Segmental Odontomaxillary Dysplasia (SOD) is a rare, non-hereditary developmental disorder affecting one side of the maxilla, impacting hard and soft tissue as well as dentition in the affected area. The condition is often associated with enlargement of soft and hard tissue on the affected area and dental anomalies, with occasional cutaneous manifestation. SOD is a non-progressive developmental disorder. Annual clinical and radiological follow-ups are recommended to monitor dentoalveolar development. Orthodontic treatments and dentoalveolar interventions are considered successful treatment options. We present the case of a patient suffering from SOD and discuss the dentist's role in early detection and the importance of the follow-up care of affected patients.

Benign fibro-osseous lesions (BFOLs) are a heterogeneous group of bone-forming pathologies characterized by replacement of normal bone with fibrocellular stroma and mineralized tissue. Their overlapping clinical, radiological, and histopathological features often complicate diagnosis. This review summarizes the clinicopathologic characteristics and recent molecular insights of BFOLs to enhance diagnostic accuracy and classification. A narrative review of English-language literature available in electronic databases upto 2025 was performed, focusing on key clinical, radiologic, histologic, and molecular findings of BFOLs. BFOLs encompass bone dysplasias including fibrous dysplasia and segmental odontomaxillary dysplasia, ossifying fibroma, and cemento-osseous dysplasia, each showing distinct biological behavior. Molecular studies have revealed GNAS mutations in fibrous dysplasia and HRPT2 alterations in ossifying fibroma, providing clues to their pathogenesis. Integrating molecular data with clinicopathologic assessment refines diagnosis, prevents misclassification, and supports more precise management of BFOLs.

Segmental odontomaxillary dysplasia (SOD) is a rare nonheritable unilateral developmental disorder characterized by dental, bone, and soft tissue abnormalities. A 13-year-old female patient presented with mild facial asymmetry. Clinical examination revealed right maxillary enlargement and gingival overgrowth. Radiologic examination revealed retention of primary teeth showing pulp chamber obliteration and irregular root resorption, agenesis of the second premolar, impaction of the first premolar, bone enlargement, and increased trabecular bone density with vertically oriented trabeculae. The combined imaging findings facilitated clear differentiation from common imaging mimickers, such as fibrous dysplasia and hemifacial hyperplasia. Bone histopathological examination revealed irregular bony trabeculae lacking an osteoblastic layer, accompanied by numerous basophilic reversal lines. This case underscores the importance of a multidisciplinary approach, combining clinical, radiologic, and histopathological evaluation in distinguishing SOD from other conditions with overlapping radiologic features.

Segmental Odontomaxillary Dysplasia (SOD) is a non-hereditary, unilateral developmental anomaly recently included in the WHO's classification of head and neck tumors. Here, we report the case of an 8-year-old boy presenting with unilateral maxillary enlargement and pain without facial asymmetry. Computed tomography revealed a hypodense area in the maxillary bone with altered bone structure and osseous expansion. An incisional biopsy showed fibrous hyperplasia in the gingiva, dysplastic dentin, and reversal lines in the bone trabeculae. Following the diagnosis of SOD, the patient was referred for treatment. To date, only 72 cases have been reported. SOD typically manifests in early childhood, with a male predominance. While the etiology remains unclear, mutations in the PIK3CA gene have been associated with its development. Further research is needed to better understand the disease and improve patient management.

ObjectiveVascular anomalies are often associated with hypertrophy and asymmetry of soft tissues and bony structures. The aim of this retrospective cross-sectional radiographic study was to evaluate dental maturation and development in patients with facial vascular anomalies and congenital infiltrating lipomatosis.DesignA sample of 342 patients with different vascular anomalies or congenital infiltrating lipomatosis involving the head and neck area was narrowed down to 31 patients with dental panoramic radiographs taken in the mixed dentition. A control group of 172 age-matched healthy subjects was used. Individual permanent teeth were given a maturation score from 1 to 12 and alveolar eruption stage according to Haavikko et al. 1970. The laterality of the anomaly was noted if applicable. Differences in dental development between affected and unaffected sides were recorded.ResultsThe study data included both syndromic and non-syndromic vascular anomalies as well as congenital infiltrating lipomatosis and segmental odontomaxillary dysplasia. Teeth on the side of the anomaly were more developed and the eruption of teeth was accelerated with canines, premolars and second molars being most affected. Interestingly all the patients with Sturge-Weber syndrome (n = 4) and infiltrating lipomatosis (n = 2) showed accelerated dental maturation of multiple permanent teeth on the side of the anomaly. Hypodontia, dental root resorption and macrodontia were also found.ConclusionsAccelerated development and eruption of permanent teeth unilaterally in patients with vascular anomalies and congenital infiltrating lipomatosis may have a significant impact on the developing occlusion and should be thus followed by an orthodontist.