Urethral Valves Research Articles

Fetal bilateral obstructive uropathy: a series of nine cases.

Bilateral obstructive uropathy is increasingly diagnosed in early pregnancy by ultrasound examination. Termination of pregnancy presented an opportunity to study uropathy in nine cases between 18 and 24 weeks of gestation. En bloc dissection of the urinary tract and subserial sectioning of the urethra revealed obstruction at the level of the membraneous urethra in all cases. No structural obstruction could be demonstrated in three cases, and urethral valves caused partial obstruction in two cases. The urethra was distorted by a fibrous band in one case. In three cases overriding urethral lumens appeared to represent incomplete or faulty canalization at the angled junction of the developing posterior and anterior urethra. Urethral obstruction is considered the major etiologic factor in the development of bilateral fetal uropathy and presumably the prune-belly phenotype.

The investigation and follow-up of urinary tract infection

Summary (Table 2) The aims of investigation are to diagnose unequivocally the first infection a child develops, particularly in infancy, and to identify risk factors predisposing to renal damage. This means all children require renal imaging and younger children MCUG. Decisions on the intensity of investigation and follow up are based on risk estimation. Follow up is aimed at negating the effects of identified risk factors, such as VUR, by careful medical or surgical management. Once renal scarring has occurred lifelong follow up for hypertension and possible functional deterioration is mandatory. 1.Allages (a)Renal USS — obstruction, abnormal morphology, size discrepancy, ? scars (b)AXR — small stones, spine defects (c)DMSA Scan — definitive diagnosis of scarring, differential function (d)Isotope Renogram — evaluation of upper tract obstruction, differential function 2.Variable with ageMCUG — for reflux, bladder size, emptying, urethral valves (i)All infants (ii)possibly all pre-school children certainly if renal scars, FH of VUR, clinical upper tract infection, neurogenic bladder suspected, recurrent episodes of UTI (iii)After age 4 years if renal scarring and recurrent UTI

Urinary tract infections

The populations at risk for urinary tract infection include the newborn, particularly the premature, prepubertal girls, young boys, sexually active young women, elderly males, and elderly females. Risk factors that contribute to lower tract infection in women include sexual intercourse, diaphragm-spermicide use, and voiding behavior. Host factors, more than bacterial virulence, are probably the most important contributors to infection. The genetic factors that are important contributors are secretor status and P blood group phenotype. Which patients to culture, when to culture, and the number of organisms required to define infection have changed in the past decade. A concentration of 10 2 colony forming units/mL can cause an acute urinary tract infection in the healthy woman. The presence of leukocytes in the urine is of increasing diagnostic importance. Complicated urinary tract infections occur in neonates with such congenital anomalies of the urinary tract as urethral valves or in patients with neurologic disease resulting in urinary stasis. In older men or women, complicated urinary tract infections occur with obstruction, instrumentation, surgery, anatomic abnormalities, or stones. Single-dose therapy of uncomplicated urinary tract infection is useful in only a small subset of patients, specifically in patients <45 years of age who have short duration of symptoms. The majority of patients with uncomplicated infections should receive treatment for 3–5 days. Response to therapy and long-term cure rates in complicated urinary tract infection are related both to the type of underlying abnormality and to the species of the infecting organism. Complicated urinary tract infections should be treated for 7–14 days.

Megalourethra

Megalourethra is a rare congenital anomaly characterised by a failure of development of erectile tissue and subsequent dilatation of the penile urethra. The term was first used by Nesbitt (1955) and since then many of these defects concerning the corpus spongiosum and the corpora cavernosa have been reported (Mortensen et al., 1985; Appel et al., 1986; Aigen et al., 1987).Concomitant malformations, which are sometimes incompatible with survival, have important implications for therapy and prognosis. Associated anomalies may involve the urinary tract (renal hypoplasia, ureterohydronephrosis, megaureter, megacystis, reflux, bladder diverticula, hypospadias, manifestations of intersex, urethral valves, prune belly syndrome) as well as other organs (teeth, heart, intestines).

Neonatal ascites and urethral valves

Neonatal ascites and urethral valves

Urethral Valves — Treatment, Results and Urodynamic Assessment

Posterior urethral valves is an uncommon condition, but it poses many diagnostic and therapeutic problems. Long term follow up of these patients revealed that the majority of these boys have long term problems. In depth assessment of 10 boys with this problem revealed that their upper urinary tracts remained stable whereas urodynamic studies showed gross micturition abnormalities. Most methods of assessing the urinary tracts in these boys are invasive (eg. Intravenous urography, micturating cystography) and may indeed show no change in upper tract radiology despite marked abnormalities of micturition detected by urodynamics and which may require further investigation and treatment. We therefore recommend regular urodynamic assessment of these boys as it is accurate and initially non-invasive. Those boys with a detectable abnormality can progress to further studies.

Sonographische Diagnostik fetaler Harntraktanomalien - Inzidenz, Therapie und Verlauf

561 severe foetal malformations were seen from 1975 to 1986 in a total of 17,057 births (3.3%). The urogenital tract was involved in 164 cases only (1.0%). The proportion of malformations of the urinary tract was 29% of all observed anomalies. In 31% of the cases (51 of 164) there were isolated malformations, and in 20% (33 of 164) combined malformations of the urinary tract. 58% (96 of 164) of the urinary tract anomalies definitely belonged to syndromes (e.g. Potter's syndrome). Of 68 newborns with correctable urogenital changes, 42 were diagnosed as obstructive uropathy, 7 of which with urethral valves. A highly critical view is taken of the possibilities of intrauterine therapy in obstructive uropathies.

Möglichkeiten der Beurteilung seitengetrennter Funktionsstörungen am oberen Harntrakt bei Säuglingen mit Urethralklappen

Upper urinary tract function was evaluated in 15 infants in whom urethral valves were diagnosed. One baby died at 16 days of age and was not completely investigated. In 4, the valves did not have untoward effect on the bladder and the upper urinary tract. In 10 others, function was evaluated using the Hodson index, creatinine, PAH, inuline clearance and renal I123-Hippuran scintigraphy with measurement of the accumulation indices. The various examination procedures were of value in providing precise evaluation of the renal performances at diagnosis as well as in prognosis and follow-up of the function of each kidney.

Fehler und Gefahren verschiedener Klappenresektionstechniken

Complications are frequently seen in patients with urethral valves, especially in the youngest age bracket. Some of the complications are due to valve obstruction and others due to the primary therapy. Valve resection can be transvesical, with or without symphysic incision, in direct view, via perineostomy or transurethral. These different methods represent a historical development. The mandatory prerequisite consists of the availability of update cystoscopic equipment for babies and infants. The main problems involved in "open" surgery concern incontinence. Transurethral approach can result in strictures; incontinence due to sphincter lesion can also occur. In recent years antegrade resection is being performed via percutaneous transvesical approach. It may be expected that this reduces the complications, but there is still insufficient experience to permit final assessment.

Zur Urodynamik der Urethralklappenresektion

Basing on the cases of 8 children with urethral valves who were diagnosed and treated during the first year of life we report on the urodynamic results. It is concluded that after valve resection enhanced pressure levels may still exist during micturition for some time even if the flow of urine has become normal. A morphologically and functionally satisfactory treatment result (separate lasix clearance on both sides) can be expected only after the pressure has dropped, a phenomenon that often occurs spontaneously.

Acute pancreatitis in six non-transplanted uraemic children

Ten clinical episodes of acute pancreatitis (AP) occurred in six patients (mean age 10 years, range 3-15 years) with chronic renal failure (CRF) during a 9-year period (1977-1986). The underlying cause of CRF was vesicoureteral reflux (2); urethral valves (1); ureterohydronephrosis (1); nephronopthisis (1) and a haemolytic uraemic syndrome which occurred 12 years before (1). In all patients a diagnosis of AP was established both on clinical grounds and with a serum amylase level of greater than 600 IU/l. In 3 patients laparotomy was performed because of suspected appendicitis. All patients required exclusive parenteral feeding (mean duration 25 days) and 2 patients had a partial pancreatectomy. No patient developed pancreatic pseudocysts, 2 patients experienced one relapse (3 and 21 months later) and 1 patient had two relapses and died. Mean duration of follow up was 3 years (range 1-10 years). Possible aetiological factors were: choledochal cyst (1); parotitis without a rise in mumps antibodies (1); familial dyslipidaemia but without AP in other family members (1), and aluminium intoxication with hypercalcaemia and convulsive encephalopathy treated with valproic acid in 1 patient. Severe hyperparathyroidism with radiological signs was absent in all patients. Transplantation had been performed either before AP in 2 patients (1 and 3 years before AP) or had followed AP in 1 patient (7 years after) without occurrence or relapse of AP.

Peel-Away Suprapubic Access Set for Percutaneous Bladder/Urethral Procedures in Children

The concept of a peel-away sheath is incorporated into endourologic procedures at the bladder and posterior urethral level. Percutaneous access to the bladder using this method allowed antegrade posterior urethral-valve ablation, antegrade catheterization of the urethra in cases of stricture disease, and easy bladder access for a variety of purposes.

Pediatric Applications of Augmentation Cystoplasty: The Johns Hopkins Experience

Since 1976, 23 children with bladder or cloacal exstrophy, meningomyelocele, sacral agenesis, the prune belly syndrome and noncompliant bladders associated with urethral valves or prior diversion underwent augmentation cystoplasty. Of these procedures 7 were combined with some type of urinary undiversion. Bowel segments used for augmentation included ileum alone in 10 patients, ileocecal segments in 4, a sigmoid patch in 8 and a hindgut patch in 1. An artificial urinary sphincter was placed at the time of bladder augmentation in 3 patients. There were no urinary fistulas or cases of urinary rediversion. Two patients required oral alkalizing agents as a result of persistent systemic acidosis. One patient required reoperation twice for ureteral obstruction, 1 had removal of the sphincter device secondary to erosion, 1 required reinforcement of the ileocecal valve owing to persistent reflux and 1 required reoperation for small bowel obstruction. Other complications included a superficial wound infection and 5 urinary tract infections, all of which were managed easily. Three patients were voiding and continent, 18 were dry with intermittent self-catheterization, 1 had giggle incontinence and 1 remained incontinent after sphincter removal. Augmentation cystoplasty appears to offer a reliable alternative to urinary diversion in the reconstructive management of children with small capacity bladders.

Pre-natal diagnosis: how useful is it?

Dilatation of the fetal urinary tract can now be detected by ultrasound imaging in pregnancy. Forty-six cases of pre-natally diagnosed dilatation were analysed to assess the contribution of this information to subsequent urological management. Positive pre-natal scans proved accurate. Dilatation was confirmed by neonatal ultrasound imaging or by autopsy in 24 of 25 consecutive inborn neonates and two aborted fetuses. Forty-four live births were studied. Twelve neonates had physical signs at birth which rendered the pre-natal diagnosis unnecessary. In 32 neonates (73%) the urinary tract anomaly was identified solely as a result of pre-natal imaging and would otherwise have remained undetected. However, in seven neonates the information was of doubtful value (mild unilateral dilatation), in 16 it was of probable value (confirmed unilateral PUJ obstruction, multicystic kidney, etc.) and in only nine neonates (20%) was it of definite value (treatable conditions affecting both kidneys or a solitary kidney, e.g. urethral valves, PUJ obstruction in a solitary kidney, etc.). Pre-natally detected bilateral dilatation (18 infants) carried a poor prognosis--33% mortality and 56% incidence of coexistent abnormalities. No infants in our series had undergone intra-uterine drainage, but the numbers of survivors and their renal function was comparable to the published results for fetal intervention.

Surgical Correction of Urinary Incontinence

Over the last 6 years, 114 patients have undergone surgery for urinary incontinence. The majority (79%) had neurologic dysfunction of the bladder because of spinal malformation (myelodysplasia, sacral agenesis, or trauma) and the remaining were a mixed group including exstrophy/epispadias, urethral valves, pelvic fractures, etc. The patients were grouped in six categories. Those with lower urethral resistance underwent bladder neck reconstruction with Young-Dees-Leadbetter procedure (five patients) or had placement of an artifical urinary sphincter (27 patients). Those with poor bladder compliance underwent primary bladder augmentation (21 patients). Those with combined urethral problems and poor compliance had combined procedures (14 patients). Thirty-seven patients previously diverted for incontinence and undergoing undiversion were considered separately, as were ten patients without any bladder precluding preoperative assessment. Of the entire group, continence was achieved in 83 patients with the initial procedure (73%). Secondary procedures have resulted in continence in 101 patients (89%). Three patients were improved but unsatisfactory, and nine remain wet; one is unknown.

Surgical correction of urinary incontinence

Over the last 6 years, 114 patients have undergone surgery for urinary incontinence. The majority (79%) had neurologic dysfunction of the bladder because of spinal malformation (myelodysplasia, sacral agenesis, or trauma) and the remaining were a mixed group including exstrophy/epispadias, urethral valves, pelvic fractures, etc. The patients were grouped in six categories. Those with lower urethral resistance underwent bladder neck reconstruction with Young-Dees-Leadbetter procedure (five patients) or had placement of an artificial urinary sphincter (27 patients). Those with poor bladder compliance underwent primary bladder augmentation (21 patients). Those with combined urethral problems and poor compliance had combined procedures (14 patients). Thirty-seven patients previously diverted for incontinence and undergoing undiversion were considered separately, as were ten patients without any bladder precluding preoperative assessment. Of the entire group, continence was achieved in 83 patients with the initial procedure (73%). Secondary procedures have resulted in continence in 101 patients (89%). Three patients were improved but unsatisfactory, and nine remain wet; one is unknown.

Application of the Pull-Through Technique of Transverse Advancement Ureteral Reimplantation

In the pull-through technique of ureteral reimplantation the ureter is divided at the hiatus and pulled through into the bladder. It is then advanced transversely as described previously for megaloureter but the technique also may be used for routine cases. The technique is particularly suitable for ureteral reimplantation in patients with urethral valves and neurogenic bladder. Pull-through ureteral reimplantation also may be used to reimplant the orthotopic ureter in complete ureteral duplication with ectopic ureterocele or with orthotopic reflux.

Urethral Valves and Ureterovesical Reflux Associated with Unsuspected Findings in a Nonfunctioning Kidney

Urethral Valves and Ureterovesical Reflux Associated with Unsuspected Findings in a Nonfunctioning Kidney

Maternal ultrasonography for the antenatal diagnosis of surgically significant neonatal anomalies.

The increased use of ultrasonography in the management and evaluation of pregnancy has provided a unique opportunity to observe the anatomy of the developing fetus from 12 weeks gestation until term. Twenty-eight surgically important anatomic abnormalities have been diagnosed in utero by sonographic examinations at our affiliated institutions over the past three and a half years. These include ascites (five cases), gastroschisis (four cases), omphalocele (three cases), sacrococcygeal teratoma, cystic hygroma, hydrocele, duodenal atresia, multicystic kidney (two cases each), and one each of jejunal atresia, conjoined twins, ureteropelvic junction obstruction, urethral valves, urethral agenesis, and hydronephrosis secondary to reflux. Prenatal diagnosis by ultrasonographic examination has signifcantly improved perinatal management. Elective caesarean section has benefited infants with lesions causing dystocia, such as sacrococcygeal teratoma, omphalocele, and conjoined twins. Advance notification of surgeons and neonatalogists has reduced the delays of postnatal evaluation and treatment that contribute, significantly, to complications and death. In addition, transfer of the pregnant mother carrying an infant with a significant surgical anomaly to a center with facilities for neonatal surgery and specialized postoperative care can be properly planned for in advance. In the near future, intrauterine fetal surgery or palliative intervention may provide increased salvage of patients with obstructive uropathy and diaphragmatic hernia, both of which carry high mortality rates secondary to in utero damage. Sonography has proven useful in following the dilatation of either intestinal or urinary tract structures in utero. In our hands, maternal sonography has improved the surgical care of the newborn and may open a new frontier of intrauterine fetal surgery in the future.

Perirenal urinary extravasation associated with urethral valves in infants: M. E. Mitchell and R. A. Garrett. J Urol 124: 688–691, (November), 1980.

Perirenal urinary extravasation associated with urethral valves in infants: M. E. Mitchell and R. A. Garrett. J Urol 124: 688–691, (November), 1980.