Bilateral Vision Loss Due to Sphenoidal Rhabdomyosarcoma

Parameningeal sinus rhabdomyosarcoma has been reported at a rate of 16%, with most cases diagnosed between the ages of 5 and 7 years. These malignancies are often challenging to diagnose given their nonspecific signs and symptoms. If untreated, these neoplasms can rapidly progress to affect nearby structures, including the orbit. We hereby present a case of rapid bilateral visual loss, a rarely reported consequence of this aggressive malignancy.

Poster 60 Bilateral Vision Loss Due to Sphenoidal Rhabdomyosarcoma Swarup Swaminathan1, Kara Cavuoto1, Carla Osigian1, Byron Lam1, Joshua Pasol1 Bascom Palmer Eye Institute, Miami, Florida, USA 1 Introduction: Parameningeal sinus rhabdomyosarcoma has been reported at a rate of 16%, with most cases diagnosed between the ages of 5 and 7 years. These malignancies are often challenging to diagnose given their nonspecific signs and symptoms. If untreated, these neoplasms can rapidly progress to affect nearby structures, including the orbit. We hereby present a case of rapid bilateral visual loss, a rarely reported consequence of this aggressive malignancy. Methods: A 7-year old female was seen in our institution's ocular emergency room due to double vision accompanied by headache and nausea. She was noted to have a febrile sinus illness for which she was receiving oral antibiotics. CT imaging just 3 weeks prior was interpreted as unremarkable. The patient was found to have a visual acuity of 20/50 in both eyes. On motility exam, she had an esotropia measuring 30 prism diopters with complete abduction deficits of the right and left eyes, leading to a diagnosis of bilateral cranial nerve VI palsies. On fundus examination, her optic nerves were full but without pallor or edema. An orbital MRI showed a 5x3x2cm lesion within the sphenoid sinus extending into the nasal cavity, epidural space, and extraconal space of both orbits. The patient was emergently referred to the pediatric emergency room for neurosurgical evaluation. Transsphenoidal biopsy confirmed the diagnosis of embryonal rhabdomyosarcoma. She underwent chemotherapy with vincristine, dactinomycin, and cyclophosphamide, and subsequent radiation therapy. One month later in the ophthalmology clinic, she was noted to have no light perception in either eye with pale optic nerves. Results: . Conclusions: Rhabdomyosarcoma can be extremely aggressive with significant sequelae. Rapid diagnosis and treatment is essential to reducing morbidity and mortality; however even with appropriate treatment, severe vision loss can occur. This case highlights the importance of expedited neuro-imaging in children with persistent, new-onset headaches and/or abnormal extraocular motility. References: Breen LA, Kline LB, Hart WM, Burde RM. Rhabdomyosarcoma causing rapid bilateral visual loss in children. J Clin Neuroophthalmol. 1984;4(3):185-8. Luu QC, Lasky JL, Moore TB, Nelson S, Wang MB. Treatment of embryonal rhabdomyosarcoma of the sinus and orbit with chemotherapy, radiation, and endoscopic surgery. J Pediatr Surg. 2006;41(6):e15-7. Machleder DJ, Banik R, Rosenberg RB, Parikh SR. An unusual case of rhabdomyosarcoma presenting as orbital apex syndrome. Int J Pediatr Otorhinolaryngol. 2005;69(2):249-54. Keywords: Tumors, Chemotherapy and radiation injury, Optic neuropathy, Pediatric neuro-ophthalmology Financial Disclosures: The authors had no disclosures. Grant Support: None. 114 | North American Neuro-Ophthalmology Society