Management of glaucoma in a patient with Klippel-trenaunay syndrome

In our case, we suspected juvenile onset glaucoma since there was no history of buphthalmus and we think that it was an important factor in the satisfactory outcome of medical treatment achieving the target IOP.

📌 3 Key Takeaways

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Objective & Hypothesis:We report a 20-year-old male diagnosed with Klippel-Trenaunay syndrome who had hemangiomas starting from the face and neck and covering almost the entire left side of the body in association with soft tissue hypertrophy, and we present the treatment that we implemented.

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Key Findings & Data:Increased scleral pigmentation, prominent scleral vessels, increased iris pigmentation, and prominent iris processes were present in the left eye.

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Conclusion & Impact:In our case, we suspected juvenile onset glaucoma since there was no history of buphthalmus and we think that it was an important factor in the satisfactory outcome of medical treatment achieving the target IOP.

🔬Design:Research Article

📍Scope:Comprehensive Sample

⚙️Variables:Domain Parameters

Abstract

We report a 20-year-old male diagnosed with Klippel-Trenaunay syndrome who had hemangiomas starting from the face and neck and covering almost the entire left side of the body in association with soft tissue hypertrophy, and we present the treatment that we implemented. Increased scleral pigmentation, prominent scleral vessels, increased iris pigmentation, and prominent iris processes were present in the left eye. The patient had not received any antiglaucoma treatment on a regular basis before, and the intraocular pressure (IOP) of the left eye was 33 mmHg on presentation. It decreased to 17 mmHg after 1 month of brimonidine+timolol maleate combination (Combigan) treatment. In our case, we suspected juvenile onset glaucoma since there was no history of buphthalmus and we think that it was an important factor in the satisfactory outcome of medical treatment achieving the target IOP.

Özet

Bu çalışmada yüz ve boyundan başlayıp neredeyse vücudun tüm sol yarısında uzanmakta olan hemanjiomları ve bu hemajiomlara eşlik eden yumuşak doku hipertrofleri mevcut olan, Klippel-Trenaunay sendromu tanısı almış, 20 yaşında bir erkek hasta ve bu hastaya uygulanan tedavi sunulmuştur. Olgumuzun sol gözünde skleral damarlarda belirginleşme, skleral pigmentasyon, iris renginde koyulaşma ve iris proseslerinde belirginleşme mevcuttu. Öncesinde düzenli bir anti-glokomatöz tedavi almamış olan hastanın, başvuru anındaki sol göz içi basıncı (GİB) 33 mmHg idi. Brimonidine+timolol maleat kombinasyonu içeren (Combigan) damla sonrası 1. ayda GİB'nı 17 mmHg'e düşmüştü. Olgumuzda buftalmus öyküsü olmadığı için glokomun, juvenil başlangıçlı olduğunu tahmin etmekteyiz ve medikal tedavi ile hedeflediğimiz GİB'na ulaşabilmiş olmamızda, bunun önemli bir etken olduğunu düşünüyoruz.

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Cite This Article

AYINTAP, E., KESKİN, U., COŞKUN, M., ÇELİK, Ö. İ., TUZCU, A. E., ÖKSÜZ, H. (2010). Management of glaucoma in a patient with Klippel-trenaunay syndrome. Glokom Katarakt, Cilt: 5 - Sayı: 4, 231–234. https://ajindex.com/articles/226568

References (1)

Rahman M, Rahman S, Rahman M, et al.: Overlapping of Stur-ge Weber syndrome and Klippel Trenaunay Weber syndrome. Mymensingh Med J. 2008;17:78-81. 2.Brodsky MC, Baker RS, Hamed LM.: Pediatric neurophthalmology. New York: Springer. 1996;413. 3.Brodsky MC.: Circumpapillary choroidal "haemorrhoid" in Klip-pel-Trenauney-Weber syndrome. Br J Ophthalmol. 2007;91:394. 4.Baskerville PA, Ackroyd JS, Browse NL.: The etiology of Klippel Trenaunay syndrome. Ann Surg. 1985;202:624-627. 5.Aelvoet GE, Jorens PG, Roelen LM.: Genetic aspects of the Klip-pel-Trenaunay syndrome. Br J Dermatol. 1992;126:603-607. 6.Saricaoğlu MS, Güven D, Karakurt A, et al.: An unusual case of Sturge-Weber syndrome in association with phakomatosis pig-mentovascularis and Klippel-Trenaunay-Weber syndrome. Retina. 2002;22:368-371. 7.Mattox C.: Glaucoma in the phakomatoses. In: Epstein DL, Al-lingham RR, Schuman JS, editors. Chandler and Grant's Glaucoma. 4th edition. Baltimore: Williams and Wilkins.l 997:435-436. 8.Yang CB, Freedman SF, Myers JS, et al.: Use of latanoprost in the treatment of glaucoma associated with Sturge-Weber syndrome. Am J Ophthalmol.1998;126:600-602.