Best practice guidelinesLong-term renal and neurodevelopmental outcome in infants with LUTO, with and without fetal intervention
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
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Lower urinary tract obstruction (LUTO) is a disease of high mortality and morbidity.
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Diagnosis may be made at second-trimester ultrasound.
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Prognosis is related to amniotic fluid volume, presence of renal cysts, and other congenital or structural anomalies.
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Intervention is available in the form of vesicoamniotic shunting, but the efficacy of this is not proven.
Research directions
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The efficacy and long-term safety of vesicoamniotic shunting (PLUTO trial).
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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.
References (25)
- et al.
Correction of congenital hydronephrosis in utero I: The model: fetal urethral obstruction produces hydronephrosis and pulmonary hypoplasia in fetal lambs
J Pediatr Surg
(1983) - et al.
Correction of congenital hydronephrosis in utero. II: decompression reverses the effects of obstruction on the fetal lung and urinary tract
J Pediatr Surg
(1982) - et al.
Prenatally detected posterior urethral valves: qualitative assessment of second trimester scans and prediction of outcome
J Urol
(1997) - et al.
Percutaneous fetal cystoscopy and endoscopic fulguration of posterior urethral valves
Am J Obstet Gynecol
(1995) - et al.
Long-term outcome in children after antenatal intervention for obstructive uropathies
Lancet
(1999) - et al.
Boys with posterior urethral valves: outcome concerning renal function, bladder function and paternity at ages 31 to 44 years
J Urol
(2005) - et al.
Prognosis for patients with prenatally diagnosed posterior urethral valves
SO J Urol
(1992) - et al.
Outcome of posterior urethral valves: to what extent is it improved by prenatal diagnosis?
J Urol
(1999) - et al.
Prenatally detected posterior urethral valves: qualitative assessment of second trimester scans and prediction of outcome
SO J Urol
(1997) - et al.
Pyospermia in an adult cohort with persistent lower urinary tract symptoms and a history of ablated posterior urethral valves
J Pediatr Urol
(2010)
Sexual function and fertility in patients treated for posterior urethral valves
J Urol
Diagnosis and outcome of fetal lower urinary tract obstruction in the northern region of England
Prenat Diagn
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