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Klin Monbl Augenheilkd 2001; 218(5): 292-297
DOI: 10.1055/s-2001-15884
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Georg Thieme Verlag Stuttgart ·New York

Diagnostic et traitement des tumeurs intra-oculaires chez l'enfant

Diagnosis and treatment of intraocular tumours in childrenAubin Balmer, Francis Munier
  • Hôpital Ophtalmique Jules Gonin, Université de Lausanne, Avenue de France 15, CH-1004 Lausanne (Directeur: Professeur L. Zografos)
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Publication Date:
31 December 2001 (online)

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Diagnose und Therapie intraokularer Tumoren beim Kind

Hintergrund Das Retinoblastom zählt zu den häufigsten und bösartigsten intraokularen Tumoren im Kindersalter. Die Prognose hängt stark von einer frühzeitigen Behandlung ab und erfordert daher eine rasche Diagnose.Material und Methoden Basierend auf der eigenen Erfahrung und der Literatur werden die diagnostischen Kriterien sowie die heute verfügbaren therapeutischen Möglichkeiten aufgezeigt.Ergebnisse Leukokorie und monolateraler Strabismus sind die zwei wichtigsten Alarmzeichen des Retinoblastoms. In 20 % der Fälle können jedoch atypische Symptome die ersten Anzeichen der Krankheit sein. Diagnostische Kriterien sind Alter, Geschlecht, Lateralität, Vererblichkeit, Größe des Auges, Verkalkungen und Absiedelungen im Glaskörper. Eine unter Narkose durchgeführte Untersuchung der gesamten Oberfläche der Retina ist unerlässlich. Die Ultraschallechographie, die Computertomographie oder die Kernspintomographie erlauben in den meisten zweifelhaften Fällen eine sichere Diagnose. Eine histologische Diagnose bleibt der Ausnahmefall. Die Therapie kann sehr verschieden sein, je nachdem ob es sich um einen bösartigen oder gutartigen Tumor handelt. Enukleation, Chemotherapie, Thermotherapie, Thermo-Chemotherapie, äußere Bestrahlung, Brachytherapie, Kryokoagulation und Photokoagulation sind die zahlreichen Techniken, die beim Retinoblastom entweder in Kombination oder einzeln angewandt werden können. Die Wahl hängt hauptsächlich vom Stadium ab. Das Medulloephitheliom in seiner bösartigen Form kann eine Enukleation erforderlich machen. Die Brachytherapie stellt in gewissen Fällen eine Alternative dar.Schlussfolgerungen Jedes Zeichen, das auf einen intra-okularen Tumor beim Kind hinweisen könnte, soll als Warnzeichen eines Retinoblastoms gewertet werden, bis das Gegenteil bewiesen ist. Es muss auf eine Sicherheitsdiagnose bestanden werden. Die Behandlung eines Retinoblastoms im Frühstadium kann Leben und Augenlicht retten.Schlüsselwörter Retinoblastom - Morbus Coats - retinale astrozytäre Hamartome - persistierender und hyperplastischer primärer Glaskörper - Medulloepitheliom

Purpose To remind of the absolute necessity for early diagnosis in the presence of ocular signs in children giving rise to possible intraocular tumours.Method Based on our own experience of intraocular tumours in children, together with findings from the literature, diagnostic criteria and methods of treatment are presented.Results Retinoblastoma is the predominant cause of intraocular tumours in children, representing over 80 % of cases under the age of 15 years. Other diseases may give rise to the same initial signs, usually leukocoria, sometimes strabismus, more rarely other atypical signs. Elements taken into account for diagnosis include age, sex, laterality, heredity, size of the globe, clinical aspect of the tumours, presence of calcifications and vitreous seeding. Full fundus examination under general anaesthetic is usually necessary. Biological examination, ultrasonography, computerized tomography and MRI enable an accurate diagnosis to be made in the majority of doubtful cases. The management of retinoblastoma is adapted for each individual case from the wide range of treatments available. Enucleation, radioactive applicators (…), brachytherapy (…), cryo- and photocoagulation represent classical measures. Primary chemotherapy, combined with other treatments such as thermotherapy, has become the treatment of choice in those cases where external beam radiotherapy has been used up to now, or in some instances before enucleation. Enucleation is usually carried out for medullo-epitheliomas, but brachytherapy may offer an alternative.Conclusion Any unexplained ocular sign in children should be considered as a possible retinoblastoma, making an accurate and certain diagnosis imperative. Early treatment may save not only the life but also the vision of patients carrying this highly malignant lesion.Key words Intraocular tumours in children - retinoblastoma - Coats' disease - medullo-epithelioma - PHPV (persistent hyperplastic primary vitreous)

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