Management kindlicher Glaukome bei Sturge-Weber-Syndrom

Zusammenfassung

Hintergrund: Das Sturge-Weber-Syndrom ist eine seltene kongenitale Erkrankung mit Gefäßmalformationen im Bereich der Haut, der Leptomeningen und des Auges. Ein Glaukom tritt in 30–70 % der Fälle auf. Die Behandlung ist aufgrund eines erhöhten Risikos für schwerwiegende Komplikationen wie z. B. expulsiven Blutungen, choroidaler Effusion und serösen Netzhautabhebungen schwierig.

Methoden: Retrospektive Fallserie, die 5 Augen von 5 Kindern (durchschnittliches Alter 5,6 Jahre) mit Sekundärglaukom im Rahmen eines Sturge-Weber-Syndroms einschließt. Augenbefunde, Behandlungsmethoden, Augendruck und Komplikationen wurden ausgewertet. Es wird ein Überblick über die aktuelle Literatur gegeben.

Ergebnisse: Ein Feuermal (Naevus flammeus) und dilatierte episklerale Gefäße lagen bei allen betroffenen Augen vor, in 4 Augen fand sich außerdem ein diffuses Aderhauthämangiom. Bei 4 Kindern wurde eine operative Augendrucksenkung durchgeführt, bei einem Kind eine alleinige konservative. Ein postoperatives reversibles uveales Effusionssyndrom mit Subluxation der Linse trat in einem Auge auf. In einem anderen Auge kam es 3 Jahre nach der Operation (unter einem Augendruck von 10 mmHg) zu einer irreversiblen exsudativen Netzhautabhebung. Beide Komplikationen traten in Assoziation mit einem diffusen Aderhauthämangiom auf.

Schlussfolgerung: Die Behandlung von Sekundärglaukomen im Rahmen eines Sturge-Weber-Syndroms ist schwierig und wird kontrovers diskutiert. Eine alleinige medikamentöse Therapie führt oft nicht zu einer ausreichenden Drucksenkung. Bei der Planung eines operativen Eingriffs muss das erhöhte Risiko für schwerwiegende Komplikationen speziell bei Vorhandensein eines diffusen Aderhauthämangioms berücksichtigt werden.

Abstract

Purpose: Sturge-Weber syndrome is a rare congenital disorder consisting of cutaneous, leptomeningeal and ocular vascular malformations. Glaucoma occurs in 30–70 % of patients. Treatment is challenging due to a high risk of severe complications such as expulsive haemorrhage, massive choroidal effusion and serous retinal detachment. Ocular findings and the occurrence of complications under different treatment modalities have been reviewed.

Methods: A retrospective analysis of a case series of 5 children (5 eyes/mean age 5.6 years) with secondary glaucoma associated with Sturge-Weber syndrome was undertaken. Ocular findings, treatment modalities, intraocular pressure and complications were assessed.

Results: Facial port-wine nevus, i.e., nevus flammeus and dilated episcleral vessels were present in all cases. Diffuse choroidal haemangiomas were seen in four eyes. Glaucoma surgery was performed in 4 children, one child was treated with antiglaucomatous medication alone. Reversible uveal effusion and subluxation of the lens appeared postoperatively in one eye, persistent serous retinal detachment occurred 3 years after surgery in another eye (with an intraocular pressure of 10 mmHg). Both complications were found in eyes with diffuse choroidal haemangioma.

Conclusion: Management of glaucoma associated with Sturge-Weber syndrome is difficult and controversial. Medical treatment often does not decrease intraocular pressure sufficiently. When planning surgical intervention an increased risk of severe complications has to be considered, especially in the presence of diffuse choroidal haemangioma.

Schlüsselwörter

Glaukom - Kinderophthalmologie - Sturge-Weber-Syndrom

Key words

glaucoma - paediatric ophthalmology - Sturge-Weber syndrome

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