Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria

Aims: To describe a Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria.Presentation of Case: A 25 years old, male, student, in regular follow-up in the Hematology sector due to Paroxysmal Nocturnal Hemoglobinuria in regular use of Eculizumab. He reports a month ago that he suddenly noticed, upon waking up in the morning, blurring and decreased visual acuity in her left eye.Discussion: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder characterized by hemolysis, thrombosis, and bone marrow failure caused by defective expression of glycosylphosphatidylinositol-anchored (GPI-anchored) complement inhibitors. Most commonly, PNH is caused by the loss of PIGA function, which is necessary for GPI biosynthesis.Conclusions: Patients with hemolytic anemia, unexplained thrombosis, especially in uncommon sites, cytopenias and bone marrow failure syndromes, dysphagia, and unexplained abdominal pain should be screened for PNH. PNH patients can benefit greatly from the treatments available, with a reduction in the risk of serious sequelae and a considerable improvement in their quality of life. So that the patient continues to be followed up in the ophthalmology department, with decreased visual acuity ipsilateral to the lesion, with a relative afferent pupillary defect on that side.

Extra-anatomical veno-venous surgical bypass for central vein occlusion in patients with ipsilateral arterio-venous fistula (AVF) for haemodialysis - A single centre experience

Objective: Central vein occlusion is a common complication related to central vein catheter insertion for haemodialysis which can be unmasked by an ipsilateral fistula creation, leading to a dysfunctional arteriovenous fistula (AVF). We describe an extra-anatomical venous bypass surgical procedure performed to maintain vascular access and reduce the symptoms of swelling of the ipsilateral upper limb, neck and face. Materials and Methods: We report 20 consecutive patients with end-stage renal failure (ESRF) who had central vein occlusion and were not amenable to endovascular intervention. They underwent extra-anatomical vein to vein surgical bypass. The axillary and iliac or femoral veins were approached via infraclavicular and extraperitoneal groin incisions respectively. In all the patients, an externally supported 6 or 8 mm polytetrafluoroethylene (PTFE) graft was used as a conduit and was tunnelled extra-anatomical. All patients had double antiplatelet (Aspirin and Clopidogrel) therapy post-operatively. Results: Substantial improvement in the facial, neck and upper limb swelling was noticed following this diversion surgery. The vein to vein bypass was patent at 12 months in 10 out of 20 patients. Graft infection occurred in two (10%) cases. Re-thrombectomy or assisted patency procedure (stent/plasty) was done in four (20%) cases. The patients with preoperative fistula flow rate of more than 1500 ml/min and post-operative graft flow rate of more than a 1000 ml/min were patent at 12 months (P=0.025 and p=0.034 respectively). Conclusion. Axillary to iliac/femoral vein bypass can salvage functioning ipsilateral fistula threatened by occluded upper central vein and relieve their upper limb obstructive venous symptoms.

Interventional treatment of central vein occlusion in maintenance hemodialysis patients / 局解手术学杂志

Objective To observe the effectiveness of the percutaneous transluminal angioplasty (PTA) and percutaneous transluminal stenting (PTS) for central vein occlusion in maintenance hemodialysis patients.Methods From January 2010 to August 2015,a total of 42 patients with center vein occlusion of arteriovenous fistula were treated with maintenance hemodialysis,and the surgery and postoperative conditions,revascularization and improvement of vascular stenosis were observed.Results Among the 42 patients,38 cases were successfully carried out with PTA,and the patency rate was 90.5%(38/42).A total of 32 intravascular stents were placed in 30 patients whose vein stenosis were still greater than 30% after PTA.After surgery,the swelling of the patient receded rapidly and the internal fistula went back to normal.Conclusion PTA and PTS are effective methods for maintenance hemodialysis patients with central vein occlusion,and they could help protecting functional access in patients with autogenous fistulas with smaller wounds and faster effects.