Correlation of changes in serum level of VEGF and peripapillary retinal thickness in patients with POEMS syndrome.

AIM: To determine whether changes in the serum levels of vascular endothelial growth factor (VEGF) after thalidomide therapy will affect the peripapillary retinal thickness (pRT) associated with optic disc oedema (ODE) in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. METHODS: This was a retrospective, observational case series of 23 right eyes of 23 treatment-naïve patients with POEMS syndrome and ODE whose intracranial pressure was within the normal range. The pRT was determined by spectral-domain optical coherence tomography, and the serum level of VEGF was determined by ELISA at baseline and 6 months after the thalidomide therapy. We determined whether a change in the pRT from baseline was significantly correlated with the serum level of VEGF from that at 6 months after the thalidomide treatment. RESULTS: Six months after treatment, the mean serum level of VEGF was significantly reduced from 7153±4214 pg/mL to 1067±769 pg/mL (p<0.001), and the pRT was significantly decreased from 471.2±203 µm to 318.1±53.9 µm (p<0.001). The change in the pRT from baseline was significantly and linearly correlated with the change in the serum level of VEGF from that at 6 months after treatment (r=0.67, p=0.00039). CONCLUSIONS: The close relationship between the pRT and the serum level of VEGF may offer clues on the pathogenesis of POEMS syndrome and potentially add a new candidate cause for the pathogenesis of ODE.

Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome.

AIMS: To determine whether there is a significant correlation between the peripapillary retinal thickness (pRT) and the serum level of vascular endothelial growth factor (VEGF) in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. METHODS: This was a cross-sectional, observational case series. We studied 34 eyes of 17 treatment-naïve patients with POEMS syndrome whose intracranial pressure was within the normal range. The spectral-domain optical coherence tomographic (SD-OCT) examinations consisted of circle scans of 3.45 mm diameter around the optic disc. The pRT was automatically measured in the SD-OCT images and was used for the statistical analysis. The serum level of VEGF was measured by ELISAs, and the correlation between the pRT and the serum level of VEGF was determined. Multivariable logistic regression analyses were used to identify independent factors that were correlated with the pRT. RESULTS: There was a significant positive correlation between the serum levels of VEGF and the average pRT of the two eyes of each patient (r=0.81, p<0.0001). There was a significant correlation between the pRT of the right and left eyes with an intraclass correlation coefficient of 0.839. Multiple regression analysis showed that the serum levels of VEGF were independent contributors to the pRT (standard regression coefficient=0.59, p=0.012). CONCLUSIONS: The significant correlation between the pRT and the serum level of VEGF suggests that the higher serum level of VEGF might be associated with the development of the optic disc oedema in patients with POEMS syndrome.

Meningitis associated with bilateral optic papillitis following Mycoplasma pneumoniae infection.

Mycoplasma pneumoniae (M. pneumoniae) is a frequent cause for human infection, and central nervous system disease associated with M. pneumoniae infection is being reported with increasing frequency. We described herein a 60-year-old woman who developed meningitis associated with bilateral optic papillitis following M. pneumoniae infection and discussed the possible mechanisms. To our knowledge, there have been only a few reports that described the meningitis complicated by bilateral optic papillitis in association with M. pneumoniae infection. Our case highlighted the protean neurological manifestations of M. pneumoniae infection.

Bilateral diabetic papillopathy and metabolic control.

OBJECTIVE: The pathogenesis of diabetic papillopathy largely is unknown, but case reports suggest that it may follow rapidly improved metabolic control. The present study was designed to investigate this hypothesis. DESIGN: Retrospective case-control study. PARTICIPANTS: Two thousand sixty-six patients with type 1 diabetes. METHODS: Review of clinical, photographic, and clinical chemistry records from a large diabetology and ophthalmology unit between 2001 and 2008. MAIN OUTCOME MEASURES: Simultaneous, bilateral diabetic papillopathy. RESULTS: The mean follow-up was 4.9 years. During 10,020 patient-years of observation, bilateral diabetic papillopathy developed in 5 patients. During the year preceding this incident, all 5 patients had experienced a decrease in glycosylated hemoglobin A1(c) (HbA1(C)) at a maximum rate of -2.5 (mean) percentage points per quarter year, which was significantly different from the changes in HbA1(C) observed in the remainder of the study population (P<0.001). Photographs recorded before the onset of bilateral diabetic papillopathy showed that all 5 patients had small cup-to-disc diameter ratios in both eyes (P<0.001). CONCLUSIONS: Diabetic papillopathy was associated markedly with a drastic recent reduction in glycemia and a small cup-to-disc diameter ratio. This supports the hypothesis that diabetic papillopathy may be an early worsening phenomenon occurring in anatomically predisposed patients in response to a recent rapid decrease in glycemia. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Trisulfonated porphyrazines: new photosensitizers for the treatment of retinal and subretinal edema.

A new series of water-soluble, mononaphthotrisulfobenzoporphyrazines, bearing an alkynyl side chain of varying lengths on the naphtho ring, were prepared and tested for their efficacy to inhibit plasma extravasation when used as photosensitizers during photodynamic therapy (PDT) of the retina in the rat. The hexynyl substituted photosensitizer was the most potent, and was able to produce complete inhibition, at low doses of photosensitizer and light.

Optic disc edema, cystoid macular edema, and elevated vascular endothelial growth factor in a patient with POEMS syndrome.

A 48-year-old man with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome had bilateral optic disc edema (ODE), bilateral cystoid macular edema (CME), anasarca, and elevated serum vascular endothelial growth factor (VEGF). This is the first reported example of ophthalmoscopic, angiographic, and optical coherence tomographic evidence of the combination of ODE and CME in this syndrome. This combination of features suggests that the ODE in this condition may be due to increased vascular permeability.

Optic disk manifestation in diabetic eyes with low serum albumin: late fluorescein staining and high blood flow velocities in the optic disk.

PURPOSE: To evaluate ocular findings and blood flow characteristics in diabetes mellitus (DM) patients with low serum albumin (ALB). METHODS: We studied 106 eyes of 53 type II DM patients without proliferative diabetic retinopathy and panretinal photocoagulation. All underwent standard ophthalmic examinations, color Doppler imaging, and serum ALB measurements. The patients were divided into Group 1 (34 cases) with a serum ALB > or =3.8 g/dl and Group 2 (19 cases) with ALB<3.8 g/dl. RESULTS: All of the optic disks appeared normal by Ophthalmoscopy except one case with the lowest serum ALB. The number of optic disks showing late fluorescein staining was significantly higher in Group 2 than in Group 1. Peak systolic velocities in the central retinal artery (CRA, P=0.02) and central retinal vein (CRV, P<0.001) were significantly higher in Group 2, and significantly correlated with the serum ALB value (CRA; r=0.41, P=0.003 and CRV; r=0.60, P<0.001). CONCLUSIONS: We suggest that low serum ALB enhances the permeability of the vessels in or around the optic disk and induces subclinical optic disk edema, which is characterized by late fluorescein staining and high blood flow velocities in the CRA and CRV.

Recurrence of idiopathic intracranial hypertension after weight loss: the carrot craver.

PURPOSE: To describe a patient with stable idiopathic intracranial hypertension whose papilledema worsened. METHOD: Case report. RESULTS: A patient with documented idiopathic intracranial hypertension had resolution of disc edema with weight loss. Recurrence of papilledema led to the discovery that she consumed large quantities of raw carrots to help maintain her weight. Her increased vitamin A levels normalized, and the disc edema resolved when she stopped eating carrots. CONCLUSION: Patients with idiopathic intracranial hypertension should be counseled regarding carrot intake.

Optic disc edema associated with spinocerebellar degeneration.

A 58-year-old man presented with optic disc edema as a rare association with spinocerebellar degeneration (SCD). The patient also had chronic idiopathic intestinal pseudo-obstruction with hypoalbuminemia. No elevation of intraspinal pressure and no intracranial lesion was observed. The hypoalbuminemia reacted promptly to treatment, whereas the optic disc edema regressed gradually. An association between SCD and optic atrophy has often been described, but to our knowledge this is the first report of SCD in association with optic disc edema.

Association between measures of vitamin A and the ocular fundus findings in cerebral malaria.

OBJECTIVE: To investigate the relationship between serum vitamin A levels and conjunctival impression cytology and retinal whitening present in Malawian children with cerebral malaria. METHODS: Standard retinal examination and conjunctival impression cytology were performed at hospital admission on 101 consecutively admitted children with cerebral malaria. Blood samples were drawn from 56 children at 24 hours, frozen at -20 degrees C, and transported for assessment of vitamin A levels by high-performance liquid chromatography. Associations among fundus findings and vitamin A measurements were sought. RESULTS: The whitening of the retina that we have previously described in children with cerebral malaria was found to be associated with a mean+/-SD serum vitamin A level of 0.29+/-0.1 micromol/L, compared with a mean vitamin A level of 0.41+/-0.2 micromol/L in children without retinal whitening. Children with retinal whitening were 2.77 (95% CI, 1.06-7.3) times more likely to have abnormal conjunctival impression cytology results than those without whitening. No child had any clinical or ophthalmologic evidence of chronic vitamin A deficiency. CONCLUSIONS: The retinal whitening described in children with cerebral malaria is associated with low serum vitamin A levels and with abnormal conjunctival impression cytology results and may be due to acute vitamin A deficiency at the tissue level.

Lupus anticoagulant and bilateral optic disc edema: a case report.

We present the case of a 29-year-old woman with the rare combination of lupus anticoagulant and bilateral optic disc edema. The linked etiopathologic problems are discussed to provide a proper clinical and ophthalmologic definition.

Pseudotumor cerebri: an observation and review.

In five consecutive cases of pseudotumor cerebri we observed an elevation in the erythrocyte sedimentation rate that we were able to correlate with the changes in the degree of papilledema. In spite of an extensive medical workup, no other cause for the increased ESR was found. Our findings suggest that possibly serial ESR determinations might be of assistance in following cases of pseudotumor cerebri.