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PubMed2006

National rates of birth defects among hospitalized newborns.

BACKGROUND: The Healthcare Cost and Utilization Project (HCUP) family of hospital discharge databases offer an unprecedented opportunity to generate national estimates of newborn infants with birth defects. This report estimates national hospital admissions for newborn infants diagnosed with birth defects computed from HCUP and compares them to pooled prevalence figures computed from state birth defect surveillance systems. METHODS: HCUP-derived rates of 36 birth defects from 1997 through 2001 were compared to rates derived from pooled data reported by 26 state-based surveillance systems stratified by inclusion of elective terminations in case definitions. Rate ratios (RRs) were calculated for each birth defect by dividing the rate derived from HCUP by the rate derived from the relevant surveillance systems. RESULTS: HCUP newborn hospitalization rates for birth defects closely approximate pooled birth defect rates for surveillance systems that do not include elective terminations. HCUP rates were not significantly different for 35 of 36 defects. Overall, 20 HCUP rates were within 10% of state rates, 11 more were within 20% of state rates, and only 1 differed by more than 50%. HCUP rates compared most closely to state rates for cardiovascular (VSD RR = 0.98, ASD = 0.96, pulmonary valve atresia and stenosis = 0.92), orofacial (cleft palate RR = 1.10, cleft lip = 1.06), and genitourinary defects (obstructive genitourinary RR = 1.01, bladder exstrophy = 0.97). HCUP rates compared less favorably to rates derived from surveillance systems that included elective terminations. CONCLUSIONS: HCUP data approximate state-based surveillance system data for defects that are easily recognized in the newborn period and infrequently a cause for elective termination. HCUP data can be used to examine the impact of public health efforts on the number of infants born with birth defects as well as the cost and consequences of variations in the hospital management of birth defects.

Child, Hospitalized↗
PubMed2001

Prenatal diagnosis: does it alter outcome?

The prenatal detection of urinary tract anomalies is changing paediatric practice but in many areas the impact on clinical outcome remains difficult to quantify. However it is already apparent that termination of pregnancy has reduced the numbers of infants with lethal pulmonary hypoplasia and renal dysplasia who would previously have been liveborn but destined to succumb as neonates. Similarly, referrals of major non lethal abnormalities such as bladder exstrophy are declining as parents increasingly opt for termination. Fetuses at greatest risk of early onset postnatal renal failure can now be identified with considerable accuracy on prenatal ultrasound. Termination, prompted by quality of life considerations, could result in reduced numbers of infants and young children requiring end stage renal failure treatment in the first few years of life. Pre natal detection of anomalies such as PUJ obstruction and reflux undoubtedly provides an opportunity to avert functional deterioration and minimise urinary infection. But the proportion of children who genuinely benefit has proved difficult to assess. The prenatal detection of mild dilatation is of doubtful benefit in all but a minority of cases. Clinically significant underlying pathology is rare yet this common prenatal finding often generates disproportionate parental anxiety.

Fetal Diseases↗
PubMed1995

Spurious rise in total carbon dioxide and chloride with negative anion gap after cystogram.

We report a case of spurious hyperchloraemia, elevated total carbon dioxide and negative anion gap 9 days following a voiding cystourethrogram (VCU) in a patient with bladder exstrophy and obstructive uropathy. We believe that the spurious laboratory results were due to analytical interference of the absorbed iodine with the method of bicarbonate and chloride measurement. The absorbed iodine was retained in the circulation for an extended period because of associated renal impairment. Our patient was also on piperacillin which might have interfered with iodine clearance. Physicians and laboratory pathologists should be aware of this effect when interpreting laboratory results of patients who have undergone a VCU in association with obstructive uropathy and impaired renal function.

Acid-Base Equilibrium↗
PubMed2005

The future of pediatric nephrology.

The delineation of renal disease in children dates to the 1880s with descriptions of Henoch's purpura, bladder exstrophy, renal rickets and nephritis. The discipline of pediatric nephrology mainly emerged during the 20th century in response to problems in fluid and electrolyte balance, characterization of the nephrotic syndrome, use of renal biopsy, antibiotic therapy of urinary tract infections, dialysis and transplantation in children, growth problems associated with chronic renal failure, detection and therapy of hypertension, and the creation of both national and international pediatric nephrology societies and a journal now in its 18th year. The development of molecular and cell biology, genetic and genomic techniques and bioinformatics methods underlie many future directions. We should anticipate further elucidation of single gene disorders, of complex trait analysis of disorders, such as diabetic nephropathy and hypertension, the interplay of developmental genes and gene products and interactions within the podocyte. Specific therapies directed against inflammation, vascular damage, cyst development, the ravages of proteinuria and graft rejection (or induction leading to tolerance) will be developed. Stem cell therapies may replace lost renal mass, even of specific nephron sites. Novel therapies will also modulate the cell cycle, tyrosine kinase signaling and apoptosis. In addition, drugs will be specifically tested in children for many renal conditions. Larger and more specialized registries will be developed; epidemiologic studies and exploration of large data sets will lead to clinical guidelines that are evidenced-based. There is a need for more careful measurement of glomerular filtration rate (GFR), proteinuria and cytokines, and a fuller appreciation of the nutritional and hormonal role of the kidney. Finally, the antecedents of adult renal disease and the need to intervene in a proactive fashion will be realized. Despite these impressive advances in care, the greatest challenges will be in providing children with renal disease access to well-trained pediatric nephrologists, especially in Asia (1 billion children), Africa, Central and South America, and in immigrant and refugee populations. Included in this challenge is the capacity to have affordable access to use of contemporary techniques, and effective medications and prevention strategies. The International Pediatric Nephrology Association (IPNA), its journal, and pediatric advocates will need to use their energies to take on these challenges.

Child↗
PubMed2006

The risk of birth defects in multiple births: a population-based study.

OBJECTIVES: To determine if multiple births have higher risks of birth defects compared to singletons and to identify types of birth defects that occur more frequently in multiple births, controlling for seven sociodemographic and health-related variables. METHODS: A retrospective cohort study was conducted of all resident live births in Florida during 1996-2000 using data from a population-based surveillance system. Birth defects were defined as in the 9th edition of the International Classification of Diseases-Clinical Modification (ICD-9-CM) code for the 42 reportable categories in the Centers for Disease Control and Prevention (CDC) Birth Defects Registry list and eight major birth defects classifications. Relative risks (RR) before and after adjusting for control variables and 95% confidence intervals (95% CI) were calculated. The control variables included mother's race, age, previous adverse pregnancy experience, education, Medicaid participation during pregnancy, infant's sex and number of siblings. RESULTS: This study included 972,694 live births (27,727 multiple births and 944,967 singletons). Birth defects prevalence per 10,000 live births was 358.50 for multiple births and 250.54 for singletons. After adjusting for control variables, multiple births had a 46% increased risk of birth defects compared to singletons. Higher risks were found in 23 of 40 birth defects for multiple births. Five highest adjusted relative risks for birth defects among multiple births were: anencephalus, biliary atresia, hydrocephalus without spina bifida, pulmonary valve atresia and stenosis, and bladder exstrophy. Increased risks were also found in 6 out of 8 major birth defects classifications. CONCLUSIONS: Multiple births have increased risks of birth defects compared to singletons.

Adult↗
PubMed1991

Inguinal hernia in children: factors affecting recurrence in 62 cases.

This report analyzes factors associated with 71 recurrent inguinal hernias in 62 children treated between 1976 and 1988. Cases were evaluated for sex, age, type of initial repair, interval to recurrence, the presence of comorbid conditions, and type of reoperation. There were 57 boys and 5 girls. Sixty percent of patients were less than 6 months old and 72% were less than 1 year of age at the time of the initial repair. Recurrence was on the right in 74%, left in 24%, and bilateral in 2%. Recurrence was noted by 6 months in 50%, by 2 years in 76%, and by 5 years in 96%. Comorbid conditions were present in 60% of cases, including increased intraabdominal pressure (ventriculoperitoneal [VP] shunts), growth failure, prematurity, chronic pulmonary disease, bladder exstrophy, connective tissue disorders, cryptorchism, seizure disorder, and malnutrition. Incarceration was a factor in four of the 62 cases. Seven patients had multiple recurrences. Fifty-one recurrences were indirect, whereas 20 were direct inguinal hernias. Inadequate high ligation (three with chromic catgut), wound infection, and groin hematoma were other findings. The direct hernias may be related to injury to the floor of the canal during initial repair. Recurrent repair included high ligation of the sac alone (20), high ligation plus snugging of a large internal ring (11), and high ligation with repair of the iliopubic tract in patients with VP shunts, connective tissue disorder, or weak floor (20). All direct hernias had a Cooper's ligament (McVay) repair. Two direct hernias recurred again and were successfully repaired using a preperitoneal approach.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1975

Urinary incontinence: management with electronic stimulation of muscles of pelvic floor.

The evolution of the management of incontinence with electronic techniques is reviewed. Seven patients with urinary incontinence due to various causes were treated with external stimulators connected to intra-anal plug electrodes. One cure, 2 controls, 3 improvements, and 1 failure were noted. There are encouraging signs in the management of incontinence following closure of bladder exstrophy.

Aged↗
PubMed2006

Congenital anomalies are commonly associated with exomphalos minor.

AIM: The objective of this study is to define the incidence of chromosomal and congenital anomalies in neonates with exomphalos major and minor. BACKGROUND: Incidence of major congenital anomalies varies from 35% to 81% in exomphalos. It is unclear whether these malformations are more common with exomphalos major. MATERIAL AND METHODS: The case notes of 82 antenatal diagnoses of exomphalos, made between January 1998 and December 2004, were retrospectively reviewed. Exomphalos major was defined as a defect 5 cm or greater and exomphalos minor a defect less than 5 cm in diameter. RESULTS: There were 72 live births, 6 still births, and 4 terminations of pregnancy. There was no statistical significance between exomphalos major and minor regarding mode of delivery, gestational age at birth, birth weight, major cardiac anomalies (21% vs 23%), and renal and external genitalia abnormalities (11% vs 18%). Chromosomal anomalies, syndromes, and dysmorphism were common in exomphalos minor 17 (39%, P = .0001). Congenital malformations of the gastrointestinal tract (14% vs 27%), central nervous system (0 vs 21%), and Wilms' tumor (0 vs 5%) occurred commonly in exomphalos minor. Limb abnormalities (25% vs 5%), ectopia cordis (11% vs 0), and bladder exstrophy (7% vs 0) occurred predominantly in exomphalos major. Mean follow-up was 34 months. Three neonates with exomphalos major died. Overall mortality was 4%. CONCLUSIONS: Chromosomal anomalies and syndromes occur more commonly in exomphalos minor. Exomphalos minor and major seem to have a predilection for associated anomalies of specific organ systems. This predisposition may help in counseling parents, planning investigations, and organization of multidisciplinary management strategy.

Abnormalities, Multiple↗
PubMed1986

Omphalocele: a 25-year experience.

Between 1958 and 1983, 92 neonates with omphalocele were admitted to Ste-Justine Hospital. The male to female ratio was 3:2. Birth weight ranged from 1,450 to 5,100 g (mean 2,786 g). Associated anomalies, apart from malrotation, were present in 45%. They were cardiovascular (18.4%), vesico-intestinal fissure or bladder exstrophy (11.9%), Beckwith Wiedeman (6.5%), and chromosomal abnormalities (7.5%). Seven patients were not treated. Topical applications were used in seven cases (1 survivor). In 65%, primary closure was achieved; the mortality rate was 20% with a mean hospital stay of 17.5 days. Silastic was used in 12 cases with a mortality of 5/12 and a mean hospital stay of 82.1 days. The mean number of reductions was 6.7. Omphalocele rupture did not influence mortality. Prior to 1974 the mortality rate was 50%. Since 1974 it has decreased to 31.5%. The advent of total parenteral nutrition (TPN) was an important factor in decreasing the mortality. Prior to 1974, 23 patients survived, only one with a major associated anomaly (Fallot). After 1973, 35 survived, 13 having a major associated anomaly. Overall mortality was 9% when no other major malformations were present. A retrospective study of 92 cases of omphalocele over a 25-year period reveals an overall mortality rate of 37%. Death was associated almost exclusively with additional congenital anomalies. Birthweight in itself was not a determining prognostic factor. With the advent of TPN and a better knowledge of the mechanical ventilation of the neonate the results are better and involve the survival of a greater number of patients with serious associated malformations.

Birth Weight↗
PubMed2006

Victor Fray Marshall: twentieth century renaissance urologist.

PURPOSE: Victor Fray Marshall (1913 to 2001) contributed to advancements in a variety of fields within urology, including incontinence, oncology, stone disease and pediatrics. MATERIALS AND METHODS: We reviewed historical records from the New York Hospital archives as well as the original publications of Marshall. Personal experiences of former residents and others were recounted. RESULTS: While Marshall may be most recognized for Marshall-Marchetti-Krantz vesicourethral suspension, designed to correct stress urinary incontinence, he was among the earliest advocates of surgical extirpation for bladder cancer and helped introduce the use of urinary cytology for the diagnosis of urothelial malignancy. At the same time Marshall contributed to the development of ureteroscopy for stone disease. His contributions to pediatric urology include the description of a nonrefluxing ureteral re-implantation technique for the surgical correction of reflux as well as his investigation of the embryology and surgical repair of bladder exstrophy. CONCLUSIONS: The contributions of Victor Marshall to urology are notable in breadth and significance.

History, 20th Century↗
PubMed2001

Sigmoid reconfigured vaginal construction in children.

PURPOSE: We present a modified technique of sigmoid neovaginal construction in children that protects the sigmoid pedicle from traction, allows easy adjustment of caliber and reorients the mucosal fold in a longitudinal direction. MATERIALS AND METHODS: From 1997 to 2000, 10 genetically male (46 XY) children 1 to 13 years old underwent construction of a neovagina with sigmoid, incorporating the Yang-Monti concept of intestinal reconfiguration. The diagnosis was androgen insensitivity in 7 patients, congenital adrenal hyperplasia in 2 due to 17 alpha-hydroxylase deficiency and 3 beta-hydroxysteroid dehydrogenase deficiency, respectively, and bladder exstrophy in 1 who required sex reassignment. RESULTS: Eight children had an adequate caliber neovagina after an initial period of systematic dilation. In 1 case a relevant stricture required reoperation using the same technique and the outcome was good. In another child a stricture developed in the middle of the reconfigured sigmoid segment and a regular dilation schedule is still being followed after 23 months of followup. CONCLUSIONS: The new sigmoid reconfiguration technique enables the use of smaller dimension intestinal segments and construction of a long vaginal conduit of adequate caliber. Its optimal adequacy for penetration must be assessed in the future after these patients begin sexual activity.

Adolescent↗
PubMed1994

Intraureteral metallic self-expanding endoprosthesis (Wallstent) in the treatment of difficult ureteral strictures.

Intractable and recurrent ureteral stricture presents a continuous challenge to the urologist. We report on 5 patients with severe ureteral stricture who were successfully treated with self-expanding metallic stents. Ureteral stricture occurred at ureteroileal anastomotic sites after neoplasm resection in 2 cases, multiple upper ureteral strictures were related to multiple surgical procedures for correction of bladder exstrophy in 1 and a ureteral kink developed in 1. Treatment with transluminal balloon dilation provided poor results but self-expanding metallic stents were used successfully with no major complications. In the last patient the stent and the overlying ureter were removed due to recurrent reflux; the gross and histological ureteral changes are discussed in detail. The technical approach is described, alternative therapeutic options are considered and pertinent literature is reviewed.

Adult↗
PubMed1992

The Cantwell-Ransley technique for repair of epispadias.

A total of 26 boys with bladder exstrophy (20) and epispadias (6) underwent initial urethral reconstruction between 1988 and 1991 using the Cantwell-Ransley technique. Penile reconstruction included wide mobilization of the urethral plate from the underlying corpora based on a mesentery from the ventral penoscrotal skin, corporeal lengthening by dividing the suspensory ligaments and attachments to the undersurface of the pubis, urethral and glandular tubularization, chordee correction by medial incision of the corpora with anastomosis dorsal to the urethra and penile skin coverage. All exstrophy patients had adequate phallic length, with 13 having an intact urethral plate and 13 having had prior paraexstrophy skin flap interposition. Postoperatively, repairs were intubated with silicone stents for 10 days. Two urethrocutaneous fistulas developed, 1 of which closed spontaneously. One patient had a small degree of penile skin loss that did not affect the neourethra. All patients currently have a cosmetically acceptable penis and all but 1 (previously diverted) are voiding per urethram. The neourethra in such patients allows for easier access for endoscopy and the ventral position aids in maintaining correction of the dorsal chordee. The low complication rate of this procedure coupled with the better anatomical configuration of the neourethra makes it useful for urethral and penile reconstruction in the exstrophy and epispadias patient.

Adolescent↗
PubMed1991

Preperitoneal approach for hernia repair: clinical application in pediatric urology.

The preperitoneal approach for inguinal hernia repair rarely is indicated in children. However, we report on its clinical usefulness for children in whom the perivesical space must be exposed, such as during surgical repair of bladder exstrophy, ureterocele and ureteral reimplantation. This approach allows for a true high ligation of the hernia sac, and the repair is safe, fast and effective.

Child↗
PubMed2002

Pregnancy in women with ureterosigmoidostomy.

OBJECTIVES: To review our experience with four pregnancies during the past 5 years with special attention to the diagnostic and therapeutic aspects of ureterosigmoidostomy. Pregnancy in women with ureterosigmoidostomy is a rare condition that differs in many ways from pregnancies in women with other forms of urinary diversion. METHODS: From 1995 to 2000, we observed four pregnancies in 3 women with ureterosigmoidostomy. Two women had had bladder exstrophy, and one had had interstitial cystitis. During pregnancy, we performed urologic examinations every 4 weeks with renal ultrasonography, calculation of the resistive index, and tests of serum electrolytes, urea, creatinine, and blood gas analysis. In all pregnancies, antibiotic prophylaxis was performed. RESULTS: All women had recurring urinary tract infections before pregnancy. In all cases, reversible dilation of the upper urinary tract was observed during pregnancy. The resistive index never increased to a pathologic range (greater than 0.7). With administration of sodium-potassium-hydrogen citrate, no acidosis was observed. With antibiotic prophylaxis, the women only had one episode each of urinary tract infection during pregnancy. One patient developed preeclampsia that led to a cesarean section at week 36 of gestation. Delivery was achieved by cesarean section in two more cases and vaginally in 1 case. We did not observe any postpartum or neonatal complications related to pregnancy. CONCLUSIONS: Under regular urologic and gynecologic control, there is no contraindication to pregnancy in patients with ureterosigmoidostomy. Antibiotic prophylaxis seems to be recommended.

Adult↗
PubMed1994

Current indications for cutaneous ureterostomy.

OBJECTIVE: To assess the current indications for cutaneous ureterostomy in children. METHOD: A total of 32 children underwent cutaneous ureterostomy at Texas Children's Hospital from 1975 to 1990. Medical records were reviewed to determine the urologic diagnosis, the indication for urinary diversion, the type of ureterostomy performed, and the outcome for each patient. RESULTS: Twenty patients underwent loop cutaneous ureterostomy (LCU) or pyelostomy and 12 patients underwent end cutaneous ureterostomy (ECU). The main indication for LCU was obstructive uropathy unresponsive to lower urinary tract drainage, and the most common cause was posterior urethral valves. Other indications for LCU included obstruction requiring delayed surgical correction, high-grade reflux into a solitary kidney, and obstruction with infection. The children who required ECU were an older, more diverse group than those who underwent LCU. The main indication for ECU was poor bladder function secondary to a variety of congenital anomalies, including prune-belly syndrome, posterior urethral valves, bladder exstrophy, and urogenital sinus defect. CONCLUSION: LCU is easy to perform and is an excellent method for achieving temporary upper urinary tract drainage. ECU is suited for long-term or permanent urinary diversion in children with at least one dilated ureter, and can provide a socially acceptable stoma when delayed reconstruction is necessary. Many children who were "permanently" diverted by ECU may be undiverted using newer reconstructive techniques.

Child, Preschool↗
PubMed2002

A ventral rotational skin flap to improve cosmesis and avoid chordee recurrence in epispadias repair.

OBJECTIVE: To describe a technical modification that facilitates dorsal skin closure, improves cosmesis and eliminates chordee recurrence secondary to contracture of the dorsal penile skin in the repair of epispadias. PATIENTS AND METHODS: Eleven patients with penopubic epispadias (mean age 1.8 years) had the epispadias repaired using a modified ventral penile skin flap. Four patients had isolated epispadias and seven had had a previous primary closure of bladder exstrophy. Nine patients underwent the Cantwell-Ransley technique, leaving the meatus in a glanular position. Two patients were repaired using the penile disassembly technique of Mitchell and Bägli, because they had a short urethral plate. A ventral island skin flap was fashioned, starting at the base of the penis. Dissection was carried ventrally into the scrotum to allow for adequate dorsal flap transposition. The flap was rotated laterally to shift the suture line from the midline and to cover the dorsal aspect of the penis with untouched penile shaft skin. Redundant ventral foreskin was discarded. RESULTS: All patients had an uneventful course after surgery. Dorsal penile skin was viable in every case and no patient developed recurrence of chordee or a urethrocutaneous fistula. The cosmetic result was excellent in all patients. CONCLUSIONS: Dorsal skin closure using lateral rotation of ventral penile skin flap improves cosmesis after epispadias repair and eliminates the recurrence of chordee secondary to midline dorsal scarring.

Cicatrix↗