Inflammatory Myofibroblastic Tumor of the Orbit in a 5-Month-Old Infant.

Inflammatory myofibroblastic tumors are mesenchymal neoplasms composed of spindle cells and inflammatory infiltrate. The authors describe a 5-month-old infant with orbital inflammatory myofibroblastic tumor, the youngest patient currently reported in the literature. The histo-pathology, orbital apex location, and patient's age led to a chemotherapy-driven treatment using crizotinib with near-complete resolution of the tumor. [J Pediatr Ophthalmol Strabismus. 2022;59(2):e25-e28.].

Bilateral optic nerve compression secondary to skull hyperostosis from vitamin A deficiency.

A 15-year-old boy with a history of autism spectrum disorder presented with bilateral progressive blurry vision and nyctalopia. Initial examinations, including optical coherence tomography scans of the macula and optic nerve, were within normal limits. Subsequent examination revealed trace pallor of the right optic nerve. Computed tomography and magnetic resonance imaging of the brain demonstrated diffuse thickening of calvarial bone with bilateral optic nerve compression. Laboratory evaluation was notable for profound vitamin A deficiency. The patient underwent optic nerve decompression and vitamin A supplementation with postoperative improvement in visual acuity of both eyes.

Experience of a non-pediatric-trained ophthalmic hospitalist at a children's hospital.

The relatively novel role of ophthalmic hospitalist at our institution includes evaluation and management of all inpatient and emergency department ophthalmology consultations. This report reviews the management of consultations at a children's hospital by the non-pediatric-trained ophthalmologist in this role. Within the study period of 2 months, 61 pediatric patients were evaluated by the ophthalmic hospitalist service. The pediatric ophthalmology department was consulted in 15 cases (25%), 10 of which were for discussion alone. This model of care illustrates an effective way to provide an ophthalmology consult service at a children's hospital in conjunction with pediatric ophthalmology subspecialists for management.

Management of ocular manifestations of autosomal recessive congenital ichthyosis 4B, harlequin type, in the perinatal period.

Autosomal recessive congenital ichthyosis (ARCI4B [OMIM #242500]), also known as harlequin ichthyosis, presents at birth with extreme hyperkeratosis and thick-fissured plaques, leading to tightness of the skin around the eyes, mouth, ears, chest, abdomen, and extremities. Ocular manifestations include cicatricial ectropion and exposure keratitis. We present 2 infants with ARCI4B and cicatricial ectropion who were managed with aggressive nonsurgical therapy. Both infants avoided severe ocular sequelae and maintained corneal clarity, highlighting that management of exposure keratopathy with frequent ophthalmic ointment application can prevent severe ocular surface pathology in ARCI4B.

Congenital lymphocytic choriomeningitis virus-an underdiagnosed fetal teratogen.

Congenital lymphocytic choriomeningitis virus (LCMV) infection is associated with high mortality and morbidity. Although the number of cases reported in the literature has been increasing, it might still be clinically an underdiagnosed human fetal teratogen. We report 2 more cases of serologically proven congenital LCMV infection. One case presented with Aicardi-like syndrome features. Since congenital LCMV infection may mimic Aicardi syndrome, serologic testing should be considered in the workup of patients with Aicardi syndrome to rule out LCMV infection.

Conjunctival melanoma: outcomes based on tumor origin in 382 consecutive cases.

PURPOSE: To evaluate prognostic factors based on origin of conjunctival melanoma. DESIGN: Interventional case series. PARTICIPANTS: Three hundred eighty-two consecutive patients. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: Melanoma-related metastasis and death. RESULTS: The melanoma arose from primary acquired melanosis (PAM; n = 284; 74%), from pre-existing nevus (n = 26; 7%), and de novo (n = 72; 19%). The mean tumor base was 11 mm for melanoma arising from PAM, 6 mm for melanoma arising from nevus, and 10 mm for those arising de novo. At 5 years (10 years), melanoma metastasis occurred in 19% (25%) in melanoma arising from PAM (P = 0.003), 10% (26%) in melanoma from nevus (P = 0.193), and 35% (49%) in those de novo. Factors predictive of metastasis by multivariable analysis included tumor origin de novo (P = 0.001), palpebral location (P<0.001), nodular tumor (P = 0.005), and orbital invasion (P = 0.022). At 5 years (10 years), melanoma-related death occurred in 5% (9%) in melanoma arising from PAM (P<0.001), 0% (9%) in melanoma arising from nevus (P<0.057), and 17% (35%) in those arising de novo. Factors predictive of death by multivariable analysis included tumor origin de novo (P<0.001), fornix location (P = 0.04), and nodular tumor (P = 0.001). CONCLUSIONS: Melanoma arising de novo carries a higher risk of melanoma-related metastasis and death compared with those cases arising from PAM or nevus.

Regulated translation of listeriolysin O controls virulence of Listeria monocytogenes.

Listeria monocytogenes is an intracytosolic bacterial pathogen that escapes from the phagosome using a secreted cytolysin, listeriolysin O (LLO). In the host cytosol, LLO activity is minimized to prevent pore formation in the host plasma membrane; premature lysis of the infected host cell exposes the bacteria to extracellular immune defences of the host and is detrimental to infection. Here we identified nucleotide substitutions in the coding sequence of the LLO gene (hly) that did not alter the protein sequence, yet caused over-production of LLO, cytotoxicity and loss of virulence. These phenotypes were independent of the promoter and, under conditions in which the mutants produced more LLO protein than wild type, levels of hly mRNA were similar. Finally, negative regulation of LLO was maintained even when bacteria were engineered to produce elevated levels of the wild-type hly transcript. Together, our data demonstrate that translational regulation of LLO is critical for L. monocytogenes pathogenesis.