Etiology and management of early pregnancy renal anhydramnios: Is there a place for serial amnioinfusions?
Prenat Diagn
; 40(5): 528-537, 2020 04.
Article
em En
| MEDLINE
| ID: mdl-32003482
ABSTRACT
Early pregnancy renal anhydramios (EPRA) comprises congenital renal disease that results in fetal anhydramnios by 22 weeks of gestation. It occurs in over 1 in 2000 pregnancies and affects 1500 families in the US annually. EPRA was historically considered universally fatal due to associated pulmonary hypoplasia and neonatal respiratory failure. There are several etiologies of fetal renal failure that result in EPRA including bilateral renal agenesis, cystic kidney disease, and lower urinary tract obstruction. Appropriate sonographic evaluation is required to arrive at the appropriate urogenital diagnosis and to identify additional anomalies that allude to a specific genetic diagnosis. Genetic evaluation variably includes karyotype, microarray, targeted gene testing, panels, or whole exome sequencing depending on presentation. Patients receiving a fetal diagnosis of EPRA should be offered management options of pregnancy termination or perinatal palliative care, with the option of serial amnioinfusion therapy offered on a research basis. Preliminary data from case reports demonstrate an association between serial amnioinfusion therapy and short-term postnatal survival of EPRA, with excellent respiratory function in the neonatal period. A multicenter trial, the renal anhydramnios fetal therapy (RAFT) trial, is underway. We sought to review the initial diagnosis ultrasound findings, genetic etiologies, and current management options for EPRA.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Anormalidades Congênitas
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Obstrução Ureteral
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Obstrução Uretral
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Anormalidades Múltiplas
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Oligo-Hidrâmnio
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Doenças Renais Císticas
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Rim
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Nefropatias
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Pulmão
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Pneumopatias
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Prognostic_studies
Limite:
Female
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Humans
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Pregnancy
Idioma:
En
Revista:
Prenat Diagn
Ano de publicação:
2020
Tipo de documento:
Article