Rare cases of (SMMCI) solitary median maxillary central incisor syndrome: Identification and management

A. Kamboj, Paras Angrish, S. Chopra, Mrinalini Rathore, Aarti Sharma
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Abstract

Condition where there is agenesis of central maxillary incisor is unique and very rare with association of peculiar stomatognathic features is called (SMMCI) Solitary median maxillary central incisor syndrome which may or may not have systemic involvement, and the condition is found to affect 1:50,000 live births. SMMCI is a very rare abnormality in developing stage of fetus involving the tooth germs of central incisor. The most common systemic association is with holoprosencephaly, and diagnosis in early stage is of utmost importance for managing dental aesthetic issue patient generally suffers from the syndrome. The objective of current article is to discuss two cases of SMMCI syndrome; one with systemic involvement having esophageal Artesia, a rare entity with SMMCI syndrome and the other one without systemic involvement. In both cases, patient’s parents were not aware of the dental anomaly the child was suffering, and parents were highlighted on treatment options in managing such dental cases. As there are a significant number of SMMCI syndrome cases with systemic involvement and other developmental problems, it becomes prudent to have a quick and prompt diagnosis in initial stages of life and simultaneously the management approach becomes multidisciplinary.
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罕见的(SMMCI)单发上颌正中切牙综合征病例:识别与治疗
上颌中切牙缺失是一种独特而罕见的情况,伴有奇特的口颌特征,这种情况被称为(SMMCI)上颌中切牙缺失综合征(Solitary median maxillary central incisor syndrome),可能会也可能不会累及全身,这种情况对活产婴儿的影响为1:50,000。SMMCI是胎儿发育阶段的一种非常罕见的异常,涉及中切牙的牙胚。最常见的全身性并发症是全口畸形,因此早期诊断对于处理该综合征患者的牙科美学问题至关重要。本文旨在讨论两例 SMMCI 综合征病例:一例全身受累,伴有食道阿特西亚(一种罕见的 SMMCI 综合征);另一例无全身受累。在这两例病例中,患者的父母都不知道孩子患有牙科异常,医生向父母强调了处理此类牙科病例的治疗方案。由于有大量 SMMCI 综合征病例伴有全身性受累和其他发育问题,因此在生命的最初阶段进行快速、及时的诊断就显得尤为重要,同时,治疗方法也应是多学科的。
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