Adams-Oliver syndrome associated with refractory glaucoma.

Adams-Oliver syndrome (AOS) is a rare inherited disorder characterized by aplasia cutis congenita, cutis marmorata telangiectatica congenita, and terminal limb defects. Ocular associations have been rarely reported. We report a 6-month-old boy with AOS associated with refractory glaucoma, megalocornea, and anterior polar cataract. To our knowledge, this is the first case of glaucoma to be reported in association with AOS.

Glaucoma in Uveitic Eyes: Long-Term Clinical Course and Management Measures.

BACKGROUND: The management of glaucoma in uveitis is challenging. A meticulous combination of anti-glaucoma and anti-inflammatory agents is often needed to control the intraocular pressure (IOP) and preserve the visual status in an otherwise blinding disease. AIM: To study the clinical course and management of glaucoma in uveitic eyes. METHODS: A retrospective study analyzing the case records of patients over 12 years, referred for the management of uveitic glaucoma in the last two decades. RESULTS: The analysis of 582 uveitic glaucoma eyes of 389 patients was done, and the mean IOP at baseline was 25.89 (±13.1) mmHg. Non-granulomatous uveitis (102 eyes) was the most common diagnosis. Granulomatous uveitis was the most common diagnosis among the treatment failure eyes and in eyes requiring more than one surgical intervention for glaucoma. CONCLUSION: An appropriate and adequate combination of anti-inflammatory and IOP-lowering therapy will lead to better clinical outcomes.

Two routes of administration of mitomycin C in a trabeculectomy.

Background: The introduction of mitomycin C (MMC) as an adjunct to trabeculectomy was a major advance in the ability to improve the Intra ocular pressure lowering efficiency of the procedure. The time tested traditional way of administration of MMC is via a sponges soaked in it, duration and concentration varies depending on risk of failure. A subconjunctival injection of MMC instead of these sponges is recently being used as a promising alternative. Purpose: Here is the video demonstrating the 2 possible ways to prepare and apply MMC during a trabeculectomy surgery. Synopsis: Intraoperative injection of MMC in trabeculectomy has several advantages over conventional method ofsponge application. A large MMC treatment area produces more diffuse and elevated blebs. Large-area MMC application also seems to increase long-term success without increasing the complication rates in trabeculectomies. Direct and diffuse application of MMC by injection may promote less scarring and vascularization of the bleb. To achieve the same surface area of exposure with sponges, i.e.achieved with injection, the surgeon must use multiple sponges, all of which must be carefully collected thereafter. The injection method,therefore,eliminates the risk of retained sponges. Highlights: injection of MMC may be as safe and as effective as conventional sponge application of MMC with comparable estimated complete treatment success with relatively lower complication rates. Video link: https://youtu.be/RLEbK4IeRtU.

Long-Term Surgical Outcomes of Primary Congenital Glaucoma in a South Indian Population.

PURPOSE: To evaluate the long-term surgical outcomes of children with primary congenital glaucoma (PCG) in a South Indian population. DESIGN: Retrospective cohort study. PARTICIPANTS: Children with PCG who underwent primary surgery from 1997 through 2000 at a tertiary eye center in India with minimum of 5 years of follow-up. METHODS: This retrospective cohort study included children with PCG who underwent trabeculotomy, trabeculectomy, or combined trabeculotomy and trabeculectomy (CTT) as primary surgery from 1997 through 2000 at a tertiary eye center in India with minimum of 5 years of follow-up. Survival analyses were performed to determine cumulative probability of complete and qualified success. MAIN OUTCOME MEASURES: Intraocular pressure (IOP) control, glaucoma medication use, surgical success rates, and complications. RESULTS: The study included 50 eyes of 31 patients. Mean age at initial surgery was 5 months (range, 5 days-48 months) and 19 patients (61.3%) showed bilateral disease. Mean duration of follow-up was 10.9 ± 3.10 years (range, 6-18 years). Mean IOP was reduced from 28.58 ± 9.78 mmHg (range, 10-59 mmHg) before surgery to 17.13 ± 7.62 mmHg after surgery (range, 5-42 mmHg; P < 0.001) at final follow-up. Survival analysis showed that the probability of surgical success with CTT was 77.8% at 1 year, 66.2% at 2 years, 53.0% at 5 years, and 16.6% at 15 years. Visual acuity at last available follow-up correlated with surgical success (P = 0.042). CONCLUSIONS: Surgical success after long-term follow-up of children with PCG is low. The probability of surgical success declines over time. Children with PCG require life-long follow-up and management, even after initial surgical success, to prevent visual impairment and blindness.

Outcomes after long-term mechanical ventilation of cancer patients.

BACKGROUND: The probability of weaning and of long-term survival of chronically mechanically ventilated cancer patients is unknown, with incomplete information available to guide therapeutic decisions. We sought to determine the probability of weaning and overall survival of cancer patients requiring long-term mechanical ventilation in a specialized weaning unit. METHODS: A single-institution retrospective review of patients requiring mechanical ventilation outside of a critical care setting from 2008 to 2012 and from January 1 to December 31, 2018, was performed. Demographic and clinical data were recorded, including cancer specifics, comorbidities, treatments, and outcomes. Overall survival was determined using the Kaplan-Meier approach. Time to weaning was analyzed using the cumulative incidence function, with death considered a competing risk. Prognostic factors were evaluated for use in prospective evaluations of weaning protocols. RESULTS: Between 2008 and 2012, 122 patients required mechanical ventilation outside of a critical care setting with weaning as a goal of care. The cumulative incidence of weaning after discharge from the intensive care unit was 42% at 21 days, 49% at 30 days, 58% at 60 days, 61% at 90 days, and 61% at 120 days. The median survival was 0.16 years (95% CI, 0.12 to 0.33) for those not weaned and 1.05 years (95% CI, 0.60 to 1.34) for those weaned. Overall survival at 1 year and 2 years was 52 and 32% among those weaned and 16 and 9% among those not weaned. During 2018, 36 patients at our institution required mechanical ventilation outside of a critical care setting, with weaning as a goal of care. Overall, with a median follow-up of 140 days (range, 0-425 days; average, 141 days), 25% of patients requiring long-term mechanical ventilation (9 of 36) are alive. CONCLUSIONS: Cancer patients can be weaned from long-term mechanical ventilation, even after prolonged periods of support. Implementation of a resource-intensive weaning program did not improve rates of successful weaning. No clear time on mechanical ventilation could be identified beyond which weaning was unprecedented. Short-term overall survival for these patients is poor.

A Rare Case Report of Frank Ter Haar Syndrome in a Sibling Pair Presenting With Congenital Glaucoma.

Frank Ter Haar syndrome (FTHS) is a rare autosomal recessive disorder with characteristic skeletal, cardiac, ocular, and craniofacial abnormalities. We report a sibling pair presenting with clinical features typical of FTHS, born to consanguineous parents, with a novel mutation in the SH3PXD2B gene on chromosome 5q35.1 that results in premature truncation of the protein encoded. The children presented with brachycephaly, multiple joint contractures, cardiac valvular defects, bilateral megalocornea, and congenital glaucoma. Trabeculotomy combined with trabeculectomy was performed in both siblings to control intraocular pressure. The characteristic clinical features with the underlying genetic defects confirmed the diagnosis of FTHS. Early diagnosis and treatment of congenital glaucoma preserved vision in the children.

Waste generated during glaucoma surgery: A comparison of two global facilities.

PURPOSE: We measured waste from glaucoma surgeries at an eye care facility in Southern India and compared these results to a community hospital in the United States. METHODS: The waste produced in the glaucoma operating room at Aravind Eye Hospital, Madurai, India from June 22 to July 15, 2015 was weighed and compared to the waste produced in the glaucoma surgical clinic in a Baltimore-area community hospital from one day of surgeries in August 2015. RESULTS: The average waste produced per trabeculectomy at Aravind was 0.5 ±â€¯0.2 kg, compared to an average of 1.4 ±â€¯0.4 kg per trabeculectomy (p < 0.05) at the Baltimore-area hospital. Waste from device surgeries and trabeculectomy with phacoemulsification was also quantified at Aravind, with averages of 0.4 ±â€¯0.2 kg and 0.7 ±â€¯0.2 kg respectively. CONCLUSIONS AND IMPORTANCE: The amount of waste per trabeculectomy at the Aravind Eye Hospital was significantly lower than the waste per trabeculectomy in the Baltimore-area hospital, even though the used and the apparent complication rates between Aravind and American eye hospital are comparable. Given efforts to decrease the environmental impact of health care, it is necessary to examine the waste produced from surgeries to determine if policy and legal changes in the United States could decrease surgical waste while not affecting the surgical complication rate.

A rare case of overlapping Sturge-Weber syndrome and Klippel-Trenaunay syndrome associated with bilateral refractory childhood glaucoma.

A 6-year-old girl presented with blurred vision and was found to have elevated intraocular pressure (IOP) and glaucomatous optic disc damage in both eyes. She also displayed capillary malformations on the face (port-wine stain), upper back and all four limbs, angiomatosis in the brain and had hypertrophy of the left upper and lower limbs typical of overlapping Sturge-Weber syndrome and Klippel-Trenaunay syndromes. She was initially managed with IOP lowering topical medications but required trabeculectomy in the right eye followed by Ahmed valve implantation in both eyes. Despite multiple measures over a 7-year period, her IOP still remained uncontrolled with gradual progression of the glaucomatous damage. This case exhibits a very rare occurrence of overlapping syndromes reported only a handful of times in literature. Most cases with Sturge-Weber syndrome have ipsilateral glaucoma affecting the eye on the same side as the port-wine stain. This case presented with bilateral refractory childhood glaucomas, which is exceedingly rare.

The Effect of Counseling on Cataract Patient Knowledge, Decisional Conflict, and Satisfaction.

PURPOSE: Cataract is the leading cause of non-refractive preventable blindness, and comprehensive strategies to increase cataract surgery rates are imperative, including high-quality supportive patient education. We evaluated the effectiveness of non-physician pre-surgical counselors teaching patients about cataract and cataract surgery in improving patient knowledge, decisional conflict, and satisfaction. METHODS: A survey was given before and after 61 newly-diagnosed cataract patients underwent pre-surgical counseling at the Aravind Eye Hospital, Madurai, India. The survey measured change in cataract knowledge and decisional conflict, a measure of anxiety surrounding the decision to undergo surgery, along with patient satisfaction. Multiple regression was used to identify factors that influenced change in knowledge. RESULTS: Both patient knowledge scores and decisional conflict scores improved following counseling (mean difference +2.0, p = 0.004 and +8.4, p < 0.0001, respectively). Multiple regression analysis identified female sex (ß = 2.5, p < 0.001) and being illiterate (ß = 1.7, p = 0.04) as important predictors of increased knowledge post-counseling. CONCLUSION: Counseling both improved knowledge and reduced decisional conflict about cataract surgery, particularly among patients who had traditionally had more limited access to healthcare such as women and illiterate patients. Increased use of high quality counseling might help to further reduce the global burden of cataract and other forms of blindness.

Ribosomal RNA gene sequencing for early diagnosis of Blastomyces dermatitidis infection.

Prompt detection and identification of fungal pathogens at the genus and species level is critical in order to provide timely antifungal therapy. Here, we highlight the vital role of molecular diagnostics in achieving a fast and definitive diagnosis of disseminated blastomycosis in a diabetic patient presenting as a brain mass initially thought to be tumoral in nature. A broad-range PCR amplification and sequencing of the fungal ribosomal RNA genes on brain biopsy tissue obtained during elective craniotomy revealed a final microbial identification of Ajellomyces dermatitidis (telemorph of Blastomyces dermatitidis).

α1-Antitrypsin activates protein phosphatase 2A to counter lung inflammatory responses.

RATIONALE: α1-Antitrypsin (A1AT) was identified as a plasma protease inhibitor; however, it is now recognized as a multifunctional protein that modulates immunity, inflammation, proteostasis, apoptosis, and cellular senescence. Like A1AT, protein phosphatase 2A (PP2A), a major serine-threonine phosphatase, regulates similar biologic processes and plays a key role in chronic obstructive pulmonary disease. OBJECTIVES: Given their common effects, this study investigated whether A1AT acts via PP2A to alter tumor necrosis factor (TNF) signaling, inflammation, and proteolytic responses in this disease. METHODS: PP2A activity was measured in peripheral blood neutrophils from A1AT-deficient (PiZZ) and healthy (PiMM) individuals and in alveolar macrophages from normal (60 mg/kg) and high-dose (120 mg/kg) A1AT-treated PiZZ subjects. PP2A activation was assessed in human neutrophils, airway epithelial cells, and peripheral blood monocytes treated with plasma purified A1AT protein. Similarly, lung PP2A activity was measured in mice administered intranasal A1AT. PP2A was silenced in lung epithelial cells treated with A1AT and matrix metalloproteinase and cytokine production was then measured following TNF-α stimulation. MEASUREMENTS AND MAIN RESULTS: PP2A was significantly lower in neutrophils isolated from PiZZ compared with PiMM subjects. A1AT protein activated PP2A in human alveolar macrophages, monocytes, neutrophils, airway epithelial cells, and in mouse lungs. This activation required functionally active A1AT protein and protein tyrosine phosphatase 1B expression. A1AT treatment acted via PP2A to prevent p38 and IκBα phosphorylation and matrix metalloproteinase and cytokine induction in TNF-α-stimulated epithelial cells. CONCLUSIONS: Together, these data indicate that A1AT modulates PP2A to counter inflammatory and proteolytic responses induced by TNF signaling in the lung.

Extraction of permanent maxillary teeth by only buccal infiltration of articaine.

OBJECTIVE: The aim of this study is to demonstrate whether articaine hydrochloride administered alone as a single buccal infiltration in maxillary tooth removal, can provide favourable palatal anesthesia as compared to buccal and palatal injection of lidocaine. STUDY DESIGN: The study population consisted of 30 patients who were undergoing orthodontic treatment, and who required bilateral extraction of maxillary permanent premolars as per their orthodontic treatment plan. On the experimental side, 4 % articaine/HCl was injected into the buccal vestibule of the tooth to be extracted. On the control side, 2 % lignocaine HCl was injected both into the buccal and the palatal side of the tooth to be extracted. Following tooth extraction all patients completed a 100-mm visual analogue scale (VAS) and faces pain scale (FPS) to rate the pain on extraction. RESULTS: According to the VAS and FPS scores, the pain on extraction between buccal infiltration of articaine and the routine buccal and palatal infiltration of lignocaine was statistically insignificant. CONCLUSIONS: The routine use of a palatal injection for the removal of permanent maxillary premolar teeth may not be required when articaine/HCl is used as the local anesthetic.

Frontal sinus mucoceles presenting in the upper eyelid: an easily missed diagnosis.

Frontal sinus mucoceles are epithelium-lined mucus-containing sacs that are capable of bony expansion causing a spectrum of ophthalmological symptoms. If left untreated, they can erode the thin sinus wall causing life-threatening complications such as meningitis. We would like to alert the clinicians to this diagnosis that can be easily misdiagnosed. The first patient appeared to have an allergic blepharo-conjunctivitis that was not responding to topical and systemic medications. The second patient presented with recurrent preseptal cellulitis unresponsive to oral antibiotics. CT imaging of both patients revealed frontal sinus mucoceles. Both patients recovered well with sinus surgery. Management of these patients needed a close liaison with our ENT and radiology colleagues to warrant a good outcome.