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Objective: To evaluate the medium-term efficacy and safety of Ex-PRESS shunt implantation in the treatment of secondary glaucoma associated with Sturge-Weber Syndrome (SWS). Methods: This was a retrospective case series study. Medical records of patients diagnosed with secondary glaucoma due to SWS who underwent Ex-PRESS shunt implantation at Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, between January 2013 and February 2020 were collected. Only the right eye were included in the analysis when both eyes were affected. Follow-up visits were scheduled at postoperative week 1, 3 months, 6 months, 1 year, 2 years, and 3 years. Clinical data including intraocular pressure (IOP), vertical cup-to-disc (C/D) ratio, corneal horizontal diameter, visual field, anterior chamber condition, anti-glaucoma medication usage, and additional surgeries were evaluated and analyzed before and after the procedure. Surgical success rates and procedure-related complications at each follow-up time point were assessed. Statistical analyses were performed using t -test, Mann-Whitney U test, and χ 2 test. Results: A total of 21 patients (21 eyes) were included in the study, comprising 10 females and 11 males. Among them, 15 eyes were on the right side, and 6 eyes were on the left side. The patients' ages ranged from 3 to 51 years, with a median age of 8.1 (6.3, 11.9) years. The follow-up period ranged from 3 to 53 months, with a median of 11 (6, 24) months. Preoperatively, the IOP in the 21 operated eyes was (32.9±9.1) mmHg (1 mmHg=0.133 kPa), significantly higher than the IOP in the 19 healthy eyes, which was (17.1±4.3) mmHg ( t =5.80, P <0.001). The C/D ratio in the operated eyes was (0.75±0.13), also significantly higher than that in the healthy eyes, which was (0.32±0.10) ( t =11.22, P <0.001). At the 1-year, 2-year, and 3-year follow-up, 8 eyes out of 16 operated eyes, 6 eyes out of 9 operated eyes, and 7 eyes out of 10 operated eyes achieved overall surgical success (complete success+conditional success), respectively. The number of eyes with complete success at the three follow-up time points was 3, 2, and 4, respectively. The IOP in the operated eyes was significantly reduced at all follow-up time points compared to preoperative values (all P <0.05), while there was no statistically significant difference in C/D ratio before and after surgery (all P >0.05). Two operated eyes experienced retinal detachment or choroidal leakage postoperatively, both of which recovered after conservative treatment. One eye developed postoperative degree Ⅰ shallow anterior chamber, and it resolved spontaneously on the third day after surgery. No serious surgical complications, such as bleb-related complications, malignant glaucoma, expulsive choroidal hemorrhage, or endophthalmitis, were observed postoperatively. Conclusions: Ex-PRESS shunt implantation for the treatment of secondary glaucoma in SWS demonstrated a relatively high level of safety. The medium-term IOP in the operated eyes significantly decreased compared to preoperative values. However, the majority of operated eyes did not achieve complete surgical success.

According to the latest study of the pathogenesis of Sturge-Weber syndrome and its related syndromes, all signs and symptoms arise from localized primary venous dysplasia. Venous hypertension will be transmitted to nearby areas through persisting communicating venous passageways and compensatory collateral venous channels. The port-wine stains is a vascular disorder rather than a neural disorder. Symptoms depend upon the extent and location of the venous dysplasia. This hypothesis is supported by published data and by researches and the Doppler ultrasonographic examinations of orbital venous flow in patients with Sturge-Weber syndrome. This new pathophysiologic mechanism also elucidates the reason for tissue hypertrophy. Therapies are now aimed at reducing collateral venous blood-flow passageways so as to obliterate port-wine stains. But in some instances, this reduction may worsen blood stasis within the brain and potentially aggravate symptoms of nerve. Supported by National Natural Science Foundation of China (Grant No. 30672329). [ABSTRACT FROM AUTHOR]

Objective: To analyze the clinical manifestations and neuroimaging characteristics of Sturge-Weber syndrome (SWS), to describe the manifestations of facial port-wine stains (PWS) of SWS, and to explore the screening opinions for SWS., Methods: A retrospective analysis was performed on the general condition, clinical manifestations, and neuroimaging results of 24 SWS patients from the dermatology department of West China Hospital of Sichuan University between 2017 and 2019. Three different facial PWS distribution methods (traditional anatomical distribution, facial trigeminal nerve distribution, and facial embryological vasculature distribution) in SWS patients were Analysed., Results: Among the 24 patients, 50% were male and 50% were female, with an average age of (18.9±14.0) years (range 1 to 54 years old). 12 cases were SWS type Ⅰ, and the other 12 cases were type Ⅱ. All patients had facial PWS at birth, and the facial PWS of 13 cases (54.2%) were thickened. According to the anatomical division, all the PWS involved the upper and middle face (above the oral commissure); according to the trigeminal nerve distribution, 100% (24/24) patients involve the V2 area; according to the distribution of facial embryological vasculature, 95.8% (23/24) of the patients involved frontal region. 22 patients had ophthalmic abnormalities, the most common was glaucoma (70.8%), and 4 patients had a history of epilepsy. The typical neuroimaging presentations of SWS include leptomeningeal enhancement, cortical calcification, enlarged choroid plexus, focal cerebral atrophy, abnormal intracranial vessels, and local thickening of the skull., Conclusion: Early intervention is recommended for facial PWS in patients with SWS , and ophthalmological screening should be performed on children with PWS found in any part of the upper and middle face after birth. Moreover, neuroimaging examination (MRI) for patients with high suspicion of SWS should be performed after 1 year old, and regular ophthalmological examination and intraocular pressure measurement is necessary., (Copyright© by Editorial Board of Journal of Sichuan University (Medical Science Edition).)

Sturge-Weber Syndrome is a sporadic rare disease. Its characteristic feature of Port-wine Stain in patient's face has been an important indicator for screening secondary glaucoma, which further provides patients with possibility of early diagnosis and treatment of Sturge-Weber Syndrome induced glaucoma. This paper focuses on the pathogenesis, risk factors and treatment of Sturge-Weber Syndrome secondary glaucoma. (Chin J Ophthalmol, 2018, 54: 229-233) .

Objective: To investigate the clinical characteristics of Sturge-Weber syndrome (SWS) in the patients with Port-wine stain (PWS). Methods: A total of 279 PWS patients, 164 males, 115 females with a median age of first visit 17.00 (4.75, 56.00) months. Most of the PWS patients were referred to the Ophthalmology Department for screening eye problems when the cutaneous angiomas involved the ophthalmic division of the trigeminal nerve distribution. The intraocular pressure (IOP), cup to disk ratio (C/D), corneal condition and other essential measurements were examined to screen glaucoma or choroidal hemangioma. The differences of age, gender and vascular ectasia in the ipsilateral eyes were compared among PWS and SWS patients with chi-square test. The differences about the first visit time, IOP, C/D and corneal diameters were evaluated with independent-sample T test or nonparametric test followed by Mann-Whitney U test. Results: A total number of 66 out of 279 PWS patients (23.7%) were confirmed as SWS with glaucoma. The IOP of the ipsilateral eye with vascular ectasia in PWS and SWS was 13.00 mmHg (1 mmHg=0.133 kPa) (IQR: 9.75, 17.00) and 23.00 mmHg (20.00, 32.00), respectively ( Z=- 8.212, P< 0.001); the IOP differences between the ipsilateral and contralateral eye in PWS and SWS was 1mmHg (0, 2) and 7 mmHg (3, 11) respectively; the C/D in the ipsilateral eye and the contralateral eye was 0.30 (0.30, 0.35) and 0.7 (0.6, 0.8) respectively in SWS cases with secondary glaucoma. Conclusions: There is a high proportion of SWS with glaucoma in ophthalmic division affected PWS patients. Fundus examinations were necessary for this type of patients. (Chin J Ophthalmol, 2017, 53:753-757) .