Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism

Abstract Background The median solitary maxillary central incisor syndrome (SMMCI) is a rare malformative syndrome consisting of multiple defects, mainly found on the body midline. It can be correlated to the etiopathological and phenotypic pattern of panhypopituitarism. This case-report describes t...

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Autores principales:	Alessandro Nota, Shideh Ehsani, Laura Pittari, Giorgio Gastaldi, Simona Tecco
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Panhypopituitarism Dentistry Hormonal deficiency Congenital syndromes Children disease Dental anomalies Specialties of internal medicine RC581-951
Acceso en línea:	https://doaj.org/article/1ec7450c5ed343e89594111915e76a76
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spelling	oai:doaj.org-article:1ec7450c5ed343e89594111915e76a762021-11-28T12:09:59ZRare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism10.1186/s13005-021-00300-31746-160Xhttps://doaj.org/article/1ec7450c5ed343e89594111915e76a762021-11-01T00:00:00Zhttps://doi.org/10.1186/s13005-021-00300-3https://doaj.org/toc/1746-160XAbstract Background The median solitary maxillary central incisor syndrome (SMMCI) is a rare malformative syndrome consisting of multiple defects, mainly found on the body midline. It can be correlated to the etiopathological and phenotypic pattern of panhypopituitarism. This case-report describes the rare case of a patient suffering from SMMCI and panhypopituitarism, showing an unusual craniofacial morphology. Case presentation From the cephalometric analysis, a skeletal class III was identified (despite the other cases described in literature described as skeletal class II), derived from hypomaxillia and mandibular protrusion. A convex lip profile, with tendency to mandibular hyper-divergency, airway patency, anterior and posterior cross-bite were observed. At the clinical examination, a maxillary cant was evident on the frontal plane that appeared asymmetric, with the prevalence of the third lower part of the face. There were some dysmorphic signs such as: small nose, rectilinear eyelid line and reduced interocular distance. Conclusions The present clinical case shows how, despite the literature, SMMCI can be associated with a III skeletal class, with maxillary hypoplasia and mandibular protrusion. The interdisciplinary collaboration between dentist and pediatrician is therefore important for the early interception of the malocclusions associated with these syndromes.Alessandro NotaShideh EhsaniLaura PittariGiorgio GastaldiSimona TeccoBMCarticlePanhypopituitarismDentistryHormonal deficiencyCongenital syndromesChildren diseaseDental anomaliesSpecialties of internal medicineRC581-951ENHead & Face Medicine, Vol 17, Iss 1, Pp 1-7 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Panhypopituitarism Dentistry Hormonal deficiency Congenital syndromes Children disease Dental anomalies Specialties of internal medicine RC581-951
spellingShingle	Panhypopituitarism Dentistry Hormonal deficiency Congenital syndromes Children disease Dental anomalies Specialties of internal medicine RC581-951 Alessandro Nota Shideh Ehsani Laura Pittari Giorgio Gastaldi Simona Tecco Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism
description	Abstract Background The median solitary maxillary central incisor syndrome (SMMCI) is a rare malformative syndrome consisting of multiple defects, mainly found on the body midline. It can be correlated to the etiopathological and phenotypic pattern of panhypopituitarism. This case-report describes the rare case of a patient suffering from SMMCI and panhypopituitarism, showing an unusual craniofacial morphology. Case presentation From the cephalometric analysis, a skeletal class III was identified (despite the other cases described in literature described as skeletal class II), derived from hypomaxillia and mandibular protrusion. A convex lip profile, with tendency to mandibular hyper-divergency, airway patency, anterior and posterior cross-bite were observed. At the clinical examination, a maxillary cant was evident on the frontal plane that appeared asymmetric, with the prevalence of the third lower part of the face. There were some dysmorphic signs such as: small nose, rectilinear eyelid line and reduced interocular distance. Conclusions The present clinical case shows how, despite the literature, SMMCI can be associated with a III skeletal class, with maxillary hypoplasia and mandibular protrusion. The interdisciplinary collaboration between dentist and pediatrician is therefore important for the early interception of the malocclusions associated with these syndromes.
format	article
author	Alessandro Nota Shideh Ehsani Laura Pittari Giorgio Gastaldi Simona Tecco
author_facet	Alessandro Nota Shideh Ehsani Laura Pittari Giorgio Gastaldi Simona Tecco
author_sort	Alessandro Nota
title	Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism
title_short	Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism
title_full	Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism
title_fullStr	Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism
title_full_unstemmed	Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism
title_sort	rare case of skeletal third class in a subject suffering from solitary median maxillary central incisor syndrome (smmci) associated to panhypopituitarism
publisher	BMC
publishDate	2021
url	https://doaj.org/article/1ec7450c5ed343e89594111915e76a76
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Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism

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