97th DOG Annual Meeting 1999

P506

UNILATERAL LOSS OF VISUAL ACUITY AS FIRST SYMPTOM OF ALVEOLAR RHABOMYOSARCOMA OF THE PARANASAL SINUSES AND ORBIT IN A 14-YEAR-OLD BOY

A. Walter, W. Huk, L. M. Holbach


Background: Clinical signs of orbital rhabdomyosarcoma include most commonly lid swelling, proptosis and impaired motility. We report on a 14-year-old boy who presented with acute visual loss as the first sign of alveolar rhabdomyosarcoma.

Case report: A 14-year-old boy presented with progressive visual loss OD for ten days. Systemic steroid therapy from his practitioner for a diagnosis of retrobulbar neuritis was associated with temporary improvement of visual acuity. Visual acuity was light perception OD and 1.25 OS. A relative afferent pupillary defect and 2 mm proptosis were detectable in the right eye. Coronal CT and MRI revealed a mass in the ethmoidal sinuses, the nasal cavity, the medial orbit, the sphenoidal sinus and the anterior cranial cavity. Bony defects of the medial orbital wall were detectable. A biopsy was performed endonasally and allowed the histopathologic diagnosis of alveolar rhabdomyosarcoma. The patient underwent chemotherapy and visual acuity improved to 1/35 with eccentric fixation five weeks after the onset of chemotherapy.

Conclusion: Alveolar rhabdomyosarcoma of the orbit may masquerade as retrobulbar neuritis. Loss of visual acuity may be due to compression of the optic nerve, tumor cell infiltration and/or concurrent sinusitis. Differential diagnosis of acute visual loss includes retrobulbar neuritis, sinusitis and neoplastic conditions such as alveolar rhabdomyosarcoma.

Department of Ophthalmology and Eye Hospital, University Erlangen-N├╝rnberg, Schwabachanlage 6, D-91054 Erlangen, Germany


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