Analysis of Surgical and Medical Management of Glaucoma in Sturge-Weber Syndrome

Management of childhood glaucoma in Sturge Weber Syndrome: A challenge

“…Several modalities of management had been suggested in the literature. Many surgeons prefer goinotomy (Shaffer et al, 1970;Iwach et al, 1990) or trabeculotomy (Rothkoff et al, 1979;Iwach et al, 1990) as the operation of choice but the success rate is less than that for primary congenital glaucoma (Shaffer et al 1970;Rothkoff et al 1979). The relatively low success rates and high risk of choroidal effusion with goniotomy and trabeculotomy has led to the opinion that an additional filtering surgery to bypass the episcleral veins such as Nepal J Ophthalmol 2015;7(14): 194-197 trabeculectomy or aqueous shunting procedure is current approach in patients refractive to medical therapy (Bellows et al 1979, Ali et al 1990Iwach et al 1990;Hamush et al 1999;Budenz et al 2000).…”

Primary single-plate Molteno tube implantation for management of glaucoma in children with Sturge-Weber syndrome

“…The excessive healing process in young patients and the stimulatory effect of abnormal episcleral vessels which enhance scarring may explain the lower success of trabeculectomy in patients with SWS. Despite acceptable results of trabeculectomy in some of these patients, one must consider the higher rate of serious complications, such as expulsive choroidal hemorrhage, choroidal effusion and prolonged flat anterior chamber [5,6]. Cyclocryotherapy alone and combined with trabeculectomy have also been described in patients with SWS with variable results [15,16].…”

“…Those patients that present at birth or during the first year of life are probably secondary to trabecular dysgenesis and are managed with trabeculotomy or goniotomy [3,4]. In older children, the cause of glaucoma is increased episcleral venous pressure, and if the intraocular pressure cannot be controlled with medications, the current approach consists of filtering procedures (trabeculectomy, nonpenetrating deep sclerectomy and tube-shunt surgery) [5][6][7]. Trabeculectomy has been reported to have good short-term results but carries the risk of massive choroidal effusion or expulsive hemorrhage [5,6].…”

“…In older children, the cause of glaucoma is increased episcleral venous pressure, and if the intraocular pressure cannot be controlled with medications, the current approach consists of filtering procedures (trabeculectomy, nonpenetrating deep sclerectomy and tube-shunt surgery) [5][6][7]. Trabeculectomy has been reported to have good short-term results but carries the risk of massive choroidal effusion or expulsive hemorrhage [5,6]. The risk of these complications may be less in nonpenetrating deep sclerectomy if the latter is technically feasible [7].…”

Sindrom Sturge Weber

“…1,2 Angka kejadian SSW di Amerika Serikat 1 : 50.000; 1,3 dari 13 kasus SSW didapatkan 5 anak laki-laki dan 8 anak perempuan berumur antara [8][9][10][11][12][13][14][15] tahun. 4,5 Riviello JJ 2 melaporkan 2 dari 60 (3,3%) pasien SSW meninggal, sedangkan Oakes W 6 dikutip oleh Riviello JJ melaporkan 4 dari 30 (14%) pasien SSW meninggal.…”