Anterior segment anomalies of the eye, growth retardation associated with hypoplastic pituitary gland and endocrine abnormalities: Jung syndrome or a new syndrome?

We report on two children from an inbred Arab family with anterior segment anomalies of the eyes, growth retardation, associated with small pituitary gland, and endocrine abnormalities. The features in the sibs in this report are similar to those described in Peters-plus syndrome. However, small pituitary gland associated with growth hormone deficiency has not been reported in Peters-plus syndrome. In addition, sequencing of the B3GALTL gene, the gene implicated in Peters-plus syndrome did not reveal any mutation in the sibs reported here. The association of anterior segment anomalies of the eye, growth retardation, and endocrine problems has previously been described by Jung et al. in 1995. We suggest that the features in the children in this report could represent variable manifestation of this syndrome or previously not described syndrome.

Malignant non-teratoid medulloepithelioma of ciliary body with retinoblastic differentiation: a case report and review of literature.

We present a 6-year-old child with intraocular and extraocular mass and high intraocular pressure. The tumor mass involved a disorganized anterior segment and extended through the medial cornea and sclera. A preliminary diagnosis of retinoblastoma with extraocular extension was made. An exenteration of the left globe and orbital tissue was performed. Histological examination showed that the lesion, which occupied the posterior chamber, involved the ciliary body, extending into the iris, sclera and cornea, projecting beyond the cornea anteriorly and extending to the retina posteriorly. The tumor cells were diffusely immunoreactive to vimentin, neuron specific enolase and CD 138. The medulloepithelioma cells were focally positive to cytokeratin (AE1/AE3), cytokeratin 18, CD56,CD57, S100, HMB-45 and bcl2 while areas of retinoblastic differentiation showed diffuse immunoreactivity to synaptophysin, neurofilament and CD138 with focal immunoreactivity to calretinin. All tumor cells showed no immunoreactivity to cytokeratin 7, cytokeratin 20, epithelial membrane antigen, carcinoembryonic antigen, desmin, GFAP, and chromogranin. Nuclear staining for P53 was seen in 80% of tumor cells. The ki-67 index was 90%. The tumor was described as malignant intraocular non-teratoid medulloepithelioma with retinoblastic differentiation arising from the ciliary body. Tumor satellites were seen in the adjacent periocular soft tissue. The treatment involved exenteration of the left globe and orbital tissue with secondary skin graft following chemotherapy. The patient is well and has no recurrence after 1 year of treatment. We report that medulloepithelioma can present as a case of infantile glaucoma, can show signs of intraocular calcifications and can show retinoblastic differentiation.

Prevalence of diabetes mellitus and its complications in a population-based sample in Al Ain, United Arab Emirates.

AIMS: To determine the prevalence of diabetes mellitus (DM) and its complications in the adult population of the United Arab Emirates (UAE) and assess the degree of metabolic control in subjects with diagnosed DM. METHODS: A random sample of houses of Emirati citizens living in Al Ain, UAE was surveyed. Fasting blood glucose was determined by glucose meter and an oral glucose tolerance test (OGTT) was conducted if blood sugar was <7 mmol/l. DM was defined according to the WHO criteria. Pre-diabetes status was based on fasting venous blood glucose concentration of 5.6-6.9 mmol/l or 2h post-OGTT venous blood glucose level of 7.8-11.0 mmol/l. RESULTS: There were 2455 adults (>18) living in the 452 surveyed houses of which 10.2% reported having the diagnosis of DM. A total of 373 men and non-pregnant women underwent testing, and after adjustment for factors affecting participation probability the prevalence of diagnosed DM, undiagnosed DM and pre-diabetes was 10.5, 6.6 and 20.2%, respectively. Age-standardized rates for DM (diagnosed and undiagnosed) and pre-diabetes among 30-64 years old were 29.0 and 24.2%, respectively. Logistic regression analysis showed that only age and body mass index (BMI) were significantly independently related to undiagnosed DM. In patients with diagnosed DM, the prevalence rates for retinopathy, neuropathy, nephropathy, peripheral vascular disease and coronary heart disease were 54.2, 34.7, 40.8, 11.1 and 10.5%, respectively. A significant proportion of subjects with undiagnosed DM and pre-diabetes also had micro- and macro-vascular complications. The proportion of subjects with diagnosed DM who achieved internationally recognized targets for HbA1c (<7%), LDL-C (<2.6 mmol/l) and blood pressure (<130/80 mmHg) was 33.3, 30.8 and 42.1%, respectively. CONCLUSION: This study confirms the previously reported high prevalence of DM in the UAE. Diabetic complications were highly prevalent among subjects with diagnosed and undiagnosed DM. Metabolic control was suboptimal in most subjects with diagnosed DM. Greater efforts are urgently needed to screen early and effectively treat DM in the UAE in order to prevent long-term complications.

Delayed suprachoroidal hemorrhage following viscocanalostomy.

Delayed suprachoroidal hemorrhage is a rare complication of conventional glaucoma surgery. Viscocanalostomy is one of the new surgical techniques used in glaucoma surgery that may theoretically protect against suprachoroidal effusion and hemorrhage due to the nonpenetrating nature of the procedure. Delayed suprachoroidal hemorrhage developed in a 92-year-old white woman following viscocanalostomy. This case demonstrates that the risk of suprachoroidal hemorrhage may not be completely eliminated after a nonpenetrating glaucoma procedure such as viscocanalostomy.