Bilateral Orbital Compartment Syndrome Preceding Cerebellar Herniation in Neuropsychiatric Systemic Lupus Erythematosus.

Orbital compartment syndrome poses a significant risk for vision loss if not promptly treated. The authors report a patient with neuropsychiatric systemic lupus erythematosus presenting with bilateral orbital compartment syndrome and rapid decompensation leading to uncal and tonsillar herniation. While extremely rare, bilateral orbital compartment syndrome should prompt consideration of systemic etiologies including neuropsychiatric systemic lupus erythematosus.

Rheumatoid Arthritis-Associated Orbital Vasculitis.

A patient with a history of rheumatoid arthritis presented with bilateral orbital lesions while on abatacept. Biopsy of the lesions yielded pathologic assessment showing vasculitis with a mixed inflammatory infiltrate notable for numerous eosinophils. Despite initial improvement on cyclophosphamide therapy, she developed progression of the orbital lesions and lesions elsewhere on her body. All serologic studies were normal except for increased levels of rheumatoid factor and eosinophilia. The patient underwent a second biopsy, which was consistent with her initial pathology. The patient was started on high-dose mycophenolate mofetil treatment with a good response. After 1 year of treatment, the patient's lesions have nearly completely resolved. This patient illustrates the diagnostic and therapeutic challenges encountered when treating orbital vasculitis. Furthermore, this case shows the importance of not categorizing vasculitis with unclear etiology under the umbrella diagnosis of atypical granulomatosis with polyangiitis. This patient did not exhibit the serologic or pathologic characteristics of granulomatosis with polyangiitis, and she did not respond to treatment as would be expected for granulomatosis with polyangiitis. Maintaining an open approach to diagnosis and treatment was crucial in obtaining a favorable outcome for this patient.

Effect of mannitol on globe and orbital volumes in humans.

PURPOSE: To determine the effect of intravenous mannitol on globe and orbital volumes. METHODS: Retrospective chart review of a consecutive series of Cleveland Clinic Neurosurgical Intensive Care Unit patients who underwent computed tomographic imaging before and after intravenous mannitol administration. Volume measurements were performed according to a previously described technique by averaging axial image areas. Measurements before and after mannitol administration were compared using paired t-test. RESULTS: Fourteen patients (28 eyes) met inclusion criteria. Average globe volume decreased 186 mm3 (-2.5%, p = 0.02) after mannitol administration, while average orbital volume increased 353 mm3 (+3.5%, p = 0.04). Average globe volume change for subjects with follow-up scan less than 4.7 hours (mean 1.9 hours; range 0.2-4.5 hours) after mannitol administration was -125 mm3 (-1.7%, p = 0.24) and average orbital volume change was +458 mm3 (+5.1%, p = 0.11). Average globe volume change after mannitol administration for those with follow-up more than 4.7 hours (average 13.9 hours, range 4.9-24.7 hours) was -246 mm3 (-3.3%, p = 0.05) and orbital volume change was +248 mm3 (+2.2%, p = 0.24). Dividing the study population into groups based on mannitol dose did not yield any statistically significant change. CONCLUSIONS: Human globe volume decreases after intravenous mannitol administration, while orbital volume increases. These volume changes occur during the time period when intraocular pressure normalizes, after the pressure-lowering effects of the drug. This novel volumetric information improves our understanding of mannitol's mechanism of action and its effects on human ocular and periocular tissues.

Orbital Decompression for Thyroid Eye Disease.

Although most cases of thyroid eye disease (TED) can be managed medically, some refractory or severe cases are treated surgically with orbital decompression. Due to a lack of randomized controlled trials comparing surgical techniques for orbital decompression, none have been deemed superior. Thus, each case of TED is managed based on patient characteristics and surgeon experience. Surgical considerations include the extent of bony wall removal, the surgical approach, the choice of incision, and the use of fat decompression. Outcomes vary based on surgical indications and techniques; hence, vision can improve or worsen after the surgery.

Molecular biology and genetics of embryonic eyelid development.

The embryology of the eyelid is a complex process that includes interactions between the surface ectoderm and mesenchymal tissues. In the mouse and human, the eyelids form and fuse before birth; they open prenatally in the human and postnatally in the mouse. In the mouse, cell migration is stimulated by different growth factors such as FGF10, TGF-α, Activin B, and HB-EGF. These growth factors modulate downstream BMP4 signaling, the ERK cascade, and JNK/c-JUN. Several mechanisms, such as the Wnt/ß-catenin signaling pathway, may inhibit and regulate eyelid fusion. Eyelid opening, on the other hand, is driven by the BMP/Smad signaling system. Several human genetic disorders result from dysregulation of the above molecular pathways.

Orbital Radiation Therapy in Thyroid Eye Disease.

PURPOSE: To review the current literature summarizing the state of knowledge on the use of orbital radiation therapy (ORT) in thyroid eye disease. METHODS: A systematic review and analysis of the literature were performed. MEDLINE/PubMed, Cochrane Library databases, SCOPUS, and recent relevant journal articles were searched. RESULTS: Thyroid eye disease is an autoimmune disorder that has the propensity to affect multiple orbital tissues and can cause permanent vision loss. In moderate to severe disease, treatment may be warranted and can include multiple therapeutic modalities. Orbital radiation therapy is frequently used in this setting and may act to break the inflammatory cycle of glycosaminoglycan production and deposition. There has been a wealth of data regarding the effectiveness of ORT in thyroid eye disease resulting in the publication of treatment algorithms and management guidelines; however, there continues to be a lack of conformity among experts on the exact role of ORT in thyroid eye disease. CONCLUSION: The multiple different thyroid eye disease classification schemes and the concurrent use of other therapeutic modalities challenge the interpretation of studies evaluating the effectiveness of thyroid eye disease. Despite these limitations, the current literature indicates that ORT is generally safe and well tolerated. Orbital radiation therapy may have a modest effect on motility and proptosis early in the disease process. It is unclear whether ORT leads to improved quality of life. There are some data to support the use of ORT to prevent compressive optic neuropathy.

A case of Listeria monocytogenes endophthalmitis with recurrent inflammation and novel management.

BACKGROUND: Listeria monocytogenes is a rare cause of endogenous endophthalmitis. In the limited number of reported Listeria endophthalmitis cases, visual acuity outcomes have been very poor. FINDINGS: Here, we report a case of Listeria endophthalmitis that was complicated by recurrent inflammation. The patient required treatment with both intravitreal and long-term systemic antibiotics. An anterior chamber washout was necessary for the patient to regain 20/20 visual acuity. CONCLUSIONS: This case highlights the importance of considering Listeria early in the disease course, as it has low sensitivity to standard empiric antibiotic therapy. It also stresses the importance of addressing damaging inflammation in infectious conditions.

Rotational Flap Repair of Full Thickness Eyelid Defects without a Posterior Lamellar Graft or Flap.

BACKGROUND: To determine the safety and effectiveness of full thickness eyelid reconstructions using a semicircular rotational flap without reconstructing the posterior lamella. METHODS: The charts of all patients undergoing semicircular flap closure of full thickness eyelid defects by one surgeon (JDP) at the Cole Eye Institute between March 2000 and October 2012 were reviewed. Charts were reviewed for patient demographic information, as well as for the size of the defect, the type of flap used, length of follow-up and complications. RESULTS: Fifty eyelids of 50 patients underwent a semicircular flap repair without posterior lamellar reconstruction during the study period, and 41 charts were available for review. Average patient age was 74 years (range, 40-92 years). Average follow-up was 9.8 months (range, 1-84 months). Average defect size was 19.1 mm (range, 14-30 mm, SD 4.6). Complications included pyogenic granuloma (10 patients, 24.4%), exposure keratopathy (7 patients, 17.1%) lagophthalmos (5 patients, 12.2%), ectropion (6 patients, 14.6%), lateral canthal dystopia (2 cases, 4.9%), eyelid notch (2 cases, 4.9%) and trichiasis (4 cases, 9.8%). Two patients underwent subsequent tarsorrhaphy and one patient underwent ectropion repair. There were no cases of wound dehiscence, diplopia or fornix inadequacy, and the recruited aspect of the eyelid healed well in each case. No case required reconstruction of the eyelid margin or fornix. CONCLUSIONS: Semicircular flap repair of full thickness eyelid defects without flap or graft repair of the posterior lamella results in an adequate fornix and a low rate of secondary surgery.

Microanatomical Location of Hyaluronic Acid Gel Following Injection of the Temporal Hollows.

PURPOSE: To examine the microanatomical location of hyaluronic acid gel injected within the temporal hollows of cadaver specimens. METHODS: The temporal hollows were injected subcutaneously with hyaluronic acid gel in 6 fresh frozen human cadaver hemifaces. Temporal soft tissues were dissected to a preperiosteal plane and fixated in 95% alcohol. A soft tissue section extending from skin to temporal bone was obtained for each specimen. Histologic examination was performed using hematoxylin and eosin stain. RESULTS: In 5 of 6 specimens, at least 95% of the hyaluronic acid was located within the subcutaneous fat. In 1 of 6 specimens, approximately 35% of the material was located within the subcutaneous fat and 60% was located within the superficial temporal fascia. Two specimens had 5% located within the temporalis muscle. In 1 specimen, hyaluronic acid was found to encompass a superficial muscular artery within the superficial temporal fascia. CONCLUSIONS: This study elucidates the location of hyaluronic acid gel after subcutaneous injection within the temporal hollow. Histology confirmed consistent placement of the gel within the subcutaneous tissues, but it also showed that injection in this region may produce unintended deeper location of filler, and a significant perivascular collection of the material. The proximity of dense temporal fascial and muscle arterial networks in this region may pose risk for perivascular injection and associated complications.

Differentiation of high lipid content from low lipid content adrenal lesions using single-source rapid kilovolt (peak)-switching dual-energy multidetector CT.

PURPOSE: This study aimed to determine if a single-source rapid kilovolt (peak)-switching dual-energy (RSDE) multidetector computed tomography (CT) can differentiate high lipid content (HLC) from low lipid content (LLC) incidental adrenal lesions. METHODS: A retrospective intrapatient study of 40 consecutive adults with known hepatic or pancreatic pathology who underwent multiphasic abdominal RSDE for nonadrenal-related clinical indications and had adrenal lesions was done. Arterial phase was acquired with RSDE, conventional unenhanced (CU) images with standard MDCT. RSDE measurements included lesion attenuation in Hounsfield units on simulated monochromatic 140-keV images and density (in milligrams per milliliter) on material decomposition images, using water-iodine and fat-iodine basis pairs. Each variable was correlated with CU Hounsfield units (Pearson coefficient). RSDE lesion values were compared with analysis of variance and Tukey HSD test. Receiver operating characteristic (ROC) analysis was performed to identify RSDE thresholds comparable to 10 HU on unenhanced MDCT. RESULTS: Twenty-nine HLC and 18 LLC lesions were evaluated in 40 subjects (21 men; mean age, 66.5 years). RSDE variables correlated with CU Hounsfield units, r = 0.90-0.92, P < 0.001. Myelolipomas, HLC, and LLC lesions were different by analysis of variance, P < 0.001 for all dual-energy variables. Excluding myelolipomas from ROC curve analysis, ROC areas for Hounsfield unit 140-keV images, fat(-iodine), and water(-iodine) were 0.929 (0.039), 0.917 (0.046), and 0.912 (0.048), respectively (P < 0.001); using a specificity of 94.4%, 64% of adenomatous lesions had 140 keV values of less than 9.5 HU, 59% had fat(-iodine) values of less than 987 mg/mL, and 50% had water(-iodine) values of less than 994 mg/mL. CONCLUSIONS: There is a strong correlation between RSDE measures and accepted MDCT attenuation values for HLC and LLC adrenal lesions. In some patients undergoing postcontrast RSDE who are found to have incidental adrenal nodules, further unenhanced CT or adrenal-protocol CT or magnetic resonance imaging may not be necessary.