Phacomatosis pigmentovascularis with sturge-weber syndrome and congenital glaucoma: A rare case report.

Phacomatosis pigmentovascularis (PPV) is a rare congenital disease characterized by the co-existence of cutaneous vascular malformation and pigmentary nevi with or without extracutaneous systemic involvement. Here, we present a 2-month old child diagnosed with phacomatosis cesioflammea type of PPV with Sturge-Weber syndrome and secondary congenital glaucoma of the left eye. She underwent combined trabeculotomy and trabeculectomy in the left eye for glaucoma and was started on anti-epileptics for seizure control following pediatric evaluation. Early screening and treatment initiation can prevent blindness and other systemic complications associated with PPV.

Glaucoma and optical coherence tomography changes in migraine: A comparative cross-sectional study.

PURPOSE: To study the prevalence of glaucoma among adults with migraine and the effect of migraine on peripapillary retinal nerve fiber layer (pRNFL) and central macular thickness (CMT) using optical coherence tomography (OCT) compared to those without migraine headache, i.e. in tension-type headache (TTH) and normal group. METHODS: One hundred and eleven patients (222 eyes) were recruited in three groups. migraine, TTH, and normal subjects visiting hospital outpatient services. After noting demographic details and pertinent history, ophthalmological evaluation including optic disc for glaucomatous changes along with computerized visual field testing and OCT for pRNFL thickness and CMT was performed in all eyes. Continuous variables were compared using ANOVA or Kruskal-Wallis test, while categorical variables including the association of glaucoma with migraine were analyzed using Chi-square or Fisher's exact test. RESULTS: Prevalence of glaucoma in migraine group (12.2%) was more than in comparison groups (6.8% in TTH, 4.1% in normal) which was however not significant (Fisher's exact P = 0.207). Average pRNFL thickness (103.59 ± 12.82 µm) and thickness in nasal (90.49 ± 19.19 µm) and temporal quadrants (70.58 ± 16.13 µm) and CMT (213.78 ± 19.81 µm) were significantly reduced (ANOVA P < 0.05) in migraine patients when compared to the other groups and this was independent of the presence of glaucoma. CONCLUSION: Prevalence of glaucoma is not significantly higher in migraine patients. However, migraine causes thinning of retinal layers on OCT that is statistically significant.

Orbital plasmacytoma as the presenting feature in multiple myeloma.

A 50-year-old female patient presented with protrusion of the left eye for 1 month. Examination showed abaxial proptosis, restriction of extraocular movements, and elevated intraocular pressure. Computed tomography of the orbits showed soft tissue enhancing lesion in the superolateral aspect of the left orbit with lytic lesions in calvarium. Fine needle aspiration cytology of the lesion revealed a diagnosis of plasmacytoma with positive CD138 and CD38 immunohistochemical stains. Erythrocyte sedimentation rate, C-reactive protein and serum lactate dehydrogenase were elevated. Serum protein electrophoresis revealed hypergammaglobulinemia, and bone marrow biopsy revealed 6% plasma cells. The patient was started on chemotherapy with bortezomib, dexamethasone and lenalidomide by the medical oncologist. Significant improvement in proptosis and extraocular movements noted on follow-up. Orbital myeloma may be the first manifestation of systemic disease.