Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome.

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Treatment of the capillary vascular malformation (port-wine stain) in Sturge-Weber syndrome with the use of a laser is helpful cosmetically. However, concerns have been raised that laser obliteration of port-wine stains may result in ocular hypertension. The aim of this study was to review clinical features and management of ocular complications of SWS and assess the effects of dermatological laser treatment on the incidence of glaucoma or ocular hypertension.

Related collections

Most cited references 17

Record: found
Abstract: found
Article: not found

Sturge-Weber syndrome: a review.

Sanjeev V. Thomas, Bernard L Maria, D Vaslow (2004)

Sturge-Weber syndrome is a rare disorder that occurs with a frequency of approximately 1 per 50,000. The disease is characterized by an intracranial vascular anomaly, leptomeningeal angiomatosis, most often involving the occipital and posterior parietal lobes. Facial cutaneous vascular malformations, seizures, and glaucoma are among the most common symptoms and signs. Stasis results in ischemia underlying the leptomeningeal angiomatosis, leading to calcification and laminar cortical necrosis. The clinical course is highly variable and some children experience intractable seizures, mental retardation, and recurrent strokelike episodes. In this review, we describe the syndrome's characteristic features, clinical course, and optimal management.

0 comments Cited 43 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

The pathogenesis of glaucoma in Sturge-Weber syndrome.

C Phelps (1978)

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.

0 comments Cited 22 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Treatment of children with port-wine stains using the flashlamp-pulsed tunable dye laser.

O Tan, Logan K. Sherwood, B Gilchrest (1989)

Thirty-five children, three months to 14 years of age, with disfiguring port-wine stains were treated with a flashlamp-pulsed tunable dye laser. All had complete clearing of the stains after an average of 6.5 laser treatments to each lesional area; skin over bony prominences required approximately half as many sessions as skin on the cheek. Children less than seven years old required fewer sessions (mean +/- SD, 5.8 +/- 1.1) than older children (7.1 +/- 1.1; P less than 0.05). Treated skin was identical in texture and color to adjacent normal skin in 33 (94.3 percent) of the children, whereas 2 (5.7 percent) had small, isolated, depressed scars in areas accidentally traumatized soon after laser treatment. The only other side effect was transient hyperpigmentation, which occurred in 20 patients (57 percent). These results can be attributed to two distinguishing characteristics of the flashlamp-pulsed tunable dye laser: an emission wavelength of 577 nm, theoretically ideal for selective absorption by the intravascular target oxyhemoglobin, and a pulse duration of 360 microseconds, which closely matches the thermal relaxation time for dermal blood vessels and hence avoids diffuse nonspecific thermal necrosis with subsequent scarring of the treated skin.