Visual rehabilitation in a child with diffuse choroidal hemangioma by using aggressive amblyopia therapy with low-dose external beam irradiation.

Diffuse choroidal hemangioma is a congenital vascular hamartoma often associated with hemangiomatous lesions of the brain, orbit, and periocular skin (nevus flammeus) in the Sturge-Weber syndrome. Visual loss from diffuse choroidal hemangioma may result from chronic serous retinal detachments causing retinal pigment epithelial, photoreceptor or cystoid degeneration, and glaucomatous optic atrophy. Low-dose external beam irradiation has successfully resolved exudative retinal detachment and caused shrinkage of the choroidal hemangioma.(1-3) Visual loss in Sturge-Weber syndrome with diffuse choroidal hemangioma often begins during amblyogenic years. Visual rehabilitation may thus require not only therapeutic intervention to address organic disease but also amblyopia therapy. Though many of the previously reported cases address treatment of the diffuse choroidal hemangioma with laser or radiotherapy, none advocate or emphasize treatment of nonorganic amblyopia associated with this condition. We report the case of a child with Sturge-Weber syndrome and unilateral diffuse submacular choroidal hemangioma who developed an exudative retinal detachment that responded to the combination of low-dose external beam irradiation and aggressive amblyopia therapy.     

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.     

Bilateral diffuse choroidal hemangiomas with unilateral facial nevus flammeus in Sturge-Weber syndrome

PURPOSE: To report bilateral choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome. METHODS: Case report. RESULTS: A 6-year-old male with a left facial nevus flammeus and a history of a left cerebral angioma had been followed 2 years for increasing esotropia. Examination demonstrated bilateral diffuse choroidal hemangiomas with overlying exudative retinal detachments. After bilateral external beam radiotherapy, the retinal detachments resolved and vision improved. CONCLUSION: Patients with Sturge-Weber syndrome and unilateral facial nevus flammeus may harbor bilateral choroidal hemangiomas. Clinical manifestations of the Sturge-Weber syndrome are characteristically unilateral and ipsilateral to the facial nevus flammeus. Bilateral choroidal hemangiomas associated with bilateral facial nevus flammeus are rare. 1,2 We report bilateral diffuse choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome.     

Sturge-Weber Syndrome Associated with Early Diffuse Choroidal Hemangiaoma： A Case Report

Purpose:To report indocyanine green angiography (ICGA) findings in one patient of diffuse choroidal hemangioma associated with Struge-Weber syndrome. Methods: Color fundus photography, fluorescein angiography (FA) and ICGA were performed in a patient with diffuse choroidal hemangioma associated with Sturge-Weber syndrome, Results: Three findings were unveiled by ICGA : rapid filling of diffuse choroidal hemangio‘s vascular network in the early stages; diffuse hyperfluorescence visual up to the late phase; no “wash-out“ phenomenon was observed in the late phase. Conclusion:Indocyanine green angiography can provide information that is not detected by clinical or fluorescence angiographic examination in the patient with Sturge-Weber syndrome. ICGA may be important and sensitive in detecting the diffuse choroidal hamangioma associated with Sturge-Weber syndrome. Eye Science 2004:20:168-170     

Indocyanine green angiographic findings in diffuse choroidal hemangioma associated with Sturge-Weber syndrome

Purpose: To analyze indocyanine green angiography (ICGA) features in one case of diffuse choroidal hemangioma associated with Sturge-Weber syndrome. Methods: Color fundus photography, fluorescein angiography (FA) and ICGA were performed in a patient with diffuse choroidal hemangioma associated with Sturge-Weber syndrome. Results: The diffuse choroidal hemangioma was not identified by FA. ICGA revealed that the vascular tissue of the diffuse hemangioma filled rapidly with dye during the arterial phase of the choroidal angiogram; copious dye leakage appeared early and persisted into the late phase of angiography. Late ”wash-out” phenomenon was not observed 30 minutes after dye injection. Sectors of reduced choroidal perfusion in the upper or lower half of the midperiphery were present. Conclusion: ICGA may be an important and sensitive technique in detecting the diffuse choroidal hemangioma associated with Sturge-Weber syndrome. Received: 25 November 1999 Revised: 1 February 2000 Accepted: 3 February 2000     

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、...     

A case of circumscribed choroidal hemangioma in Sturge-Weber syndrome in China

We present a case of circumscribed choroidal hemangioma (CCH) in Sturge-Weber syndrome in a 30-year-old woman with congenital port-wine stains on the left side of face involving the upper eyelid, cheek and the nose, and she had undergone facial hemangioma surgery 3 years ago suggestive of Sturge-Weber syndrome. She presented with a 1-month history of rapidly decreased visual acuity (VA) to counting fingers in the left eye which had no prior history of visual problem. And there was no evidence of glaucoma. At 3 months after the treatment of the standard photodynamic therapy (PDT) the VA was 20/200. For some reasons, we have no idea about the changes of tumor thickness and subretinal fluid. We confirmed the curative effect of PDT treatment for CCH because of the significantly improved VA in the bad eye.     

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.     

Glaucoma and expulsive hemorrhage mechanisms in the Sturge-Weber syndrome.

An eye from an adult with Sturge-Weber syndrome and glaucoma is described histologically following enucleation for massive choroidal hemorrhage. The specimen is noted to contain hemangiomas of the episclera and choroid as well as a partial angle cleavage abnormality. Histologic evidence reviewed herein supports the concept that infantile glaucoma associated with this syndrome may be associated with marked filtration angle cleavage deformities. Some Sturge-Weber patients who develop unilateral glaucoma later as juveniles or adults appear to have milder expressions of angle cleavage abnormality or a forme fruste of this angle disorder. Any surgical procedure for this glaucoma preferably should be one which minimizes or eliminates operative hypotony, thus avoiding a possible expulsive hemorrhage.     

Resolution of Persistent Exudative Retinal Detachment in a Case of Sturge-Weber Syndrome with Anti-VEGF Administration

Purpose: Report the resolution of a persistent exudative retinal detachment in a patient with Sturge-Weber syndrome following intravitreal pegaptanib injection. Design: Case report. Methods: A 13-year-old male with Sturge-Weber syndrome presented with a choroidal hemangioma associated with an exudative retinal detachment that failed to resolve 6 months after external beam radiation therapy. Results: A single intravitreal pegaptanib injection resulted in 50% resolution of the detachment within 1 week and complete resolution within a month. Despite anatomic success, vision remained poor. Conclusions: VEGF likely plays a role in the pathogenesis of Sturge-Weber choroidal hemangioma-associated exudative retinal detachment and offers potential treatment.     

Hemangioma of the choroid. A clinicopathologic study of 71 cases and a review of the literature

This is a clinicopathologic study of 71 hemangiomas of the choroid. The cases were divided into three groups: Group 1 consisted of 45 cases of solitary choroidal hemangiomas (not related to any systemic disease); Group 2 comprised 17 cases with unequivocal evidence of Sturge-Weber syndrome; and Group 3 included 9 cases, 6 of which were classified as “probably Sturge-Weber syndrome.” Clinically, many differences were found that established a clear distinction between Groups 1 and 2. Histopathologically, the solitary hemangiomas were well-circumscribed tumors that showed a sharply demarcated pushing margin causing compression of melanocytes and choroidal lamellae. About 70% of these tumors were located temporally (at the posterior pole). In contrast, the lesions in the Sturge-Weber syndrome showed a diffuse angiomatosis involving more than one-half of the choroid, as well as the episcleral and intrascleral perilimbal plexuses. The cause and pathogenesis of these lesions are discussed.     

Choroid effusion in an antiglaucoma operation in a child with Sturge-Weber syndrome

The authors describe a case of expulsive choroidal effusion which occurred in the course of a fistulating operation in a child with Sturge-Weber syndrome. This is the youngest patient with this complication so far reported in the literature. The persistent postoperative choroidal and retinal detachments in this case led to a considerable diminution of visual acuity.     

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.     

Sturge-Weber syndrome: medical management of choroidal hemangiomas

PURPOSE: To investigate the outcome of irradiation of complicated choroidal hemangiomas in Sturge-Weber syndrome. PATIENTS AND METHODS: The charts of 6 patients (7 eyes) with Sturge-Weber syndrome and choroidal hemangiomas were reviewed. An exudative retinal detachment was the indication for treatment in all cases. The mean age of the 6 patients was 13 years (range, 4 to 20 years). The minimum follow-up time was 1 year. Patients were checked for initial and final best-corrected visual acuity, fundus examination, fluorescein angiography, and tumor thickness on B-scan ultrasonography. The patients were treated with radiotherapy. A total dose of 20 Grays was applied to 7 eyes: 2 with a circumscribed choroidal hemangioma underwent proton therapy and 5 with diffuse hemangioma were treated by external beam irradiation. RESULTS: Complete resolution of the subretinal fluid was achieved in all cases with the tumor height decreased. Visual acuity improved to 1 line or more in 5 eyes and remained stable in 2 eyes. Two cases that underwent proton therapy developed radiation retinopathy. CONCLUSION: External beam radiation is an effective and safe option in the management of choroidal hemangiomas complicated by retinal detachment. Based on our experience, proton therapy should be reserved for sporadic circumscribed choroidal hemangioma.     

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.     

Central aleolar choroidal dystrophy in sibilings coexisting with alopecia

Central areolar choroidal dystrophy is localized in macular region and is characterized by atrophy of pigment epithelium, photoreceptors and choriocapillaris. This paper presents the history of two sibilings at the age of 23 and 30, with central aleolar choroidal dystrophy coexisting with alopecia. The results of erg, eog and fluorescein angiography are presented. Conclusion: The results of therapy for glaucoma associated with the Sturge-Weber syndrome are often disappointing.     

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.     

Patterns of indocyanine green videoangiography of choroidal tumours.

BACKGROUND--Indocyanine green video-angiography is a recently popularised technique for imaging the retina and choroid. Little is known about the value of this technique in the evaluation of choroidal tumours. METHODS--Indocyanine green video-angiograms of 51 patients with choroidal tumours, including 32 with choroidal melanoma, five with choroidal naevus, four with choroidal metastasis, four with choroidal haemangioma, one with choroidal osteoma, one with choroidal vortex vein varix, and four with irradiated choroidal melanoma were reviewed in a masked fashion. RESULTS--The choroidal melanoma group achieved maximal fluorescence at an average of 18.2 (range 0.4 to 60) minutes after injection. At maximum fluorescence, the pattern varied from hypofluorescent in 17 cases, to isofluorescent in eight cases, and hyperfluorescent in seven cases. Non-pigmented choroidal melanoma generally showed an earlier average onset of fluorescence than the pigmented choroidal melanoma (mean 28 versus 185 seconds, respectively) (p = 0.04). The two subgroups otherwise overlapped substantially in angiography characteristics. The choroidal metastasis group demonstrated maximal fluorescence at an average of 8.9 (range 1.7 to 13) minutes. All choroidal metastases had a homogeneous diffuse fluorescence with late isofluorescence. The choroidal metastases allowed a subtle blurred perception of the normal choroidal pattern through the tumour. The choroidal haemangioma group revealed very characteristic findings with an onset of filigree fluorescence at an average of 0.6 (range 0.4 to 0.7) minutes with little variation. The maximal fluorescence was typically hyperintense in all cases and was achieved at an average of 1.2 minutes and was reached by 1.8 minutes in all cases. In these cases the fluorescence appeared as a lacy diffuse 'fluorescent mulberry' pattern with visibility of the vascular channels and demonstrated 'washout' of the dye in the late frames. CONCLUSION--Indocyanine green may be a useful adjunct in the differentiation of amelanotic choroidal melanoma, choroidal metastasis, and choroidal haemangioma. The different patterns of fluorescence may be explained by the intrinsic choroidal vascular architecture in these three tumours.     

Choroidal hemangioma

Choroidal hemangioma is an uncommon benign vascular tumor of the choroid that can be circumscribed or diffuse. Circumscribed choroidal hemangiomas are usually diagnosed between the second to fourth decade of life when they cause visual disturbances owing to the development of an exudative retinal detachment. Circumscribed tumors occur sporadically, without any associated local or systemic anomalies. Diffuse choroidal hemangiomas are usually evident at birth and generally occur as a part of neuro-oculo-cutaneous hemangiomatosis (Sturge-Weber syndrome).     

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.