Unilateral exophthalmos associated with ipsilateral mucosal turbinate hypertrophy: benign exophthalmos syndrome (BES). A description of a new clinical condition.
PURPOSE: To report the clinical and radiologic characteristics of a group of patients who experienced unilateral exophthalmos associated with ipsilateral mucosal turbinate hypertrophy. The clinical features of these patients are presented and a hypothesis proposed to explain this condition for which the authors introduce the term benign exophthalmos syndrome (BES). METHODS: Retrospective, noncomparative case series. PARTICIPANTS: Four patients experienced slow progressive unilateral exophthalmos associated with ipsilateral mucosal turbinate hypertrophy, with no evidence of orbital mass or extraocular muscles involvement. INTERVENTION: Main Outcome Measures. Symptomatic outcome and measurement of the degree of relative exophthalmos. RESULTS: The onset of exophthalmos was associated with clinical and radiologic features that resemble BES. In all patients, radiologic examination demonstrated an ipsilateral mucosal turbinate hypertrophy and not the presence of orbital disease or expanding lesions of paranasal sinus. After daily intranasal spray of steroid, in three of the four cases the globe returned to within 1 mm of exophthalmometry of the contralateral eye. CONCLUSIONS: The relationship between the feature of paranasal sinus disease and the development of ipsilateral exophthalmos has been described in the literature. The four cases described herein appear peculiar for the slow progressive onset of the exophthalmos, without inflammatory and mass effect signs. This condition associated in all cases with ipsilateral hypertrophy of the nasal mucosa provides a guide to a hypothetical mechanism for BES. According to these hypothesis, the therapy should be devoted to the nasal disease more than the orbital.