Abstract
INTRODUCTION AND IMPORTANCE: Rhabdomyosarcoma (RMS) can have various unusual presentations. We report an unusual presentation of RMS as orbital apex syndrome in a six-year-old boy involving the petrous part of the temporal bone. CASE PRESENTATION: A six-year-old boy presented with drooping of the left eyelid for seven days associated with headache, left-sided hearing loss, and nasal blockage. Contrast-enhanced magnetic resonance imaging (CE-MRI) brain and orbit revealed an ill-defined expansile vascular lesion centered at the petrous part of the left temporal bone with extension to the cavernous sinus, which was confirmed as RMS on histopathology and immunohistochemistry. The patient was managed by chemotherapy and radiotherapy. CLINICAL DISCUSSION: RMS is the most common aggressive malignant soft tissue tumor in the pediatric population. It accounts for 4-8 % of all malignancies in children below 15 years of age, with strong male preponderance. The most common site for RMS is head and neck (45 %), having maximum incidence during the first decade of life. CONCLUSION: Total external ophthalmoplegia in a child is an acute emergency; it should be properly worked up, and neuroimaging should always be advised. Prompt diagnosis and management by a multidisciplinary team can be both life and sight-saving.