97th DOG Annual Meeting 1999
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
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.
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.
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-Nürnberg, Schwabachanlage 6, D-91054 Erlangen, Germany