Inadvertent Orbital Mitomycin C Injection as a Cause of Ptosis and Ophthalmoplegia from Orbital Necrosis.

Mitomycin C is an alkylating agent with the ability to suppress fibroblast proliferation and activity, making it a powerful antifibrotic. It has therefore become popular in glaucoma filtering surgeries, used both intraoperatively during bleb formation and postoperatively as an adjunct to bleb needling. This report presents a rare but serious risk of bleb needling with Mitomycin C at the slit lamp, where inadvertent movement of the patient resulted in an orbital injection. The patient quickly developed focal tissue inflammation and necrosis, presenting one day after the procedure with complete ptosis, ophthalmoplegia, and a palpable orbital mass. After appropriate imaging and an excisional biopsy to exclude infectious, infiltrative, and neoplastic causes, this was managed with close observation and continued improvement, and resolution of most orbital sequelae.

Ocular manifestations and long-term complications of rhabdomyosarcoma in children.

BACKGROUND/OBJECTIVES: The purpose of the study was to describe the ocular manifestations of rhabdomyosarcoma in a large cohort of children. SUBJECT/METHODS: This was a retrospective observational cohort study. The medical records of all pediatric patients with head and neck rhabdomyosarcoma diagnosed between 1997 and 2021 at a tertiary-care pediatric hospital were analyzed. The main outcome measures were the incidence and prognostic role of ocular findings at presentation and long-term ocular complications. RESULTS: There were 77 children with head and neck rhabdomyosarcoma in the study cohort with 38 patients showing ocular manifestations at presentation. Median age at diagnosis was 6.0 years, the median follow-up was 5.7 years and 54.5% were male. At last follow-up, 70.1% had no evidence of progression, 26.0% were deceased, and 2.6% were on palliative treatment. Orbital signs were common (44.2%). The most common ocular findings were proptosis (18.2%), restriction of extraocular motility (28.6%), strabismus/diplopia (22.1%) and ptosis (16.9%). The most common long-term complications were bony hypoplasia/facial asymmetry (40.3%) and keratopathy/dry eye (31.2%). Poor visual acuity (≤20/200) was noted in 13 (16.9%) patients with 5 (6.5%) patients requiring an exenteration. Survival was 100% in primary orbital RMS (p = 0.02), whereas any or a combination of cranial nerve palsies carried a poor prognosis (42% survival, p = 0.008). CONCLUSIONS: In our cohort, half of children with rhabdomyosarcoma had ocular manifestations at presentation with about one-third showing orbital tumor involvement. Cranial nerve involvement carried a significantly worse prognosis for survival.

Clinical features and management of keratoconjunctivitis associated with inadequate tear drainage.

OBJECTIVE: To describe clinical features and management of toxic keratoconjunctivitis associated with punctal and (or) canaliculus stenosis (toxic soup syndrome). DESIGN: Retrospective observational case series. METHODS: Electronic medical record database search for the keywords "toxic soup syndrome" and retrospective chart review were performed. Thirty-five eyes of 25 patients were seen at a tertiary cornea clinic between January 2017 and December 2021. Sex, age, distance-corrected visual acuity, topical medications, symptoms, clinical signs, and outcomes after medical and surgical interventions were analyzed. The main outcome measured was improvement in ocular surface signs and symptoms after interventions. RESULTS: Overall, 35 eyes of 25 patients with a mean age of 66.8 ± 12.8 years, of which 72% were female, were included. The primary complaint was hyperemia and epiphora in all patients. Rosacea or meibomian gland disease were present in all patients, 7 (28%) had glaucoma, and 7 (28%) developed limbal stem cell deficiency. Twenty-two patients (88%) were using topical medications on presentation. All eyes had either punctal plugs, cauterized puncta, or punctal and (or) canaliculus stenosis. Management of all patients consisted of suspension of all preserved topical medications and institution of some type of anti-inflammatory therapy. Nineteen patients (76%) improved after improvement of lacrimal drainage. One patient with severe resistance in the canaliculus required ongoing preservative-free topical steroids. CONCLUSIONS: Chronic drug-induced or pooled inflammatory mediators causing toxic conjunctivitis may be aggravated by punctal and (or) canaliculus stenosis, leading to toxic soup syndrome. Clearance of punctal obstruction leads to improvement in most patients.

The Isabel Differential Diagnosis Generator for Orbital Diagnosis.

PURPOSE: The Isabel differential diagnosis generator is one of the most widely known electronic diagnosis decision support tools. The authors prospectively evaluated the utility of Isabel for orbital disease differential diagnosis. METHODS: The terms "proptosis," "lid retraction," "orbit inflammation," "orbit tumour," "orbit tumor, infiltrative" and "orbital tumor, well-circumscribed" were separately input into Isabel and the results were tabulated. Then the clinical details (patient age, gender, signs, symptoms, and imaging findings) of 25 orbital cases from a textbook of orbital surgery were entered into Isabel. The top 10 differential diagnoses generated by Isabel were compared with the correct diagnosis. RESULTS: Isabel identified hyperthyroidism and Graves ophthalmopathy as the leading causes of lid retraction, but many common causes of proptosis and orbital tumors were not correctly elucidated. Of the textbook cases, Isabel correctly identified 4/25 (16%) of orbital cases as one of its top 10 differential diagnoses, and the median rank of the correct diagnosis was 6/10. Thirty-two percent of the output diagnoses were unlikely to cause orbital disease. CONCLUSION: Isabel is currently of limited value in the mainstream orbital differential diagnosis. The incorporation of anatomic localizations and imaging findings may help increase the accuracy of orbital diagnosis.

Gaze-evoked amaurosis from idiopathic orbital inflammation.

Gaze-evoked amaurosis is a transient monocular vision loss elicited by eccentric gaze and has been reported in many orbital conditions but is most classically associated with intraconal tumors, such as cavernous hemangioma and optic nerve sheath meningioma. Here, the authors report a case of gaze-evoked amaurosis due to idiopathic orbital inflammation. The patient was a 35-year-old man who presented with vision loss only when abducting his left eye. He had a history of sclerosing idiopathic orbital inflammation with a left orbital intraconal mass diagnosed 15 months prior to the current presentation. The patient had difficulty with immunosuppressive therapy, which was stopped 5 months prior to presentation. Repeat imaging during the current presentation revealed enlargement of the mass. This case demonstrates that idiopathic orbital inflammation should be considered in the differential diagnosis for gaze-evoked amaurosis, which may be the first symptom of disease progression.

Diplopia Following the Insertion of a Canalicular Bypass Tube: Etiology, Risk Factors, Management, and Outcomes.

PURPOSE: To report the etiology, management, and possible risk factors for diplopia after canalicular bypass surgery. METHODS: A multicenter retrospective, noncomparative case series of patients who developed diplopia following canalicular bypass surgery were assessed. RESULTS: Twenty-four cases of diplopia were identified across 12 institutions. Tubes were inserted as a primary procedure with external dacryocystorhinostomy (DCR) (1; 4%) or without DCR (10; 42%) or as a secondary procedure after external (8; 33%) or endonasal (5; 21%) DCR. Factors predisposing to local damage were noted in 17 (71%): these factors included preexisting autoimmune/inflammatory condition (7 cases), medial canthal tumor resection (5 cases), preoperative radiotherapy (2 cases), 2 drug treatments (topical and systemic), and 1 local surgery. Horizontal diplopia was due to restriction of abduction and first noted at a median of 3.5 months (mean: 17.8 months, range: 1 day to 112 months) and persisted in 23 (96%) cases with a mean restriction of -2, affecting primary gaze in 4 patients and activities of daily living in 13 (42%). Seventeen patients received various treatments: 10 were operated on resulting in cure in 1 and improvement in 9. A stable degree of diplopia persisted in all but one patient. CONCLUSIONS: Restriction of abduction causing horizontal diplopia is a rare complication with canalicular bypass surgery and a notably high proportion occurred after tube placement without DCR; carunculectomy was not ubiquitous. Although in some the diplopia may be improved with intervention, the chance of cure is low. This complication should probably be included during informed consent for canalicular bypass tubes.

Guidelines for vismodegib in the management of periocular basal cell carcinoma.

OBJECTIVE: The management of advanced basal cell carcinoma (BCC) in the periocular region remains a clinical challenge. Vismodegib (ErivedgeTM) has been approved in 2013 by Health Canada for adult patients with "histologically confirmed metastatic BCC or locally advanced BCC inappropriate for surgery or radiation." An expert consensus was sought to create a standardised approach in the use of this novel treatment. METHODS: Fourteen practicing oculoplastic surgeons across Canada were involved in formulating and reviewing guidelines until consensus was reached. A consultancy meeting was followed by further ratification of guidelines over email. Two voting surveys were performed of the group to objectively assess agreement over each statement within the guidelines. Ratification continued until at least two-thirds of the group agreed on every guideline statement. RESULTS: The guidelines summarize 21 statements in a major and minor criteria format. A multidisciplinary team review is suggested for each patient with the involvement of recommended specialists. The internal survey revealed 100% agreement over 9 statements, 91.7% agreement over 8 statements, 83.3% agreement over 4 statements, and 2 statements had 66.7% and 58.7% agreement each. All statements with less than 91.7% agreement were surveyed again, and they were kept, modified, or removed on the basis of a consensus of over 66.7%. CONCLUSIONS: These guidelines serve to act as a framework for physicians considering vismodegib for the medical management of patients with advanced or metastatic periocular BCC. Future applications, including neoadjuvant uses of the drug, may become apparent through further research.

Acute Unilateral Ptosis and Myositis Following the H1N1 Influenza Vaccine.

The authors present a rare case of sudden onset unilateral complete ptosis occurring hours after administration of the H1N1 influenza vaccine. A complete workup for autoimmune and neurological disease was negative. MRI of the orbits demonstrated unilateral orbital myositis involving the left superior rectus/levator complex, superior oblique, and medial rectus muscles. Ptosis resolved spontaneously over 2 months without intervention. An autoimmune mechanism may explain rare cases of orbital myositis following administration of the H1N1 influenza vaccination.A case of acute onset and spontaneous resolution of complete unilateral ptosis and orbital myositis post H1N1 influenza vaccination.

Management of a Large Congenital Hemangioma Obstructing Visual Axis: A Case Report and Review of Literature.

Congenital hemangiomas comprise a subset of vascular tumors with clinicopathologic features that are distinct from the more common infantile hemangioma. The authors present a patient with a large congenital hemangioma involving the forehead and brow which obstructed the visual axis and created significant risk for deprivational amblyopia. Management of the congenital hemangioma involved customized headgear to clear the visual axis and early vascular embolization of feeder vessels with the subsequent successful surgical resection at 23 days of life.A large amblyogenic congenital hemangioma required a multidisciplinary approach involving early vascular embolization of feeder vessels and subsequent surgical resection at 23 days of life.

Management of orbital rhabdomyosarcoma in a child with Li-Fraumeni syndrome.

This case highlights the management of orbital rhabdomyosarcoma in a child with Li Fraumeni syndrome (LFS). Treatment with chemotherapy and eventual orbital exenteration enabled margin-free control of the tumor. Radiation therapy was avoided to reduce the risk of inducing additional malignancy. Reactive orbital hyperostosis was observed postoperatively and was confirmed with surgical biopsy of the orbital roof. In this case, systemic surveillance imaging, which is necessary in patients with LFS, revealed an adrenal cortical carcinoma.

A Case of Pseudomonas Orbital Cellulitis Following Glaucoma Device Implantation.

PURPOSE: Orbital cellulitis is a rare complication of aqueous tube shunt surgery. Nine cases have been described in the literature, though the microbiologic etiology is rarely reported. Management with intravenous antibiotics and/or explantation has been described. METHODS: This is a case report and literature review. CASE: A 64-year-old woman developed pain, periorbital swelling, limited extraocular motility, proptosis, and conjunctival injection 3 days following implantation of an Ahmed Glaucoma Valve. Computed tomography of the orbits with contrast showed soft tissue fat stranding consistent with orbital inflammation. Initial medical management with topical and intravenous ceftriaxone and vancomycin was unsuccessful. Surgical removal of the implant was performed and intraoperative cultures demonstrated florid Pseudomonas aeruginosa growth. Antibiotic coverage was changed to Piperacillin-Tazobactam for 3 days, with eventual resolution of her orbital symptoms. CONCLUSIONS: We report the first case of orbital cellulitis after implantation of a glaucoma device associated with P. aeruginosa. Failure of intravenous and topical antibiotics led to explantation of the valve and targeted intravenous antibiotic therapy with subsequent clinical improvement.

Perceptions and use of computer-assisted surgery (CAS) in the orbit.

Computer-assisted surgery (CAS) plays a prominent role in certain surgical disciplines. We investigated the current perceptions and use of this technology for orbital surgery. An online survey was emailed to members of the American Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Society of Oculoplastic Surgery, and British Oculoplastic Surgery Society. Respondents were asked to describe their practice type and seniority, their frequency of orbital surgery, experience, use, and accessibility of CAS, and their opinion on the technology. There were a total of 151 responses across the societies. 105 respondents (69.5%) had been in attending/consultant practice for over 10 years, with over half (54.7%) working in academic/teaching hospitals. The majority (66.7%) had superficial or no experience with CAS. In total, 84.8% of respondents rarely or never use CAS for orbital surgery (n = 128). Posterior orbital surgery (64.2%) and orbital decompression (49.0%) were the two most useful reasons to implement CAS. Longer operating time (58.3%) and cost (54.8%) were the two most selected weaknesses for CAS, whereas improved accuracy in attaining surgical end point(s) (80.8%) and patient safety (63.6%) were the principal advantages. Type of practice was significantly associated with CAS availability/accessibility (p < 0.05). Proportion of orbital surgery performed in practice was significantly associated with both CAS experience and use (p < 0.05). Our study confirms an expected variation in the perception and use of CAS for orbital surgery. Demonstrated patient benefit and integration of refined and cost-effective CAS systems into operating room environments may influence its future role.

Complete ophthalmoplegia in Ipilmumab and Nivolumab combination treatment for metastatic melanoma.

Ipilimumab and Nivolumab are novel monoclonal antibodies that have recently been used successfully for treatment of metastatic melanoma. Ipilimumab is a human monoclonal antibody against Cytotoxic T Lymphocyte Antigen 4 (CTLA4) receptor, which suppresses T-cell proliferation and stimulates an inflammatory response against cancer cells. Nivolumab is an IgG4 monoclonal antibody against the cytotoxic T lymphocyte associated programmed death 1 receptor (PD-1). Ipilimumab and Nivolumab combination treatment has been shown to induce remission and prolong survival in patients with metastatic melanoma. The side effect profile of these medications has not been well studied. One entity of the side effects reported in the literature is immune-related adverse events (irAEs). There have been few case reports where these events were serious and irreversible. In this case report, we describe a fatal and severe diffuse panmyositis that involved the cardiac, respiratory, and extraocular muscles in a patient with metastatic melanoma secondary to combination treatment with Ipilimumab/Nivolumab.

Endovascular Management of a Traumatic Infraorbital Pseudoaneurysm Causing Orbital Compartment Syndrome.

An 89-year-old woman presented after blunt injury to the left orbit from a fall. Examination findings were suggestive of left-sided orbital compartment syndrome, unresponsive to emergent lateral canthotomy and cantholysis. CT revealed a left-sided orbital floor blowout fracture involving the infraorbital canal, with a large maxillary and infraorbital hematoma. Angiography revealed a pseudoaneurysm supplied by the infraorbital artery. Interventional neuroradiology successfully achieved hemorrhage control by endovascular obliteration of the parent artery close to the pseudoaneurysm. To our knowledge, this is the first reported case of successfully managing active intraorbital hemorrhage causing orbital compartment syndrome by endovascular vessel sacrifice of an infraorbital artery pseudoaneurysm.