Mandibular osseous and dental involvement in progressive hemifacial atrophy: a case report

Abstract

Clinical Presentation

This case report describes a 19-year-old male patient with progressive hemifacial atrophy (PHA), also known as Parry-Romberg syndrome, a rare condition characterized by gradual atrophy of facial hard and soft tissues of unknown etiology. The patient sought consultation to correct facial asymmetry, which had been progressive since childhood. Clinical examination revealed a smaller right lower half of the face compared with the left. There was no linear scleroderma present. The patient had no history of trauma. The malar region and external ear were normal. A craniofacial cone beam computed tomography scan was acquired for presurgical planning and submitted for oral and maxillofacial radiology review. Radiologic examination revealed hypoplasia of the right body of the mandible but normal temporomandibular joint structures. Dental abnormalities also were noted, including root resorption and a missing mandibular premolar on the affected side. There was severe atrophy of the facial soft tissues on the right, particularly near the symphyseal/parasymphyseal area.

Differential Diagnosis

Diagnosis of PHA requires ruling out other causes of unilateral facial hypoplasia including idiopathic or trauma-induced mandibular hypoplasia and oculo-auricular-vertebral spectrum. The normal temporomandibular joint structures and severe atrophy of the facial soft tissues with a history of progressive changes during childhood favor a diagnosis of PHA.

Diagnosis and Management

On the basis of the clinical presentation and radiographic findings, a diagnosis of PHA was made. The management plan included onlay mandibular angle and body implant surgery to address the facial asymmetry, with a second step planned for fat grafting to address the soft tissue deficiency.

Conclusion

This case report presents features of PHA including rarely-reported osseous and dental changes in the mandible. The diagnosis relies on a combination of radiographic exclusions and clinical examination. Management involves correction of osseous and soft tissue defects to help mitigate the aesthetic and functional impact of this condition.