Diffuse choroidal hemangioma in pregnancy: Symptomatic diffuse choroidal hemangioma spontaneously resolving postpartum.

We present the case of a pregnant young woman with symptomatic diffuse choroidal hemangioma (CH), diagnosed with Sturge-Weber syndrome (SWS). Three months postpartum, there was full spontaneous resolution of the subretinal fluid and improvement in the visual acuity (VA). A 29-year-old, 31-week pregnant female with a coagulation disorder and enoxaparin (clexane) treatment, complained of a left visual disturbance of 2 weeks duration. On examination, the right eye was normal; left eye VA was 6/12 (20/40) with a left eyelid port-wine stain and on funduscopic examination there was a serous retinal detachment. On ultrasound, there was diffuse thickening of the choroid with a focus superior and nasal to the disc with active vascularization - a diagnosis of diffuse CH was made. Magnetic resonance imaging (MRI) and MR venography studies of the brain and orbit revealed engorged orbital vasculature, and the diagnosis of SWS was made. Three months after Cesarean section with birth of a healthy baby boy, VA returned to 6/9 with full resorption of subretinal fluid. On fundoscopic exam, there were no retinal or vein occlutions but there were tortuous blood vessels with A-V crossing. This is a rare case of newly diagnosed SWS with symptomatic diffuse CH in pregnancy. Postpartum, there was full resolution of the subretinal fluid. A watchful waiting approach can be considered in such cases.

Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence.

Adult onset xanthogranulomatous disease is a rare orbital disease. IgG4-related disease is a systemic disease that can often manifest in the orbit. In this communication, we present the case of a patient with a xanthelasma-like lesion on the upper eyelid, and an enlarged lacrimal gland, which on biopsy was diagnosed as an orbital xanthogranuloma. Detailed serological workup showed that the patient was found to have elevated serum IgG4 levels. The orbital specimen was re-stained and found to be positive for IgG4. The patient was treated with oral steroids with partial resolution of the lesion. This is an interesting case of both histopathological adult onset xanthogranuloma (AOX) and IgG4-related orbital disease (IgG4-ROD). The early evidence suggests that the diagnosis of one of these disorders should point the physician to investigate for the presence of the other, especially if xanthogranulomatous disease is diagnosed first.

Masquerading solitary plasmacytoma; an eyelid lump in disguise.

Solitary plasmacytoma is a rare orbital lesion, most commonly appearing in patients with multiple myeloma. We report a case of a 75-year-old woman who presented with a left upper eyelid lesion, initially misdiagnosed and treated as a chalazion. Histopathological testing revealed plasmacytoma originating from the frontal sinus. This case demonstrates a rare presentation for this malignancy.

Telemedicine comes of age during coronavirus disease 2019 (COVID-19): An international survey of oculoplastic surgeons.

PURPOSE: The Coronavirus disease 2019 (COVID-19) pandemic is an ongoing healthcare crisis that continues its worldwide spread. Ophthalmologists are at high risk of acquiring and transmitting the virus. Telemedicine platforms have evolved and may play an important role in attenuating this risk. For patients, these platforms provide the possibility of clinic consultation without the concerns of a clinic visit. We aimed to assess the utilization of telemedicine by oculoplastics specialists worldwide during the COVID-19 pandemic. METHODS: A 13-item survey was distributed internationally to practicing oculoplastic surgeons. Collected data included demographics, clinical practice variables and perceptions regarding telemedicine. Significance of associations and single survey items was evaluated by Chi-squared and z-score of proportions tests, respectively. RESULTS: The questionnaire was completed by 70 oculoplastic surgeons (54.3% male, mean age 47.3 years, median experience 10 years) from eight countries, practicing in various clinical settings (50.0% hospitals, 45.7% private clinics, 4.3% community clinics). Most respondents reported telemedicine to be an effective tool for oculoplastic consultations (67.1%, p = 0.004), while only 12.8% (p < 0.00001) had incorporated this modality into clinical practice prior to the pandemic. Even though a vast majority (98.6%) of participants had limited outpatient activity, most (55.7%) felt unprotected from the virus. Telemedicine had been incorporated by 70.5% (p = 0.001) of respondents during the COVID-19 pandemic, whereas most (57.1%) predicted continued use of the modality. CONCLUSION: Telemedicine can be effectively and rapidly incorporated into the clinical practice of oculoplastic surgeons during the COVID-19 pandemic. Further research into the most effective utilization of these platforms appears warranted.

The impact of IgG-4-ROD on the diagnosis of orbital tumors: A retrospective analysis.

This study was to determine the prevalence of immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD) among patients who have previously undergone biopsy and were diagnosed to have idiopathic orbital inflammatory disease (IOID) or orbital lymphoproliferative disease (OLD), namely, lymphoma and benign reactive lymphoid hyperplasia (BRLH). This is a retrospective cross-sectional study. The charts and slides of all patients who underwent biopsies and were histopathologically diagnosed to have either IOID or OLD were reviewed. Demographics, clinical features, initial histopathological diagnoses, treatment received, and final outcome were noted. Using the diagnostic criteria for diagnosis for IgG4 disease, those cases that would classify as "possible IgG4-related disease (IgG4-RD)" were reviewed, reclassified, and reassigned a diagnosis of IgG4-ROD. We reviewed 105 patients' clinical charts. Of these 105 patients, upon reviewing the histopathology, 18 (17.15%) patients were found to fit the diagnostic criteria for possible IgG4-ROD. Of these 18 patients who were now reassigned the diagnosis of IgG4-ROD, the most common previous histopathological diagnosis was found to be IOID, for eight patients (44%), then BRLH, which was noted in five patients (27.8%), followed by lymphoma, which was noted in two patients (11.1%). Previously diagnosed cases of IOID and OLD were found to fulfill the criteria for IgG4-ROD. Given the advent of recent diagnostic and histopathological techniques, all cases of suspected IOID and OLD should be screened for IgG4-ROD and all previously diagnosed cases must be closely followed up, given the systemic implication of IgG4-RD. Histopathological reassessment of previously diagnosed cases may be considered.

Extramedullary plasmacytoma of the lateral rectus muscle.

Plasmacytoma is an uncommon presentation of plasma cell malignancy, especially in the absence of multiple myeloma. Orbital plasmacytomas generally originate from bone, although few cases in the literature report orbital extramedullary plasmacytomas. We present the case of a 68-year-old man found to have a solitary extramedullary plasmacytoma in the lateral rectus muscle without further evidence of multiple myeloma. This case demonstrates a rare presentation for such a malignancy, and a review of the literature highlights the importance of proper workup and close monitoring to rule out multiple myeloma to guide management.

Dacryolithiasis: A Review.

PURPOSE: To review and summarize the clinical features, presentations, diagnostic modalities and management of dacryolithiasis. METHODS: A comprehensive PubMed search of all English articles on dacryolithiasis was reviewed. Although this review primarily relied on articles written in English, non-English-language articles that had abstracts translated into English were also reviewed. Data reviewed included epidemiology, pathogenesis, appearance and composition, clinical features, presentations, diagnostic modalities, management of dacryolithiasis and the implications of incidental dacryoliths found during lacrimal surgery. RESULTS: Although an unknown proportion of dacryolithiasis cases may remain asymptomatic; epiphora, acute and/or recurrent dacryocystitis, punctal discharge, and localized swelling are the most common presenting features of dacryolithiasis. It may also present as partial nasolacrimal duct obstruction. Dacryoliths exhibit a variety in external appearances. While some minimally invasive techniques for the removal of dacryoliths have been described, dacryocystorhinostomy with removal of the dacryoliths remain the most effective treatment in cases of symptomatic dacryolithiasis. The expression and production of certain proteins and peptides, namely those of the trifoil factor family play a significant role in the pathogenesis of dacryoliths. CONCLUSIONS: The management of dacryolithiasis is driven by the goal of resolution of secondary obstruction and/or inflammation. Although a large number of dacryoliths are incidentally found during dacryocystorhinostomy, certain clinical features such as unilateral sac distension, particularly those with a palpable firm medial canthal mass, might lead one to have a high index of suspicion. It remains unclear if the incidental finding of a dacryolith during a dacryocystorhinostomy has a favorable prognostic value.

Surgeon point-of-view recording: Using a high-definition head-mounted video camera in the operating room.

OBJECTIVE: To study the utility of a commercially available small, portable ultra-high definition (HD) camera (GoPro Hero 4) for intraoperative recording. METHODS: A head mount was used to fix the camera on the operating surgeon's head. Due care was taken to protect the patient's identity. The recorded video was subsequently edited and used as a teaching tool. This retrospective, noncomparative study was conducted at three tertiary eye care centers. The surgeries recorded were ptosis correction, ectropion correction, dacryocystorhinostomy, angular dermoid excision, enucleation, blepharoplasty and lid tear repair surgery (one each). The recorded videos were reviewed, edited, and checked for clarity, resolution, and reproducibility. RESULTS: The recorded videos were found to be high quality, which allowed for zooming and visualization of the surgical anatomy clearly. Minimal distortion is a drawback that can be effectively addressed during postproduction. The camera, owing to its lightweight and small size, can be mounted on the surgeon's head, thus offering a unique surgeon point-of-view. In our experience, the results were of good quality and reproducible. CONCLUSIONS: A head-mounted ultra-HD video recording system is a cheap, high quality, and unobtrusive technique to record surgery and can be a useful teaching tool in external facial and ophthalmic plastic surgery.

Effects of growth hormone therapy in children after cardiac transplantation.

BACKGROUND: Growth hormone (GH) is used to treat growth failure in children with GH deficiency. The safety and efficacy of GH after pediatric cardiac transplantation is not known. The objective of this study was to evaluate growth and cardiovascular effects of GH in children with growth failure after cardiac transplantation. METHODS: Pediatric cardiac transplant recipients who received GH from 1994 to 2004 were evaluated. Growth, cardiac function, hemodynamics and rejection frequency were serially monitored for 2 years before, during and after GH. Eight age-matched heart transplant recipients undergoing a natural growth spurt were evaluated as controls. RESULTS: The mean age of subjects at initiation of GH was 13 +/- 3 years (mean duration 2.5 +/- 1 years, n = 10), of whom 3 were GH-deficient. Growth velocity (GV) increased from 2.5 +/- 2 to 8.6 +/- 3 cm/year with GH. There was an increase in left ventricular (LV) shortening fraction (SF; 37 +/- 1% to 41 +/- 1%), LV mass (93 +/- 11 to 118 +/- 15 g/m2), LV volume (138 +/- 14 to 188 +/- 21 ml/m2) and cardiac index (3.1 +/- 0.7 to 4.1 +/- 0.5 liters/min/m2) during GH therapy (p < 0.05). After discontinuation of GH, SF, cardiac index and LV mass returned to normal, but LV volume did not. In control patients, LV volume increased without an increase in SF or mass. Rejection frequency did not change in either group. There were no adverse events related to GH. CONCLUSIONS: GH is safe and effective in treating growth failure in children after cardiac transplantation. GH therapy is associated with an increase in LV mass, volume and cardiac output. These changes are partially reversible after discontinuation of GH. The mechanisms and long-term consequences of these changes require further investigation.