solitary median maxillary central incisor syndrome (Monarch Disease Ontology term MONDO_0007819) is a hereditary autosomal dominant condition characterized primarily by single (unpaired) deciduous and permanent maxillary central incisors and short stature. Growth hormone deficiencies may also be present. Mutations in the SHH gene have been identified. Also known as: SMMCI, single median maxillary central incisor, single upper central incisor.