Sarcoid-like reaction of the orbit in diffuse large B-cell lymphoma.

Sarcoid-like reaction (SLR) has been reported in patients with solid tumor malignancies, lymphomas, and patients receiving immunotherapy. SLR is often incidentally found during positron emission tomography/computed tomography scans as hilar and/or mediastinal lymphadenopathy. SLR has also been found in the lung, spleen, bone marrow, and skin. Biopsy of these lesions shows noncaseating granulomas. When systemic criteria are not met for sarcoidosis, these noncaseating granulomas are termed SLR. We present the first case in the literature of a case of orbital SLR in a patient with concomitant diffuse large B-cell lymphoma and inverted papilloma of the maxillary sinus. This case highlights the importance of including malignancy in the differential for the presence of a noncaseating granuloma in the orbit.

A Case of Endophthalmitis After Bilateral Medial Rectus Recession.

A 9-month-old male infant with a history of Down syndrome underwent bilateral medial rectus recession. Two weeks postoperatively, he developed leukocoria of the left eye with a white opacity posterior to the lens, numerous undulations, necrosis, and hemorrhages in the retrolental space. His contralateral eye had white retinal lesions nasally. Ultrasound examination of the left eye showed a funnel retinal detachment, loculated debris, and a small, central, hyperechoic area concerning for calcification. He underwent enucleation because retinoblastoma could not be definitively ruled out. Given this patient's low visual potential, enucleation was a definitive and safe treatment option. Ocular pathology showed significant inflammation and necrosis. There was an area of scleral perforation by a suture adherent to the retina and vitreous, surrounded by inflammatory cells. This case uniquely demonstrates pediatric endophthalmitis following strabismus surgery, secondary to scleral perforation confirmed by histopathological analysis. Care must be taken during scleral passage of sutures to prevent inadvertent scleral perforation and the potential complication of endophthalmitis. [J Pediatr Ophthalmol Strabismus. 2017;54:e37-e41.].

Conjunctiva-Sparing Posterior Ptosis Surgery: A Novel Approach.

PURPOSE: Müller's muscle conjunctiva resection is a well-established means of correcting blepharoptosis. The primary objective of this prospective study was to determine if modifying Müller's muscle conjunctiva resection by sparing the conjunctiva would accomplish successful repair while allowing patients to retain their conjunctiva. The potential advantages of preserving conjunctiva are significant and include: conservation of an anatomically normal tissue, retention of goblet cells, reduction of suture-related complications such as corneal irritation or abrasion as the conjunctiva covers the suture during postoperative healing, and preservation of conjunctiva for potential future surgical procedures critical to ocular health. METHODS: This is a prospective study enrolling patients with mild to moderate ptosis. RESULTS: The procedure has been performed in 18 patients and 30 eyelids. Follow up at 1 week revealed normal healing with expected postoperative edema and ecchymosis. Notably, all patients were free of any corneal abrasion or irritation. Follow up at 1 month and a minimum of 3 months (average 5.7 months) revealed that all patients had satisfactory correction of their blepharoptosis with Margin Reflex Distance 1 (MRD1) improvement to within 1 mm of the anticipated goal, preserved eyelid margin contour, and good symmetry (average 0.26 mm difference in MRD1 between the sides). CONCLUSION: These results show an effective correction of blepharoptosis with a favorable cosmetic outcome and low postoperative complication rate; all while preserving the conjunctiva.

Relapsing acute lymphoblastic leukemia presenting with a rapidly enlarging and vision-threatening orbital mass.

This case report describes a patient with known acute lymphoblastic leukemia (ALL) presenting with a rapidly enlarging and vision-threatening orbital mass. Orbital disease is rare in patients with ALL, and to our knowledge only six cases have been previously described, five of which are in children. We describe a 36-year-old Caucasian female with known acute lymphoblastic leukemia who developed markedly decreased visual acuity, proptosis, and elevated intraocular pressure over the course of 12 h. She was treated with emergent surgical intervention followed by intrathecal chemotherapy and intravenous steroids. Following medical and surgical intervention, the patient demonstrated rapid resolution of symptoms and a return to baseline visual acuity. The initial presentation of acute onset proptosis with optic nerve compromise can be suggestive of infectious etiologies, however, this case suggests caution in evaluation of patients with known systemic malignancy, particularly ALL, as early intervention with systemic steroids and surgery may result in return of visual function.

Orbital liposarcoma masquerading as a hemangioma.

Liposarcomas are the most common soft tissue sarcomas in adulthood, comprising approximately 20% of all sarcomas; most present in the extremities and retroperitoneum (1). Despite the large amount of adipose tissue in the orbit, orbital liposarcomas are rare. Diagnosis can be challenging due to the rarity of the entity and the pathological similarity to benign adipose tumors (2, 3). Fortunately, the advent of immunohistochemical staining has aided in diagnosis. Management of orbital liposarcoma also can be challenging, as illustrated by the case described herein (4).

Time-dependent degenerative transformations in the lipidome of chalazia.

The aim of this prospective study was to conduct histopathologic and lipidomic analyses of chalazia, in order to evaluate time-dependent changes in the lesion. Samples of surgically excised chalazia were collected over a period of 12 months from 10 patients (mean age 41 years; range, 23-58) with clinically diagnosed chalazia, who underwent scheduled surgery. The ages of chalazia varied from 2 to 28 weeks. To confirm the clinical diagnoses, the morphology of collected tissue samples was evaluated histologically after hematoxylin and eosin staining. The lipids from individual chalazia were analyzed by high-performance liquid chromatography-mass spectrometry and compared with authentic lipid standards and with the lipids of meibum collected from normal controls. We observed gradual, lesion age-dependent transformation of the lipidome of chalazia from an almost normal meibum-like composition to a very different kind of lipidome. A rapid initial increase in the free cholesterol content was followed by a gradual replacement of extremely long chain meibomian-type lipids with a mixture of shorter-chain cholesteryl esters of the C14-C18 family, triacylglycerols, ceramides, phospholipids and sphingomyelins. In addition, a rapid disappearance of wax esters and cholesteryl esters of (1-O)-acyl-omega-hydroxy fatty acids from the lipidome of aging chalazia was observed. Our results are indicative of dramatic, time-dependent changes in the lesion that may involve cholesterol as a trigger and/or a marker of subsequent degeneration of the meibomian lipidome. We hypothesize that early inhibition of these transformations may be useful in reversing the course of the disease.

Fetal akinesia deformation sequence and neuroaxonal dystrophy without PLA2G6 mutation.

We present autopsy findings of a stillborn female infant at 20 to 21 weeks' gestation with neuroaxonal dystrophy. External examination showed features of fetal akinesia deformation sequence. Internal examination showed hypoplasia of the cerebellum, corpus callosum, and optic nerves, as well as nuclear cataracts. Light and electron microscopic examinations showed widespread axonal spheroids in the central and peripheral nervous systems. Gene sequencing failed to reveal PLA2G6 mutations, indicating that fetal neuroaxonal dystrophy presenting as fetal akinesia deformation sequence is genetically distinct from infantile neuroaxonal dystrophy and related disorders. In addition, placental examination showed α-fetoprotein-positive, eosinophilic, globular inclusions in the cytoplasm of a few villous macrophages. The significance of this novel histologic finding is unclear.

Avellino corneal dystrophy worsening after laser in situ keratomileusis: further clinicopathologic observations and proposed pathogenesis.

PURPOSE: To study the nature of the deposits in Avellino corneal dystrophy (ACD) worsening after laser in situ keratomileusis (LASIK), and suggest a mechanism for histopathogenesis. DESIGN: Interventional case report. METHODS: A 28-year-old woman previously diagnosed with bilateral ACD underwent bilateral LASIK. The corneal dystrophy progressively worsened bilaterally, one year later. A penetrating keratoplasty was subsequently performed on the right eye at 31 years of age, and in the left eye a year later. The clinical and histopathologic findings of the corneal graft of the right eye were reported in the literature, with positivity to the Masson trichrome stain, negative staining with Congo red, and heterozygosity for the Arg124His mutation by serum DNA studies. Histopathologic studies of the corneal graft of the left eye were conducted at the University of Texas Southwestern Medical Center. RESULTS: Histopathologic examination of the excised cornea showed the Masson trichrome positive deposits present from underneath the Bowman layer to the LASIK interface, with absence of deposits posterior to the latter. In contrast to the prior report describing findings in the corneal graft of the left eye, the deposits stained lightly with Congo red, but failed to show birefringence under polarized light, or fluorescence with thioflavin T. CONCLUSION: Accelerated deposits developing after LASIK in ACD eyes seem to harbor pre-amyloid features. The epithelium is likely to be the culprit, in a pathway independent of with human transforming growth hormone beta (TGF-beta), with deposits developing in the anterior stroma and the stromal interface.

Autophagy gene-dependent clearance of apoptotic cells during embryonic development.

Autophagy is commonly observed in metazoan organisms during programmed cell death (PCD), but its function in dying cells has been unclear. We studied the role of autophagy in embryonic cavitation, the earliest PCD process in mammalian development. Embryoid bodies (EBs) derived from cells lacking the autophagy genes, atg5 or beclin 1, fail to cavitate. This defect is due to persistence of cell corpses, rather than impairment of PCD. Dying cells in autophagy gene null EBs fail to express the "eat-me" signal, phosphatidylserine exposure, and secrete lower levels of the "come-get-me" signal, lysophosphatidylcholine. These defects are associated with low levels of cellular ATP and are reversed by treatment with the metabolic substrate, methylpyruvate. Moreover, mice lacking atg5 display a defect in apoptotic corpse engulfment during embryonic development. We conclude that autophagy contributes to dead-cell clearance during PCD by a mechanism that likely involves the generation of energy-dependent engulfment signals.

Secondary glaucoma associated with advanced acanthamoeba keratitis.

PURPOSE: To describe the association of acanthamoeba keratitis and glaucoma, to establish an incidence of glaucoma in patients with acanthamoeba keratitis, to discuss treatment options and outcomes in these patients, and to describe the histopathologic findings and pathogenesis of glaucoma secondary to acanthamoeba keratitis. METHODS: After Institutional Review Board approval, the charts of all patients suspected of having acanthamoeba keratitis at Aston Ambulatory Center at The University of Texas Southwestern Medical Center were reviewed. Inclusion criteria were as follows: diagnosis of acanthamoeba keratitis by positive confocal microscopy or culture, diagnosis of glaucoma or ocular hypertension secondary to acanthamoeba keratitis, and at least 6 months of follow-up. Exclusion criteria included a previous diagnosis of glaucoma or ocular hypertension and any history of intraocular surgery before the development of glaucoma. The date of keratitis development, pneumotonometry on initial and follow-up examinations, glaucoma medications used, and surgical procedures performed were tabulated. RESULTS: Twenty patients (20 eyes) were included. Six (30%) eyes developed secondary glaucoma during the review period. Of the patients treated for glaucoma with medication alone, the visual acuity of three (75%) of four became light perception or no light perception. Three of six patients required glaucoma drainage device implantation for intraocular pressure control. Of these, the vision of one eye became no light perception, and the other two eyes maintained better than 20/100 vision. Histopathologic examination showed chronic inflammation of the trabecular meshwork and angle closure. No acanthamoeba organisms were found in the angle structures. CONCLUSIONS: The development of secondary glaucoma is not uncommon in acanthamoeba keratitis and is a poor prognostic sign in patients with acanthamoeba keratitis, because most progress to light perception or no light perception vision. Histopathologic findings suggest an inflammatory angle-closure mechanism, apparently without direct infiltration of the organism. The glaucoma associated with acanthamoeba keratitis is often severe and frequently requires surgical intervention for intraocular pressure control and vision preservation.