Long-Term Survival in an Infant with Urethral Atresia

Steinhardt, George F.; Hogan, William B.; Wood, Ellen G.; Weber, Thomas; Lynch, Robert E.

doi:10.1016/s0022-5347(17)39952-4

Cited by 34 publications

(17 citation statements)

References 8 publications

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“…LUTO, usually in form of posterior urethral valves (PUVs) or urethral atresia, is more prevalent in male than in female fetuses [1][2]. Depending on the cause and degree of urethral obstruction, LUTO may be associated with 45-100% prenatal mortality [3], and even postnatal survivors often develop end-stage chronic renal impairment [4].…”

Section: Introductionmentioning

confidence: 99%

Early vesico-amniotic shunting — does it change the prognosis in fetal lower urinary tract obstruction diagnosed in the first trimester?

Dębska¹,

Kretowicz²,

Olędzka³

et al. 2017

Ginekol Pol

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Objectives: The aim of the study was to assess the outcome of vesico-amniotic shunting performed before 16 weeks of pregnancy in fetuses with severe megacystis diagnosed in the first trimester of pregnancy. Material and methods:Between January 2008 and October 2012 severe megacystis with the bladder length > 15 mm was diagnosed in 17 fetuses. The procedure of early vesico-amniotic shunting (VAS) was offered to 8 patients with presumably isolated LUTO.The procedure of VAS was performed in 6 fetuses. Before the intervention one or two procedures of vesicocentesis and urine analysis were performed. Results:In all treated cases shunts provided urinary tract decompression. All babies were born prematurely, 2 of them died due to premaurity, 3 of them survived and have normal renal function at the age of 5-6 years. In 4/5 children accompanying malformations were later diagnosed, in 1 born prematurely neonate necropsy was not performed. Conclusions:Our results suggest that early vesico-amniotic shunting in fetal LUTO is feasible and may potentially prevent not only pulmonary hypoplasia but also renal insufficiency. However, the rationale of the procedure needs further investigation due to a high risk of long-term morbidity and co-existing malformations in children Before offering the therapy detailed counseling of the parents about the possible pros and cons of the therapy is necessary.

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Section: Introductionmentioning

confidence: 99%

Early vesico-amniotic shunting — does it change the prognosis in fetal lower urinary tract obstruction diagnosed in the first trimester?

Dębska¹,

Kretowicz²,

Olędzka³

et al. 2017

Ginekol Pol

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“…The most common anatomical abnormalities are posterior urethral valves (PUVs). 1 These congenital, structural anomalies account for one-third of renal tract malformations detected at autopsy following the termination of pregnancy for ultrasound-diagnosed fetal anomaly. 2 The fetus is typically male.…”

Section: Introductionmentioning

confidence: 99%

Congenital lower urinary tract obstruction: a population‐based epidemiological study

Malin

Tonks

Morris

et al. 2012

BJOG

149

121

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Objective To determine the prevalence and outcome of lower urinary tract obstruction (LUTO), including the sensitivity of antenatal diagnosis.Design A retrospective population-based study. Setting Regional population-based congenital anomalies register (WMCAR).Population Fetuses affected by LUTO delivered between 1995 and 2007 to women resident in the West Midlands.Methods Cases were selected from the WMCAR using codes and keyword terms from the International Classification of Diseases, tenth revision (ICD10). Diagnoses were validated using additional data sets from Regional Fetal Medicine, Perinatal Pathology and Paediatric services.Main outcome measures Rates of prevalence, prenatal diagnosis and mortality, with trends.Results There were 284 LUTO cases in 851 419 births during the study period, representing a total prevalence of 3.34 (2.95-3.72) per 10 000 births, and this prevalence did not change significantly over time. The prevalence of LUTO was significantly higher in Black and minority ethnic groups when compared with white Europeans (OR 2.38; 95% CI 1.87-3.03), and are associated with area-based deprivation measures (P < 0.01). Of all LUTO cases, 221 (77.8%) were isolated, and the remainder were associated with other structural or chromosomal anomalies. The most common subtype was posterior urethral valves (PUVs; n = 179, 63%). In total there were 211 (74.3%) cases of isolated, nonfemale, singleton fetuses that fitted the referral criteria for in utero vesico-amniotic shunting, giving a prevalence of 2.48 (2.14-2.81) per 10 000 live births. Within this group, the prenatal diagnosis rate was 46.9% (99/211).Conclusion This is the largest population-based study of LUTO that has been performed to date, and provides accurate estimates for prevalence. The low prevalence and relatively low rate of antenatal detection limit the number of cases amenable to prenatal surgical intervention.

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“…Two fetuses had diagnoses other than posterior urethral valves to account for oligohydramnios, 1 with urethral atresia and 1 with unilateral multicystic dysplas- tic kidney with contralateral agenesis. Urethral atresia might be expected to have a sonographic appearance similar to urethral valves, however, the outcome is uniformly dismal, there is only one survivor in the literature [17,20]. Those fetuses with normal amniotic fluid have a much milder form of disease due to minor anatomic variations or incomplete urethral obstruction, and therefore have a much better prognosis than those with oligohydramnios.…”

Section: Discussionmentioning

confidence: 99%

Posterior Urethral Valves: Inaccuracy of Prenatal Diagnosis

1998

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This study was undertaken to determine the specificity of prenatal diagnosis of posterior urethral valves. Twenty-two fetuses were diagnosed in utero with posterior urethral valves due to the presence of persistent megacystis and hydronephrosis. Ten cases had oligohydramnios and 12 had normal or increased fluid. Confirmation of diagnosis was not available in 3 fetuses. Only 8 of 19 fetuses had postnatal confirmation of posterior urethral valves. Other anomalies included urethral atresia, ureteral duplication, megacystis/megaureter, reflux and multicystic dysplastic kidney. We conclude that in utero evidence of megacystis and hydronephrosis with or without oligohydramnios is not diagnostic of posterior urethral valves.

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Long-Term Survival in an Infant with Urethral Atresia

Cited by 34 publications

References 8 publications

Early vesico-amniotic shunting — does it change the prognosis in fetal lower urinary tract obstruction diagnosed in the first trimester?

Early vesico-amniotic shunting — does it change the prognosis in fetal lower urinary tract obstruction diagnosed in the first trimester?

Congenital lower urinary tract obstruction: a population‐based epidemiological study

Posterior Urethral Valves: Inaccuracy of Prenatal Diagnosis

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