Meshed dermal regeneration template for traumatic periocular tissue loss in the young population.

The IntegraⓇ Dermal Regeneration Template (DRT) is a bioengineered dermal substitute that is becoming increasingly popular in the field of reconstruction. Its unique properties allow for immediate wound closure while providing a scaffold for tissue regeneration. Currently, it is commonly used to treat burns, ulcers, and complex wounds. In the setting of traumatic periocular tissue loss, only two prior reports have been published on its use for primary reconstruction. We present our institution's experience with a series of four young patients who received primary reconstruction with IntegraⓇ DRT as a full-thickness skin substitute for their large traumatic periorbital skin defects.

Intraosseous hemangioma of the orbit: a case report involving pre-operative embolization with reconstruction using a custom porous polyethylene implant.

A 28-year-old female presented with a slowly enlarging, left cheek mass over two years. She underwent neuroimaging and was found to have a well-defined, low attenuating lesion with thickened vertical trabeculation of the left zygoma, consistent with intraosseous hemangioma. To minimize the risk of severe intraoperative hemorrhage, the patient underwent embolization of the mass by neuro-interventional radiology two days prior to resection. The patient subsequently underwent a left anterior orbitotomy and partial zygoma resection followed by reconstruction of the lateral orbit with a custom porous polyethylene zygomaxillary implant. The postoperative course was uneventful with a good cosmetic outcome.

Cladophialophora bantiana orbital cellulitis after penetrating injury.

A 75-year-old immunocompetent male presented with a right orbital cellulitis after a foreign body penetrating injury. He was taken for orbitotomy with foreign body removal and started on broad-spectrum antibiotics. Intra-operative cultures were positive for Cladophialophora bantiana, a mold known for causing brain abscesses with no prior reports of orbital invasion in the literature. Following culture results, the patient was managed with voriconazole and required multiple orbitotomies and washouts for infection control.

Immunoglobulin A vasculitis presenting as bilateral upper eyelid erythema: A case report and review of literature.

Immunoglobulin A vasculitis (IgAV) is the most common vasculitis of childhood characterized by petechial or purpuric rash, abdominal pain, arthralgia, and renal involvement. Ophthalmic manifestations of IgAV are uncommon. Herein, we describe a case of bilateral upper eyelid erythema presenting in a 6-year-old male, leading to a diagnosis of IgAV.

Outcomes of Patients With Thyroid Eye Disease Partially Treated With Teprotumumab.

PURPOSE: In response to the coronavirus (COVID-19) pandemic, teprotumumab production was temporarily halted with resources diverted toward vaccine production. Many patients who initiated treatment with teprotumumab for thyroid eye disease were forced to deviate from the standard protocol. This study investigates the response of teprotumumab when patients receive fewer than the standard 8-dose regimen. METHODS: This observational cross-sectional cohort study included patients from 15 institutions with active or minimal to no clinical activity thyroid eye disease treated with the standard teprotumumab infusion protocol. Patients were included if they had completed at least 1 teprotumumab infusion and had not yet completed all 8 planned infusions. Data were collected before teprotumumab initiation, within 3 weeks of last dose before interruption, and at the visit before teprotumumab reinitiation. The primary outcome measure was reduction in proptosis more than 2 mm. Secondary outcome measures included change in clinical activity score (CAS), extraocular motility restriction, margin reflex distance-1 (MRD1), and reported adverse events. RESULTS: The study included 74 patients. Mean age was 57.8 years, and 77% were female. There were 62 active and 12 minimal to no clinical activity patients. Patients completed an average of 4.2 teprotumumab infusions before interruption. A significant mean reduction in proptosis (-2.9 mm in active and -2.8 mm in minimal to no clinical activity patients, P < 0.01) was noted and maintained during interruption. For active patients, a 3.4-point reduction in CAS ( P < 0.01) and reduction in ocular motility restriction ( P < 0.01) were maintained during interruption. CONCLUSIONS: Patients partially treated with teprotumumab achieve significant reduction in proptosis, CAS, and extraocular muscle restriction and maintain these improvements through the period of interruption.

Orbital osteomas associated with Gardner's syndrome: a case presentation and review of literature.

Gardner syndrome (GS) is a rare genetic disorder characterized by numerous intestinal colon polyps with various extraintestinal manifestations. Osteomas are a known extracolonic manifestation of GS and can affect the orbit, as seen in our patient, as well as 13 other cases documented in literature. Excision of large orbital osteomas can be successful with a multi-disciplinary approach as presented in this article. Ophthalmologists can even be the first to diagnose GS, usually via the presence of congenital hypertrophy of the retinal pigment epithelium (CHRPE) lesions. Untreated, 100% of colon polyps will transform into cancer, thus it is important to be aware of this rare syndrome with ophthalmic manifestations and screen patients with osteomas for GS.

Alpha-Gal Syndrome: A New Etiology for Periorbital Edema.

Alpha-gal syndrome is characterized by an IgE-mediated hypersensitivity response to the carbohydrate galactose-alpha-1,3-galactose. It can manifest as both an immediate drug allergy to pharmaceuticals containing alpha-gal as well as a delayed hypersensitivity response to the ingestion of mammalian meat products. In the United States, sensitization to alpha-gal is thought to occur from tick bites and can cause severe angioedema, most commonly periorbitally and periorally, with high rates of anaphylaxis. We present a unique case of chronic periorbital edema in a carnivorous individual after acquiring a new red meat allergy from Alpha-Gal Syndrome.

Orbital invasion by Esthesioneuroblastoma: a comparative case series and review of literature.

PURPOSE: To review the current literature on esthesioneuroblastoma (ENB) as it pertains to clinical features, grading systems, treatment options, and survival. METHODS: A literature search in PubMed was performed to include all articles published in English with orbit involving ENB. Only articles that included each patient's demographics, tumor stage, treatment, or survival were included. A total of 22 articles with 104 patients were considered for this literature review. We also present five cases of ENB, all encountered in our health system, between 2010 and 2020. RESULTS: The median age of diagnosis of orbit involving ENB was 44.5 years. Males were more likely affected than females at 72.9%. Common presenting ocular symptoms were visual change (38.1%), periorbital pain (33.3%), and diplopia (14.3%). Common clinical exam findings were proptosis (47.6%), extraocular movement deficit (23.8%), and periorbital edema (19.0%). Twenty-seven patients (77.1%) received surgery, 22 patients (62.9%) received chemotherapy, and 30 patients (85.7%) received radiation therapy as part of their treatment. Median duration of survival was 124.0 months and 5-year overall survival (OS) was 67.1%. Hyams, Kadish, and Dulguerov T-staging showed inconsistent survival prognosis while orbital invasion and lymph node metastasis had worse outcomes. Our five cases exhibited the spectrum of disease processes evidenced above, with four involving the orbit. CONCLUSIONS: ENB is a rare sinonasal tumor that can invade the orbit. Because of its rarity, no single staging system appears superior. Resection with radiation therapy has superior survival results while the benefits of chemotherapy are currently unknown.

Occult Colon Adenocarcinoma and Multiple Myeloma Associated With Clostridium septicum Panophthalmitis With Orbital and Chiasmal Extension: A Case Report.

A previously healthy 62-year-old African American female presented with a fulminant orbital cellulitis of the right eye with diffuse scleritis and orbital inflammation extending to the optic chiasm on neuroimaging. She was taken for an emergent orbitotomy with an orbital fat biopsy and started on broad-spectrum intravenous (IV) and topical antibiotics. Within 36 hours of presentation, scleral thinning and a corneal melt ensued, ending in enucleation. Intraoperative cultures were positive for Clostridium septicum, leading to a systemic work-up exposing a previously undiagnosed colon adenocarcinoma and metastatic multiple myeloma.

Phakomatous Choristomas: A Case Report and Review of Literature.

PURPOSE: To document a case of phakomatous choristoma (PC), a rare benign periocular tumor, and to review the literature on previously reported cases. METHODS: The authors describe a case of PC and its clinical, histopathological, immunohistochemical, and radiological features, and present findings from a comprehensive review of all previously reported cases of this rare pediatric tumor. RESULTS: This case report and review highlights the benign clinical nature of PC. It typically presents at birth as a lower eyelid mass involving the orbit. Definitive diagnosis is made with hematoxylin and eosin stain showing the tumor's histological similarities to lenticular tissue. CONCLUSION: PC remains a rare entity that should be included in the differential of pediatric eyelid lesions. Surgical excision is curative, and the postoperative clinical course is unremarkable as there have been no reports of recurrence. Prompt recognition and surgical intervention may be warranted due to astigmatism and anisometropia induced by mass effect.

Congenital corneal blood staining secondary to hemorrhagic persistent fetal vasculature.

An African American girl born at 37 weeks via spontaneous vaginal delivery to a 33-year-old woman was noted on delivery to have a unilateral absent red reflex in the right eye, which was enlarged. Intraocular pressure was elevated, and the cornea had a straw-colored opacity. B-scan ultrasonography of the right eye showed diffuse hyperechoic vitreous opacities and a retrolental mass, with a hyperechoic band stretching from the optic disk to the posterior lens. Neuroimaging showed a unilateral enlarged globe, intraocular hemorrhage, and persistent fetal vasculature, with no other intracranial pathology. An anterior chamber washout revealed liquified blood; the presence of corneal blood staining was confirmed. A spontaneous intraocular hemorrhage associated with persistent fetal vasculature was suspected, leading to secondary glaucoma and corneal blood staining.

IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis.

PURPOSE: We report an uncommon case of immunoglobulin gamma 4-related ophthalmic disease (IgG4-ROD) presenting as meningitis and panuveitis. OBSERVATIONS: A 35-year-old male with no prior ophthalmic history presented with headaches, altered mental status, and fever of unknown origin. A lumbar puncture (LP) revealed an elevated white count with lymphocytic predominance, confirming a suspected meningitis. After an extensive work-up, he was discharged on oral acyclovir to cover for presumed aseptic meningitis. The patient initially improved, however, bilateral eye pain, redness, and photophobia 2 weeks after discharge prompted his first visit to the ophthalmology clinic. Exam at that time was consistent with bilateral anterior uveitis for which he was given topical prednisolone and cyclopentolate. In addition to the preceding work-up, quantitative immunoglobulin serology including IgG4 levels was added. At follow-up, he was found to have increased ocular inflammation with vitreitis, nerve head edema, and subclinical macular thickening. Visual acuity (VA) had decreased in both eyes. Serology titers for IgG had resulted in a significant elevation in IgG subclass 4 (IgG4). Optical coherence tomography (OCT) and fundus fluorescein angiography (FFA) confirmed posterior retinal involvement. The patient was diagnosed with presumed bilateral panuveitis secondary to IgG4-ROD. CONCLUSIONS AND IMPORTANCE: IgG4-RD can be a serious condition that requires careful consideration and intuition to diagnose. This report serves to encourage ophthalmologists to consider IgG4-ROD in cases of idiopathic systemic inflammation with ophthalmic involvement.

Hypertensive crisis with massive retinal and choroidal infarction: A case update.

PURPOSE: We report an update on a recently published case of uncontrolled hypertension secondary to immunoglobulin A (IgA) nephropathy resulting in massive bilateral retinal and choroidal infarction. OBSERVATIONS: In our previous report, we presented a 30-year old female with end-stage renal disease who complained of painless vision loss after many missed hemodialysis. The patient was found to be in hypertensive crisis resulting in massive retinal and choroidal infarction with severe vision loss in both eyes. The patient was treated with pan-retinal photocoagulation (PRP) with intravitreal Bevacizumab and was subsequently lost to follow-up. In this update, we report the complications that followed. After many months, she presented to clinic with a blind painful right eye. She was found to have a further decrease in vision with neovascular glaucoma in the right eye and a tractional retinal detachment in the left eye. The patient ultimately elected for enucleation of her right eye. Immunohistopathology revealed IgA deposition, confirming the presumed diagnosis of IgA nephropathy, previously unconfirmable through renal biopsy. CONCLUSIONS AND IMPORTANCE: There is a strong association between severity of retinopathy and level of kidney function. Although a rare presentation, hypertensive retinopathy is a common complication of end-stage renal disease and can be a devastating process as emphasized by this report. Those with auto-immune renal disease, such as IgA nephropathy, are at higher risk for retino-choroidal complications. It should remind all ophthalmologists and clinicians on the necessity of closer eye examinations for these patients, particularly for those with auto-immune renal disease.