Dental School and Postgraduate School of Orthodontics, Vita-Salute San Raffaele University and IRCCS San Raffaele Hospital, via Olgettina 58, Milan, Italy.
Head Face Med. 2021 Nov 23;17(1):49. doi: 10.1186/s13005-021-00300-3.
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.
孤立性上颌正中门牙综合征（SMMCI）是一种罕见的发育畸形综合征，由多个缺陷组成，主要发生在中线身体部位。它可能与垂体功能减退症的病因发病和表型模式有关。本病例报告描述了一位患有 SMMCI 和垂体功能减退症的患者的罕见病例，表现出不寻常的颅面形态。
从头影测量分析中，确定存在骨骼 III 类（尽管文献中描述的其他病例为骨骼 II 类），源于下颌骨发育不全和下颌前突。观察到凸唇轮廓，下颌过度发散倾向，气道通畅，前牙反合和后牙反合。在临床检查中，正面观察到上颌明显倾斜，表现为不对称，以下半面部为主。存在一些畸形迹象，如：小鼻子、直线型眼睑线和眼距缩小。
本临床病例表明，尽管文献中存在，但 SMMCI 也可能与 III 类骨骼相关，表现为上颌骨发育不全和下颌前突。因此，牙医和儿科医生之间的跨学科合作对于早期发现与这些综合征相关的错颌畸形非常重要。
