Konnatale Nierenerkrankungen mit Oligohydramnion (ROH)

Zusammenfassung

Ursachen für die seltene angeborene Nie -reninsuffizienz sind meist bds. Fehlbildungen der Nieren und ableitenden Harnwege sowie autosomal-rezessive Zystennieren. Folge des Oligohydramnions als mögliches Zeichen einer sich früh manifestierenden Nierenfunktionsstörung können äußere Fehlbildungen, aber auch eine evtl. lebenslimitierende Lungenhypoplasie sein. Entgegen früheren Vorstellungen ist die Prognose aber nicht grundsätzlich infaust. Durch Fortschritt im Bereich der intensivmedizinischen Neonatologie und pädiatrischen Nephrologie hat die Mehrzahl der Kinder heute realistische Chancen auf ein Überleben mit guter Lebensqualität, wenngleich auch mindestens langfristig mit einer Nierenfunktionsstörung zu rechnen ist. Die Pränatalberatung sollte interdisziplinär unter Beteiligung von Kindernephrologen erfolgen, die Entbindung grundsätzlich in einem Zentrum mit in der Versorgung dieser Patienten erfahrenem Team aus neonatologischer Intensivmedizin und Kindernephrologie mit der Möglichkeit des Einsatzes sämtlicher Nierenersatzverfahren geplant werden. Ein Großteil der überlebenden Patienten kann über viele Jahre hinweg konservativ betreut werden und hat eine gute Lebensqualität.

Summary

Congenital renal insufficiency is rare and usually due to malformations of the kidneys and urinary tract or autosomal recessive polycystic kidney disease. Oligo- or anhydramnios is a strong indicator of fetal kidney dysfunction but can also result in arthrogryposis or even pulmonary hypoplasia. However in recent years it has become clear, that overall prognosis of renal oligohydramnios is not always fatal. Progress in neonatal intensive care and pediatric nephrology has improved outcome. Mortality is highest in the immediate perinatal phase due to pulmonary hypoplasia but some infants only have mild pulmonary symptoms. Also, although all children show some degree of renal impairment in the long run, not all require renal replacement therapy. Thus, antenatal counselling can be difficult and should be multidisciplinary with participation of neonatologists and pediatric nephrologists. In summary and conclusion outcome of fetuses with renal oligohydramnion is not always fatal; due to the complexity of problems these children should be delivered in centers where all modalities of neonatal intensive care and dialysis treatment are available.

Schlüsselwörter

Angeborene Niereninsuffizienz - Oligohydramnion - Lungenhypoplasie - Dialyse - Outcome

Keywords

Renal oligohydramnios - chronic kidney disease - pulmonary hypoplasia - dialysis - outcome

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