Long-term intraocular pressure after combined trabeculotomy-trabeculectomy in glaucoma associated with Sturge-Weber syndrome

PURPOSE. To evaluate the effectiveness of surgical treatment of secondary glaucoma and to present the clinical features of the postoperative period in a patient with Sturge-Weber syndrome (SWS). METHODS. A patient with SWS, choroidal hemangioma and secondary glaucoma was examined and treated (Ahmed valve implantation) at the S.N. Fedorov National Medical Research Center "MNTK "Eye Microsurgery" in Moscow. RESULTS. Hypotension, a decrease in anterior chamber depth, serous choroidal detachment were observed on the first day after surgery on the left eye. Posterior scleral trepanation and injection of viscoelastic into the anterior chamber was performed. On the next day, IOP normalization and complete choroidal attachment were achieved, but macular detachment of neuroepithelium occurred and was successfully treated with injections of glucocorticosteroids. After one month, the retina and choroid were attached, IOP was 16 mm Hg in the left eye without hypotensive drops, visual acuity was 1.0 in both eyes. CONCLUSION. Implantation of the Ahmed valve can be used in the treatment of secondary glaucoma in patients with SWS in order to reduce the IOP and minimize the risks of complications.

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Section: клинический случайunclassified

Features of the postoperative period after glaucoma surgery in a patient with Sturge-Weber syndrome (clinical case)

et al. 2022

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“…[37][38][39]41] Combined trabeculotomy/trabeculectomy as the initial operation has been suggested in early onset glaucoma to address more distal resistance. [45][46][47][48] Recently, Sood et al [46] reported combined trabeculotomy/trabeculectomy in 24 eyes of 20 patients with SWS glaucoma with the overall success rate of 41.7% within 134.7 ± 67.7 months follow-up. Board and Shields investigated combined trabeculotomy/trabeculectomy in five SWS patients with the age ranging from 2 months to 15 years.…”

Section: Surgical Treatmentmentioning

confidence: 99%

Ocular Manifestations of the Sturge–Weber Syndrome

Hassanpour

Nourinia

Gerami

et al. 2021

JOVR

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Sturge–Weber syndrome (SWS) or encephalotrigeminal angiomatosis is a non-inherited congenital disorder characterized by neurologic, skin, and ocular abnormalities. A somatic activating mutation (R183Q) in the GNAQ gene during early embryogenesis has been recently recognized as the etiology of vascular abnormalities in SWS. Approximately, half of the patients with SWS manifest ocular involvement including glaucoma as the most common ocular abnormality followed by choroidal hemangioma (CH). The underlying pathophysiology of glaucoma in SWS has not been completely understood yet. Early onset glaucoma comprising 60% of SWS glaucoma have lower success rates after medical and surgical treatments compared with primary congenital glaucoma. Primary angle surgery is associated with modest success in the early onset SWS glaucoma while the success rate significantly decreases in late onset glaucoma. Filtration surgery is associated with a higher risk of intraoperative and postoperative choroidal effusion and suprachoroidal hemorrhage. CH is reported in 40–50% of SWS patients. The goal of treatment in patients with CH is to induce involution of the hemangioma, with reduction of subretinal and intraretinal fluid and minimal damage to the neurosensory retina. The decision for treating diffuse CHs highly depends on the patient’s visual acuity, the need for glaucoma surgery, the presence of subretinal fluid (SRF), its chronicity, and the potential for visual recovery.

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“…Earlier diagnosis of glaucoma and surgery at a median age of 3 months may afford a better success rate with a single trabeculotomy (Wu et al, ). Due to high risk of failure with single procedures, combined procedures, such as trabeculotomy‐trabeculectomy have been proposed as the first‐line approach in infants and children with ETA (Mandal, ; Mantelli et al, ; Sood, Rathore, Sood, Kumar, & Sood, ).…”

Section: Encephalotrigeminal Angiomatosis (Sturge‐weber Syndrome)mentioning

confidence: 99%

“…The type of angle surgery performed can be variable and goniotomy and trabeculotomy, which work by mechanically disrupting the anterior chamber angle structures, are often preferred as the initial surgical procedure for congenital glaucoma in children with ETA due to lower risk of adverse effects compared to filtering or tube surgery. Better IOP control can obtained by repeating the procedure, as initial goniotomy is only been proposed as the first-line approach in infants and children with ETA (Mandal, 1999;Mantelli et al, 2016;Sood, Rathore, Sood, Kumar, & Sood, 2018 (Friedman, 2002). Characteristic dermatologic features are caf e-au-lait spots and axillary freckling.…”

Section: Glaucoma Monitoring and Treatmentmentioning

confidence: 99%

Pathophysiology and management of glaucoma associated with phakomatoses

Thavikulwat

Edward

AlDarrab

et al. 2018

J of Neuroscience Research

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The phakomatoses, encephalotrigeminal angiomatosis (ETA; Sturge-Weber Syndrome), neurofibromatosis type 1 (NF1 or von Recklinghausen disease), Von Hippel-Lindau (VHL) disease, tuberous sclerosis (TSC), oculodermal melanocytosis (ODM), and phakomatosis pigmentovascularis are a group of neurocutaneous disorders that have characteristic systemic and ocular manifestations. Through many different mechanisms, they may cause glaucomatous damage of the optic nerve and subsequent vision loss varying from mild to severe. Glaucoma commonly affects patients with ETA (43-72%), orbito-facial NF1 (23-50%), and ODM (10%). Rarely, it may present as neovascular glaucoma in VHL and TSC. In ETA, glaucoma typically occurs ipsilateral to the port-wine stain, which is caused by a mutation in the GNAQ gene. Specifically, mechanical malformation of the anterior chamber angle and elevated episcleral venous pressure has been implicated as causes of glaucoma in ETA. In NF1, which is caused by a mutation in the NF1 tumor suppressor gene, glaucoma commonly occurs ipsilateral to lid plexiform neurofibromas. Histological studies of eyes with NF1 have revealed direct anterior chamber infiltration by neurofibromas, secondary angle closure, fibrovascularization, and developmental angle abnormalities as mechanisms of glaucoma. Lastly, phakomatosis pigmentovascularis is a rare combination of ODM and port-wine stain. Affected patients are at very high risk of developing glaucoma. Despite the many different mechanisms of glaucomatous damage, management follows similar principles as that for congenital glaucoma and primary open angle glaucoma. First-line therapy is topical intraocular pressure-lowering eye drops. Surgical management, including goniotomy, trabeculotomy, trabeculectomy, and tube shunt placement may be required for more severe cases.

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Long-term intraocular pressure after combined trabeculotomy-trabeculectomy in glaucoma associated with Sturge-Weber syndrome

Abstract: Combined trabeculotomy-trabeculectomy showed promising results as a treatment for SWS-associated glaucoma in children. Long-term visual and surgical outcomes are encouraging.

Cited by 11 publications

References 13 publications

Features of the postoperative period after glaucoma surgery in a patient with Sturge-Weber syndrome (clinical case)

Features of the postoperative period after glaucoma surgery in a patient with Sturge-Weber syndrome (clinical case)

Ocular Manifestations of the Sturge–Weber Syndrome

Pathophysiology and management of glaucoma associated with phakomatoses

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