Segmental odontomaxillary dysplasia: report of a series of 5 cases with long-term follow-up

Segmental odontomaxillary dysplasia: report of a case emphasizing histopathological, immunohistochemical and scanning electron microscopic features

“…Histologically, bone features in SOD include a large amount of dense or loosely arranged collagen fibers surrounded by immature cancellous bone with prominent reversal lines in a hypocellular stroma (1,(3)(4)(5). Despite the aforementioned characteristics, affected bone in the present case showed spindle cell proliferation and a large number of odontogenic epithelial cords and nests.…”

“…It is characterized by unilateral enlargement of the posterior segment of the maxilla that may cause facial enlargement and, as a result, gingival fibrous hyperplasia, bony expansion or both (3,4). Extension of maxillary swelling is variable and can include a solely alveolar process from the canine to the tuberosity or extend up to the floor of the maxillary sinus and orbit (4,5). Skin facial findings such as hypertrichosis, erythema and hypopigmentation may also be present (5); however, in this case, the patient showed only intraoral swelling caused by osseous growth without any facial alteration.…”

“…Teeth abnormalities in SOD include agenesis of one or both premolars in the affected area, delayed eruption of the primary molars or the adjacent permanent molars, malformation of both deciduous and permanent teeth including hypoplasia, enlarged crowns and roots, splayed roots, pulp stones, irregular root resorption, root abnormalities of the primary molars and abnormal Table 1 Mean Calcium (Ca) and Phosphorus (P) content values (weight percent) and Ca/P ratio identified through X-ray microanalysis in different dentinal areas from affected tooth in segmental odontomaxillary dysplasia (SOD) and from healthy first primary molar spacing between erupted teeth (2,4,5). One study has been conducted analyzing microscopic features of three primary teeth affected by SOD; its major findings included the presence of tubular dentin defects in the circumpulpal area, enlarged pulp chamber and root canals, moderate cellular and vascular pulp fibrous tissue, a number of pulp stones, irregular outline dentin, absence of a peripheral odontoblast layer, and apical external resorption (6).…”

“…Despite some typical clinical and radiological characteristics, SOD may be misdiagnosed as fibrous dysplasia, segmental hemifacial hypertrophy, regional odontodysplasia, focal osseous dysplasia and gingival fibromatosis (2,(4)(5)(6), thus reinforcing the importance of describing the clinicopathologic features of such a rare disorder.…”

The palatomaxillary suture revisited: A histological and immunohistochemical study using human fetuses

“…These studies provided a basis for understanding partial anomalies of the palate, such as segmental odontomaxillary dysplasia (Becktor et al, 2002;Whitt et al, 2011). Less is known, however, about the double layered bony palate, such as whether the bony deposition and reconstruction process starts in the fetal palatomaxillary suture.…”