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Parry-Romberg syndrome is a rare acquired disorder characterized by unilateral idiopathic progressive atrophy of the skin and soft tissues of the face, resulting in a sunken appearance. The muscles, cartilage, and underlying bony structures may also be affected. The etiology remains unclear and is based on several hypotheses. The incoherence of atrophy and the development of associated symptoms make the diagnosis, prognosis, and management of patients difficult. Here, we report the case of a 10-year-old boy who presented to the Department of Pediatric Dentistry at the Mohamed V Military Training Hospital in Rabat with progressive left hemifacial atrophy and was diagnosed by a pediatric rheumatologist as having Parry-Romberg syndrome. On extraoral examination, the patient presented a slight facial asymmetry and a small, dark, linear scar in the left zygomatic region. Intraoral examination revealed a left lateral open bite and atrophy of the left side of the tongue. Panoramic radiography showed incomplete eruption of the left mandibular first and second premolars (34/35), with significant root atrophy giving a narrowed appearance to the corresponding pulp chambers confirmed on retro alveolar radiographs. The mandible had a slightly reduced ramus height on the affected side confirming the patient's facial asymmetry. A better understanding of this syndrome will help to improve oral care in young patients.
Parry-Romberg syndrome is a rare acquired disorder characterized by unilateral idiopathic progressive atrophy of the skin and soft tissues of the face, resulting in a sunken appearance. The muscles, cartilage, and underlying bony structures may also be affected. The etiology remains unclear and is based on several hypotheses. The incoherence of atrophy and the development of associated symptoms make the diagnosis, prognosis, and management of patients difficult. Here, we report the case of a 10-year-old boy who presented to the Department of Pediatric Dentistry at the Mohamed V Military Training Hospital in Rabat with progressive left hemifacial atrophy and was diagnosed by a pediatric rheumatologist as having Parry-Romberg syndrome. On extraoral examination, the patient presented a slight facial asymmetry and a small, dark, linear scar in the left zygomatic region. Intraoral examination revealed a left lateral open bite and atrophy of the left side of the tongue. Panoramic radiography showed incomplete eruption of the left mandibular first and second premolars (34/35), with significant root atrophy giving a narrowed appearance to the corresponding pulp chambers confirmed on retro alveolar radiographs. The mandible had a slightly reduced ramus height on the affected side confirming the patient's facial asymmetry. A better understanding of this syndrome will help to improve oral care in young patients.
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