Postnatal Management in Congenital Lower Urinary Tract Obstruction With and Without Prenatal Vesicoamniotic Shunt

Purpose: Congenital lower urinary tract obstruction (cLUTO) includes a heterogeneous group of conditions caused by a functional or mechanical outlet obstruction. Early vesicoamniotic shunting (VAS) possibly reduces the burden of renal impairment. Postpartum, pediatric urologists are confronted with neonates who have a shunt in place and a potentially impassable urethra with a narrow caliber. Early management of these patients can be challenging. Here, we would like to share the approach we have developed over time.Materials and Methods: We conducted a single-center retrospective analysis from 2016 to 2020 and included all patients diagnosed with cLUTO. Data focusing on time point and type of intervention was collected. Furthermore, patients with temporary diversion via a percutaneous VAS were selected for a more detailed review.Results: In total, 71 cases of cLUTO were identified during the study period. Within this group, 31 neonates received postnatal management and surgical intervention in our center. VAS was performed in 55% of these cases (N = 17). The postnatal treatment varied between transurethral or suprapubic catheterization and early Blocksom vesicostomy. In five infants with VAS, the urinary drainage was secured through the existing VAS by inserting a gastric tube (N = 1) or a 4.8 Fr JJ-stent (N = 4). To our knowledge, this is the first report of a stent-in-stent scheme, which can remain indwelling until the definite treatment.Conclusion: Having a secure urine drainage through a VAS allows the often premature infant to grow until definite surgery can be performed. This avoids placing a vesicostomy, which requires anesthesia.

Evaluation of Sequential Urine Analysis when Selecting Candidates for Vesicoamniotic Shunting in Lower Urinary Tract Obstruction

Objective: The objective of our study was to assess the utility of sequential fetal urine analysis in severe lower urinary tract obstruction (LUTO) when selecting cases suitable for vesicoamniotic shunting. Material and Methods: This was a retrospective cohort study of cases of severe LUTO treated in our fetal medicine center from 1994 to 2013. Two fetal bladder samples were taken 24–48 h apart to assess renal function. A vesicoamniotic shunt was inserted in case of improvement in urinary biochemistry between the 2 samples. We assessed perinatal morbidity and mortality and renal function at 5 years. Results: Among a total of 26 LUTO cases with sequential urine analysis, 5 showed normal urinary biochemistry, 13 were abnormal, and 8 improved between the 2 samples. These 8 cases underwent vesicoamniotic shunt placement, leading to the birth of 6/8 (75%) live infants, 5/6 (83%) of whom had normal renal function at 5 years. The 5 cases with normal biochemistry occasioned 2 neonatal deaths and 3 children with normal renal function at 5 years. Elective termination of pregnancy was requested by parents for the fetuses exhibiting abnormal biochemistry. Conclusion: An improvement in urinary biochemistry between 2 sequential fetal bladder punctures in severe LUTO could be an effective criterion in the selection of candidates for vesicoamniotic shunting. However, the benefit of a shunt in fetuses with normal amniotic fluid remains to be evaluated in clinical trials.

Evaluation of Fetuses Diagnosed With Megacystis During Prenatal Period

<p><strong>Objectives:</strong> Fetal megacystis is very rare; however this problem may be associated with other structural or chromosomal abnormalities leading to morbidity and mortality. Due to renal injury and pulmonary hypoplasia, prognosis is poor especially in fetuses with early onset oligohydramnios. In this study, we evaluated the management strategies and outcomes of fetal megacystis in our hospital.</p><p><strong>Study Design:</strong> The data of cases who were prenatally diagnosed with megacystis between 1 January 2017 and 31 December 2019 were analyzed. Ultrasonography findings and antennal interventions were withdrawn from computerized database. Information about postpartum status was also received. The data were analyzed in terms of diagnostic methods, fetal interventions (vesicosynthesis (VS), vesicoamniotic shunt (VAS)), potential prognostic markers and short/long-term prognostic results.</p><p><strong>Results:</strong> A total of 15 megacystis patients were detected. One of the fetuses was female and the remaining cases were male. Six of the patients were isolated. Ten patients underwent invasive procedures for prenatal aneuploidy diagnosis and one of them diagnosed with trisomy 18. Totally, 4 patients underwent VS; however no VAS was recorded. Four fetuses were terminated and intrauterine fetal demise occurred in 2 fetuses. Four patients were lost during follow up. Survival rate was 33.3%.</p><p><strong>Conclusion: </strong>Fetal megacystis is an important ultrasonographic finding which may be a component of chromosomal / genetic anomalies. This problem may also be isolated in some cases. Because of the poor outcome, parents should be well informed and all interventions should be offered to families.</p>

Diagnosis and Medical Management of Prune Belly (Triad) Syndrome

Incidence of prune belly syndrome (PBS) has remained stable for several decades; advances have been made in prenatal diagnosis with the aid of advanced US and magnetic resonance technology. Use of fetal MRI as an adjunct to US, especially in the setting of oligohydramnios, anhydramnios, and maternal obesity, makes additional imaging methods potentially beneficial. MRI is currently accepted as a valuable technique for fetal anomalies assessment. Addition of three- and four-dimensional US has improved specificity to the traditional two-dimensional imaging. The following discusses the in utero assessment, neonatal and childhood evaluation, and management of PBS. This review contains 5 figures, 2 tables, and 48 references. Key Words: Eagle-Barrett, fast-scanning MRI, fetal ultrasonography, in utero hydronephrosis, prune belly syndrome, triad syndrome, vesicoamniotic shunt,pediatric renal insufficiency

Fetal Lower Urinary Tract Obstruction: An Update

Fetal lower urinary tract obstruction [LUTO] represents a rare, heterogeneous group of uropathies involving mechanical or functional blockage at the bladder outlet of varying severity. While ultrasound is a fair screening tool for the antenatal detection of this condition, it cannot reliably determine the underlying etiology of LUTO presentations. Sonographic assessment of renal appearance and amniotic fluid volume, as well as urinary analyte analysis, are the most useful predictors of preserved renal function in fetal LUTO, although they, too, are limited in their ability to predict outcomes on an individual basis. Animal models suggest that obstructive uropathy likely causes irreparable damage to the renal and urinary collecting system injury early in pregnancy. When LUTO is associated with abnormal amniotic fluid volume, long-standing oligohydramnios may result in lethal pulmonary hypoplasia. Data evaluating the efficacy of vesicoamniotic shunt therapy for the treatment of fetal LUTO are inconclusive. While vesicoamniotic shunting may modify disease lethality by restoring fluid volume, renal and other genitourinary benefits are minimal if not altogether absent, arguably supporting the belief that bladder decompression is being performed after irreversible injury has already been sustained.