Management von obstruktiven Harntransportstörungen

Zusammenfassung

Kongenitale obstruktive Harntransportstörungen bilden eine heterogene Gruppe von Erkrankungen, die durch einen unterschiedlichen natürlichen Verlauf gekennzeichnet sind. Obstruktive Harntransportstörungen können unilateral oder bilateral auftreten und entweder auf Höhe des ureteropelvinen Übergangs, im Bereich des distalen Harnleiters, auf Blasenausgangsebene oder im Bereich der Urethra liegen. Zu den häufigsten angeborenen obstruktiven Uropathien gehören die ureteropelvine Stenose, der primär obstruktive Megaureter sowie die Harnröhrenklappen bei Jungen. Während sich Obstruktionen des oberen Harntraktes häufig spontan zurückbilden, sind infravesikale Obstruktionen durch eine hohe Morbidität und Mortalität gekennzeichnet. Nach wie vor gehören die obstruktiven Uropathien zu den Hauptursachen chronisch terminaler Niereninsuffizienz im Kindesalter. Im Folgenden berichten wir über die Pathogenese, Diagnostik und Therapie einer kongenitalen obstruktiven Uropathie. Obstruktive Harntransportstörungen sind multifaktoriell bedingt und können potenziell zur Niereninsuffizienz im Kindesalter führen.

Summary

Congenital obstructive uropathy is a heterogeneous group of pathologies with a varying natural course. Urinary tract obstruction can be unilateral or bilateral and localizes to the ureteropelvic junction, the ureterovesical junction, to the bladder outlet or to the urethra. The most frequent forms of congenital obstructive uropathies are ureteropelvic junction obstructions, primary obstructive megaureters as well as posterior urethral valves. Whereas upper urinary tract obstruction may resolve spontaneously, lower urinary tract obstruction shows a high morbidity and mortality. Obstructive uropathies represent a major cause for end-stage renal disease in children. This review will give a short overview on the pathogenesis, the diagnostic and therapeutic procedures in congenital obstructive uropathies and demonstrate the multifactorial history and the risk for renal failure in childhood.

Schlüsselwörter

Harntransportstörung - ureteropelvine Stenose - Harnröhrenklappen - primär obstruktiver Megaureter

Keywords

Hydronephrosis - ureteropelvic junction obstruction - urethral valves - primary obstructive megaureters

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