: case report
title_full_unstemmed Dental management and oral manifestations in child with Robinow syndrome: case report
title_short Dental management and oral manifestations in child with Robinow syndrome: case report
title_sort dental management and oral manifestations in child with robinow syndrome case report
topic Craniofacial anomalies
Oral manifestations
Pediatric dentistry.
url https://www.revistaodontopediatria.org/index.php/alop/article/view/762
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