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Tatalaksana kegagalan filtrasi pasca trabekulektomi pada pasien anak dengan glaukoma sekunder dan sindrom Sturge Weber: sebuah laporan kasus

Abstract

ABSTRACT

Background: Sturge-Weber syndrome is a neurocutaneous disorder with angiomas involving the leptomeninges and facial skin. Glaucoma ipsilateral to port-wine stain develops in 30% -70% of patients. Trabeculectomy has been the gold standard and is the most commonly used surgical procedure. The risk of trabeculectomy failure may be due to the perioperative inflammation associated with failed bleb and increased inflammatory cytokines in the aqueous humor.

Case Illustration: A 9-years old girl with mental retardation and port-wine stain on the right side of the face, patient's right eye had a trabeculectomy surgery with 5-Fluorouracil four years ago and routinely used 0.5% timolol maleate drops every 12 hours in the right eye. Filtration failure characterized by failed bleb and failure to achieve intraocular pressure target led to a repeat trabeculectomy with 5-Fluorouracil administration. Repeated trabeculectomy procedures performed in this patient showed no significant complications and demonstrated a reduction of intraocular pressure.

Discussion: This case meets the diagnostic criteria for type 1 Sturge-Weber syndrome. Unilateral right eye glaucoma occurred, corresponding to the location of a port-wine stain on the right side of the face. Bleb failure is usually caused by the production, contraction, and remodeling of collagen, which causes excessive scarring of the conjunctiva and episclera. Ophthalmological examination showed a flat bleb with minimal vascularity, indicating failure of filtration and bleb formation. Glaucoma surgery was chosen when intraocular pressure cannot be controlled by medication. The initial trabeculectomy performed in this patient showed uncontrolled intraocular pressure after four years, despite 0.5% timolol maleate administration every 12 hours, so a repeat trabeculectomy was performed to achieve the intraocular pressure target. Repeated trabeculectomy, in this case, appears to be effective in controlling intraocular pressure.

Conclusion: Repeated trabeculectomy can be considered as the management of initial trabeculectomy failure in cases of secondary glaucoma in Sturge-Weber syndrome.

 

Latar Belakang: Sindrom Sturge-Weber adalah kelainan neurokutan dengan angioma yang melibatkan leptomeninges dan kulit wajah. Glaukoma ipsilateral hingga port-wine stain terjadi pada 30% -70% pasien. Trabekulektomi telah menjadi baku emas dan merupakan prosedur bedah yang paling umum digunakan. Risiko kegagalan trabekulektomi mungkin disebabkan oleh peradangan perioperatif yang berhubungan dengan kegagalan bleb dan peningkatan sitokin inflamasi dalam aqueous humor.

Laporan Kasus: Seorang anak perempuan berusia 9 tahun dengan retardasi mental dan tampak port-wine stain pada sisi kanan wajah, mata kanan pasien telah dilakukan operasi filtrasi trabekulektomi dengan 5-Fluorourasil empat tahun yang lalu, dan rutin menggunakan obat tetes timolol maleate 0,5% setiap 12 jam di mata kanan. Kegagalan filtrasi yang ditandai dengan kegagalan bleb dan tidak tercapainya target tekanan intraokular membuat dilakukannya trabekulektomi ulangan dengan pemberian 5-Fluorourasil. Prosedur trabekulektomi berulang yang dilakukan pada pasien ini menunjukkan tidak adanya komplikasi yang signifikan dan menunjukkan penurunan TIO pada pasien.

Diskusi: Laporan kasus ini memenuhi kriteria diagnosis Sindrom Sturge-Weber tipe 1 yaitu klasik dengan adanya angioma leptomeningeal dan fasial, adanya Port Wine Stain dan glaukoma. Glaukoma unilateral terjadi di mata kanan sesuai dengan lokasi Port Wine Stain. Kegagalan bleb cenderung biasanya disebabkan oleh produksi, kontraksi, dan remodeling kolagen yang menyebabkan jaringan parut yang berlebihan pada episklera. Pemeriksaan oftalmologi menunjukkan bleb yang yang mendatar dengan vaskularitas minimal, hal ini menunjukkan adanya kegagalan filtrasi dan pembentukan bleb yang dijumpai empat tahun pasca tindakan trabekulektomi inisial. Operasi glaukoma dilakukan pada kondisi TIO tidak dapat dikontrol oleh obat-obatan. Trabekulektomi inisial yang dilakukan pada pasien ini menunjukkan TIO yang belum terkontrol baik setelah empat tahun, walaupun dengan pemberian timolol maleate 0,5% setiap 12 jam, sehingga dilakukan trabekulektomi ulangan untuk mencapai target TIO. Trabekulektomi berulang pada kasus ini tampaknya efektif dalam mengontrol TIO.

Simpulan:  Trabekulektomi ulang dapat dipertimbangkan sebagai manajemen pada kegagalan trabekulektomi inisial pada kasus glaukoma sekunder pada Sindrom Sturge-Weber.

References

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How to Cite

Prabasari, C. I. W., Suryaningrum, I. G. A. R. ., & Kusumadjadja, I. M. A. . (2024). Tatalaksana kegagalan filtrasi pasca trabekulektomi pada pasien anak dengan glaukoma sekunder dan sindrom Sturge Weber: sebuah laporan kasus. Intisari Sains Medis, 15(1), 14–21. https://doi.org/10.15562/ism.v15i1.1934

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