Recalcitrant Herpes Zoster Ophthalmicus in a Patient Discovered to Have Underlying Functional Natural Killer Cell Deficiency.

PURPOSE: To report the management of a patient with chronic herpes zoster ophthalmicus with previously undiagnosed natural killer cell deficiency. METHODS: A 50-year-old female presented with herpes zoster ophthalmicus that despite appropriate treatment progressed to uveitis and expansion of the lesions. Multiple attempts to wean the patient off therapeutic anti-viral medications continued to result in reoccurrence of the disease. Discovery of underlying natural killer cell deficiency prompted indefinite treatment with therapeutic anti-viral medication. RESULTS: After multiple flares of herpes zoster ophthalmicus over 3 years, seven prior to the discovery of the immunodeficiency, she developed unilateral corneal scarring on the affected side. At this time, her visual acuity was measured to be uncorrected at 20/150 with improvement to 20/25-1 with refraction. CONCLUSIONS: This case highlights the need for further immunological investigation and unconventional medical management with recurrent viral infections to prevent visual morbidity.

Spontaneous hyphema associated with ocular syphilis.

The purpose of this case series is to illustrate a novel presentation of ocular syphilis. Two cases of ocular syphilis presenting with spontaneous hyphema are described, demonstrating that spontaneous hyphema can be caused by ocular syphilis. This association has not previously been reported.

The link between colon cancer and congenital hypertrophy of the retinal pigment epithelium (CHRPE).

PURPOSE: Clarify the differences between Familial Adenomatous Polyposis (FAP)-associated Congenital Hypertrophy of the Retinal Epithelium (CHRPE) and benign variants with regards to lesion characteristics and associated risk. OBSERVATIONS: An eighteen-year-old man with no past medical history was found to have multiple lesions in both eyes that were consistent with FAP-associated CHRPE. Although family history was negative for colon cancer, a colonoscopy was performed, and hundreds of polyps were found extending from the rectum to the distal colon with pathological findings of tubular adenoma. Genetic testing was consistent with a possible de novo Adenomatous Polyposis Coli (APC) mutation. CONCLUSIONS: FAP is an autosomal dominant syndrome that causes colorectal cancer by age thirty-five in ninety-five percent of cases. There has been no established relationship between the benign variants of CHRPE and FAP, and patients with benign variants have no increased risk of colon cancer. While the lack of distinction in nomenclature and similar lesion appearance often leads to misdiagnosis and overtreatment, there are distinct ocular exam features that can provide the correct diagnosis. The exam findings that distinguish FAP-associated CHRPE lesions are (1) bilateralism, (2) occurrence in multiple quadrants, (3) pisiform shape, and (4) irregular borders. Knowing these features can be of great aid, especially in the setting of suspected de novo Familial Adenomatous Polyposis.

Intrinsic and Extrinsic Cardiac Pseudotumors: Echocardiographic Evaluation and Review of the Literature.

Echocardiography is the most common imaging modality for the assessment of cardiovascular tumors, followed by more advanced imaging modalities, such as cardiac computed tomography or cardiac magnetic resonance imaging. Non-neoplastic lesions that may simulate a true neoplasm on imaging are termed "cardiac pseudotumors." As echocardiography is the initial imaging modality where pseudotumors are identified, it is imperative to have a fundamental understanding of pseudotumors evaluation using echocardiography. There is paucity of the literature describing the different kinds of pseudotumors. This review is an attempt to describe common cardiac pseudotumors and to classify them based on their origin. The tumors arising from cardiac structures, such as epicardium, endocardium, or myocardium, were termed as "intrinsic" while the pseudotumors with no cardiac origin were termed as "extrinsic." The more common pseudotumors are described in detail with pertinent echocardiographic features and examples.

Electrolyte and hemodynamic changes following percutaneous left atrial appendage ligation with the LARIAT device.

PURPOSE: The left atrial appendage (LAA) is a well-known source of atrial natriuretic peptide (ANP) and therefore plays an important role in homeostasis. The neurohormonal impact of epicardial exclusion of the LAA with the LARIAT procedure is unknown. In this proof-of-concept study, we postulated that LAA exclusion would impact homeostasis as evidenced by changes in electrolytes and blood pressure (BP). METHODS: A total of 76 patients who underwent successful LAA exclusion were enrolled in this retrospective observational study utilizing a prospective registry. Electrolytes, BP, and heart rate (HR) were monitored before LARIAT and post-LARIAT (24 and 72 h and 6 months). RESULTS: There was a significant reduction of systolic BP (mmHg) at 24 h (113.3 ± 16.0; p < 0.0001) and 72 h (119.0 ± 18.4 mmHg; p < 0.0001) post-LARIAT when compared with pre-LARIAT BP (138.2 ± 21.3). The reduction in systolic BP persisted at 6-month follow-up (128.8 ± 17.3; p = 0.0005). There was significant reduction in serum sodium (mmol/L) at 24 h (135.4 ± 3.6; p < 0.0001) and 72 h (136.3 ± 3.7; p < 0.001) post-LARIAT when compared to pre-LARIAT (138.7 ± 3.2). The reduction in sodium was not persistent at 6-month follow-up (138.4 ± 3.3; p = 0.453). CONCLUSIONS: LAA exclusion results in an early and persistent decrease in systolic BP. Additionally, there is an early decline in serum sodium, which normalizes at long-term follow-up. The underlying mechanism leading to these changes is not entirely clear; however, it is likely related to neurohormonal changes post LAA exclusion.