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Laryngorhinootologie 2014; 93(S 01): S185-S202
DOI: 10.1055/s-0033-1363216
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© Georg Thieme Verlag KG Stuttgart · New York

Extrakranielle vaskuläre Fehlbildungen (Hämangiome und vaskuläre Malformationen) im Kindes- und Jugendalter – Diagnostik, Klinik und Therapie

Extracranial Vascular Anomalies (Hemangiomas and Vascular Malformations) in Children and Adolescents – Diagnosis, Clinic, and Therapy
B. Eivazi
1   Marburger Universitätsklinik für Hals-, Nasen- und Ohrenheilkunde, UKGM, Angiomzentrum Marburg
,
J. A. Werner
1   Marburger Universitätsklinik für Hals-, Nasen- und Ohrenheilkunde, UKGM, Angiomzentrum Marburg
› Author Affiliations
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Publication History

Publication Date:
07 April 2014 (online)

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Zusammenfassung

Das Gebiet der extrakraniellen vaskulären Anomalien wird als ein spezieller Schwerpunkt der Kinder-HNO-Heilkunde betrachtet und zeigt eine rasante Entwicklung in den letzten Jahren. Ausschlaggebend hierfür ist nicht zuletzt die globale Akzeptanz der von der ISSVA (International Society for the Study of Vascular Anomalies) eingeführten Klassifikation. Hämangiome sind die Hauptvertreter der vaskulären Tumore. Die systemische Propranololtherapie gilt heutzutage als Therapie der ersten Linie für behandlungsbedürftige Hämangiome. Zunehmend wird der Einsatz vom Betablocker-haltigen Topika diskutiert, wobei die Anwendung im Kopf-Hals-Gebiet erfahrungsgemäß limitiert zu sein scheint. Vaskuläre Malformationen werden nach der Morphologie des betroffenen Anteils des Gefäßsystems in arterielle, venöse, arterio-venöse, lymphatische, kapilläre und kombinierte vaskuläre Malforma­tionen unterteilt. Die konventionelle Chirurgie, die Sklerosierungstherapie und die Laserbehandlung stellen die invasiven therapeutischen Optionen für lymphatische Malformationen dar. Für venöse Malformationen im Kopf-Hals-Bereich ist es gelungen, die therapeutischen Möglichkeiten in den letzten Jahren deutlich zu optimieren. Hierzu zählen der Einsatz von Nd:YAG Laser, die konservative Behandlung der lokalisierten Verbrauchskoagulopathie mit niedermolekularem Heparin, die Wiederentdeckung des Bleomycin als potentes Sklerosierungsmittel und die Verfeinerung von alkoholbasierten Embolisationsmitteln. Die Farbstofflaserbehandlung gilt heute als die bevorzugte Therapie für kapilläre Malformationen und ist für diese Entität anderen Therapiemodalitäten wie z. B. der photodynamischen Therapie überlegen. Arterio-venöse Malforma­tionen als Hauptvertreter der Hochflussläsionen zählen zu den gefährlichsten Läsionen. Nicht selten werden sie mit malignen Kopf-Hals-Tumoren verglichen, vor allem dann, wenn bei einer diffusen oder multifokalen Ausdehnung eine kurative Behandlung nicht mehr gewährleistet werden kann und die Erkrankung einen progressiven Verlauf aufweist. Die gezielte, kombinierte Therapie aus Embolisationen und chirurgischer Resektion und gegebenenfalls konsekutiver Defektdeckung hat sich für arterio-venöse Malformationen bewährt. Inkurable Befunde stellen weiterhin ein großes Problem dar. Trotz der Einführung von antiangiogenetischen Medikamenten in der Onkologie konnte sich ein medikamentöser Therapieansatz für arterio-venöse Malformationen bis dato nicht etablieren.

Abstract

Extracranial Vascular Anomalies (Hemangiomas and Vascular Malformations) in Children and Adolescents – Diagnosis, Clinic, and Therapy

The field of extracranial vascular anomalies is considered as special focus of pediatric otolaryngology and it has shown a rapid development during the last years. The reason for this interest is finally also due to the global acceptance of the classification introduced by the ISSVA (International Society for the Study of Vascular Anomalies). Hemangiomas are the most frequently observed vascular tumors. Today the systemic propranolol therapy is mostly used for therapy of hemangiomas requiring treatment. Increasingly, the topical application of beta blocker is discussed while the benefit in the head and neck seems to be limited. Vascular malformations are classified according to the morphology of the affected part of the vascular system in arterial, venous, arterio-venous, lymphatic, capillary, and combined vascular malformations. Conventional surgery, sclerosing therapy, and laser treatment are invasive options for the treatment of lymphatic malformations. The options for the treatment of venous malformations could be significantly improved during the last years. In this context, the use of Nd:YAG laser, the conservative treatment of the localized disseminated intravascular coagulation with low-molecular weight heparin, the re-discovery of bleomycin as effective sclerosing agent, and the improvement of alcohol-based embolization agents must be mentioned. Today the treatment with dye laser is the preferred therapy for capillary malformations and it is superior to other therapeutic options as for example photodynamic therapy. Arterio-venous malformations as representatives for high-flow lesions are the high-risk lesions. Frequently they are compared to malignant head and neck tumors, in particular when a curative treatment can no longer be assured because of diffuse or multifocal extent and when the disease shows a progressive course. The combined treatment of embolization and surgical resection and if necessary consecutive defect reconstruction have turned out to be appropriate for arterio-venous malformations. Incurable findings are still a major challenge. Despite the introduction of antiangiogenetic drugs in oncology, the medicamentous therapeutic approach could not be established for arterio-venous malformations up to now.

Schlüsselwörter

Hämangiome - Vaskuläre Malformationen - Lymphatische Malforma­tionen - Kapilläre Malformationen - Venöse Malformationen - Arteriovenöse Malforma­tionen

Key words

Hemangiomas - Vascular malformation - Lymphatic malformation - Capillary malformation - Venous malformation - Arteriovenous malformation
 

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