色素血管性斑痣性错构瘤及其研究进展

色素血管性斑痣性错构瘤(phacomatosis pigmentovascularis,PPV)是以全身皮肤弥漫血管畸形合并全身皮肤弥漫色素增多为特征的先天性综合征,病变还可累及颅脑、眼部、全身静脉、骨骼等多个器官。PPV发病极为罕见,眼部表现特殊,可表现为青光眼、眼周皮肤鲜红斑、视网膜血管异常、脉络膜血管瘤、眼黑变、...     

Histopathological study of a case with glaucoma due to Sturge-Weber syndrome

PURPOSE: To investigate the cause of the secondary glaucoma in a case of Sturge-Weber syndrome by histopathology. CASE: A 10-year-old boy with Sturge-Weber syndrome and glaucoma in the right eye was studied. Trabeculectomy was performed because of uncontrolled intraocular pressure, and the trabeculectomy specimen was examined histologically by both light and electron microscopy. RESULTS: Histological examination of the trabeculectomy specimen showed that the ciliary muscle was dislocated anteriorly, and the Schlemm canal was not present. The spaces in the juxtacanalicular connective tissue (JCT) were replaced by vascular structures and connective tissue. There were two kinds of vascular structures: in one, the endothelium was surrounded by pericytes; and in the other, the endothelium was not surrounded by pericytes. CONCLUSIONS: Developmental abnormalities of the Schlemm canal and the JCT may have caused the glaucoma. These observations suggest that the developmental abnormalities of both the mesoderm and the neural crest might be involved in the pathogenesis of the glaucoma in cases of Sturge-Weber syndrome.     

A case of acute angle-closure glaucoma secondary to posterior scleritis in patient with Sturge-Weber syndrome

BACKGROUND: Sturge-Weber syndrome has been known to be frequently associated with facial cutaneous angioma and ipsilateral glaucoma. However, as far as we know, no cases accompanied by acute angle-closure glaucoma have been reported in patients with Sturge-Weber syndrome. CASE: A 14-year-old boy with unilateral acute angle-closure glaucoma secondary to posterior scleritis associated with Sturge-Weber syndrome is described. OBSERVATIONS: Slit-lamp examination revealed diffuse episcleral venous hemangioma in the right eye. With ultrasound biomicroscopy, a forward shift of the lens-iris diaphragm, a swelling of the ciliary body, and an anterior rotation of the ciliary processes with annular choroidal effusion were detected. The patient responded well to treatment with systemic corticosteroids and cycloplegics. CONCLUSIONS: In our patient, inflammatory changes of the sclera, including swelling of the ciliary body, choroidal effusion, an anterior rotation of the ciliary processes at the scleral spur, and swelling of the lens, leading to closure of the anterior chamber angle, were suggested to be the major mechanisms of intraocular pressure elevation.     

Histopathological study of a case with glaucoma due to Sturge-Weber syndrome]

PURPOSE: To investigate by histopathology the cause of secondary glaucoma due to Sturge-Weber syndrome. CASE: A 10-year-old boy with Sturge-Weber syndrome and glaucoma in the right eye is reported. Trabeculectomy was performed because of uncontrolled intraocular pressure and the trabeculectomy specimen was examined histologically by both light and electron microscopy. RESULTS: Histological examination of the trabeculectomy specimen showed the ciliary muscle dislocated anteriorly and there was no Schlemm's canal. The spaces in juxtacanalicular connective tissue(JCT) were replaced by vascular structures and connective tissue. There were two kinds of vascular structures, one where the endothelium was surrounded by pericytes and the other where it was not surrounded by pericytes. CONCLUSIONS: Developmental abnormalities of Schlemm's canal and JCT may have caused glaucoma in this case. These observations suggested that development of both mesoderm and neural crest might be involved in the pathogenesis of glaucoma due to Sturge-Weber syndrome.     

Latanoprost in glaucoma associated with Sturge-Weber syndrome: benefits and side-effects.

PURPOSE: This study was conducted to evaluate the ocular hypotensive efficacy, safety, and side effects of latanoprost 0.005% administered as adjunctive therapy in patients with Sturge-Weber syndrome (SWS) and glaucoma. METHODS: Commercially available latanoprost 0.005% was added as a single drop once daily to other antiglaucoma medications. Intraocular pressure (IOP) was measured at 1, 3, and 6 months of treatment. A successful response was defined as a reduction of at least 20% in IOP at the final follow-up evaluation without additional medical or surgical therapy and no adverse events related to latanoprost. RESULTS: 18 eyes of 18 patients with SWS and glaucoma were enrolled from 9 clinical centers. Mean baseline IOP was 28.4 +/- 7.1 mmHg (range, 17-42 mmHg). Using Kaplan-Meier analysis, a successful response to latanoprost was observed in 3 of 18 (16.7%) patients at the 6-month interval. Seven (38.9%) patients required surgery; three (16.7%) patients required additional medical therapy, seven (38.9%) patients had no change in therapy. One (5.6%) patient discontinued latanoprost treatment because of intolerable conjunctival hyperemia. Two successfully treated patients had significantly greater episcleral vessel engorgement after initiation of latanoprost therapy. CONCLUSION: Patients with SWS and glaucoma respond poorly to adjunctive latanoprost therapy and often require additional medical or surgical intervention. Increased episcleral vascular engorgement might result in greater operative risks should filtration surgery become necessary in these patients.     

The pathogenesis of glaucoma in Sturge-Weber syndrome

What is the cause of glaucoma in Sturge-Weber syndrome? Looking for the answer to this puzzling question, we examined 21 patients with the disease. Sixteen patients had gglaucoma: three bilateral and 13 unilateral. Episcleral hemangiomas were visible in all glaucomatous eyes. In general, the more extensive the hemangioma, the more severe was the glaucoma. During gonioscopy, blood could easily be made to reflux into Schlemm's canal of glaucomatous eyes. Often the canal separated into multiple fine channels. Episcleral venous pressure, which we measured in 11 patients, was high in all glaucomatous eyes. These observations suggest that glaucoma in Sturge-Weber syndrome is caused by elevated episcleral venous pressure. Most likely, veins draining aqueous from the canal of Schlemm are part of an intrascleral or episcleral hemangioma. The canal of Schlemm itself may be part of the hemangioma. Arteriovenous shunts in the hemangioma raise episcleral venous pressure, which in turn elevates intraocular pressure.     

Sturge-Weber综合征伴发青光眼临床特征

目的　Ｓｔｕｒｇｅ－ Ｗｅｂｅｒ 综合征伴发青光眼的临床特征。方法　对１９７８ 年１ 月至１９９９年３ 月收治的１９ 例２２ 眼ＳＷＳ青光眼患者的一般状况、眼部表现、以及伴发青光眼的治疗和预后进行回顾性分析。结果　平均患病年龄１０－７ａ,１９ 例２２ 眼均伴发青光眼,以开角型青光眼为主,伴中胚叶组织残留。３ 例双眼患病,３ 例伴脉络膜血管瘤。药物降压治疗效果不佳,行手术治疗共１８ 例,手术方式有小梁切开术、小梁切除术、巩膜下灼瘘术、虹膜嵌顿术、睫状体冷冻术。眼压从术前平均３５－４５ｍ ｍＨｇ（１ｍ ｍＨｇ＝ ０ ．１３３ｋＰａ） 降至术后平均１８－２４ｍ ｍＨｇ。主要手术并发症为脉络膜上腔出血,前房出血,术后浅前房,脉络膜脱离等。结论　Ｓｔｕｒｇｅ－Ｗｅｂｅｒ 综合征伴发青光眼发生率高,青光眼治疗预后差,与诊断发现较晚有关,提示临床应注意Ｓｔｕｒｇｅ－ Ｗｅｂｅｒ 综合征中青光眼的早期诊断。     

Two-staged Baerveldt glaucoma implant for childhood glaucoma associated with Sturge-Weber syndrome

PURPOSE: To report the outcome and complications of 10 eyes of 9 children with Sturge-Weber syndrome (SWS) who underwent two-stage insertion of a Baerveldt glaucoma implant (BGI) for glaucoma. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: The authors reviewed the medical records of children under the age of 14 years with SWS who underwent two-stage BGI for glaucoma at two tertiary care referral centers. MAIN OUTCOME MEASURES: Intraoperative and postoperative complications, intraocular pressure (IOP), number of glaucoma medications, visual acuity, and further surgical intervention. RESULTS: Ten eyes of nine patients were included in the study. Ages of the nine patients at time of first stage BGI ranged between 6 weeks and 13 years. With average follow-up of 35 months (range, 10-50), all eyes had adequate IOP control (< or = 21 mmHg) without the need for additional glaucoma surgery. Intraocular pressure was reduced from a mean (+/- standard deviation) of 24.8 +/- 6.2 mmHg preoperatively to 16.9 +/- 2.3 mmHg at last follow-up visit (P = 0.001). The number of medications used for control of glaucoma was reduced from a mean (+/- standard deviation) of 1.8 +/- 1.0 preoperatively to 1.1 +/- 1.4 at last follow-up visit (P = 0.2). One eye had serous choroidal effusions with overlying serous retinal detachment that resolved spontaneously after 7 days with no permanent visual loss, and one eye had low choroidal effusion that lasted 4 days. There were no intraoperative or postoperative suprachoroidal hemorrhages. At last follow-up, visual acuity had improved by one or more lines in all patients in whom vision was measurable. CONCLUSIONS: Two-stage BGI surgery appears to be a safe and effective treatment for refractory glaucoma in children with SWS.     

Primary combined trabeculotomy-trabeculectomy for early-onset glaucoma in Sturge-Weber syndrome.

OBJECTIVE: To evaluate the safety and efficacy of primary combined trabeculotomy-trabeculectomy in the management of early-onset glaucoma associated with Sturge-Weber syndrome (SWS). DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Ten eyes of nine patients were included in this study. All patients with SWS who underwent primary combined trabeculotomy-trabeculectomy from January 1993 through December 1996 were included. One patient had bilateral surgery. INTERVENTION: Primary combined trabeculotomy-trabeculectomy. MAIN OUTCOME MEASURES: Pre- and postoperative intraocular pressures (IOPs), corneal clarity and diameters, visual acuities, success rate, bleb characteristics, time of surgical failure (if any), and complications. RESULTS: The mean preoperative IOP was 28.2 mmHg +/- 7.35 mmHg with medication (mean, 1.2 +/- 0.6; range, 0-2). The mean postoperative IOP was 11.8 mmHg +/- 1.8 mmHg, with a mean percent reduction of 55.8 +/- 12.6 in IOP (P < 0.0001). All eyes maintained a postoperative IOP < 16 mmHg without medication over a mean follow-up of 27.6 +/- 16.4 months (range, 12-64 months). Normal corneal clarity was achieved in all eight eyes that had corneal edema. There were no significant intraoperative complications. Postoperatively, one patient developed a shallow anterior chamber with choroidal detachment, which was successfully managed conservatively. CONCLUSION: Primary combined trabeculotomy-trabeculectomy is safe, effective, and sufficiently predictable to be considered the first choice of surgical treatment in early-onset glaucoma associated with SWS.     

Sturge-Weber syndrome: glaucoma with elevated episcleral venous pressure

The present report describes clinical findings in 13 patients with Sturge-Weber syndrome, 11 of whom had glaucoma (22-52 mm Hg). The episcleral venous pressure (EVP) was high in all glaucomatous eyes (14-23 mm Hg). Tonography showed a marked decrease in outflow facility. The observations suggest that glaucoma in Sturge-Weber syndrome is caused by a) elevated episcleral venous pressure and b) secondary damage of the trabecular meshwork with reduction of outflow facility. The cause and pathogenesis of these lesions are discussed. In nine patients a diffuse angiomatosis involving most of the choroid (1.5-4.0 mm thick), as well as the episcleral perilimbal plexus, was demonstrated by ultrasonography.     

Ahmed glaucoma valve implant for management of glaucoma in Sturge-Weber syndrome

PURPOSE: To evaluate the safety and efficacy of the Ahmed glaucoma valve implant in patients with glaucoma as a result of Sturge-Weber syndrome. METHODS: Eleven eyes (10 patients) with glaucoma resulting from Sturge-Weber syndrome had placement of an Ahmed glaucoma valve implant from May 1993 to June 1996 at the Jules Stein Eye Institute. Success was defined by intraocular pressure at the last two consecutive visits of less than 21 mm Hg, no additional glaucoma surgery, no expulsive choroidal hemorrhage, and no retinal detachment. RESULTS: Mean intraocular pressure on the first postoperative day was 14.0 mm Hg (SD +/- 6.7). The cumulative probability of success was 79% (95% confidence interval [CI], 52% to 100%) at 24 months, 59% (95% CI, 20% to 98%) at 42 months, and 30% (95% CI, 0% to 75%) at 60 months. CONCLUSIONS: On the basis of limited follow-up, the Ahmed glaucoma valve implant appears to be a relatively useful drainage device in eyes with glaucoma resulting from Sturge-Weber syndrome.     

Trabeculectomy with cyclocryotherapy for infantile glaucoma in the Sturge-Weber syndrome

Various mechanisms have been proposed to explain the pathogenesis of infantile glaucoma in the Sturge-Weber syndrome. We report the results of treating six patients with combined trabeculectomy and cyclocryotherapy. Successful control of the intraocular pressure occurred in all patients. Two patients required a second procedure at another site. We feel that the success of this procedure provides evidence that the glaucoma is secondary to elevated episcleral venous pressure in this condition.     

Non-penetrating deep sclerectomy for glaucoma associated with Sturge-Weber syndrome

PURPOSE: To report the results of non-penetrating deep sclerectomy (NPDS) in the treatment of glaucoma associated with Sturge-Weber syndrome (SWS). METHODS: We carried out a retrospective case series analysis of patients who underwent NPDS for glaucoma associated with SWS between 1998 and 2003. The control of glaucoma after NPDS, the results of surgery on intraocular pressure, the need for additional medical treatment and surgical complications were studied. RESULTS: Twelve eyes of nine patients, aged 11 days to 24 years, underwent filtering surgery: nine NPDS procedures were performed and three surgical procedures had to be converted to trabeculectomy because NPDS was not technically achievable. The mean follow-up after surgery was 26.3 months (range 6-48 months). Two trabeculectomies were complicated by choroidal effusion, which resolved in both cases. Good control of glaucoma was obtained during follow-up. CONCLUSIONS: Non-penetrating deep sclerectomy is transiently efficient in the treatment of SWS-associated glaucoma. Further studies of NPDS for the treatment of glaucoma associated with SWS are warranted.     

重视Sturge-Weber综合征继发青光眼的治疗

Sturge-Weber综合征继发青光眼系难治性青光眼之一.目前对其治疗存在的主要问题是,药物治疗效果较差且存在潜在副作用,手术操作难度大,术中及术后脉络膜渗漏等严重并发症的发生率较高,术后远期瘢痕化严重.在手术治疗时应根据患者的具体情况注重手术适应证的选择、完善手术前检查、采取多种措施预防手术并发症的发生.对现有的手术方式、远期效果与安全性应开展前瞻性多中心临床试验进行验证.     

Analysis of surgical and medical management of glaucoma in Sturge-Weber syndrome

Management of glaucoma associated with Sturge-Weber syndrome (SWS) is difficult. The authors reviewed 36 eyes of 30 SWS patients with either early or late-onset glaucoma with a mean follow-up of 122 months (range, 24-253 months). Intervals between required surgical or medical interventions were analyzed. Intervention was attributed to elevated intraocular pressure (IOP) in 55% of cases and disc change in 45%. Median stable postoperative interval with goniotomy was 12 months; with trabeculotomy, 21 months; with trabeculectomy, 34 months; with argon laser trabeculoplasty, 25 months; and with medications, 57 months. Survival analysis shows statistically significant differences between goniotomy and medications. Intraoperative choroidal expansions developed in 24% of cases receiving a trabeculectomy, and none developed with either goniotomy or trabeculotomy.