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113:Orofacial and ophthalmological Manifesteations of Long-Standing Parry-Romberg Syndrome[AAOM2020}
EP36002
Poster Title: 113:Orofacial and ophthalmological Manifesteations of Long-Standing Parry-Romberg Syndrome[AAOM2020}
Submitted on 29 Mar 2021
Author(s): Rebecca Oliva1 & Mahnaz Fatahzadeh2
Affiliations: Rutgers School of Dental Medicine
This poster was presented at 2021 American Academy of Oral Medicine Virtual Conference
Poster Views: 255
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Poster Information
Abstract: Introduction

Parry-Romberg Syndrome (PRS) is a rare acquired disorder characterized by progressive hemiatrophy of soft and sometimes hard facial tissues typically starting before 20. Although aetiology is unknown, some experts consider it a variant of localized scleroderma and limited evidence for regression of disease activity with immunomodulator therapy support a possible autoimmune or inflammatory nature. Diagnosis is primarily clinical and PRS has highly variable course and severity including serious neurological, ophthalmological and orofacial sequela. We report a young male with long-standing PRS and multitude of complications.

Case

A 24-year-old male presented for evaluation of facial asymmetry starting with appearance of a shiny skin patch under his right eye during adolescence. He was subsequently diagnosed with morphea and treated with topical steroids to no avail. Considering his condition merely a skin disease, he had not sought additional care ever since. He denied history of trauma, burn, extracranial anomalies and PMH.

Extraoral exam revealed sunken-in right face with a focal shiny plaque below the orbit and only a thin skin covering the depressed temporal, zygomatic and mandibular region. Other notable findings were right-sided ear protrusion, enophthalmos, lower lid ectropion, sparse facial hair, limited mouth opening and upward deviation of lip corner with ipsilateral exposure of teeth. Intraorally, right tongue was atrophic, depapillated, had poor muscle tone and deviated ipsilaterally. Proprioception of dental instruments was also diminished on the affected side of his mouth. His maxillary midline was shifted causing Class III occlusion with posterior crossbite and open bite on the right side. Panoramic radiograph revealed right-sided dental crowding, delayed eruption and shorter roots as well as ipsilateral reduction in ramus height, body size, gonial prominence and mental tubercle. Differential also included facial lipoatrophy and hemifacial microsomia but history, clinical, radiographic and laboratory (ANA:1:80, nucleolar pattern associated with systemic sclerosis ) findings supported diagnosis of Parry-Romberg Syndrome with complications and he was referred to specialists for management.

Conclusion

Clinicians should include PRS in the differential diagnosis of patients with facial asymmetry. Timely diagnosis, patient education and early multidisciplinary collaboration are necessary to halt progression of active disease, ameliorate symptoms and prevent serious sequela
Summary: We report a young male with long-standing PRS and multitude of complications.

Ask the author questions about this poster:fatahza@sdm.rutgers.edu
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