Robert A. Hyde, Yunxiang Liu, Vinay K. Aakalu & Pete Setabutr
We report the case of an anterior orbital tumor in a young woman that enlarged during pregnancy. The mass was excised and found to be a spindle cell tumor with immunohistochemical reactivity consistent with a solitary fibrous tumor, a rare entity in the spectrum of fibroblastic mesenchymal tumors. The tumor was strongly positive for the progesterone receptor, consistent with its clinical growth during the antenatal and postnatal periods. To our knowledge, a primary orbital tumor with these characteristics has rarely been reported in the literature.
Bipasha Mukherjee, Uma Devi & Sumita Agarkar
Ossifying fibroma (OF) is a benign fibro-osseous neoplasm which may be mistaken for other similar lesions due to overlapping clinical and radiological features. We report a 5-year-old male child with recurrent benign OF of the orbit. The child had two episodes of recurrence in a span of 18 months. Computed tomography (CT) of orbit showed a large, lobulated expansile fibro-osseous lesion involving the greater wing of sphenoid and orbital roof without intracranial extension. An excisional biopsy was done though an orbital approach. Histopathology showed fibroblast rich stroma with bony trabeculae. Osteoblastic rimming without any mitotic activity was suggestive of juvenile OF. The child developed a recurrence 6 months following the initial excision, and surgical excision was done by a neurosurgeon using a bicoronal approach. The patient had another recurrence after 1 year requiring further surgery. At 2-year follow up there was no recurrence. Juvenile OF is the most aggressive variant that commonly occurs in children, the other benign fibro-osseous lesions being fibrous dysplasias (FDs), osseous dysplasias, and familial gigantiform cementomas.
Seanna R. Grob, Natalie Wolkow, Frederick A. Jakobiec & Daniel R. Lefebvre
A 53-year-old male presented with a bony lesion over the superior orbital rim increasing in size over several months. CT imaging showed a circumscribed, osseous lesion involving the outer table of the right frontal bone and superior orbital rim with a honeycomb appearance. Anterior orbitotomy revealed an osseous lesion along the superior orbital rim with purple cavernous spaces. Histopathological examination demonstrated cavernous vascular channels with variably-sized lumens and variably-thickened vascular walls interspersed among bony trabeculae consistent with an osseous cavernous hemangioma.
Amun Sachdev, Declan Costello & Simon Madge
In ophthalmology, there have been few reports of botulinum toxin type-A (BTX-A) injection into the lacrimal gland to treat epiphora. In ENT, adductor and abductor (ABSD) spasmodic dysphonia are often treated with BTX-A injections into the respective overacting vocal cord muscles. We describe a 53-year old male with Parkinson’s disease who did not respond to BTX-A injections to either the lacrimal gland, for epiphora secondary to Parkinsonian-related blink lagophthalmos, or posterior cricoarytenoid (PCA) muscles for ABSD. Subsequent BTX type-B (BTX-B) injections into the lacrimal gland remarkably improved his epiphora. BTX-B injections into the PCA muscle also greatly improved his dysphonia. We describe the first reported case of (1) BTX-B injection into the lacrimal gland for epiphora, (2) use of Botox in treating epiphora due to blink lagophthalmos/reduced blink frequency secondary to Parkinson’s disease, (3) BTX-B use in treating ABSD, and (4) association between ABSD and Parkinson’s disease.
Justin B. Hellman, Ilana Traynis & Lily Koo Lin
We report the first case of fatal myocarditis presenting as bilateral ptosis in a patient on combination therapy with pembrolizumab and epacadostat. An 83 year-old man with stage III high-grade urothelial carcinoma presented with acute onset droopy eyelids one month after starting pembrolizumab and epacadostat. Exam showed myogenic ptosis and ophthalmoplegia. He was later found to have acute myocarditis with complete heart block and subsequently passed away. Pembrolizumab in combination with epacadostat can induce a potentially fatal myocarditis. Although immune mediated myocarditis is a rare established side effect, more reported fatalities are needed in the literature to highlight the urgency for standardized cardiac monitoring of even asymptomatic patients to prevent fatal outcomes, as well as a consensus on treatment protocols. Cancer immunotherapy complications are not well known to ophthalmologists. This case is unique in that the presenting sign was ptosis, which prompted the patient to call his ophthalmologist first.
Verona E. Botha, Kent T. K. Chow, Paul J. M. Salmon, Rami H. El-Khayat, Michelle T. Sun, Dinesh Selva & Stephen G. J. Ng
Desmoplasia is the formation of a dense collagenous stroma around a neoplasm. It occurs in a variety of malignancies including squamous cell carcinoma (SCC). While desmoplasia is uncommonly seen in cutaneous SCC, it is an independent risk factor for recurrence and metastasis. We report a case series of desmoplastic SCC in the periorbital region. Seven cases were identified: the median age was 68, four were men. The mean follow-up was 48 months. Two patients (29%) had aggressive local recurrence: one required salvage surgery including orbital exenteration, parotidectomy, and neck dissection to excise involved parotid and cervical lymph nodes; the other required repeat excision and adjuvant radiotherapy. Desmoplastic SCC is an uncommon but highly aggressive subtype. In the periorbital region, due to the high risk of orbital invasion, it is potentially sight and life-threatening.
Ann Q. Tran, Daniela P. Reyes-Capó, Nimesh A. Patel, Joshua Pasol, Hilda Capó & Sara T. Wester
A 51-year-old female underwent four upper zygomatic dental implants (ZI) and one upper and four lower conventional implants. Immediately postoperatively, the patient had pain and diplopia upon manual elevation of the edematous eyelid. Panoramic x-ray showed a malpositioned right upper ZI, requiring removal of the right upper ZI the following day. The patient had delayed referral to ophthalmology one month later for persistent diplopia. Computed tomography scan and magnetic resonance imaging demonstrated a right inferolateral fracture with fibrosis surrounding the inferior oblique muscle. Clinical exam showed right lower eyelid retraction, right hypotropia, and inability to elevate in adduction, consistent with a right inferior oblique paresis. Surgical exploration revealed incarceration of lid and orbital tissue into the fracture. After repositioning of the prolapsed tissue, a high-density porous polyethylene implant was placed for fracture repair. The inferior fornix was reconstructed with amniotic membrane and 5-fluorouracil was injected into the scar tissue. Six months later, the patient underwent strabismus surgery with resolution of symptoms.
Pavindran Gounder, Chandrashan Perera, Glenn Moore, Neil Powers & Kayvan Arashvand
A 9-year-old girl was reviewed by a tertiary ophthalmology service after being hit in her right upper eyelid by a fish whilst swimming. Initial wound exploration demonstrated fish scales in the wound. She was first treated conservatively with washout of the 5-mm wound and was discharged with oral ciprofloxacin. Five days later, the patient re-presented with a worsened ptosis due to periorbital swelling. Ultrasound of the upper lid demonstrated a foreign body in the upper lid. The patient was taken to theatre and, with the assistance of intraoperative ultrasound, the jaws of a needle fish were removed from the upper lid. The case highlights the importance of ultrasound and its intraoperative utility in cases of trauma and a suspicion of retained foreign bodies as well as the potential danger of fish injuries off the West Australian coast.
Nicolien A. van der Poel, Kornelis D. de Witt, René van den Berg, Maartje M. de Win & Maarten P. Mourits
Purpose: To present nine new cases of superior ophthalmic vein thrombosis (SOVT) and compare these with the literature, and to assess the impact of SOVT for the clinician.
Methods: Using the data bases of the Department of Ophthalmology of the AMC, we searched for patients with radiologically evidenced SOVT between January 2006 and December 2014. In addition, a PubMed search, using the mesh term ‘superior ophthalmic vein thrombosis’, was done.
Results: We found nine patients with SOVT. In three patients, SOVT was related to dural arteriovenous fistulae. In one patient, it was caused by the acute reversal of warfarin by vitamin K. In two patients, an infectious cause was found. In three patients, the cause of SOVT was not found despite screening for coagulation and other disorders. All patients presented with eyelid swelling, proptosis, and/or motility impairment. We found complete recovery in four patients. Three patients had mild sequelae and two patients had severe visual impairment. In the literature, we found 60 cases reporting on SOVT with various aetiologies. Clinical presentation, treatment modalities, and outcomes were comparable to our findings.
Conclusion: Our case series and literature review show that SOVT can occur simultaneously with cavernous sinus thrombosis (CST) but can also be a separate entity. Clinical presentation can mimic orbital cellulitis (OC) or CST and when no signs of OC can be found, an alternative cause for SOVT should be sought. When timely and adequate treatment is conducted, the prognosis is predominantly favourable.
Samuel S. Y. Wang, Mitchell B. Lee, Adarsh George, Sarah B. Wang, Jonathan Blackwell, Steve Moran & Ian C. Francis
Purpose: Multiple myeloma is an insidious haematological malignancy characterised by monoclonal proliferation of plasma cells in the bone marrow. Extramedullary plasmacytoma is a rare manifestation of multiple myeloma and usually occurs in the upper respiratory tract. Orbital involvement is particularly uncommon, but may be associated with devastating visual impairment and poor clinical outcomes. Therefore, this article aims to highlight the need for multidisciplinary management of orbital extramedullary plasmacytoma.
Methods: This is a retrospective observational case series of five patients. All presented to the authors for management of orbital extramedullary plasmacytomas from 2004 to 2015 at Prince of Wales and Mater Hospitals in Sydney, Australia. Medical records were reviewed for pertinent information including demographics, disease features, management strategy, and clinical progress. The study met Medical Ethics Board standards and is in accordance with the Helsinki Agreements.
Results: This case series of five patients underscores the poor prognosis of orbital extramedullary plasmacytoma. Despite aggressive multidisciplinary management, four of these five patients succumbed to their illness during the study period. However, multidisciplinary management did manage to minimise symptoms and preserve quality of life.
Conclusions: On a case-by-case basis, patients may derive palliative benefit from orbital surgery in conjunction with radiotherapy and chemotherapy. Orbital surgeons are encouraged to work within a multidisciplinary framework of medical specialists, including haematologists and radiation oncologists, when determining the optimal management plan in cases of orbital extramedullary plasmacytoma.