Assessment of the Hallmarks of Wilson Disease in CT Scan Imaging.

INTRODUCTION: Owing to nonspecific clinical symptoms, Wilson disease (WD) diagnosis is often missed or delayed; hence, many patients reach end-stage liver disease. When cirrhosis takes place, it is difficult to distinguish between WD and other causes of cirrhosis by imaging alone. This study outlines abdominal computed tomography (CT) imaging findings that occur more frequently in patients with WD cirrhosis. MATERIAL AND METHODS: Fifty-seven patients with WD who had referred for liver transplantation took part in this study and underwent dynamic liver CT examination before transplantation. Qualitative and quantitative parameters including liver density, contour irregularity, dysmorphia, hypertrophy of caudate lobe, presence of focal parenchymal lesion, thickness of perihepatic fat layer, periportal thickness, lymphadenopathy, and other associated findings were recorded and evaluated. RESULTS: Among these patients, 85.9% had contour irregularity, 28% had hepatic dysmorphia, and periportal thickening and cholelithiasis were found in 25.5% and 12.3% of patients, respectively. Splenomegaly, lymphadenopathy, and portosystemic shunting were observed in all patients. Also, hyperdense nodules (>20 mm) and honeycomb pattern were detected in 65.2% and 15.2% of patients, respectively, in the arterial phase. In the portal phase, these findings were detected only in 13% and 4.3% of patients. Hypertrophy of caudate lobe was seen only in 12.2% of patients. CONCLUSION: WD-associated cirrhosis has many CT imaging findings, although most of them are nonspecific. Some findings, such as hyperdense nodules and honeycomb pattern in non-contrast-enhanced CT scan and arterial phase of triphasic CT scan with lack of hypertrophy of caudate lobes, are hallmarks of WD.

Results of Ahmed glaucoma valve implantation in primary congenital glaucoma.

PURPOSE: To evaluate the outcome of Ahmed glaucoma valve (AGV) implantation in primary congenital glaucoma (PCG). METHODS: The medical records of patients with PCG refractory to trabeculotomy and medical therapy who had undergone AGV implantation with a minimum follow-up of 6 months were retrospectively reviewed. The primary outcome measure was cumulative probability of success, defined as intraocular pressure (IOP) of 6-21 mm Hg, with or without medication, and no serious complications, additional glaucoma surgery, or loss of light perception. RESULTS: A total of 33 eyes of 22 children with a mean age (and standard deviation) of 2.7 ± 3.1 years were included. The patients had a mean follow-up time of 32.6 ± 18.3 months. The mean IOP was 32.8 ± 7.3 mm Hg preoperatively and 16.8 ± 4.0 mm Hg postoperatively. The number of glaucoma medications was 2.5 ± 0.7 preoperatively and 2.2 ± 0.7 postoperatively. The cumulative probability of success (and standard error) was 97% ± 3.0% in the 1st year, 85% ± 7.0% in the 2nd year, and 56% ± 14.8% in the 5th year. When only the first operated eye per patient was included, the cumulative probability of success was 96% ± 4.4% in the 1st and the 2nd years and 72% ± 15.3% in the 5th year. Major complications comprised tube-endothelial touch (3 eyes), cataract (2 eyes), shunt extrusion (1 eye), and retinal detachment (1 eye). CONCLUSIONS: AGV implantation in patients with PCG offers moderate success in controlling the IOP with a low rate of complications. Most continued to require medications.