Lacrimal gland botulinum toxin injection for epiphora management.

PURPOSE: Epiphora remains an often difficult to manage ocular complaint for ophthalmologists in all subspecialties. This review seeks to examine the safety and efficacy of botulinum toxin injection for management of chronic epiphora. METHODS: The authors conducted a Pubmed search for studies on the use of lacrimal and transplanted salivary gland botulinum toxin injections for the management of epiphora within the past 20 years. Studies included had a minimum of four glandular injections. RESULTS: The authors identified 14 studies and divided them by indication for injection; either functional epiphora, non-functional epiphora, or mixed studies. Seven studies examined injections for cases of functional epiphora, four for non-functional epiphora, and four for mixed cases. The number of glandular injections reported ranged from 4 to 65. Side effects reported were limited to diplopia, eyelid or lacrimal gland hematoma, papillary conjunctivitis, dry eye, ptosis, and bleeding. CONCLUSIONS: Glandular botulinum toxin injection should be considered as a viable treatment strategy for both functional and nonfunctional epiphora. From the studies reviewed, botulinum toxin injection was shown to be effective in both children and adults. Injection can be performed in the outpatient setting, is minimally invasive, technically easy to administer, has a favorable side effect profile, and good efficacy. Furthermore, repeat injections can be performed with similar efficacy.

Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma of the Orbit: Three Cases and Review of the Literature.

The authors present 3 patients from this retrospective case series to review the clinical findings, imaging, pathology, and treatment of orbital atypical lipomatous tumor/well-differentiated liposarcoma. Pathology of biopsy specimens ranged from spindle cell proliferations mimicking neurofibroma to proliferations of well-differentiated adipocytes. Immunohistochemical stains were positive for murine double minute 2 in 1 case, and fluorescent in situ hybridization showed amplification of murine double minute 2 in 2 cases. Treatments ranged from serial debulking, proton beam irradiation, and exenteration. None of the patients developed metastases. A literature review supported the low-grade nature of this lesion. Orbital atypical lipomatous tumor/well-differentiated liposarcoma is a low-grade, indolent liposarcoma that may be locally invasive. The histologic diagnosis is enhanced with immunohistochemical staining for murine double minute 2 and fluorescent in situ hybridization analysis for amplification of murine double minute 2. Although treatment may vary according to the individual, conservative therapies may be attempted prior to radical surgery.

Use of percutaneous bleomycin sclerotherapy for orbital lymphatic malformations.

PURPOSE: Bleomycin sclerotherapy has been shown to be a viable treatment for lymphatic malformations. However, its use for these lesions confined to the orbit is becoming increasingly documented in the literature. In this study, we summarize the clinical manifestations and outcomes observed following percutaneous bleomycin sclerotherapy for orbital lymphatic malformation. METHODS: A 5-year retrospective chart review of patients with clinical, radiographic, and/or biopsy-confirmed diagnoses of orbital lymphatic malformation that received bleomycin sclerotherapy was conducted at the Emory Hospital and Clinics. Data examined included patient demographics, patient history and symptoms, clinical findings, radiographic findings, route of bleomycin delivery, and outcome. RESULTS: Of the 10 patients who met inclusion criteria, the median age of treatment was 7 years. The most common presenting symptoms included vision change and proptosis. Nine of 10 patients demonstrated macrocysts (>1 cm) on imaging. Seven of 10 patients had histories of prior interventions including resections, cyst drainage, and debulking. Because 2 of these 10 patients were lost to follow-up, 8 patients remained for post-procedural evaluation. Four of these eight showed improvement of visual acuity after post-bleomycin sclerotherapy. In seven of eight patients, extraocular motility either improved or remained stable. Pretreatment and posttreatment exophthalmometer measurements obtained in four patients revealed an average improvement in proptosis of 65% from their average pretreatment measurements. CONCLUSIONS: Our findings suggest that percutaneous bleomycin sclerotherapy is a viable option for treatment of orbital lymphatic malformations, with potentially greater benefit to those with macrocystic features.

A 15-year review of secondary and tertiary optic nerve sheath fenestration for idiopathic intracranial hypertension.

PURPOSE: Optic nerve sheath fenestration (ONSF) is a common surgical option for patients with idiopathic intracranial hypertension (IIH) with vision loss refractory to medical management. Little is known about the visual benefit of repeated ONSF. The authors aimed to assess the efficacy of secondary and tertiary ONSF in patients with IIH. METHODS: A retrospective chart review was performed on all patients with repeat ONSF for IIH at Emory University from 1999 to 2016. Primary outcome measures included visual acuity, optic nerve head findings, and visual field results. RESULTS: A total of nine eyes in seven patients (five females and two males) with repeat ONSF were identified. Two of the seven patients had repeat ONSF in both eyes, while the remaining five patients had only one eye repeated. Five of seven patients (five eyes) improved or remained stable after the secondary ONSF. Two patients (three eyes) continued to worsen despite the secondary fenestration surgery and underwent tertiary ONSF at an average of 13.2 months (SD 5.5 months) after the failed secondary ONSF. Both patients that underwent the tertiary fenestration showed improvement. Six of the patients had either improvement or stability in their clinical findings at their last documented follow-up, but one continued to worsen despite intervention. CONCLUSIONS: This study suggests that secondary and tertiary nerve sheath fenestration is a viable management option for patients with progressive vision loss from IIH. Repeat ONSFs do not appear to have increased complication or failure rates compared to prior documented studies regarding primary fenestrations.

Review of periorbital nerve enlargement and biopsy techniques.

Periorbital nerve enlargement commonly indicates perineural invasion of malignancy or inflammatory conditions. This study reviews the role of supraorbital and infraorbital nerve biopsies in patients presenting with radiographic enlargement and to elucidate the surgical technique involved. A retrospective chart review (1997-2014) was performed at a single tertiary center. Patients with radiographic confirmation of enlarged supraorbital/infraorbital nerves that underwent biopsy were included. Charts were reviewed for: patient demographics and history, clinical symptoms and findings, radiographic findings, surgical method, and treatment. Five patients (4 female, 1 male) met inclusion criteria. Average age was 72.4 years (range 36-90). Four patients had history of cutaneous malignancy. All presented with diplopia and/or dysesthesias. Clinical examination confirmed decreased V1 and/or V2 sensation for 4 patients. Imaging revealed enlargement of V1, V2, and/or V3 in all patients. Infraorbital nerve biopsies were performed in 3 patients via transconjunctival fornix-based orbitotomy with subperiosteal dissection along orbital floor followed by unroofing of infraorbital canal. The remaining 2 underwent supraorbital nerve biopsy via sub-brow incision onto superior orbital rim with reflection of periosteum. Biopsies confirmed squamous cell carcinoma(3), mucoepidermoid carcinoma(1), and idiopathic orbital inflammation(1). Three patients initiated treatment in <1 month. One decided to follow-up closer to home, one was lost to follow-up. For patients presenting with enlarged supraorbital/infraorbital nerves, biopsy can rapidly confirm the underlying condition and facilitate early treatment. A sub-brow approach offers direct access to supraorbital nerve while transconjunctival fornix-based anterior orbitotomy with canal unroofing allows access to infraorbital nerve.

Giant Chondroid Syringoma of the Lower Eyelid.

Chondroid syringoma is a benign mixed tumor characterized by sweat gland elements in a cartilaginous stroma. This rare tumor accounts for only 0.01% of all primary skin tumors and occurs only rarely in the periorbital region. Usually between 0.5 cm and 3.0 cm, risk of malignancy increases in chondroid syringomas greater than 3.0 cm in size. Here, the authors report a rare case of giant chondroid syringoma arising in the lower eyelid, characterized by keratinized stratified epithelium in a cartilaginous stroma. This case illustrates the importance of considering a possible diagnosis of chondroid syringoma in the evaluation of eyelid masses.

Pleomorphic Adenoma With Prominent Clear Cell Myoepithelioma Component of the Lacrimal Gland.

A case of a pleomorphic adenoma of the lacrimal gland with a prominent clear cell myoepitheliomatous component was reported. An 81-year-old Caucasian woman experienced a 2-month history of right supraorbital swelling and proptosis. Excisional biopsy revealed a multicomponent lesion including a stromal component featuring glandular structures made of small epithelioid and spindle cells and a trabecular component with small islands of vacuolated cells, displaced nuclei, and clear cytoplasm. Immunohistochemical analysis revealed strong cytokeratin AE1/3 reactivity and focal smooth muscle actin positivity. The pathologic findings including immunohistochemistry results were consistent with a pleomorphic adenoma with prominent clear cell myoepithelioma component.

A modification to the Hughes tarsoconjunctival flap for a challenging case of recurrent lower eyelid retraction.

PURPOSE: To present a modification of the Hughes tarsoconjunctival flap to correct a case of refractory lower eyelid retraction. METHODS: The authors report a 49-year-old man with cicatricial right lower eyelid retraction that failed multiple surgical interventions, including ear cartilage grafts to the posterior lamella, tarsal strip, alloplastic implant, and Sub-Orbicularis Oculi Fat (SOOF) lift. A modified Hughes tarsoconjunctival flap was used for repair of a nonmarginal defect of the lower eyelid. A marginal defect was purposely created with a full-thickness blepharotomy that compensated for the amount of lower eyelid retraction. The posterior lamellar defect was filled with the tarsoconjunctival-Muller's flap and the anterior lamella defect covered with a full-thickness retroauricular skin graft. Mueller's muscle was included in the flap to increase the vascular supply to the reconstructed eyelid. Likewise, the authors waited 9 weeks to divide the flap because of concerns that the vascular supply of the surrounding eyelid might be compromised from multiple previous surgeries. RESULTS: A single case of cicatricial right lower eyelid retraction status after multiple failed surgical interventions successfully completed a full-thickness blepharotomy with a modified Hughes tarsoconjunctival flap. Two months after the procedure, the patient had good cosmesis and function with resolution of his signs and symptoms of exposure keratopathy. CONCLUSIONS: The Hughes tarsoconjunctival flap combined with a full-thickness blepharotomy may be a useful technique in selected patients for repair of nonmarginal defects of the lower eyelid when other standard techniques have failed.

Periorbital ecthyma gangrenosum: a case report and review of the literature.

Ecthyma gangrenosum is a cutaneous manifestation of a Pseudomonas aeruginosa infection that is extremely rare in the periorbital region. The authors present a 48-year-old woman with a newly diagnosed acute promyelocytic leukemia with necrosis of her bilateral upper and lower eyelids and the lacrimal system. Following treatment with intravenous antibiotic and surgical debridement with delayed reconstruction, the patient had healed well.

A cyst-like foreign body reaction to porcine decellularized membrane (TarSys).

A 58-year-old Caucasian woman with thyroid eye disease underwent a bilateral lower eyelid blepharoplasty with porcine decellularized membrane (TarSys) eyelid spacer graft placement. Three months postoperatively, she developed unusual cyst-like masses in both lower eyelids that were excised. These were found to be consistent with inflammatory cysts with a foreign body reaction. No such reaction has ever been reported.

Association of clinical response and long-term outcome among patients with biopsied orbital pseudotumor receiving modern radiation therapy.

PURPOSE: To retrospectively evaluate institutional outcomes for patients treated with modern radiation therapy (RT) for biopsied orbital pseudotumor (OP). METHODS AND MATERIALS: Twenty patients (26 affected orbits) with OP were treated with RT between January 2002 and December 2011. All patients underwent biopsy with histopathologic exclusion of other disease processes. Sixteen patients (80%) were treated with intensity modulated RT, 3 (15%) with opposed lateral beams, and 1 (5%) with electrons. Median RT dose was 27 Gy (range 25.2-30.6 Gy). Response to RT was evaluated at 4 months post-RT. Partial response (PR) was defined as improvement in orbital symptoms without an increase in steroid dose. Complete response (CR) 1 and CR 2 were defined as complete resolution of orbital symptoms with reduction in steroid dose (CR 1) or complete tapering of steroids (CR 2). The median follow-up period was 18.6 months (range 4-81.6 months). RESULTS: Seventeen patients (85%) demonstrated response to RT, with 7 (35%), 1 (5%), and 9 (45%) achieving a PR, CR 1, and CR 2, respectively. Of the 17 patients who had ≥ PR at 4 months post-RT, 6 (35%) experienced recurrence of symptoms. Age (>46 years vs ≤ 46 years, P=.04) and clinical response to RT (CR 2 vs CR 1/PR, P=.05) were significantly associated with pseudotumor recurrence. Long-term complications were seen in 7 patients (35%), including 4 with cataract formation, 1 with chronic dry eye, 1 with enophthalmos, and 1 with keratopathy. CONCLUSIONS: RT is an effective treatment for improving symptoms and tapering steroids in patients with a biopsy supported diagnosis of OP. Older age and complete response to RT were associated with a significantly reduced probability of symptom recurrence. The observed late complications may be related to RT, chronic use of steroids/immunosuppressants, medical comorbidities, or combination of factors.

Atypical bilateral orbital metastases of lobular breast carcinoma.

Breast carcinoma metastasizing to the orbit is a well-recognized entity. The authors present a 50-year-old woman with bilateral upper and lower eyelid masses and generalized dysmotility. MRI showed gadolinium-enhancing homogenous soft tissue masses filling both orbits from the eyelids to the apex and a biopsy that confirmed metastatic breast carcinoma. To the authors' knowledge, orbital metastasis of breast carcinoma has never presented in this manner.

Lupus erythematosus profundus masquerading as idiopathic orbital inflammatory syndrome.

Idiopathic orbital inflammatory syndrome (IOIS) is a nonspecific inflammation of orbital tissue. As it is a diagnosis of exclusion, systemic testing and, at times biopsy, is utilized to rule out other inflammatory etiologies. Since some inflammatory etiologies that masquerade as typical IOIS can be vision or life threatening, it is important to consider these diagnoses. Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune process that can affect the eye and visual system in 20% of individuals. In this idiopathic process, the deposition of pathogenic autoantibodies and immune complexes damage tissues and cells. Some common ocular manifestations of SLE include keratoconjunctivitis sicca, periocular skin lesions, orbital inflammation, retinal hemorrhages and vasculitis, retinal vaso-occlusive disease, iritis, scleritis, optic neuritis and optic neuropathy. One rare clinical entity in the SLE spectrum is panniculitis, also known as lupus erythematosus profundus (LEP), which is a nodular inflammation of adipose tissue. Panniculitis involving orbital structures as the primary presenting symptom of SLE is quite unusual and has only rarely been previously reported in the literature and has not been reported presenting as IOIS. This uncommon presentation can make the diagnosis more difficult. We describe a patient who had presented with ptosis evolving to orbital inflammation, which was consistent with IOIS by laboratory and histologic examinations. The patient later developed extensive panniculitis and a final diagnosis of LEP was made.

Multiple intratarsal keratinous cysts of the eyelid.

Intratarsal keratinous cysts are uncommonly reported entities that originate within the tarsal plate. Previously reported cysts have always been solitary, whether primary or recurrent. The authors present an unusual case of a 50-year-old man with 11 intratarsal keratinous cysts on a single eyelid. Complete excision of all the cysts was curative for at least 2 months.

Malignant (anaplastic) transformation of orbital clear cell meningioma.

A 69-year-old woman with a history of a previously resected right sphenoid wing meningioma and radiation presented with a 4-month history of a rapidly progressive right orbital swelling. MRI revealed a sphenoid wing mass extending in the right orbit and depressing the right globe. An incisional biopsy revealed a clear cell meningioma with anaplastic features. Histopathologic examination showed that the tumor was composed of sheets of polygonal cells with clear cytoplasm consistent with clear cell meningioma. Nuclear atypia with a high mitotic activity was also evident; thus, the tumor was classified as anaplastic. Clear cell meningioma is a rare form of meningioma usually located in the cerebellopontine or spinal cord areas.

Trichoblastoma of the eyelid.

A 50-year-old woman developed a slow-growing tumor of the right medial canthus. Excisional biopsy and histologic examination showed a trichoblastoma. This nodule was excised using Mohs micrographic and reconstructive surgery. Trichoblastoma is a rare, slow-growing neoplasm derived from the hair follicle. Though rarely undergoing malignant transformation, these nodules histologically resemble basal cell carcinoma and generally require complete excision.

Results of lacrimal gland botulinum toxin injection for epiphora in lacrimal obstruction and gustatory tearing.

PURPOSE: To describe the author's experience with the use of botulinum toxin (Botox, Allergan Inc., Irvine, CA, U.S.A.) injection in the palpebral lobe of the lacrimal gland for symptomatic epiphora due to lacrimal obstruction or gustatory tearing. METHODS: This is a retrospective review of 46 patients treated by the author with botulinum toxin injection in the palpebral lobe of the lacrimal gland for symptomatic epiphora due to lacrimal obstruction or gustatory tearing from 2001 through 2008. All patients were injected with 2.5 units of botulinum toxin, and the patients' subjective responses were assessed 1 to 2 weeks later. If there was insufficient response, they were reinjected with an additional 2.5 units of botulinum toxin and re-evaluated in 1 to 2 weeks. The response to the treatment and complications were evaluated. RESULTS: Overall, 74% of patients treated felt that tearing was mostly or completely improved. The only complication was temporary ptosis in 11% of the patients. CONCLUSION: Botulinum toxin injection in the palpebral lobe of the lacrimal gland can be used effectively and safely for symptomatic epiphora due to lacrimal obstruction and gustatory tearing. Although the beneficial results are temporary, the patient satisfaction in selected patients is high.

Giant corneal keloid: case report and review of the literature.

PURPOSE: To report a patient with a tumor-like corneal keloid. METHODS: Retrospective review of clinical features and histopathologic findings. RESULTS: A 68-year-old woman with a remote history of an orbital tumor treated with radiation developed a corneal tumor. A biopsy of the tumor showed fibrocellular tissue, and her blind painful eye was enucleated. Pathologic findings in the enucleated eye showed that the corneal mass was consistent with a large keloid. CONCLUSIONS: A corneal keloid may clinically appear as a large corneal tumor.

Myxofibrosarcoma of the orbit: a clinicopathologic case report.

A 27-year-old woman developed a rapidly progressive left orbital tumor that extended in the cranial fossa. MRI revealed a heterogenous enhancing lesion confined to the left frontal bone and superior orbit. An incisional biopsy was performed, and histopathologic examination of the specimen showed findings diagnostic of a high-grade myxofibrosarcoma. Complete excision with postoperative adjuvant radiation therapy and chemotherapy was performed, and the patient had no evidence of tumor recurrence within 6 months' follow-up. Myxofibrosarcoma is a fibroblast-derived soft tissue neoplasm with up to a 60% local recurrence rate, and metastasis may be associated with intermediate to high-grade tumors.

Mucinous eccrine adenocarcinoma of the eyelid: report of 6 cases.

OBJECTIVE: To report on patients with mucinous eccrine adenocarcinoma of the eyelid. DESIGN: Retrospective case series. PARTICIPANTS: Biopsy specimens of 6 patients with eyelid neoplasm were obtained. The pathologic diagnoses were mucinous eccrine adenocarcinomas. METHODS: The medical records, including demographic, clinical, histopathologic, and follow-up information, were reviewed. RESULTS: Five patients underwent surgical removal of the lesion by means of Mohs micrographic surgery. Four of these patients were doing well; 1 of them, with positive margins of resection, had 2 recurrences. One case was lost to follow-up. CONCLUSIONS: Mucinous eccrine adenocarcinoma is an uncommon adnexal tumour that can involve the eyelid, has low metastasis and mortality, but can be invasive or locally recur. Mohs micrographic surgery is a recommended treatment of mucinous eccrine adenocarcinoma of the eyelid.