Elsevier

Early Human Development

Volume 87, Issue 9, September 2011, Pages 607-610
Early Human Development

Best practice guidelines
Long-term renal and neurodevelopmental outcome in infants with LUTO, with and without fetal intervention

https://doi.org/10.1016/j.earlhumdev.2011.07.004Get rights and content

Abstract

Congenital lower urinary tract obstruction (LUTO) is a heterogeneous group of pathologies, the most common being posterior urethral valves (PUV) or urethral atresia. The bladder neck obstruction in utero leads to a spectrum of disease including mild oligohydramnios with normal renal function to a picture of severe oligohydramnios associated with chronic obstructive macro/microcystic renal parenchymal disease leading to chronic renal impairment. These anomalies may be isolated or complex; the latter being associated with other structural or chromosomal abnormalities. If isolated, the congenital bladder neck obstruction may be amenable to in-utero therapy. In a significant proportion of babies affected by LUTO there is severe oligohydramnios (occurring before 20 weeks gestation) and associated with pulmonary hypoplasia, a scenario almost always associated with perinatal death. For those babies that survive the perinatal period there is a significant risk of renal impairment, often necessitating renal dialysis or transplantation in childhood. In addition, there may be other morbidities such as chronic filling anomalies of the bladder that may require treatment.

Section snippets

Background

Congenital abnormalities of the genitourinary tract are one of the most common sonographically identified anomalies with obstructive uropathies accounting for the majority of cases. The obstruction can be defined according to the relationship with the bladder being either upper or lower. Congenital lower urinary tract obstruction (LUTO) of bladder neck at the level of the urethra occurs secondary to several pathologies. The most common are posterior urethral valves (PUV) or urethral atresia [1]

Perinatal outcomes

The natural history of LUTO in the perinatal period is of high fetal and perinatal mortality. Data from the West Midlands Congenital Anomaly Register demonstrates that over the 13 year time period studied there were 69 termination of pregnancies (33%), 11 intra-uterine deaths (3.9%) and 190 live births (66.9%) associated with the condition. The perinatal mortality rate was 458 per 1000 births for complex cases and 120 per 1000 live births for isolated cases [4].

A systematic review of

Long term outcomes

Data relating to the natural history of LUTO further than the perinatal period is difficult to identify. A unique study from Finland presents 10 year outcomes in babies with PUV (n = 46) comparing outcomes in those diagnosed prenatally (with no intervention n = 23) and those diagnosed postnatally (with apparently ‘normal’ ultrasound appearances in the majority = 23) [16]. Despite postnatal treatment, 13% ended up developing chronic renal failure with 17% ending the study with end-stage renal disease.

Future advances

As antenatal imaging advances and the use of 3D ultrasound and fetal magnetic resonance imaging becomes more widely available we may see and improvement in the antenatal diagnosis of LUTO and indeed prospective factors identified that lead to prognosis being more completely defined. Improvements in fetal cystoscopy techniques may offer the opportunity for diagnosis of the underlying pathology and thus appropriate intervention antenatally. One of the major difficulties with this morbid condition

Conclusion

Congenital LUTO is a perinatal condition associated with bladder neck obstruction secondary to heterogeneous pathologies. It's natural history of high fetal perinatal morbidity and mortality. Despite options for antenatal intervention survivors of the neonatal period are still at high risk of severe renal impairment, bladder dysfunction, poor growth and male infertility which track through childhood and adolescence. The efficacy of antenatal interventions both diagnostic and therapeutic still

Key guidelines

  • Lower urinary tract obstruction (LUTO) is a disease of high mortality and morbidity.

  • Diagnosis may be made at second-trimester ultrasound.

  • Prognosis is related to amniotic fluid volume, presence of renal cysts, and other congenital or structural anomalies.

  • Intervention is available in the form of vesicoamniotic shunting, but the efficacy of this is not proven.

Research directions

  • The efficacy and long-term safety of vesicoamniotic shunting (PLUTO trial).

  • The predictive accuracy of ultrasound markers and fetal urinalysis for postnatal renal function.

Acknowledgement

This work is funded by Wellbeing of Women, the Hannah Eliza Guy Charity (Birmingham Children's Hospital Charity) and the NIHR HTA programme (ref 07/01/44). Dr R K Morris is funded by an NIHR Clinical Lectureship.

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