scholarly journals A rare case of bilateral ocular manifestations of Sturge-Weber syndrome

2021 ◽  
Vol 9 (12) ◽  
pp. 3721
Author(s):  
Pranami Basyach
Keyword(s):  
Normal Limit ◽  
Retinal Nerve Fibre Layer ◽  
Ocular Manifestations ◽  
Corrected Visual Acuity ◽  
Weber Syndrome ◽  
Operative Complications ◽  
Sturge Weber Syndrome ◽  
Nerve Fibre Layer ◽  
Systemic Examination

Sturge–Weber syndrome (SWS) is a group of phakomatoses characterized by hamartomas involving brain, skin and eyes. A 36 years old female presented with pain and diminution of vision in both eyes since last 1 year. On examination she has bluish sclera in B/L eyes with characteristic facial port-wine stain. Best corrected visual acuity (BCVA) in R/E- 6/18, N6 and in L/E- 6/6, N6. Intra-occular pressure (IOP) in R/E – 27 mm Hg and in L/E –18 mmHg. On gonioscopy – open angle B/E. On direct ophthalmoscopy and slit-lamp examination with 90 D lens, cup-disc ratio in R/E – 0.9 and in L/E – 0.7 with thinning of neuro-retinal rim in B/E. In optical coherence tomography (OCT), retinal nerve fibre layer (RNFL) and optic nerve head (ONH) it was confirmed. Other systemic examination – within normal limit. Initially, she had been on conservative treatment. As intraocular pressure remained high after several weeks of treatment, trabeculectomy in R/E done under local anaesthesia. No post-operative complications were seen. After 7 days of follow-up IOP – within normal limit, vision in R/E unchanged and had been frequently followed-up.

scholarly journals Half Fluence Photodynamic Therapy in Peripapillary Circumscribed Choroidal Hemangiomas

2021 ◽  
Author(s):  
Ashok Kumar ◽  
Poninder Kumar ◽  
Sanjay Kumar Mishra ◽  
Mayank Jhanwar ◽  
Arun Gupta ◽  
...  
Keyword(s):  
Photodynamic Therapy ◽  
Case Series ◽  
Retinal Nerve Fibre Layer ◽  
Post Treatment ◽  
Single Session ◽  
Corrected Visual Acuity ◽  
Nerve Fibre Layer ◽  
Safe Treatment Option ◽  
Sd Oct

Abstract PurposeTo evaluate the safety and efficacy of half fluence photodynamic therapy (PDT) as treatment for symptomatic peripapillary circumscribed choroidal haemangiomas (CCHs). MethodsIn this prospective, interventional case series; 11 patients with symptomatic peripapillary CCHs presenting to a single centre were treated with half fluence PDT using verteporfin 6 mg/m2 with fluence of 25 mJ/cm2 (standard is 50 mJ/cm2) and other standard settings. Patients were evaluated at baseline, four weeks, twelve weeks and twenty-four weeks post-PDT treatment with best corrected visual acuity (BCVA), ultrasonography, spectral domain- optical coherence tomography (SD-OCT), visual evoked potential and angiographic studies. ResultsEleven patients with peripapillary CCHs received half fluence PDT. The BCVA significantly improved to 0.558 + 0.118 at four weeks post treatment (P=0.014), to 0.494 + 0.114 at twelve weeks (P=0.006) and 0.441 + 0.125 at twenty-four weeks (P=0.007) from baseline levels of 1.017 + 0.075 on log MAR scales. Similar improvement was observed in central macular thickness (CMT) of 78.50 + 13.73 um(P=0.001) at four weeks; 114.70 + 27.73 um(P=0.003) at twelve weeks and 174.60 + 23.13 um(P=0.001) at twenty-four weeks post treatment. A single session of re-treatment was required in 18%(n=2) of patients which also showed complete resolution at last follow-up. No complications were observed without any significant change in retinal nerve fibre layer (RNFL) thickness at six months follow-up(P=0.088).ConclusionsHalf fluence PDT can be an effective and safe treatment option for peripapillary CCHs which results in both anatomical and functional improvements with no observable complications.

scholarly journals Ruthenium-106 Plaque Therapy for Diffuse Choroidal Hemangioma in Sturge-Weber Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Agnieszka Kubicka-Trząska ◽  
Joanna Kobylarz ◽  
Bożena Romanowska-Dixon
Keyword(s):  
Retinal Detachment ◽  
External Beam Radiotherapy ◽  
Tumor Thickness ◽  
Exudative Retinal Detachment ◽  
Therapy Outcomes ◽  
Corrected Visual Acuity ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Diffuse Choroidal Hemangioma

Diffuse choroidal hemangiomas associated with Sturge-Weber syndrome (SWS) are classically treated with external beam radiotherapy (EBR), but there are a few reports usually of single cases indicating the usefulness of plaque therapy. We present our observations on two cases of diffuse choroidal hemangiomas with exudative retinal detachment associated with SWS treated with Ruthenium-106 plaque therapy. Outcomes included best-corrected visual acuity (BCVA) and regression in tumor thickness measured by ultrasonography. The initial BCVA of the affected eyes was counting fingers at 1 meter and light projection. Pretreatment tumors thickness was 3.5 mm and 4.7 mm. In a follow-up period of 18–24 months, significant reduction in thickness of choroidal hemangiomas up to 1.2 mm and 1.4 mm with prompt resolution of exudative retinal detachment was observed. BCVA achieved 20/200 and 20/400, respectively. The findings in this paper indicate that Ruthenium-106 plaque therapy is effective in treatment of diffuse choroidal hemangiomas associated with SWS.

Sturge Weber syndrome

2019 ◽  
Vol 16 (2) ◽  
pp. 46-49
Author(s):  
Yam Bahadur Roka
Keyword(s):  
Cerebral Atrophy ◽  
Partial Seizures ◽  
Weber Syndrome ◽  
Daily Dose ◽  
History Of ◽  
Scalp Wound ◽  
Skin Nodules ◽  
Sturge Weber Syndrome ◽  
Neurocutaneous Syndrome

Sturge-weber syndrome is a type of neurocutaneous syndrome/ neurooculo cutaneous/ phakomatoses that is characterized by facial capillary Port Wine stain, leptomeningealangioma and glaucoma with a incidence of 1 per 20-50,000 live births. A case is reported that presented to the outpatient clinic with discharging wound over the left side of his head for a week. On examination he had a large left facial PW stain involving all the divisions of the Trigeminal nerve. Further examination revealed he had history of partial seizures since last 10 years with incomplete and irregular treatment. He also had right sided hemiatrophy of the limbs along with medial gaze deviation of the left eyeball. Skull skiagram was done outside which revealed calcifications in his left hemisphere and CT was then advised for his headache and seizure that revealed Left hemispheric Tram track calcifications along with cerebral atrophy and loss of cortical volume. EEG was unremarkable. He was managed with antibiotics for the scalp wound and discharged with Leveteracetam 1000 mg daily dose and asked to attend eye hospital for the reduced vision. With the PW stain, skin nodules, eye findings and the radiological features he was diagnosed as SWS and counseled for regular follow up, use of anti-epileptic medications, probable side effects and surgery for eye abnormalities.

Evaluation of the Optic Nerve and Retinal Nerve Fibre Layer in Myopic Individuals

2012 ◽  
Vol 06 (05) ◽  
pp. 280
Author(s):  
Ahmad A Aref ◽  
Donald L Budenz ◽  
◽  
Keyword(s):  
Nerve Fibre ◽  
False Positive Rate ◽  
Field Testing ◽  
Retinal Nerve Fibre Layer ◽  
Histopathological Study ◽  
Glaucomatous Optic Neuropathy ◽  
Neuroretinal Rim ◽  
Fibre Layer ◽  
Nerve Fibre Layer

Clinical discrimination between myopic tilted optic discs and glaucomatous optic neuropathy is often challenging, especially when considering that myopia is a risk factor for the development of glaucoma. Myopic tilted discs are usually larger than average, with associated relative cupping and thinner neuroretinal rim tissue. Histopathological study has revealed thinner parapapillary retinal tissue in these eyes. Optical coherence tomography (OCT)-measured average retinal nerve fibre layer (RNFL) thickness has been found to decrease with longer axial length and higher myopic refractive error. Parapapillary RNFL quadrant and clock-hour analyses result in a higher false-positive rate in myopic eyes. Careful slit-lamp examination, quality baseline stereoscopic disc photographs and frequent serial visual field testing are essential to the follow-up of myopic individuals with suspected glaucoma. A novel diagnostic parameter, OCT-derived ganglion cell analysis, may prove to be useful in the diagnosis and follow-up of these individuals.

Utility of the transcranial doppler in the evaluation and follow-up of children with Sturge-Weber Syndrome

2020 ◽  
Vol 27 ◽  
pp. 60-66
Author(s):  
María Jiménez-Legido ◽  
Amelia Martínez-de-Azagra-Garde ◽  
Beatriz Bernardino-Cuesta ◽  
Inés Solís-Muñiz ◽  
Víctor Soto-Insuga ◽  
...  
Keyword(s):  
Transcranial Doppler ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Long-term intraocular pressure after combined trabeculotomy-trabeculectomy in glaucoma associated with Sturge-Weber syndrome

2017 ◽  
Vol 28 (2) ◽  
pp. 210-215 ◽  
Author(s):  
Devindra Sood ◽  
Aanchal Rathore ◽  
Ishaana Sood ◽  
Dinesh Kumar ◽  
Narender N. Sood
Keyword(s):  
Visual Acuity ◽  
Intraocular Pressure ◽  
Vision Loss ◽  
Intraoperative Complications ◽  
Repeat Surgery ◽  
Antiglaucoma Medication ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Purpose: Vision loss in Sturge-Weber syndrome (SWS), a rare congenital disorder, is primarily due to glaucoma. Methods: We reviewed the data of all consecutive SWS-associated glaucoma cases in patients who had undergone combined trabeculotomy-trabeculectomy (CTT) at a tertiary glaucoma facility between January 1993 and December 2015. We analyzed the preoperative and postoperative intraocular pressure (IOP), corneal clarity, visual acuity, success rate, need for repeat surgery, and number of topical antiglaucoma medications needed at last follow-up. Results: Twenty-six eyes of 20 patients with SWS (surgical age 0.7-96 months; mean 18.64 ± 29.74 months) had undergone primary CTT. The mean preoperative IOP was 32.76 ± 7.86 mm Hg (range 22-54 mm Hg) with medication (mean 3.11 ± 1.17; range 1-5). At the last follow-up (61-288 months); mean SD 134.73 ± 67.77 months), two eyes had IOP <6 mm Hg. Twenty-four eyes analyzed had an IOP of 13.63 ± 6.11 (mean ± SD; range 9-41) mm Hg. All these had an IOP <15 mm Hg at last follow-up except one, which had an IOP of 41 mm Hg. There was a mean reduction of 54.62% ± 31.33% in IOP from baseline. The antiglaucoma medication score at last follow-up visit was 0-3. No eye achieved predefined complete success or modified complete success. A total of 41.7% (10/24) of eyes attained both qualified and modified qualified success. Eleven eyes needed repeat surgeries. No intraoperative complications were noted. Visual acuity was below 6/60 in four eyes. Conclusions: Combined trabeculotomy-trabeculectomy showed promising results as a treatment for SWS-associated glaucoma in children. Long-term visual and surgical outcomes are encouraging.

The Ocular Manifestations of the Sturge-Weber Syndrome

1992 ◽  
Vol 29 (6) ◽  
pp. 349-356 ◽  
Author(s):  
Timothy J Sullivan ◽  
Michael P Clarke ◽  
J Donald Morin
Keyword(s):  
Ocular Manifestations ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Neuro-ophthalmological consequences of acute influenza A encephalitis in a genetically predisposed child

2020 ◽  
Vol 13 (9) ◽  
pp. e235636
Author(s):  
Catarina Xavier ◽  
Miguel Boncquet Vieira ◽  
Cristina Ferreira ◽  
Joana Tavares Ferreira
Keyword(s):  
Clinical Picture ◽  
Influenza A ◽  
Retinal Nerve Fibre Layer ◽  
Rare Entity ◽  
Acute Encephalopathy ◽  
Progressive Encephalopathy ◽  
Nerve Fibre Layer ◽  
Acute Necrotising Encephalopathy ◽  
Subsequent Improvement

Acute necrotising encephalopathy (ANE) is a rare disease that corresponds to a rapidly progressive encephalopathy induced by a viral infection. It is frequently associated with a mutation on the RAN-binding protein 2 (RANBP2) gene–ANE1. We present a case of a 5-year-old boy with a clinical picture of influenza aggravated to an acute encephalopathy picture after the 3rd day. Complementary examinations came back positive for the influenza A virus, and MRI showed aspects compatible with ANE. He was treated accordingly with subsequent improvement of the clinical picture. During ambulatory follow-up, a mutation was detected on the RANBP2 gene and, at the ophthalmological level, bilateral peripheral constriction on the campimetry and a significant reduction of bilateral peripapillary retinal nerve fibre layer was reported. Our case contributes to the enrichment of the neuro-ophthalmological literature and expands the spectrum of sequelae of this rare entity in the Caucasian population.

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