Successful orthotopic liver transplantation in an adult patient with sickle cell disease and review of the literature

Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and underwent successful orthotopic liver transplantation. Both pre and post-operatively, he was maintained on red cell transfusions. He remains stable with improved liver function 42 months post transplant. The role for orthotopic liver transplantation is not well defined in patients with sickle cell disease, and the experience remains limited. Although considerable challenges of post-transplant graft complications remain, orthotopic liver transplantation should be considered as a treatment option for sickle cell disease patients with end-stage liver disease who have progressed despite conventional medical therapy. An extended period of red cell transfusion support may lessen the post-operative complications

Fluid Accumulation After Neonatal Congenital Cardiac Surgery; Clinical Implications and Outcomes

BACKGROUND To determine the association between fluid balance metrics and mortality and other postoperative outcomes after neonatal cardiac surgery in a contemporary multi-center cohort. METHODS Observational cohort study across 22 hospitals in neonates (≤30 days) undergoing cardiac surgery. We explored overall % fluid overload, postoperative day 1 % fluid overload, peak % fluid overload, and time to first negative daily fluid balance. The primary outcome was in-hospital mortality. Secondary outcomes included postoperative duration of mechanical ventilation, and intensive care unit (ICU) and hospital length of stay. Multivariable logistic or negative binomial regression was used to determine independent associations between fluid overload variables and each outcome. RESULTS The cohort included 2223 patients. In-hospital mortality was 3.9% (n=87). Overall median peak % fluid overload was 4.9%, (interquartile range 0.4-10.5%). Peak % fluid overload and postoperative day 1 % fluid overload were not associated with primary or secondary outcomes. Hospital resource utilization increased on each successive day of not achieving a first negative daily fluid balance and was characterized by longer duration of mechanical ventilation (incidence rate ratio 1.11, 95% confidence interval 1.08-1.14, ICU length of stay (incidence rate ratio 1.08, 95% confidence interval 1.03-1.12), and hospital length of stay (incidence rate ratio 1.09, 95% confidence interval 1.05-1.13). CONCLUSIONS Time to first negative daily fluid balance, but not % fluid overload is associated with improved postoperative outcomes in neonates after cardiac surgery. Specific treatments to achieve an early negative fluid balance may decrease postoperative care durations

Coats’ Disease-Related Macular Edema Treated with Combined Aflibercept and Laser Photocoagulation

Purpose. To describe the clinical response of refractory macular edema associated with Coats’ disease following treatment with aflibercept and laser photocoagulation. Methods. Case report. Results. A 17-year-old female presented with decreased vision of the left eye. Ophthalmic exam demonstrated intraretinal hemorrhages and exudation with associated edema centrally. Angiographic evaluation revealed central leaking microaneurysms and peripheral capillary dropout. These findings and a systemic work-up that yielded an incidental Factor V Leiden mutation lead to a diagnosis of Coats’ disease. Initial treatment consisted of laser photocoagulation and intravitreal bevacizumab but with poor response. Switching to intravitreal aflibercept resulted in resolution of the refractory macular edema and improvement of visual acuity to 20/25 in the left eye. Conclusion. We describe a case of refractory macular edema which responded more favorably to intravitreal aflibercept compared with bevacizumab when combined with laser photocoagulation in a patient with Coats’ disease

Successful Orthotopic Liver Transplantation in an Adult Patient with Sickle Cell Disease and Review of the Literature

Sickle cell disease can lead to hepatic complications ranging from acute hepatic crises to chronic liver disease including intrahepatic cholestasis, and iron overload. Although uncommon, intrahepatic cholestasis may be severe and medical treatment of this complication is often ineffective. We report a case of a 37 year-old male patient with sickle cell anemia, who developed liver failure and underwent successful orthotopic liver transplantation. Both pre and post-operatively, he was maintained on red cell transfusions. He remains stable with improved liver function 42 months post transplant. The role for orthotopic liver transplantation is not well defined in patients with sickle cell disease, and the experience remains limited. Although considerable challenges of post-transplant graft complications remain, orthotopic liver transplantation should be considered as a treatment option for sickle cell disease patients with end-stage liver disease who have progressed despite conventional medical therapy. An extended period of red cell transfusion support may lessen the post-operative complications

Early Evolution of the Vitreomacular Interface and Clinical Efficacy After Ocriplasmin Injection for Symptomatic Vitreomacular Adhesion

BACKGROUND AND OBJECTIVE: To determine early evolution of the vitreomacular interface and clinical efficacy and safety profile after ocriplasmin treatment. PATIENTS AND METHODS: Retrospective, multicenter, observational case series. Patients with vitreomacular adhesion (VMA) confirmed on optical coherence tomography (OCT) received a single intravitreal ocriplasmin injection. Changes in the vitreomacular interface were evaluated by spectral-domain OCT. Adverse events were monitored at all visits. RESULTS: Of 22 patients treated with ocriplasmin, 14 (64%) had VMA resolution, with six (43%) achieving VMA release within the first week. Eight patients (36%) showed improvement in visual acuity (VA) of at least two Snellen lines. Rate of VMA resolution was 79% for VA less than 20/40 and 38% for VA of 20/40 or greater. Safety findings include changes in the ellipsoid layer (n = 3) and transient increases in subretinal fluid (n = 6). CONCLUSION: Ocriplasmin was effective for VMA resolution, with a rapid onset of action. Patients with worse baseline VA showed a higher VMA resolution rate.status: publishe