Clinical Features and Outcomes of Post-Traumatic Silent Sinus Syndrome.

PURPOSE: To identify demographic features and clinical outcomes associated with post-traumatic silent sinus syndrome. METHODS: A retrospective review was carried out at 3 academic medical centers to identify all cases of post-traumatic silent sinus syndrome. Clinical features and management strategies were recorded. Postoperative outcomes were assessed, and statistical analyses were performed via a dedicated computerized software package. RESULTS: Twenty cases were identified (14 men and 6 women, mean age = 44.2 years). Seven patients underwent sinus surgery as the sole means of treatment, and the mean pre- and postoperative enophthalmos measurements were 2.86 and 1.93 mm. Alternatively, 13 patients underwent combined orbital reconstruction and sinus surgery, respectively; the mean pre- and postoperative enophthalmos measurements were 3.42 and 0.39 mm, respectively. The change in enophthalmos was statistically significantly greater in patients who underwent sinus surgery and orbital reconstruction (p = 0.00028). Among patients who underwent sinus surgery alone, one patients (14.2%) experienced complete resolution of enophthalmos, as compared with 10 patients (76.9%) who underwent combined procedures. CONCLUSIONS: This study represents the largest published cohort of patients with post-traumatic silent sinus syndrome. Combined orbital reconstruction and sinus surgery results in greater reductions of enophthalmos and a markedly improved chance of postoperative symmetry of globe position.

Idiopathic Orbital Inflammation Associated With Necrotizing Scleritis and Temporal Bone Inflammation.

The authors present a case of aggressive idiopathic orbital inflammation producing necrotizing scleritis along with synchronous tumefactive fibroinflammatory lesion of the temporal bone. A young woman with no medical history presented with sectoral scleritis and mildly reduced vision. Response to initial treatment, which included topical and systemic corticosteroids, as well as systemic nonsteroidal anti-inflammatory drugs, was limited. Over the following months, signs of orbital inflammation developed, including ptosis, proptosis, and limited extraocular motility. MRI revealed both orbital and ipsilateral temporal bone masses. An orbital biopsy was performed revealing a mixed inflammatory infiltrate, whereas a biopsy of the temporal bone mass revealed a tumefactive fibroinflammatory lesion. Biopsy showed no histopathologic evidence of infection nor neoplasm. The patient eventually responded to treatment with systemic prednisone, azathioprine, and rituximab.

Temporal Artery Calciphylaxis Presenting as Temporal Arteritis in a Case of Rhinoorbitocerebral Mucormycosis.

Mucormycosis is a rare often fatal opportunistic fungal infection. It is typically described in patients with diabetes in ketoacidotic status and is rare in renal transplant recipients. Calciphylaxis is a rare and highly morbid disease of vascular calcification affecting patients with end-stage renal disease (ESRD). The first case of a renal transplant recipient who was inflicted with both rhinoorbitocerebral mucormycosis and calciphylaxis is reported. A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness. He had undergone renal transplant for ESRD 7 months earlier with resultant immunosuppressive therapy. MRI and nasal biopsy confirmed rhinoorbitocerebral mucormycosis. Immunosuppressive therapy was stopped and antifungal therapy begun. He had orbital exenteration for progressive rhinoorbitocerebral mucormycosis. Two months later, the patient reported new-onset intermittent bitemporal headache and bilateral swollen, tender temporal arteries. Temporal artery biopsy revealed features consistent with calciphylaxis. Clinical presentation, treatment course, and follow up are discussed.

Orbital schwannomatosis in the absence of neurofibromatosis.

PURPOSE: The aim of this study was to describe 3 cases of primary orbital schwannomatosis without associated systemic neurofibromatosis. METHODS: This is a retrospective interventional study of 3 patients who presented with multiple, distinct masses in the orbit (n = 3) as well as in the hemiface (n = 1). The clinical presentation, imaging features, surgical procedures, and outcomes were defined. RESULTS: Two women and a man presented with of exophthalmos and diplopia. Pain was the most prominent complaint in 2 patients. None of the patients had associated systemic neurofibromatosis by history or examination. Radiologic evaluation with computed tomography or magnetic resonance imaging of orbit revealed multiple well-demarcated intraconal and extraconal masses. Masses were excised, and histopathology confirmed all masses to be schwannomas. Postoperative follow-up was uneventful with alleviation of primary complaints in all patients. CONCLUSIONS: Multiple orbital schwannomas (primary orbital schwannomatosis) may be observed in patients without systemic association of neurofibromatosis. Management includes surgical excision of the tumors to achieve relief from their mass effects.

Papillary cystadenocarcinoma of the lacrimal gland after radiation for bilateral retinoblastoma.

The authors report a case of papillary cystadenocarcinoma of the lacrimal gland after irradiation for bilateral retinoblastoma. A 32-year-old man with a history of bilateral retinoblastoma, diagnosed shortly after birth, was treated with enucleation of the OS and a single session of radiation to the OD. Over 30 years later, he presented with an orbital mass of the right lacrimal gland that on biopsy demonstrated papillary cystadenocarcinoma.

Iatrogenic cerebrospinal fluid oculorrhea.

Cerebrospinal fluid leakage into the orbit (CSF orbitorrhea) or through the orbit to the exterior (CSF oculorrhea) occurs when there is a communication between orbit and subarachnoid space. It has rarely been described. We report a case of CSF oculorrhea following surgery for recurrent sphenoid wing meningioma. A 67-year-old patient who underwent craniotomy for a recurrent sphenoid wing meningioma complained of tearing from the ipsilateral eye. Fluid collection was observed in the right periorbital area contiguous with craniotomy wound. With wide retraction of eyelids, a 1-cm longitudinal full-thickness laceration through the conjunctiva toward the lateral orbital rim was visualized in the area of the lateral canthal tendon. Steady leakage of pink-tinged serous fluid through the forniceal laceration was observed. With a presumed diagnosis of iatrogenic CSF oculorrhea, the patient was treated conservatively with resolution by postoperative day 6.

Pilomatrixoma in a patient with bilateral retinoblastoma.

A 3-year-old girl with a history of bilateral retinoblastoma presented with a new right lower periorbital mass that showed calcifications on ultrasound. She had previously undergone systemic and intra-arterial chemotherapy for retinoblastoma but had no evidence of active disease for at least 6 months previously. Her family and oncologists feared that this mass was an extraocular metastasis of her retinoblastoma. On excision, it was diagnosed as a pilomatrixoma, an uncommon benign neoplasm that originates from the matrix of the hair root. This is the first reported case of pilomatrixoma in a patient with retinoblastoma.

Interferon for ocular surface squamous neoplasia in 81 cases: outcomes based on the American Joint Committee on Cancer classification.

PURPOSE: To assess the efficacy of interferon alpha-2b (IFNα2b) in the management of ocular surface squamous neoplasia (OSSN). METHODS: This is a retrospective, nonrandomized interventional case series study of 80 patients with 81 tumors treated with IFNα2b eye drops and/or injection combined with surgical excision when necessary. The main outcome measure was complete response or partial response based on the American Joint Committee on Cancer classification. RESULTS: The OSSN was classified as Tis (n = 10, 12%), T1 (n = 13, 16%), T2 (n = 6, 7%), T3 (n = 51, 63%), and T4 (n = 1, 1%). IFNα2b was used as immunotherapy alone (n = 22, 27%) or combined with surgery (n = 59, 73%). Overall (n = 81), complete response was achieved in 90% Tis, in 100% T1, in 100% T2, in 94% T3, and in 100% T4. Specifically for immunotherapy (n = 22), IFNα2b alone achieved complete response in 75% (3/4) Tis, in 100% (8/8) T1, and in 70% (7/10) T3. Planned IFNα2b plus surgery (n = 59) achieved control in 100% (6/6) Tis, in 100% (5/5) T1, in 100% (6/6) T2, in 100% (41/41) T3, and in 100% (1/1) T4. Tumor recurrence was noted in 5% (4/81) of cases over a median follow-up of 1 year. Ocular side effects included conjunctival hyperemia (n = 4, 5%), ocular irritation (n = 3, 4%), superficial punctate keratitis (n = 3, 4%), and conjunctival follicles (n = 1, 1%). Systemic side effects included postinjection flu-like syndrome for 1 day (n = 7, 9%). CONCLUSIONS: IFNα2b, when appropriately combined with surgical excision for OSSN, provides complete control in 95% of cases overall, specifically in 90% Tis, in 100% T1, in 100% T2, in 94% T3, and in 100% T4.

Pars plana Baerveldt tube insertion with pars plana vitrectomy for refractory glaucoma.

BACKGROUND: Glaucoma drainage implants (GDIs) are used for managing recalcitrant glaucoma and are usually placed in the anterior chamber. This approach may lead to complications such as corneal decompensation, and so a pars plana approach is used in at risk eyes. AIMS: To compare functional outcomes and complications of 250 mm(2) and 350 mm(2) pars plana Baerveldt tube insertion with pars plana vitrectomy (PPV) (both 20- and 23-gauge) for managing refractory glaucoma. SETTINGS AND DESIGN: A retrospective chart review of 38 patients (39 eyes) undergoing combined PPV-Baerveldt procedure for glaucoma recalcitrant to maximal medical treatment or previous filtering procedures with >6 weeks of follow-up. MATERIALS AND METHODS: Main outcome measures were visual acuity, intraocular pressure (IOP), number of glaucoma medications, and postoperative complications. STATISTICAL ANALYSIS USED: A paired 't' test was used to evaluate changes in IOP and glaucoma medications, Fisher's exact test was used to compare complication rates, and Kaplan-Meier survival curves were constructed for comparison of overall outcomes. RESULTS: Mean patient age was 62.2 years. Mean follow-up period was 33.7 months, with 36 (92%) eyes followed for ≥6 months. Mean±SD preoperative IOP and number of glaucoma medications were significantly reduced by the combined procedure (P<0.05). Thirty-five (90%) eyes maintained final IOP between 6 and 21 mmHg. Vision improved by ≥2 lines in 10 (26%) eyes, remained stable in 15 (38%) eyes, and decreased in 14 (36%) eyes. Two (5.1%) eyes developed no light perception vision, with one (2.6%) eye becoming phthisical. Twenty-four (62%) eyes developed complications managed with conservative measures. Five (13%) eyes required ≥1 surgeries within a year of the combined procedure. CONCLUSIONS: Pars plana Baerveldt tube implantation with PPV can preserve vision, reduce IOP, and decrease the number of glaucoma medications necessary to achieve target IOP in patients with recalcitrant glaucoma.

Topical interferon alfa-2b for management of ocular surface squamous neoplasia in 23 cases: outcomes based on American Joint Committee on Cancer classification.

OBJECTIVE: To evaluate the efficacy of topical interferon alfa-2b in the management of ocular surface squamous neoplasia (OSSN). METHODS: Clinically visible OSSN in 20 patients (23 tumors) was managed with topical interferon alfa-2b, 1 million IU/mL, 4 times daily. Tumor control and complications were evaluated according to American Joint Committee on Cancer classification. RESULTS: Complete tumor resolution was achieved in 19 tumors (83%) following topical interferon alfa-2b treatment for a median period of 6 months (mean, 7 months; range, 1-12 months) and maintained for up to 24 months of follow-up. Of the 4 tumors with partial resolution (17%), tumor surface area was reduced 44% (median) during 4 months (median) without further response and alternative therapy was used. Based on American Joint Committee on Cancer classification, complete control was achieved in 2 of 3 Tis (67%), 17 of 20 T3 (85%), 19 of 23 N0 (83%), and 19 of 23 M0 (83%) category tumors. Tumors involving the cornea responded earlier compared with those without corneal involvement (P = .01). Initial tumor size did not correlate with time to response (P = .27). Recurrence was noted in 1 case (Tis, 4%) at 3 months. Adverse effects included conjunctival hyperemia (2 [10%]), follicular hypertrophy (2 [10%]), giant papillary conjunctivitis (1 [5%]), irritation (1 [5%]), corneal epithelial defect (1 [5%]), and flulike symptoms (1 [5%]); all resolved within 1 month of medication discontinuation. CONCLUSION: According to American Joint Committee on Cancer classification, complete control with topical interferon alfa-2b can be achieved in 67% of Tis, 85% of T3, and 83% of all OSSN.

Giant ocular surface squamous neoplasia managed with interferon alpha-2b as immunotherapy or immunoreduction.

OBJECTIVE: To evaluate the efficacy of interferon alpha-2b (IFNα2b) for extensive ocular surface squamous neoplasia (OSSN). DESIGN: Retrospective, interventional case series. PARTICIPANTS: Eighteen eyes in 18 patients. METHODS: Each patient with giant OSSN (a single tumor ≥15 mm basal diameter or ≥6 limbal clock-hours) was managed with topical IFNα2b (1 million IU/ml) 4 times daily or with injection IFNα2b (a portion of 10 million IU/ml vial) with follow-up every 1 to 3 months. MAIN OUTCOME MEASURES: Tumor response, recurrence, and treatment complications were evaluated. RESULTS: Eighteen patients with giant OSSN (median diameter [MD], 20 mm; median clock-hours [MCH], 6) were treated with topical IFNα2b (n = 12), injection IFNα2b (n = 3), or both (n = 3). The IFNα2b achieved complete tumor control (immunotherapy) in 13 eyes and partial tumor control with reduction in size (immunoreduction) in 5 eyes. Topical IFNα2b alone (n = 12) provided complete immunotherapy in 7 eyes (MD, 12 mm; MCH, 9) over a median period of 5 months and immunoreduction by 74% in 5 eyes (MD, 20 mm; MCH, 3), allowing for subsequent surgical excision (n = 3), photodynamic therapy (n = 1), or cryotherapy (n = 1) for tumor control. Injection IFNα2b alone (n = 3; median, 1 injection) provided complete control of giant tarsal conjunctival OSSN (MD, 20 mm) over a 1-month period. A combination of topical and injection IFNα2b (n = 3; median, 3 injections) completely resolved larger tumors (MD, 30 mm; MCH, 6) over a 6-month period. Complications of IFNα2b included transient flu-like symptoms (n = 3), corneal epithelial defect (n = 2), and conjunctival hyperemia (n = 1). During a median follow-up of 11 months, there were no tumor recurrences, but 2 new tumors appeared at a remote site from the original tumor, requiring operative intervention. CONCLUSIONS: In 72% of giant OSSNs IFNα2b achieved complete control (immunotherapy); there was a reduction in size (immunoreduction) in 28% of giant OSSNs.

Fungal rhinosinusitis and imaging modalities.

This report provides an overview of fungal rhinosinusitis with a particular focus on acute fulminant invasive fungal sinusitis (AFIFS). Imaging modalities and findings that aid in diagnosis and surgical planning are reviewed with a pathophysiologic focus. In addition, the differential diagnosis based on imaging suggestive of AFIFS is considered.