Rare case of skeletal third class in a subject suffering from Solitary Median Maxillary Central Incisor syndrome (SMMCI) associated to panhypopituitarism.
Children disease
Congenital syndromes
Dental anomalies
Dentistry
Hormonal deficiency
Interceptive orthodontics
Panhypopituitarism
Pituitary gland
SMMCI
Journal
Head & face medicine
ISSN: 1746-160X
Titre abrégé: Head Face Med
Pays: England
ID NLM: 101245792
Informations de publication
Date de publication:
23 Nov 2021
23 Nov 2021
Historique:
received:
22
01
2021
accepted:
08
11
2021
entrez:
24
11
2021
pubmed:
25
11
2021
medline:
26
11
2021
Statut:
epublish
Résumé
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. 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. 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.
Sections du résumé
BACKGROUND
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
METHODS
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
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.
Identifiants
pubmed: 34814931
doi: 10.1186/s13005-021-00300-3
pii: 10.1186/s13005-021-00300-3
pmc: PMC8609825
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
49Informations de copyright
© 2021. The Author(s).
Références
Clin Endocrinol (Oxf). 2013 Apr;78(4):551-7
pubmed: 22967285
Int J Paediatr Dent. 2006 Mar;16(2):128-34
pubmed: 16430527
Am J Med Genet A. 2004 May 15;127A(1):93-5
pubmed: 15103725
Clin Endocrinol (Oxf). 2001 Dec;55(6):735-40
pubmed: 11895214
Eur J Orthod. 2001 Feb;23(1):63-73
pubmed: 11296511
J Med Genet. 1990 Mar;27(3):192-3
pubmed: 2325094
Am J Med Genet. 2001 Jul 22;102(1):1-10
pubmed: 11471164
Br Dent J. 1968 Feb 20;124(4):186-8
pubmed: 5236205
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998 May;85(5):548-51
pubmed: 9619672
Cranio. 2019 Mar;37(2):77-84
pubmed: 29072541
Biomed Res Int. 2018 Jul 9;2018:2694517
pubmed: 30112371
Orphanet J Rare Dis. 2006 Apr 09;1:12
pubmed: 16722608
Brain Dev. 2010 Mar;32(3):217-22
pubmed: 19398181
Minerva Pediatr. 2010 Feb;62(1):113-8
pubmed: 20212404
Lancet. 2007 Apr 28;369(9571):1461-1470
pubmed: 17467517
Acta Odontol Scand. 2001 Dec;59(6):361-6
pubmed: 11831485