Lower Urinary Tract Obstruction (LUTO) – Krankheitsbild, pränatale Diagnostik und Therapiemöglichkeiten

Zusammenfassung

Die Ätiologie der Obstruktion des Blasenhalses (LUTO) ist heterogen. Die häufigsten Entitäten sind isolierte posteriore Urethralklappen oder eine Urethralatresie beim männlichen Feten. LUTO bei weiblichen Feten ist häufig Teil eines komplexeren Fehlbildungsgeschehens. Der natürliche Verlauf bei LUTO ist charakterisiert durch eine hohe Mortalität und Morbidität aufgrund sich entwickelnden Lungenhypoplasie, die im Zusammenhang mit einer ausgeprägten Oligo- oder Anhydramnie zum Zeitpunkt der kannaliculären Phase der Lungenentwicklung (16.-24. SSW) steht. Die begleitende Nierenschädigung ist variabel und reicht von geringen Einschränkungen der Nierenfunktion im Kleinkindalter bis hin zur Dialysepflichtigkeit und Notwendigkeit der Nierentransplantation. Die Möglichkeit der pränatalen Intervention zur Umgehung der Obstruktion ist biologisch plausibel und technisch erfolgreich durchführbar. Vesiko-amniales Shunting und, zur Zeit noch seltener, die fetoskopische Zystoskopie mit Laserablation der posterioren Urethralklappen sind mögliche minimal-invasive Therapieverfahren. Die bisherigen Ergebnisse zeigen, daß die perinatale Sterblichkeit durch die pränatale Therapie (vesiko-amniales Shunting oder fetoskopische Zystoskopie mit Laserablation der posterioren Urethralklappen) verringert werden kann, jedoch derzeit keine Verbesserung der Anzahl der Kinder mit postnatal normaler Nierenfunktion zu erreichen ist. Die Auswahl der betroffenen Ungeborenen mit LUTO die von einer fetalen Therapie profitieren können, wird derzeit dadurch beeinträchtigt, dass durch die Sonografie und die serielle fetale Urinanalytik keine definitiv verlässlichen Prognosekriterien für die postpartale Nierenfunktion abgeleitet werden können. Eine Schwäche der derzeit vorliegenden Untersuchungen ist die fehlende Randomisierung. Aufgrund einer relevanten Komplikationsrate der intrauterinen Eingriffe bei nicht klar belegter Effektivität sollten derartige Eingriffe nur in hochspezialisierten Zentren erfolgen. Weitere Studien zur verbesserten Auswahl betroffener Feten und zur früheren Therapie in der Schwangerschaft sind erforderlich. Die Daten der prospektiv randomisierten multizentrischen PLUTO Studie der Universität Birmingham, England (percutaneous shunting in lower urinary tract obstruction) könnten zu diesen Fragen Aufschluss geben. Bis dahin sollte die Indikation zur intrauterinen Harnableitung mit größter Sorgfalt und nach interdisziplinärer Beratung gemeinsam mit den Neonatologen, den Kindernephrologen und den werdenden Eltern gestellt werden. Eine allgemeingültige Handlungsanweisung zur fetalen Therapie bei LUTO kann auf dem Boden der derzeitigen Daten nicht gegeben werden.

Abstract

The aetiology of urinary tract obstructions (LUTO) is heterogeneous. The most common entities are isolated posterior urethral valves or urethral atresia in male foetuses. In female foetuses LUTO is frequently a part of complex malformations. The natural history of LUTO is characterised by high morbidity and mortality due to the development of severe pulmonary hypoplasia caused by oligo- or anhydramnios affecting the cannalicular phase (16–24 weeks of gestation) of pulmonary development. The degree of renal damage is variable and ranges from mild renal impairment in infancy to end-stage renal insufficiency, necessitating dialysis and transplantation. Foetal interventions in order to bypass the obstruction are biologically plausible and technically feasible. Vesico-amniotic shunting as well as (currently less frequent) foetoscopic cystoscopy and laser ablation of posterior urethral valves are minimally invasive treatment options. Previous reports indicate that prenatal therapy is suitable to reduce perinatal mortality but does not improve postnatal renal function. Selection of foetuses who may profit from prenatal intervention is aggravated by the lack of reliable prognostic criteria for the prediction of postnatal renal function in both ultrasound and foetal urine analysis. Furthermore, there is no randomised trial available at the time of writing. Because of a relevant complication rate and still no clear evidence for foetal benefit, interventions should be performed in specialised centres. Further studies are necessary to improve case selection of affected foetuses and to evaluate the impact of interventions in earlier gestational weeks. The data from the PLUTO trial (percutaneous shunting in lower urinary tract obstruction) conducted by the University of Birmingham may help to answer these questions. In the meantime selection of foetuses for prenatal intervention puts high requirements on interdisciplinary counselling in every case. A general treatment algorithm for foetal therapy is not available at the moment.

Schlüsselwörter

LUTO - Harnwegsobstruktion - obstruktive Uropathie - Nierenfunktion - Fetus

Key words

LUTO - urinary tract obstruction - obstructive uropathy - renal function - foetus

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