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ORIGINAL RESEARCH |
From the 1Center for Fetal Diagnosis and Treatment, at The Children’s Hospital of Philadelphia, and the University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
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ABSTRACT |
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Methods: Clinical outcomes in 20 pregnancies with a singleton male fetus, oligo/anhydramnios, and lower urinary tract obstruction were studied using chart review and phone and written clinical questionnaire for parents, pediatricians, and urologists.
Results: Overall 1-year survival was 91%. Two neonatal deaths occurred from pulmonary hypoplasia. Mean gestational age at delivery was 34.6 weeks, mean days from shunting to delivery were 84.4, and mean birth weight was 2,574 g. Prenatal urinary prognosis was good in 13, borderline in 2, and poor in 3 of the survivors. Mean age at follow-up was 5.83 years. Posterior urethral valves were confirmed in 7 males, urethral atresia in 4, and prune belly syndrome in 7. Eight children had acceptable renal function, 4 had mild insufficiency, and 6 required dialysis and eventual renal transplant. Eleven children had normal bladder function with spontaneous voiding, 6 required catheterization, and 1 child still had a vesicostomy. Height and weight were below the 25th percentile in 9 children. Persistent respiratory problems were present in 8, musculoskeletal problems in 9, and frequent urinary tract infections were reported in 9. Health-related quality of life results in our group with lower urinary tract obstruction were similar to those in a healthy child population.
Conclusion: Male children who underwent prenatal bladder shunting were neurodevelopmentally normal. Although one third of the surviving babies required dialysis and transplantation, the majority have acceptable renal and bladder function and report satisfactory quality of life.
Level of Evidence: III
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MATERIALS AND METHODS |
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ABSTRACTMATERIALS AND METHODSRESULTSDISCUSSIONREFERENCES |
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Patients underwent detailed prenatal evaluation as summarized in Figure 1. Complete anatomic survey was performed to rule out the presence of other severe anomalies, and amnioinfusion was performed to restore amniotic fluid volume and enhance ultrasound imaging when required. Fetal karyotype was obtained from transabdominal chorionic villus sampling or amniotic fluid. Renal status was assessed by a minimum of 3 percutaneous bladder drainages performed at 48- to 72-hour intervals, analyzing for electrolytes, osmolality, and protein composition. On the basis of the vesicocentesis results, fetuses were categorized as good prognosis (sodium < 100 mmol/L, chloride < 90 mmol/L, calcium < 8 mg/dL, osmolality < 200 mmol/L, ß-2 microglobulin < 6 mg/L, total protein < 20 mg/dL), borderline (maximum of 2 values above good threshold criteria), and poor prognosis (
3 values above good threshold criteria). Parents were offered prenatal intervention if a normal male karyotype, isolated lower urinary tract obstruction, and a good or borderline urine profile was present. Three cases with poor predicted prognosis underwent shunting at parental request. Intervention was offered only for fetuses with clear evidence of urethral obstruction and oligo/anhydramnios. Prenatal records were reviewed for gestational ages at diagnosis, vesicocentesis, and vesicoamniotic shunting. Prenatal ultrasound findings, urine results, prenatal complications, birth weight, gestational age at delivery, mean days after shunting to delivery, associated malformations, neonatal follow-up, and final confirmed urology diagnosis were reviewed.
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Parents and physicians (pediatricians, urologists, and nephrologists) completed a brief questionnaire that detailed the child’s medical history, including height, weight, dietary needs, surgical procedures, and a systematic review of major body systems. Measures were converted to percentiles and referenced to standard graphs provided by the National Center for Health Statistics. Creatinine clearance was calculated by the Schwarz equation.16 Acceptable renal function was defined as creatinine clearance of more than 70 mL/min. Renal insufficiency was defined as a creatinine clearance of less than 70 mL/min that did not require renal replacement therapy. End-stage renal disease was defined as the requirement of renal replacement therapy, either dialysis or renal transplant. When creatinine clearance was not available, serum creatinine level was used and compared with normal, age-correlated reference values. The lowest creatinine clearance or serum creatinine level within the first year of life and the last creatinine clearance or serum level were used for renal function evaluation. Voiding habits were defined as spontaneous voiding, intermittent catheterization, or catheterization only. Social continence was defined as absence of urinary urgency, leaking accidents, or delay in toilet training with voiding accidents during the day or night time. Urinary tract infections refer to febrile infections or infections requiring antibiotic therapy, with frequent defined as more than 3 infections in a given year.
The PedsQL 4.0 (Pediatric Quality of Life Inventory) scale includes a child self-report and parent proxy-report scale developed to measure health-related quality of life in children and adolescents ages 2–18. The assessment consists of 23 items applicable for healthy populations and pediatric populations with acute and chronic health conditions. The test has been validated as a reliable measure of psychosocial well-being in children with chronic health issues.17,18 Values are reported as means ± standard deviation.
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RESULTS |
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All pregnancies were singletons with karyotype-confirmed male fetuses, oligo/anhydramnios, and lower urinary tract obstruction. Prenatal data are summarized in Table 2. Complications following vesicoamniotic shunt included shunt displacement (n = 5) requiring a second procedure in 4 cases and abdominal omental herniation (n = 2) observed postnatally through the shunting site following shunt displacement. Premature rupture of the membranes occurred in one case 4 days after placement.
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Neonatal data are summarized in Table 3. Four newborns were diagnosed as large for gestational age, and none were growth restricted. All babies had a complete urological evaluation in the newborn period to confirm the anatomic etiology of the obstruction.
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Long-term outcomes from physician and parental questionnaires are displayed in Tables 4 to 7. Neurological development was reported as normal in the majority, with one child described as being delayed in onset of walking and with high frequency hearing loss. Three children required speech and physical therapy.
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DISCUSSION |
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The natural history of obstructive uropathy is highly variable and dependent on etiology, severity, duration, and age of onset of the obstruction. Outcomes are usually measured in terms of newborn survival and are dependent on 2 factors: pulmonary development and renal function. Pulmonary hypoplasia is the leading cause of mortality in obstructive uropathy. Nakayama et al5 demonstrated a 45% mortality rate for posterior urethral valves that can be directly attributed to pulmonary insufficiency. Early midgestation oligohydramnios carries a poor prognosis for the fetus, and when associated with urethral obstruction, the mortality rate has been estimated as high as 95%.3 Therefore, fetuses in our study represent the most severe end of the obstructive uropathy spectrum. The survival rate after 1 year of age in our population that underwent prenatal treatment was high (91%). Two newborns died shortly after birth from pulmonary hypoplasia. One (good prenatal prognosis group) developed anhydramnios by 28 weeks gestation despite good shunt placement within the bladder, and the other (poor prognosis group) developed anhydramnios within 2 weeks of shunting and delivered at 26 weeks gestation due to preterm labor. No evidence of chorioamnionitis was found at time of the delivery. Despite these 2 cases, it appears that vesicoamniotic shunt in our selected population played a role by restoring amniotic fluid and allowing pulmonary development.
In 1999, we reported on the long-term outcomes after vesicoamniotic shunting in 14 children affected by lower urinary tract obstruction.13 However, this was a very heterogeneous population of children in which the etiology of the obstruction varied. The present study was designed to focus on the long-term outcomes of children with specific documented urethral obstructions. We again found growth and development abnormalities in these children, with height or weight below the 25th percentile in 50%. Although failure to thrive has been reported in boys with posterior urethral valves19,20 and prune belly syndrome,21 poor growth remains a cause of concern in this population despite acceptable renal function. However, the incidence and severity of growth problems in our present study appears to be less severe than previously reported, where 50% of children were below the 5th percentile for height and 36% for weight,13 and may reflect the more recent use of additional nutritional support in these children.
Half of the children report ongoing respiratory concerns (asthma and frequent infections). The majority (65%) of patients with recurrent pulmonary infections had prune belly syndrome, a predisposition previously described.21 Two patients had limitation in their daily activities, with functional tests showing obstructive and restrictive pulmonary disease in one child.
Musculoskeletal abnormalities were identified in 50% of our children, with the prune belly syndrome children more frequently affected. However, this was not unexpected, given that pectus excavatum, scoliosis, or limb deformities were found in 45% of children with prune belly syndrome reported by Loder et al.22
Our study showed that infants from the posterior urethral valves group less frequently had renal failure, less frequently were transplanted, or were transplanted later in childhood than infants from the prune belly syndrome and urethral atresia groups. Children with urethral atresia had the worst renal outcomes, with 50% transplanted in infancy. Proper assessment of the effect of prenatal intervention on postnatal renal function is still difficult. Despite advances in the use of fetal electrolytes and proteins, assessment can only estimate prenatal renal injury. Most importantly in the postnatal period, pre-existing prenatal renal injury may be exacerbated by febrile urinary tract infections, reflux, and residual bladder dysfunction. Therefore long-term creatinine measurements or late progression to renal failure may not reflect antenatal injury or the effectiveness of prenatal intervention but may represent progressive morbidity caused by postnatal urinary tract dysfunction and infections. In future studies, to better assess the effect of antenatal intervention on renal function, we will need to compare our prenatal evaluations with the lowest creatinine concentration in the first year of life and with the most recent creatinine concentration in later childhood to differentiate prenatal from postnatal renal morbidity.
Although the majority of children can expect to have acceptable social continence, they remain at increased risk for recurrent infections. Children in the prune belly syndrome group appear to have the highest incidence of voiding abnormalities, with only 43% of the children achieving spontaneous voiding. This is likely due to histologic changes in the urinary tract, including decreased bladder smooth muscle and increased connective tissue components, leading to reduced detrusor contractility.23
Health-related quality of life has emerged as an important patient-based health outcome and is conceptualized as a patient or parental perception of the impact of the disease and treatment functioning in a variety of dimensions, including physical, mental, and social domains. Varni et al18 compared PedsQL 4.0 scores from a healthy population with those from a chronically ill population and found them to be statistically different. In the healthy population, 717 parents and 401 children had mean scores (± standard deviation) of 87.61 (± 12.33) and 83 (± 14.79), respectively. In a chronically ill population, 662 parents and 367 children had scores of 74.22 (± 18.4) and 77.19 (± 15.53), respectively. Our results showed that children prenatally shunted for lower urinary tract obstruction believe that their life is worthwhile, with a mean score of satisfaction as high as that described in a healthy population,18 despite the presence of ongoing medical problems. Scores in the prune belly syndrome group were lower than those of children with posterior urethral valves and urethral atresia, likely reflecting their higher incidence of respiratory and musculoskeletal problems as well as bladder dysfunction.
In summary, prenatal intervention for obstructive uropathy appears to increase pulmonary survival and improve renal outcomes, compared with a natural history of untreated, prenatally diagnosed lower urinary tract obstruction in the setting of oligo/anhydramnios. Given our data, parents can be counseled that, although their children will require attentive medical and surgical care throughout childhood, they can be expected to have normal cognitive development and the majority will achieve acceptable social continence and maintain a satisfactory quality of life. In the future, efforts will continue to focus on improving postnatal medical and surgical management to prevent additional morbidity and increasing the percentage of children that maintain acceptable renal function during their lifetimes and not require renal replacement.
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Footnotes |
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Corresponding author: Mark P. Johnson, MD, The Center for Fetal Diagnosis and Treatment, 5th Floor –Wood Center, CHOP, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104-4399; e-mail: Johnsonma{at}email.chop.edu.
doi:10.1097/01.AOG.0000171117.38929.eb
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REFERENCES |
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2. Johnson MP, Freedman AL. Fetal uropathy. Curr Opin Obstet Gynecol 1999;11:185–94.[Medline]
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16. Schwartz GJ, Brion LP, Spitzer A. The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents. Pediatr Clin North Am 1987;34:571–90.[Medline]
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18. Varni JW, Seid M, Kurtin PS. PedsQL 4.0: Reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient population. Med Care 2001;39:800–12.[Medline]
19. Krueger RP, Hardy BE, Churchill BM. Growth in boys with posterior urethral valves. Urol Clin North Am 1980;7:265–72.[Medline]
20. Reinberg Y, de Castano I, Gonzalez R. Influence of initial therapy on progression of renal failure and body growth in children with posterior urethral valves. J Urol 1992;148:532–3.[Medline]
21. Geary DF, MacLusly IB, Churchill BM, McLorie G. A broader spectrum of abnormalities in the prune belly syndrome. J Urol 1986;135:324–6.[Medline]
22. Loder RT, Guiboux J, Bloom DA, Hensinger RN. Musculoskeletal aspects of prune belly syndrome. Am J Dis Child 1992;146:1224–9.[Abstract]
23. Kinahan TJ, Churchill BM, McLorie G, Gilmour RF, Khoury AF. The efficiency of bladder emptying in the prune belly syndrome. J Urol 1992;148:600–3.[Medline]
This article has been cited by other articles:
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  R K Morris, K S Khan, and M D Kilby Vesicoamniotic shunting for fetal lower urinary tract obstruction: an overview Arch. Dis. Child. Fetal Neonatal Ed., May 1, 2007; 92(3): F166 - F168. [Full Text] [PDF]
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 I. Klaassen, T. J. Neuhaus, D. E. Mueller-Wiefel, and M. J. Kemper Antenatal oligohydramnios of renal origin: long-term outcome Nephrol. Dial. Transplant., February 1, 2007; 22(2): 432 - 439. [Abstract] [Full Text] [PDF]
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