Fetal renal anomaly: Factors that predict survival

ObjectiveTo find out the relative prevalence of renal anomalies detected in the antenatal period, and to look at factors that predict the postnatal outcome.MethodsIn this prospective study, all antenatal-detected renal anomalies booked at the tertiary health centre were evaluated and counselled. Aspects such as type of renal anomaly, oligohydramnios and presence of additional anomalies were noted. Stillborn babies underwent autopsy; all live born babies were followed for one year. Appropriate statistical analyses were performed to compare the antenatal factors with outcomes.ResultsRenal anomalies were detected in 136 out of 587 cases with major fetal anomalies. Most of the women were primiparous (65.4%). The mean gestation at presentation was 30 weeks; in 12 cases, diagnosis was possible before 20 weeks (8.8%). Antenatal hydronephrosis was the most commonly seen anomaly, with 61 cases; this was followed by bilateral cystic kidney in 50 cases. Out of the 136 cases, 12 (8.8%) underwent termination of pregnancy and 60 (44.1%) babies were stillborn. Autopsy was performed in 58 out of 72 (80.6%) cases after consent. Karyotyping was performed in 49 cases and abnormalities were detected in two (4.1%) of them. A total of 64 (47.1%) babies were live born; after one year, 49 (36.0%) of them were alive. Postnatal survival was highest in unilateral disease (85.7%). In cases with oligohydramnios, there was only 3.4% survival after one year; none of the cases with cystic kidney and oligohydramnios survived. The period of gestation at presentation of non-survivors was 25.9 weeks compared to 32.5 weeks with survivors. Among the cases with extra renal anomaly, 7.0% survived; none of the cases with associated cranio-vertebral defect or polydactyly survived after a year.ConclusionOut of the different renal pathologies that were diagnosed, survival was highest in the unilateral group. The factors associated with poor prognosis included bilateral disease, absence of amniotic fluid and presence of associated malformation.     

Vesico‐amniotic shunting for lower urinary tract obstruction in a fetus with VACTERL association

Newborn cases of VACTERL association with lower urinary tract obstruction (LUTO) are rare and there have been no reports on those patients undergoing fetal therapy in English literature. We successfully performed vesico‐amniotic shunting in a fetus having LUTO caused by abnormality of the external genitalia at 16 weeks’ gestation. Although fetal karyotype was normal 46XY, follow‐up fetal ultrasound examinations revealed ventriculomegaly in the brain, a small stomach and a right multicystic dysplastic kidney. MRI at 31 weeks’ gestation suggested lobar type holoprosencephaly. Diagnosis of VACTERL association was confirmed postnatally. We consider that vesico‐amniotic shunting is indicated for a fetus of VACTERL association with LUTO if the parents wish the procedure after genetic counseling and explanations about the fetal conditions.     

Surgical interventions and anesthesia in the 1st year of life for lower urinary tract obstruction

Background

Patients with a prenatal diagnosis of lower urinary tract obstruction (LUTO) may undergo prenatal interventions, such as vesicoamniotic shunt (VAS) placement, as a temporary solution for relieving urinary tract obstruction. A recent FDA communication has raised awareness of the potential neurocognitive adverse effects of anesthesia in children. We hypothesized as to whether a prenatal LUTO staging system was predictive of the number of anesthesia events for prenatally diagnosed LUTO patients.

Cumulative incidence of outcomes and urologic procedures after augmentation cystoplasty

ObjectiveAugmentation cystoplasty (AC) is a major surgery that can be associated with long-term morbidity. This study aimed to describe the cumulative incidence of outcomes and urologic procedures in a large cohort of children who underwent AC, identify significant sources of morbidity, and to evaluate baseline factors associated with outcomes of interest.MethodsChildren ≤18 years who underwent AC in the Pediatric Health Information System from 1999 to 2010 were included. All follow-up encounters up to June 2012 were included. Cumulative incidences for 15 outcomes and urologic procedures were calculated using non-informative censoring. Sensitivity analyses were performed to determine effect of censoring assumptions and including hospitals without complete datasets. As an exploratory analysis, baseline patient factors were evaluated for associations with outcomes and urologic procedures of interest using multivariable Cox proportional hazards models adjusted for clustering by hospital.Results2831 AC patients were identified. Based on cumulative incidence calculations and sensitivity analyses; the cumulative incidence ranges of outcomes and procedures at 1, 3, 5, and 10 years were calculated. Examples of 10-year cumulative incidence ranges are given for the following outcomes and procedures: bladder rupture (2.9–6.4%), small bowel obstruction (5.2–10.3%), bladder stones (13.3–36.0%), pyelonephritis (16.1–37.1%), cystolithopaxy (13.3–35.1%), and reaugmentation (5.2–13.4%). The development of chronic kidney disease was strongly associated with a diagnosis of lower urinary tract obstruction (HR 13.7; 95% CI 9.4–19.9). Bladder neck surgery and stoma creation at time of AC were associated with an increased hazard of bladder rupture (HR 1.9; 95% CI 1.1–3.3) and bladder stones (HR 1.4; 95% CI 1.1–1.8) respectively.ConclusionsOutcomes of interest and urologic procedures after AC are common. Results from this large cohort can be used to counsel patients and families about expectations after AC. Pyelonephritis, chronic kidney disease, further reconstructive surgery, and calculus disease appear to cause significant morbidity. Collaborative efforts are needed to further reduce morbidity in this patient population.     

Successful treatment of lower urinary tract obstruction with peritoneal-amniotic and vesicoamniotic shunting

Lower urinary tract obstruction (LUTO) which is most commonly caused by posterior urethral valves or urethral atresia, is often associated with high perinatal mortality and morbidity and long-term childhood renal dysfunction or even renal failure. LUTO is an uncommon clinical entity that can be diagnosed by ultrasonographic examination and is particularly presented by enlarged bladder, hydronephrosis, hydroureter and oligohydramnios. Despite being a rare pathology of the genitourinary tract, detection rates of it is higher than other congenital abnormalities with ultrasonography. Moreover, ultrasonography has become the most widely used diagnostic tool in the diagnosis of LUTO. Pulmonary hypoplasia resulting from severe oligohydramnios is the most common cause of death. Bladder drainage has a critical role on perinatal survival in fetuses with LUTO especially in those predicted to have a poor prognosis. Further interventions and observations of renal therapies may be required in childhood.     

Urodynamic performance in boys with Y-type urethral duplication

Purpose

The aim of this study was to elucidate the urodynamic features of patients with Y-type urethral duplication.