A case of orbital schwannoma with coexisting ptosis and proptosis

Schwannomas comprise 1% of orbital tumors with wide variation in clinical presentations, but the most common presentation is painless proptosis.[1,2] Orbital schwannoma is an extremely rare manifestation of ptosis which is usually missed due to its slow progression.[3] We hereby present a case of isolated orbital schwannoma presenting with ptosis. A 21-year-old male presented to our outpatient clinic with chief complaints of drooping of the right upper lid for 4 years, insidious in onset, gradually progressive, and worse in the morning [Figure 1]. He had a history of trauma to the right eyebrow 9 years ago. His best-corrected vision in both eyes was 6/6, visual axis was parallel, extraocular movements were normal. Hertel’s exophthalmometry showed right eye four millimeters exophthalmos.Figure 1: Right eye upper lid ptosisRight eye showed moderate ptosis with fair levator palpebrae superioris (LPS) action. Rest of the eye examination was normal in both eyes. Facial sensations were intact. Computed tomography (CT) of the orbit (1 mm cuts) showed mildly enhancing extraconal soft tissue lesion along the superior aspect of right orbit abutting the superior rectus muscle, suggestive of benign etiology in sagittal, coronal and axial sections [Figures 2-4]. His provisional diagnosis was right orbital neurofibroma. He underwent anterior orbitotomy through sub-brow incision and excision of the lesion under general anaesthesia with support from the Neurosurgeon. The specimen was sent for histopathology examination. Histopathology exam showed an encapsulated tumor showing hypercellular Antoni A areas and myxoid hypocellular Antoni b areas along with multiple verocay bodies suggestive of a schwannoma. At 4 months’ postoperative, there was no change in right eye ptosis [Figure 5]. CT orbits with contrast showed no evidence of residual or recurrent tumor. He underwent right LPS resection 13 months after the first orbital surgery. At 1-week postoperative, his ptosis was corrected, as shown in Figure 6. The duration at the final follow-up was 16 months and the patient was satisfied with the surgical outcome. Orbital schwannomas are noninvasive benign slow-growing peripheral nerve sheath tumors arising from Schwann cells which are responsible for myelin production. They occur mostly in superior orbit.[1,2] Common manifestations are painless, nonpulsatile ocular proptosis, globe displacement, or a palpable orbital mass.[2] According to a study conducted by El-Hajj et al., while proptosis was the most common presenting complaint in the patients of orbital schwannoma, 19% of the patients of presented with ptosis.[4] The acquired ptosis in such cases is due to weakness of the levator muscle.[5] In cases with fair levator action, like our patient, levator resection is the choice of surgery based of the amount of ptosis.[6]Figure 2: Sagittal sections of plain computed tomography orbits 1-mm cuts showing a mass in the upper aspect of the right orbit with yellow arrowFigure 3: Coronal sections of plain computed tomography orbits 1-mm cuts showing a mass in the upper aspect of the right orbit with yellow arrowFigure 4: Axial sections of plain computed tomography orbits 1-mm cuts showing a mass in the upper aspect of the right orbit with yellow arrowFigure 5: Four months postop with right eye moderate ptosisFigure 6: One week after right eye ptosis correctionFollowing are our conclusions from this case: This case highlights the importance of identifying ptosis in the presence of more apparent proptosis in such cases intraorbital tumors Patients with acquired ptosis require careful evaluation. Data availability statement The data supporting the findings of this study will be made available upon reasonable request. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.