Transition von Jugendlichen mit Blasenekstrophie-Epispadie Komplex in die Erwachsenenmedizin: Implikationen des Langzeitverlaufes für das Management

Zusammenfassung

Menschen mit seltenen kongenitalen Anomalien wie dem Blasenekstrophie-Epispadie Komplex (BEEK) werden heute meist interdisziplinär und in der Kindheit auf hohem Niveau und mit großem Engagement betreut. Werden die Betroffenen jedoch jugendlich oder gar erwachsen, finden sie in unserem Gesundheitssystem oft keinen Ansprechpartner, der mit den Besonderheiten dieser kongenitalen Anomalien betraut ist. Die Überleitung oder Transition der Betroffenen in eine Schwerpunktbehandlung der Erwachsenenmedizin hat in Deutschland leider noch kein konkretes Gesicht.

Seit Jahren beschäftigen wir uns systematisch mit relevanten Langzeitergebnissen nach Rekonstruktion des BEEK im Neugeborenenalter und versuchen ergebnisorientierte Rückschlüsse für die primären Behandlungsstrategien abzuleiten. Thematisiert wurden neben der wichtigen Frage prognostisch relevanter prädiktiver Faktoren der späteren Kontinenzentwicklung und eines langfristigen Blasenerhaltes, die Genitalfunktion und Fertilität, postoperative Beckenbodenmorphologie mit gynäkologischem Outcome, orthopädische Implika­tionen sowie die psychosexuelle und psychosoziale Entwicklung von Individuen mit BEEK.

Als konkrete Konsequenz entwickeln wir derzeit ein strukturiertes, an den Langzeitaspekten orientiertes, die jeweiligen alters- und geschlechtsspezifischen Bedürfnisse der Kinder mit BEEK berücksichtigendes Nachsorgekonzept. Diese Langzeitergebnisse lehren uns aber auch die enorme Wichtigkeit einer interessierten und kooperativen Zusammenarbeit von Kinderurologen/Kinderchi­rurgen, Urologen, (Kinder-) Orthopäden, Gynäkologen, Andrologen, Psychologen und Urotherapeuten von Anfang an, und unterstreichen dringend einen notwendigen Wissenstransfer und damit eine hoffentlich erfolgreiche „Übernahme“ der Individuen mit BEEK in die Erwachsenmedizin.

Abstract

Today, young individuals with rare congenital anomalies as the Exstrophy-Epispadias-Complex (EEC) are mostly monitored interdisciplinary with a high standard of care and enthusiasm during childhood. However, when growing up through adolescence to adulthood adequate care-givers are not available at the moment in adult medicine in Germany and a concrete transition process has yet not been established. Over the past years, we put much effort in systematic evaluation of long-term outcome after reconstruction of the EEC in the newborn period to further improve outcome results. Beside predictive parameters for continence and long-term bladder function, genital function and fertility, as well as postoperative pelvic floor morphology and gynecological outcome, orthopedic results and psychosexual and psychosocial development in EEC were of major interest. As a consequence we currently develop a German-wide follow-up concept in EEC patients regarding age- and gender specific outcome issues. Long-term observations of the EEC outcome however, underline the unrestricted importance of careful long-term follow-up of all EEC patients, as well as the necessity of close cooperation of pediatric urologist, pediatric surgeons, urologists, orthopedic surgeons, gynecologists, andrologists, psychologists and urotherapists from early childhood and the need of knowledge transfer and hopefully a successful transition of the EEC individuals to general medicine.

Schlüsselwörter

Blasenekstrophie-Epispadie Komplex - Langzeitergebnisse - Transition - Erwachsenenalter

Key words

exstrophy-epispadias-­complex - long-term outcome - transition - adulthood

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