Clinical characteristics of infants with port-wine stain and glaucoma secondary to Sturge-Weber Syndrome.

BACKGROUND: Sturge-Weber Syndrome (SWS) is a rare disease involving the eye, skin, and brain. Port-wine stain (PWS) and glaucoma are common clinical manifestations. This study analysed the clinical characteristics of infants with PWS and glaucoma secondary to SWS. METHODS: Children with PWS and glaucoma secondary to SWS were enrolled. Data were extracted from ophthalmic and systemic examination findings. Ocular examinations included intraocular pressure, anterior segment and fundus examination, and ocular A-scan and B-scan ultrasonography. RESULTS: Fifty-seven patients were included, with a mean age of 9.9 ± 11.9 months, and 34 (59.6%) patients were male. In all, 61 eyes were diagnosed with glaucoma. Forty-one patients (71.9%) had unilateral facial PWS and glaucoma occurred on the same side. Eight patients (14.0%) had Mongolian spots and ten patients (17.5%) had epilepsy. Corneal changes included corneal oedema (n = 36 eyes, 59.0%), corneal opacity (n = 15 eyes, 24.6%), and Haab lines (n = 13 eyes, 21.3%). Mean corneal diameter and thickness in the eyes with glaucoma was larger than those in the unaffected eyes (12.2 ± 0.7 mm vs 10.8 ± 0.6 mm, P < 0.001; 681.2 ± 106.4 µm vs 578.2 ± 58.2 µm, P < 0.001). The eyes with glaucoma had higher IOP and larger axial length and C/D ratio (19.3 ± 6.2 mmHg vs 11.6 ± 4.2 mmHg, P < 0.001; 21.23 ± 1.93 mm vs 19.68 ± 1.61 mm, P < 0.001; and 0.57 ± 0.18 vs 0.24 ± 0.15, P < 0.001). Thirty-three (57.9%) and 25 (43.9%) patients showed diffuse choroidal haemangioma (DCH) and conjunctival/episcleral haemangiomas, respectively. Ten patients (17.5%) showed iris anterior insertion or hyperpigmentation in the anterior chamber angles. Six of them had Mongolian spots at the same time. CONCLUSIONS: Monocular glaucoma, DCH, and conjunctival/episcleral haemangiomas are common in SWS patients with PWS and glaucoma. Glaucomatous eyes have larger corneal diameter and axial length and thicker cornea. Patients with Mongolian spots have higher incidence of iris anterior insertion or hyperpigmentation in anterior chamber angle.

The occurrence timeline of steroid-induced ocular hypertension and cataract in children with systemic autoimmune diseases.

PURPOSE: Steroid-induced ocular hypertension (SIOH) and cataract can result in visual loss. This study evaluated the timetable of SIOH and steroid-induced posterior subcapsular cataract (SI-PSC) occurrences in children with systemic autoimmune diseases (SAD) undergoing long-term systemic corticosteroid treatment. METHODS: Thirty-seven children with SAD treated with long-term oral corticosteroids were enrolled in this study. Intraocular pressure (IOP), SI-PSC occurrences, visual field and peripapillary retinal nerve fibre layer (pRNFL) thicknesses were recorded every 3 months for at least 6 months. RESULTS: Of the 37 children, with average age 11.0 ± 2.9 years, 22 patients (59.5%) had SIOH, 2 progressed as glaucoma at the 18-month and 3-year follow-up, respectively, and 12 (32.4%) patients had SI-PSC. Among patients with SIOH, 45.5% (10/22) of them had SI-PSC occurrence, and among patients with normal IOP, 13.3% (2/15) of them had SI-PSC. Seventeen patients participated in a longitudinal study with a follow-up period of at least 18 months. The incidence of SIOH started at 1 month 52.9% (9/17) and gradually increased to 70.6% (12/17) at 6 months, then decreased to 35.3% (6/17). SI-PSC onset started at 6 months (17.6%, 3/17), and its occurrence increased to 35.3% (6/17) at 12 months and reached to 41.2% (7/17) at 18 months. The pRNFL was thicker in the children with SIOH than the healthy controls (p = 0.01). CONCLUSION: SIOH and SI-PSC are common coexistent complications in children with long-term corticosteroids treatment, and the occurrence time is during the first month and 6 months, respectively. Patients with SIOH have a higher probability of cataract.

[The application of optical coherence tomography in uveitis patients].

Uveitis is an inflammatory disease which involves the uvea, retina, retinal vessel and vitreous body. The disease mainly affects middle age and young people with a variety of clinical manifestations and is often recurrent. If not treated properly, it will cause visual impairment and even blindness. Spectral domain optical coherence tomography (SD-OCT) is a noninvasive examination which provides clear images quickly without contacting the eyes. As the development of OCT, we can obtain the sectional images with similar resolution as histological examination. Especially, when the enhanced depth image OCT (EDI-OCT) was used in clinical, the structures of choroid could also be detected. Therefore, OCT was used more and more in uveitis patients. Anterior segment OCT (AS-OCT) is used to evaluate the inflammation in anterior chamber. It also can provide the changes of iris and corneal structures during acute phase and recovery period. SD-OCT can clearly display many kinds of retinopathy in patients with uveitis including macular edema and the changes of retinal thickness. EDI-OCT can be used to observe the changes of retina and choroid at the same time. In particular, the measurement of choroidal thickness can be used to evaluate the choroidal changes and the degree of the response to treatment. Therefore, SD-OCT helps to diagnose uveitis and is easily used in the disease follow-up and treatment effect observation.

Effect of graded partial inferior rectus muscle tenotomy in treatment for superior oblique paralysis.

PURPOSE: To evaluate the surgical outcomes for patients with superior oblique paralysis (SOP) who underwent the inferior oblique weakening and the graded partial inferior rectus muscle tenotomy in the contralateral eye. METHODS: Medical records were retrospectively reviewed for those SOP patients who had hyperdeviation of 4△ -20△ in the primary position and 8△ -25△ in the downgaze position. Patients were subdivided into three subgroups according to their vertical deviation angle in the downgaze position. They underwent an ipsilateral inferior oblique muscle recession and a graded partial tenotomy of the contralateral inferior rectus muscle. The vertical deviation angles, abnormal head position, and fundus torsion were compared statistically before and after operations. All patients were followed up at least 1 year. RESULTS: Forty-seven patients were included in this study. The mean follow-up period was 17.2 months after surgery (ranging from 12 to 28 months). The vertical deviation angle was averaged preoperatively to 7.74△ ± 3.23△ in the primary position and 15.30△ ± 5.92△ in the downgaze position and reduced postoperatively to 0.85△ ± 1.15△ in the primary position and 1.53△± 1.49△ in the downgaze position (P < 0.001). All patients had an abnormal head position preoperatively and had improved significantly postoperatively. Fundus extorsion had been improved significantly postoperatively. CONCLUSION: The surgical procedure of IO weakening combined with contralateral graded partial inferior rectus muscle tenotomy is a successful intervention for the correction of small deviation in primary position of SOP.

Retinal and Choroidal Thickness in Patients with Uveitis.

PURPOSE: To study retinal thickness (RT) and choroidal thickness (CT) in patients with inactive uveitis. We also investigated the correlation between RT and CT and the duration and frequency of inflammation and visual acuity in patients with inactive uveitis. METHODS: One-hundred and forty-eight eyes from 97 patients (mean age 51.0 ± 15.7 years) and 98 eyes from 55 normal subjects (mean age 59.3 ± 14.4 years) were studied. Routine ophthalmic examination included spectral-domain optical coherence tomography with enhanced depth imaging of the retina and choroid. CT was measured from the outer border of the retinal pigment epithelium to the inner scleral border at 500-µm intervals of the horizontal section between 2.5 mm temporal and 2.5 mm nasal to the fovea. RT was measured between the inner limiting membrane and the retinal pigment epithelium. RESULTS: Mean subfoveal RT did not differ significantly between uveitis patients and controls. Mean CT at multiple locations was significantly lower in patients with uveitis compared to normal subjects; this difference was most significant at the fovea (uveitis group, 229 ± 85 µm vs. control group, 276 ± 74 µm; p < 0.001). Only CT was associated with duration and frequency of inflammation in patients with anterior uveitis (r = -0.298, p = 0.008; r = -0.349, p = 0.002, respectively). CONCLUSION: CT is reduced in patients with inactive uveitis and is associated with disease duration and frequency whereas RT does not seem to be affected by disease processes.