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.     

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.     

Dislocation of the crystalline lens in a patient with Sturge-Weber syndrome.

A moderately retarded institutionalized patient diagnosed with Sturge-Weber syndrome and bilateral retinitis pigmentosa underwent enucleation OD because of intractable pain secondary to glaucoma in 1965. Twenty-three years later, the patient had a dislocated lens OS. Although the lens dislocation might have been caused by trauma, it also might be associated with either Sturge-Weber syndrome or retinitis pigmentosa. The role of protective eye wear in one-eyed institutionalized retarded patients is discussed. To our knowledge, this combination of Sturge-Weber syndrome, bilateral retinitis pigmentosa, and a dislocated lens has not been reported previously.     

Congenital Glaucoma from Sturge-Weber Syndrome: A Modified Surgical Approach

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder that causes congenital glaucoma. Previous experiences have shown that drainage procedures are often required to control associated glaucoma. The conventional surgical approach in trabeculectomy carries a significant risk of intraoperative expulsive hemorrhage. Here, we describe a modified approach of the conventional trabeculectomy technique, which may lower the risk of expulsive hemorrhage. A viscoelastic device was employed to maintain a steady intraocular pressure throughout the procedure. Details of the surgical technique and material used are described. One patient with congenital glaucoma associated with SWS underwent a successful trabeculectomy using the modified technique. Postoperative intraocular pressure was successfully reduced and no intraoperative complications occurred. We describe a successful case of trabeculectomy in a SWS case where a modified technique was applied.     

Assessing the need for posterior sclerotomy at the time of filtering surgery in patients with Sturge-Weber syndrome

PURPOSE: Posterior sclerotomy has been recommended for prevention of intraoperative choroidal hemorrhages and choroidal effusions in patients with Sturge-Weber syndrome (SWS) or Klippel-Trenaunay-Weber (KTW) syndrome undergoing glaucoma filtering surgery. In this study, we evaluated this unproven clinical perception. DESIGN: A retrospective, noncomparative, case series. PARTICIPANTS: Seventeen consecutive patients with SWS or KTW syndrome who underwent glaucoma filtering surgery without prophylactic posterior sclerotomy or other prophylactic measures between January 1973 and March 1997 at a university-based practice. INTERVENTION: Glaucoma filtering surgery without prophylactic posterior sclerotomy. MAIN OUTCOME MEASURES: Incidence of intraoperative and postoperative choroidal effusion, choroidal detachment, or choroidal hemorrhage. RESULTS: No intraoperative choroidal effusion, choroidal detachment, or choroidal hemorrhage occurred in this series. After surgery, 6 patients had a transient choroidal effusion. Surgical drainage was not required in any of them. No suprachoroidal hemorrhages occurred after surgery. CONCLUSIONS: We did not encounter significant intraoperative suprachoroidal hemorrhage, choroidal effusions, or a combination requiring therapeutic intervention in our series. This finding leads us to question the necessity for prophylactic posterior sclerotomy to prevent the occurrence of these complications in patients with SWS and KTW syndrome undergoing glaucoma filtering surgery.     

Ocular findings in Sturge-Weber syndrome

PURPOSE: We reviewed the rare ocular findings of Sturge-Weber syndrome (SWS) and the results of implantation of the Ahmed valve in cases associated with glaucoma. METHODS: Seven patients (range 18 to 52 years) diagnosed as SWS were reviewed as clinical findings over two years. An anterior chamber maintainer was placed in patients with glaucoma to maintain stable intraocular pressure and minimize the risk of intraocular hemorrhage due to sudden pressure changes during surgery, when an Ahmed valve is implanted. RESULTS: Episcleral venous vessels were prominent in all cases. Diffuse choroidal hemangiomas were seen in three cases. Nevus of Ota was observed in only one case. Three cases had juvenile glaucoma. One also had buphthalmos. No intraocular hemorrhage or choroidal effusion was observed intraoperatively in valve-implant patients. The surgical treatment of the patients with glaucoma gave favorable outcomes. CONCLUSIONS: Rare ocular findings such as choroidal hemangioma and nevus of Ota are sometimes seen in SWS. Drainage valve implantation, with an anterior chamber maintainer, is a good choice for treatment when surgery is done in cases with glaucoma. This method may reduce the risk of intraoperative suprachoroidal effusion and expulsive hemorrhage by stabilizing intraocular pressure within normal limits during the surgery.     

The evaluation of the efficacy of treatment in glaucoma associated with Sturge-Weber syndrome

PURPOSE: To estimate the efficiency of glaucoma treatment in Sturge-Weber syndrome. MATERIAL AND METHODS: 4 patients with Sturge-Weber syndrome and the consecutive glaucoma seen at the Department of Ophthalmology in Bialystok between the years 1999-20005, were reviewed. Glaucoma associated with Sturge-Weber syndrome was diagnosed at the age of 6 months in 1 patient, of 10-11 years in 2 patients and of 14 years in 1 patients. All patients underwent surgery. 2 eyes underwent trabeculectomy and 2 eyes had nonpenetrating deep sclerectomy with SKGel implant. RESULTS: Before the surgery the intraocular pressure was 35.2 mmHg (range from 25 to 48 mmHg). All patients required anti-glaucoma medications after surgery to keep intraocular pressure less than 22 mmHg. After surgery the mean intraocular pressure was 26.25 mmHg (range from 22 to 32 mmHg). 2 patients required 2 medications: Betoptic, and/or Trusopt, and/or Xalatan and 2 patients required 1 medication. After antiglaucoma medications mean IOP was 15.2 mmHg (range from 12 to 18 mmHg). CONCLUSION: The results of therapy for glaucoma associated with the Sturge-Weber syndrome are often disappointing.     

Glaucoma in Sturge-Weber syndrome

Trabeculectomy specimens from three eyes with Sturge-Weber syndrome were examined histopathologically. Changes in the trabecular meshwork-Schlemm's canal system were similar to findings in old age and in primary open-angle glaucoma. Two mechanisms for glaucoma are theorized. In cases with buphthalmos and congenital glaucoma, the chamber angle is often anomalous, as in other types of congenital glaucoma. In later onset juvenile cases, the chamber angle more often appears normal. A premature aging of the trabecular meshwork Schlemm's canal complex, as shown by us histopathologically, is a primary cause of juvenile glaucoma. It is suggested that both mechanisms relate to the abnormal hemodynamics of episclera and chamber angle, due to persistence of Streeter's primordial vascular plexus.     

The efficacy of goniotomy/trabeculotomy in early-onset glaucoma associated with the Sturge-Weber syndrome

PURPOSE: To assess the efficacy of goniotomy/trabeculotomy as the initial surgical procedure in early-onset glaucoma associated with Sturge-Weber syndrome. METHODS: We retrospectively analyzed 16 eyes of 14 consecutive patients with Sturge-Weber syndrome-associated glaucoma diagnosed before 4 years of age. All subjects were seen at a single institution from 1978 to 1996 and underwent goniotomy or trabeculotomy as their initial surgical procedure. RESULTS: Twelve eyes underwent initial goniotomy, and 4 eyes underwent initial trabeculotomy. One subject was lost to follow-up after surgery, resulting in 15 eyes for analysis. Of the initial goniotomy eyes, two thirds required a second surgical procedure. In the initial trabeculotomy eyes, half required a second procedure. Intraocular pressure was controlled (intraocular pressure < or = 22 mm Hg) in 66.7% of the eyes (10 of 15) after one or more goniotomy or trabeculotomy procedures for a median follow-up of 5.4 years (range, 1.4 to 15 years). For eyes with only one surgical procedure, 4 of 6 eyes had controlled intraocular pressure over a median follow-up of 3.4 years (range, 3 to 12 years). Seven of the 9 eyes that required more than one procedure had controlled intraocular pressure after all procedures over a median follow-up of 4.5 years (range, 1.4 to 15 years). CONCLUSION: Initial or repeated goniotomy or trabeculotomy may be an effective management choice for treatment of glaucoma associated with Sturge-Weber syndrome presenting in early childhood.     

Late-onset pediatric glaucoma associated with cutis marmorata telangiectatica congenita managed with Molteno implant surgery: case report and review of the literature.

Cutis marmorata telangiectatica congenita (CMTC) is characterized by the appearance of telangiectasia, phlebectasia, and a persistent reticular pattern of subcutaneous vasculature at or soon after birth. Up to 90% of cases are associated with systemic abnormalities, which include body asymmetry, cutaneous atrophy, neurological abnormalities, and vascular anomalies (nevus flammeus, Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and capillary and cavernous hemangiomas). Glaucoma is the most commonly reported ocular association of CMTC, usually presenting in infancy. We report a case of CMTC associated with the previously unreported onset of glaucoma in mid-childhood that was managed by glaucoma drainage implant surgery and review the literature on glaucoma associated with this condition.     

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.     

Childhood glaucoma associated with Sturge-Weber syndrome --the efficacy of cyclofotocoagulation and other therapeutic methods

PURPOSE: Sturge-Weber syndrome is a rare congenital neurooculocutaneous disorder. Ocular involvement can include glaucoma and vascular malformations of the conjunctiva, episclera, choroid and retina. MATERIAL AND METHODS: 16 children (16 eyes) with Sturge-Weber syndrome associated with glaucoma (mean age--34 month, mean follow up 8.87 years) treated in our institution, were reviewed. In retrospective analysis were assessed: IOP and postoperative complications after diode laser cyklophotocoagulation (16 eyes), after trabeculectomy (6 eyes) and trabeculectomy with MMC (3 eyes). RESULTS: In ten eyes (62.5%) good result (IOP 6-22 mmHg) post cyclophotokoagulation--(3x) was recorded, as well as in 3 eyes (50%) post trabeculectomy and in 3 eyes (100%) post trabeculectomy with MMC 0.2/4 min. No postoperative complications occured in cyclophotocoagulation group. Complications after trabeculectomy were the following: 3 cases of hypotony, 4 cases with shallow anterior chamber and 5 with choroidal effusions. CONCLUSIONS: Diode laser cyclophotocoagulation combined with topical medication is an effective and safe treatment of glaucoma in children with Sturge-Weber syndrome.     

Sturge-weber syndrome with spina bifida: An unusual association

A 12-year-old girl with a port-wine hemangioma on the left side of her face reported worsening vision in the left eye. Intraocular pressure was 34 mm Hg. On the basis of the port-wine hemangioma, optic disc cupping, diffuse choroidal hemangioma, and gyrate calcification, a diagnosis of Sturge-Weber syndrome with juvenile glaucoma was made. The patient also had spina bifida, a previously undescrided association with Sturge-Weber syndrome. Her glaucoma was controlled with medications. The authors have stated that they do not have a significant financial interest or other relationship with any product manufacturer or provider of services discussed in this article.     

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.     

Ciliochoroidal effusion induced by topical latanoprost in a patient with sturge-weber syndrome

BACKGROUND: To report drug-induced ciliochoroidal effusion in a patient with Sturge-Weber syndrome.CASE: A 17-year-old man presented with unilateral glaucoma associated with Sturge-Weber syndrome.OBSERVATIONS: His corrected visual acuity was RE 20/20 and LE 40/60. Intraocular pressure readings by Goldmann applanation tonometry were RE 32 mm Hg and LE 12 mm Hg. Fundus examination showed marked glaucomatous disc cupping in his right eye and normal finding in his left. The patient had a port-wine stain on his right upper eyelid ipsilateral to the glaucomatous eye. Antiglaucomatous medications were begun, including topical latanoprost, with a diagnosis of juvenile onset glaucoma associated with Sturge-Weber syndrome. Ultrasound biomicroscopy showed a 360 degrees circumference ciliochoroidal effusion. Forty days after starting medication, latanoprost treatment was discontinued. Ten days later, ultrasound biomicroscopy showed a total disappearance of the ciliochoroidal effusion.CONCLUSION: Interaction of the enhanced uveoscleral outflow with latanoprost in conjunction with elevated episcleral venous pressure may have caused the congestion of the aqueous humor in the supraciliary-choroidal space, resulting in the ciliochoroidal effusion.     

Congenital glaucoma

Congenital and juvenile glaucoma are associated with goniodysgenesis and currently thought to be the result of neural crest cell abnormal terminal induction or migration. Infantile glaucoma may be primary, or may be associated with syndromes such as Sturge-Weber, Rieger's and others. Differentiation from other childhood causes of cloudy cornea such as endothelial dystrophies is essential. Evaluations under anesthesia are often needed and present their own problems in diagnosis.     

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.     

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、...     

Low Dose Irradiation for Diffuse Choroidal Hemangioma

Sturge-Weber Syndrome is a nonheritable congenital syndrome characterized by a “port-wine stain” on the face and angioma of the meninges. Ocular findings include diffuse choroidal hemangioma, retinal detachment, and various types of glaucoma. Management of diffuse choroidal hemangioma is aimed at preserving the affected eye and preventing glaucoma. In the past this has been challenging. Herein, we describe a case of Sturge-Weber Syndrome with diffuse choroidal hemangioma which was successfully treated with low dose lens-sparing external beam radiotherapy.     

THE STURGE-WEBER SYNDROME Encephalofacial Angiomatosis – Report of a Case

Sturge-Weber Syndrome is reviewed with observations on a recent theory as to the pathogenesis of glaucoma in this syndrome. It is considered that raised episcleral venous pressure is the cause of glaucoma which is best treated with a filtering operation.
A baby seen at the age of six months and who died at the age of 15 months is described together with post mortem findings.