Unilateral Exophthalmos

A 52-year-old woman presented with left-sided periocular “swelling” and sporadic double vision. The prominent clinical feature was painless unilateral exophthalmos, the left eye protruding 4 mm compared with the right eye. Magnetic resonance imaging showed osseous thickening of the left sphenoid wing, temporal bone, and occipital bone, while computed tomography revealed visible osteoblastic alteration along the sphenoid wing, with constriction of the orbit, and in the mastoid process and the occipital bone. Scintigraphy showed accumulation of activity in the left calvaria. The suspected diagnosis was Paget’s disease (deforming osteodystrophy: bone metabolism disorder/focal disorder of osteocytes). Tissue reduction by means of modeling osteotomy was carried out to confirm the diagnosis and reduce the exophthalmos. Histopathological examination of the excised tissue confirmed the diagnosis by identifying enhanced remodeling of bone, endosteal fibrosis, and the pathognomonic cement lines. The potential differential diagnoses were spheno-orbital meningioma, osteolytic metastases, fibrous dysplasia, and osteomyelitis. Any patient in whom exophthalmometry detects a discrepancy of > 1 mm between the position of the left and right eyes in the orbit should be investigated to ascertain the reason for the difference.

Figure

Axial T1 SE MRI / Axial cranial CT

Dr. med. Sebastian Paul, Prof. Dr. med. Frank Tost, Klinik und Poliklinik für Augenheilkunde der Universitätsmedizin Greifswald, [e-mail]

Björn-Christian Hübner, Klinik für Mund-Kiefer-Gesichtschirurgie/ Plastische Operation der Universitätsmedizin Greifswald

Conflict of interest statement: The authors declare that no conflict of interest exists.

Translated from the original German by David Roseveare.

Cite this as: Paul S, Hübner BC, Tost F: Unilateral exophthalmos. Dtsch Arztebl Int 2022; 119: 618. DOI: 10.3238/arztebl.m2022.0128