Herpes Zoster Ophthalmicus With Dacryoadenitis Complicated by Recurrent Orbital Inflammation.

Herpes zoster ophthalmicus represents a zoster infection in the first division of the trigeminal nerve and has potentially serious complications involving the ocular and orbital structures. Herpes zoster ophthalmicus occurs in approximately 10% to 20% of individuals with herpes zoster and can lead to significant morbidity, particularly in patients with multiple comorbidities. The authors present a case of herpes zoster ophthalmicus along with dacryoadenitis in a patient with uncontrolled diabetes and rheumatoid arthritis who was misdiagnosed, had delayed treatment, and experienced recurrence with orbital symptoms during follow up. Prompt recognition, initiation of therapy and careful monitoring and follow up are important for treating herpes zoster ophthalmicus and preventing recurrence and long-term sequelae.

Benign Reactive Lymphoid Hyperplasia of the Conjunctiva Treated With Cyclosporine.

PURPOSE: To describe a case of reactive lymphoid hyperplasia (RLH) of the conjunctiva responding to cyclosporine immunosuppressant monotherapy. METHODS: A 66-year-old man with a 2-year history of biopsy-proven bilateral RLH presented for dry eye evaluation with chief complaints of burning, stinging, and irritation in both eyes. After slit-lamp examination and positive findings of matrix metalloproteinase 9 in the patient's tear film, he was diagnosed with meibomian gland dysfunction and tear-insufficiency dry eye disease. The patient was subsequently treated with topical cyclosporine (ophthalmic emulsion 0.5 mg/mL two times per day) in both eyes. RESULTS: Examination at the 3-month follow-up visit revealed significant reduction of the RLH lesions bilaterally. CONCLUSIONS: This report represents the first case of benign ocular RLH responsive to topical cyclosporine therapy. We believe that cyclosporine could play a role in treating patients with benign ocular RLH and warrants further investigation to evaluate its full efficacy.

Alveolar rhabdomyosarcoma masquerading as embryonal subtype: the value of modern molecular diagnostic testing.

A 3-year-old child presented with a history of conjunctivitis in her OS. She had hyperglobus of the OS and a palpable mass in the left inferior orbit. An urgent MRI revealed an enhancing mass in the left orbit. The patient underwent an incisional biopsy with pathologic assessment most consistent with embryonal rhabdomyosarcoma. However, subsequent molecular genetic testing of the biopsy specimen was positive for the PAX3/PAX7-FKHR chimeric gene, indicating the tumor was actually an alveolar rhabdomyosarcoma. The patient received a more intensive chemotherapy regimen and also was treated with proton-beam radiotherapy. After chemotherapy and proton irradiation, the patient's tumor shrank considerably, and she remained in remission at over 3 years of follow up. This patient illustrates the new tools at the disposal of the orbital specialist including molecular genetic testing and proton-beam irradiation to diagnose and treat orbital rhabdomyosarcoma.

Diesel fuel injury to the orbit.

A 48-year-old man was seen in the Wills Eye Institute emergency room for pain and decreased vision after having a diesel engine fuel cylinder explosion in his face. Clinical examination showed proptosis, decreased extraocular motility, and increased intraocular pressure; CT scan imaging revealed hypodense areas within the orbit. His orbital signs rapidly worsened despite aggressive antibiotics, steroids, and surgical intervention. Two orbitotomies revealed diesel fuel infiltration of the intraconal and extraconal spaces that proved impossible to evacuate completely. The patient ended up losing vision from an intense chemical cellulitis with subsequent orbital compartment syndrome and optic neuropathy. This case highlights the severity of orbital chemical injuries and their variable prognoses despite aggressive management.

Trochlear calcification: a common entity.

PURPOSE: This study aims to quantify the prevalence of trochlear calcification. METHODS: A retrospective case review was performed on orbital CT scans ordered at Wills Eye Emergency room and Jefferson Hospital for Neuroscience for patients from June 2009 through September of 2009. RESULTS: In our series, we examined 132 scans, 61% (81/132) men, 39% (51/132) women, ranging in age from 10-83. We found a 13% (17/132) prevalence of calcification of the trochlea, 53% (9/17) unilateral and 47% (8/17) bilateral. Forty-one percent (7/17) were female, 59% male (10/17). Age (19-83) was not a factor in the presence of trochlear calcification. The calcification either came straight off of the bone or was free-floating in the trochlea. CONCLUSIONS: Our study establishes that calcification of the trochlea is common in the general population with a prevalence of 13%. Calcification of the trochlea should be considered in the differential diagnosis of hyperintense densities in the superomedial orbit, especially in cases of trauma where it can be confused with a foreign body.

Orbital hemorrhage after intravitreal injection.

PURPOSE: To describe a case of orbital hemorrhage after the administration of intravitreal ranibizumab. METHODS: Retrospective chart review of one case. RESULTS: A 73-year-old woman with a history of atrial fibrillation on coumadin anticoagulation (international normalized ratio = 3.4) presented with decreased vision and metamorphopsia in her right eye for 2 weeks duration. Examination was consistent with a diagnosis of neovascular age-related macular degeneration in the right eye. After one injection of intravitreal ranibizumab, she developed a bullous subconjunctival hemorrhage that extended into an orbital hemorrhage. CONCLUSION: Intravitreal injection may result in orbital hemorrhage, particularly in the anticoagulated patient.

Pediatric thyroid-associated orbitopathy: the Children's Hospital of Philadelphia experience and literature review.

BACKGROUND: As Graves' disease is uncommon in children, Graves' eye disease should be even more unusual. Here we report our experience with Graves' eye disease at the Children's Hospital of Philadelphia and review the literature on ophthalmic findings in children with Graves' disease. SUMMARY: A retrospective review identified 152 children with Graves' disease seen in the endocrinology clinic of the Children's Hospital of Philadelphia over a 3-year period. Of this cohort, only 26 (17%) were referred to ophthalmology because of prominent ophthalmic manifestations. The ages of the patients ranged from 4 months to 17 years. Sixteen of 26 patients were female. Most patients had mild findings consistent with Graves' disease. Proptosis was noted in 10 of 26 (38%). Lid retraction was present in 6 of 26 (23%). Mild corneal punctuate staining was identified in only 3 of the 26 patients (12%). No patients had strabismus or optic neuropathy. Three newly diagnosed Graves' patients who were seen as the retrospective review was being completed were all girls. All three had normal vision, motility, and fundus exams. Two had mild proptosis, lid retraction, and lid lag on down gaze. None had corneal, motility, or optic nerve pathology. These findings are consistent with previous studies in the literature. CONCLUSIONS: Eye findings in pediatric Graves' disease are usually mild and typically respond to local measures and control of disturbed thyroid function. Surgery is indicated in a small number of patients for cornea exposure or appearance issues. Graves' disease-associated optic neuropathy has never been reported in the pediatric population.

Erlotinib-associated trichomegaly.

A 70-year-old woman presented with a corneal ulcer resulting from aberrant and exuberant eyelash growth. She had recently been diagnosed with recurrent non-small-cell lung cancer for which she had begun a chemotherapeutic regimen that included erlotinib HCl. After several months of treatment, she noted the onset of escalating ocular irritation, which culminated in her presentation. Examination of her ocular adnexa revealed coarse, brittle, irregular aberrant eyelash growth. She has been successfully treated with regular epilation and eyelash trimming and has remained symptom free.

The histopathologic effects of balloon dacryoplasty on the rabbit nasolacrimal duct.

PURPOSE: To examine the histopathologic effects of balloon dacryoplasty on the nasolacrimal duct of the rabbit. METHODS: Sixteen nasolacrimal ducts of 8 infant white New Zealand rabbits were divided into 3 treatment groups: a control group, a 2 mm balloon dilation group, and a 3 mm balloon dilation group. The LacriCath balloon catheter (Quest, Medical, Allen, TX) was used for nasolacrimal duct dilation according to the manufacturer's recommended human protocol. The rabbits were euthanized at 1, 3, and 7 days after treatment and the nasolacrimal ducts were harvested for histological examination. RESULTS: There were no significant differences between treatment groups in the number of neutrophils in the lumen, epithelium, and stroma of the duct; the number of monocytes in the stroma of the duct; or in the number of red blood cells in the stroma of the duct. No differences were found in overall cellularity of any type in the nasolacrimal ducts of treated and untreated rabbits. Finally, no histopathologic signs of crush injury were evident. CONCLUSION: Balloon dacryoplasty does not induce significant inflammation or crush injury in the nasolacrimal duct of a rabbit model.

Anatomic and aesthetic considerations in midfacial rejuvenation.

An early sign of the aging process involves changes to the midface. The loss of the lid-cheek continuum begins with underlying laxity in the orbitomalar ligament. As the face continues to age, the malar pad drops inferomedially in position. The approach to the midface has evolved over the past 15 years with the advent of endoscopically assisted techniques of midfacial elevation. Further technology, such as thread-lifting procedures and the Endotine Midfacetrade mark, have supplemented the choices for surgical correction of this facial segment. Descriptions of the different approaches to the aging midface are reviewed.

The male eyebrow: a topographic anatomic analysis.

PURPOSE: To study and quantify the topographic anatomy of the male eyebrow and its 3-dimensional relation, based on age and location, relative to the eye and the supraorbital rim. METHODS: A prospective cohort of male patients seen for routine ophthalmic evaluation age 10 years and older had several eyebrow measurements incorporated in the examination. The presence or absence of brow ptosis was noted on the basis of observation. The location of the eyebrow relative to the center of the pupil, the lateral canthus, and the depth of the supraorbital rim was measured. The data were subjected to statistical analysis. RESULTS: The study included 444 eyebrows of 222 patients ranging in age from 10 to 86 years, with a mean age of 46.75 years. The incidence of brow ptosis was 7.4%. The mean distance between the lateral canthus and inferior brow (LIBD) was 11.23 mm. The mean distance between the pupil and the inferior brow and the superior brow border was 11.15 mm (PIBD) and 19.8 mm (PSBD), respectively. Statistical analysis demonstrated that the mean brow position did not vary with age. However, there was a significant negative correlation between the position of the eyebrow and the depth of the superior sulcus below the supraorbital rim (correlation coefficient, 0.431 for PIBD and depth and 0.381 for LIBD and depth, respectively). Deeper-set eyes had lower brow position. CONCLUSIONS: The male eyebrow sits along the supraorbital ridge and has a flat contour. In men without frank brow ptosis, the eyebrow position relative to the pupil and the lateral canthus does not significantly fall with age despite the periocular changes associated with aging. However, 3-dimensional analysis demonstrates that individuals with deep-set eyes have a lower positioned eyebrow then those with a more shallow depth below the supraorbital rim. These findings are helpful in surgical management of the eyelid-eyebrow continuum. The age-related changes in the eyebrow area should be thought of as a 3-dimensional construct with collapse of the structure and not simply descent of the eyebrow.

Comparison of monocanalicular stenting and balloon dacryoplasty in secondary treatment of congenital nasolacrimal duct obstruction after failed primary probing.

PURPOSE: To determine the success of monocanalicular stenting and balloon dacryoplasty as secondary treatment options for congenital nasolacrimal duct obstruction after failed probing surgery. METHODS: An interventional case series of consecutive secondary balloon dacryoplasty and monocanalicular stenting for congenital nasolacrimal duct obstruction was reviewed. These secondary treatments were used in cases in which a bicanalicular stent would have been used in the past. Seventy-seven nasolacrimal systems in children with epiphora after probing and irrigation surgery were treated with a monocanalicular stent or balloon dacryoplasty. The patients were then evaluated at least 3 months after surgery or after stent removal by using a dye disappearance test. Cases in which there was no significant dye at 5 minutes were considered a success. Cases with residual dye or history of persistent tearing were considered failures. RESULTS: The monocanalicular stent was used in 35 nasolacrimal systems, whereas balloon dacryoplasty was used in 42 nasolacrimal ducts. The mean age of treatment was 25.2 months for the monocanalicular stent group and 25.8 months for the balloon group. Overall, 32 of 35 (91%) nasolacrimal ducts responded to monocanalicular stenting, whereas 36 of 42 (86%) responded to balloon treatment. When the patient group was further stratified by age, the monocanalicular stenting was 94% successful in children younger than age 2 years and 89% successful for children older than 2 years. The balloon treatment had a success rate of 91% in the younger group and 79% in the older group. Chi-square statistical analysis showed no significant difference between the two treatments or on the basis of age stratification within each treatment group. CONCLUSIONS: Monocanalicular stenting and balloon dacryoplasty are excellent secondary therapies for congenital nasolacrimal duct obstruction after initial probing and irrigation surgery has failed. These two treatment options are now our procedures of choice for secondary surgery.

Heterozygous mutations of the kinesin KIF21A in congenital fibrosis of the extraocular muscles type 1 (CFEOM1).

Congenital fibrosis of the extraocular muscles type 1 (CFEOM1; OMIM #135700) is an autosomal dominant strabismus disorder associated with defects of the oculomotor nerve. We show that individuals with CFEOM1 harbor heterozygous missense mutations in a kinesin motor protein encoded by KIF21A. We identified six different mutations in 44 of 45 probands. The primary mutational hotspots are in the stalk domain, highlighting an important new role for KIF21A and its stalk in the formation of the oculomotor axis.

Cat scratch disease presenting as orbital abscess and osteomyelitis.

Ocular manifestations of cat scratch disease are uncommon. The diagnosis is usually made on the basis of increasing Bartonella henselae serum antibody titers. We report a child presenting with orbital abscess and osteomyelitis who was diagnosed with hepatosplenic cat scratch disease by detection of B. henselae DNA in the orbital abscess fluid.

Encephalocraniocutaneous lipomatosis: a neurocutaneous syndrome.

The neurocutaneous pattern syndromes are a group of disorders characterized by congenital abnormalities involving both the skin and the nervous system for which no identifiable cause has been isolated.(1) Ophthalmologic manifestations of these syndromes are common. These rare syndromes often have overlapping ophthalmic and systemic findings. Examples include encephalocraniocutaneous lipomatosis (ECCL), oculocerebrocutaneous syndrome (OCC), and linear nevus sebaceous syndrome (LNS). Clarifying the diagnostic criteria for these syndromes is a worthy goal because it will help elucidate the phenotypic spectrum of these poorly understood diseases as well as possibly facilitate genetic counseling. In this short report, a case of ECCL is used to illustrate the clinical manifestations of neurocutaneous syndromes.

Orbital presentation of posttransplantation lymphoproliferative disorder: a small case series.

OBJECTIVE: To describe a small series of patients with orbital presentation of posttransplantation lymphoproliferative disorder (PTLD). DESIGN: Retrospective, interventional case series. PARTICIPANTS: Three patients with orbital presentation of histologically diagnosed PTLD. METHODS: Review of medical records. MAIN OUTCOME MEASURES: Measured parameters included vision, proptosis, and tumor extent. RESULTS: Three cases of orbital PTLD are described. In two of the cases, the tumor initially demonstrated orbital signs and symptoms, whereas in the third case, orbital and systemic signs were synchronous. Two of three patients had disseminated disease discovered at the time of presentation. One adult patient had synchronous presentation of PTLD in the orbit and prostate. One pediatric patient had tumor dissemination to the liver at the time of presentation. The PTLD tumors were classified histologically as diffuse large cell lymphoma of monomorphic or immunoblastic type in all three cases. Treatment included local irradiation, decreased immunosuppression, and antilymphocyte monoclonal antibodies. CONCLUSIONS: Orbital presentation is a rare manifestation of PTLD. However, ophthalmologists must consider this diagnosis carefully in organ transplant recipients with subtle orbital signs and symptoms at presentation. Early detection may alter prognosis. In each case presented, the diagnosis was established via lesion biopsy and subsequent histologic or flow cytometric evaluation, or both.

Metastatic breast cancer to 4 eyelids: a clinicopathologic report.

A 47-year-old woman presented complaining of progressive, painless swelling of all 4 eyelids for 6 weeks. Her medical history was significant for breast cancer 14 months prior. Examination results showed a diffuse, firm thickening of all 4 eyelids with mild erythema of the overlying skin. Orbital computer tomography revealed extensive preseptal infiltration of soft tissue of all 4 eyelids, which enhanced with contrast. Results of a bilateral biopsy of the upper eyelids demonstrated extensive infiltration of the orbicularis muscle. Histologic features were consistent with metastatic breast cancer. Results of both the primary breast cancer biopsy taken 14 months previously and the eyelid biopsies were the same histologic type. Presentation of metastatic breast cancer to the eyelids is rare, but a recurrence must be considered in any patient with a history of breast cancer, despite the length of tumor-free survival. Bilateral involvement should not exclude metastases from diagnostic consideration, but rather, the diagnosis requires a high degree of clinical suspicion and recognition of the various cutaneous forms.