Abstract
BackgroundThe 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 presentationFrom 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.ConclusionsThe 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.
Highlights
Introduction of the clinical caseThe patient came to our attention at the San Raffaele Dental Department (Milan, Italy) at the age of 11 years
The present clinical case shows how, despite the literature, Solitary median maxillary central incisor syndrome (SMMCI) can be associated with a III skeletal class, with maxillary hypoplasia and mandibular protrusion
The most frequent deficit is about the growth hormone, which occurs with short stature and a growth speed reduction, associated with alteration of the body constitution and craniofacial anomalies such as: splanchnocranium hypoplasia, frontal drafts, saddle nose, lip/palatal cleft
Summary
The present clinical case shows how, despite the literature, SMMCI can be associated with a III skeletal class, with maxillary hypoplasia and mandibular protrusion. There is the lack of SHH gene mutation and the absence of holoprosencephaly, the patient is affected by SMMCI with concomitant pan-hypopituitarism. We can observe how the presence of SMMCI should not be considered as a simple dental anomaly, since it can be associated with other clinical features and craniofacial malformations. Interdisciplinarity is important for the early evaluation of the malocclusions associated with these syndromes
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
