TL;DR: It is suggested that infants with DS be screened with ultrasonography for renal and urological abnormalities early in life and, if abnormal, a contrast voiding cystourethrogram be performed to rule out posterior Urethral valves or other bladder or urethral abnormalities.
Abstract: Renal and urological anomalies in Down syndrome (DS) have received little attention compared with the nephrourological findings described in other chromosomal abnormalities. Renal hypoplasia, hydroureteronephrosis, ureterovesical and ureteropelvic junction obstruction, and vesicoureteral reflux, but not posterior urethral valves, have been associated with DS. We report the occurrence of posterior urethral valves in three male infants with DS at a single institution. All had multiple urological procedures for correction or palliation of obstruction. Children with DS may have an increased risk for developing posterior urethral valves and obstructive uropathy. Furthermore, they may also develop chronic renal failure secondary to posterior urethral valves. Therefore, we suggests that infants with DS be screened with ultrasonography for renal and urological abnormalities early in life and, if abnormal, a contrast voiding cystourethrogram be performed to rule out posterior urethral valves or other bladder or urethral abnormalities. A review of the renal and urological anomalies in DS reported in the literature since 1960 is presented.
TL;DR: The first reported case of hematuria in a newborn caused by a bleeding hemangioma of the bladder is reported, and 3 weeks later the follow-up ultrasound examination showed resolution of the lesion.
TL;DR: A rare variant of this condition with complete duplication of the bladder in the anteropterior (coronal) plane with no connection to the native urinary tract is reported.
TL;DR: The use of a crystal chisel contact tip firing Nd:YAG laser for transurethral sphincterotomy in 76 consecutive spinal cord injury patients with adequate voiding, minimal to absent autonomic dysreflexia and no significant symptomatic urinary tract infection is reported.
Abstract: I report here the use of a crystal chisel contact tip firing Nd:YAG laser for transurethral sphincterotomy in 76 consecutive spinal cord injury patients. Their mean age was 53 years (range 26 to 77 years). Fifty-four (72%) were complete motor (Frankel A and B) and 21(28%) were incomplete lesions (Frankel C and D) They were evaluated with multichannel urodynamic equipment and 89% of the patients showed detrusor sphincter dyssynergia and 11% showed detrusor areflexia. Forty-three patients (56%) had previous electrocautery sphincterotomy and were not voiding well. A cystoscopic examination showed that 32% had an associated enlarged prostate and/or bladder-neck stenosis and 32% had associated wide-body strictures in the bulbous urethra. The crystal chisel contact tip firing Nd:YAG laser almost mimicked a hot diathermy knife to create an intraurethral incision as well as vaporizing the tissues. For sphincterotomy, a 12 o'clock incision was used from the verumontanum to the bulbous urethra. In patients with an associated enlarged prostate or bulging lateral lobes, 3 and 9 o'clock incisions were also made from the bladder-neck to the verumontanum and also vaporized the bulging prostate tissue. We used 25 to 40 watts for cutting, and vaporization of tissue and 15-25 watts to stop bleeding. The blood loss was less than 50 ml at surgery in 97.4% patients. None of the patients were transfused. An indwelling Foley catheter was usually left in situ for about 24 h and the majority of the patients were discharged the next day. All patients have been followed up at least every 6 months for a mean period of 27 months (range 16 to 41 months). The durability of surgery has been checked with linear array transrectal sonography and by urodynamic evaluation. Sixty-nine patients (92%) had adequate voiding, minimal to absent autonomic dysreflexia and no significant symptomatic urinary tract infection. There were seven patients who required repeat laser surgery within 2 to 5 months. All subsequent patients are voiding well with wide open bladder-neck and posterior urethra as shown on a voiding cystourethrogram.
TL;DR: The preliminary results reveal no adverse outcomes associated with the selective use of VCUG in the pretransplant evaluation, and indicate that this study is of little value in the routine evaluation of patients for renal transplantation.
TL;DR: A VCUG at one month and a DMSA scan six months later, were useful for identifying patients with VUR who required prophylactic antimicrobial therapy, and (2) patients with renal scarring, the latter group may benefit from the early performance of urine culture in subsequent febrile episodes.
Abstract: Imaging studies are part of standard care following a first diagnosed urinary tract infection (UTI) in young children. Renal ultrasound (US), voiding cystourethrogram (VCUG), and dimercaptosuccinic acid (DMSA) or glucoheptonate renal scans aim to identify: unsuspected urinary tract anomalies or obstruction, vesicoureteral reflux (VUR), renal parenchymal involvement, and renal scarring. As part of an ongoing clinical trial evaluating oral vs. intravenous therapy for first time UTI, 179 children aged 1-24 mos. with fever (≥38.3° C) had a renal US and a DMSA or glucoheptonate scan within 48 hours of diagnosis, a VCUG one month following diagnosis, and a repeat scan six months later. A normal renal US was found in 157 (88%) patients, pelvocaliectasis and dilated renal pelvis in 14 (7.8%), megaureter in 5 (2.8%), duplicated collecting systems in 2 (1.1%), and a calculus in 1 (0.5%). VUR was as common in children with normal US as in those with an US showing dilatation of the upper urinary tract (50/154 vs. 7/17, NS). Renal scans performed at the time of infection showed parenchymal inflammation in 137 (77%) of 179 patients. A VCUG identified VUR in 75 (44%) of 172 patients -- grade I (18), grade II (27), grade III (25), and grade IV(5). To date, of the 134 patients who completed the 6-month follow-up, 90(67%) outcome scans were normal and 44 (33%) showed renal scarring. Scarring occurred in 44 (41%) of 108 patients who had evidence of acute pyelonephritis in the initial scan, and in none of 25 patients with normal scan at study entry. Results of US and DMSA scan at the time of presentation with UTI did not modify management. Current widespread use of prenatal US leads to identification of obstructions of the urinary tract in utero. Accordingly, selective (in patients with persistent fever or abdominal findings) rather than routine performance of US is recommended. A VCUG at one month and a DMSA scan six months later, were useful for identifying (1) patients with VUR who required prophylactic antimicrobial therapy, and (2) patients with renal scarring. The latter group may benefit from the early performance of urine culture in subsequent febrile episodes.
TL;DR: An infant male was diagnosed as having bilateral hydronephrosis on antenatal ultrasound examination and appears to have developed gross reflux nephropathy in-utero, because of sterile high-grade reflux.
Abstract: An infant male was diagnosed as having bilateral hydronephrosis on antenatal ultrasound examination. After an uneventful full-term normal delivery, this was confirmed postnatally by a follow-up ultrasound. At 2 weeks of age, a voiding cystourethrogram showed grade V vesicoureteric reflux into the lower pole moiety of duplex kidneys bilaterally. The infant was administered antibiotic therapy for this condition. No evidence of a urinary tract infection was ever documented on urine culture. A Tc-99m dimercaptosuccinic acid (DMSA) renal cortical study undertaken at 6 weeks of age showed remarkable evidence of extensive scarring of the lower poles of both duplex kidneys. This infant appears to have developed gross reflux nephropathy in-utero, because of sterile high-grade reflux.
TL;DR: It is recommended that infants with DS be screened with ultrasonography for renal and urological abnormalities early in life and, if abnormal, a contrast voiding cystourethrogram be formed to rule out posterior urethral valves or other bladder or Urethral abnormalities.
Abstract: Renal and urological anomalies in Down syn- drome (DS) have received little attention compared with the nephrourological findings described in other chromo- somal abnormalities. Renal hypoplasia, hydroureterone- phrosis, ureterovesical and ureteropelvic junction obstruc- tion, and vesicoureteral reflux, but not posterior urethral valves, have been associated with DS. We report the oc- currence of posterior urethral valves in three male infants with DS at a single institution. All had multiple urological procedures for correction or palliation of obstruction. Case 1 Children with DS may have an increased risk for devel- oping posterior urethral valves and obstructive uropathy. Furthermore, they may also develop chronic renal failure secondary to posterior urethral valves. Therefore, we sug- gests that infants with DS be screened with ultrasonography for renal and urological abnormalities early in life and, if abnormal, a contrast voiding cystourethrogram be per- formed to rule out posterior urethral valves or other bladder or urethral abnormalities. A review of the renal and ur- ological anomalies in DS reported in the literature since Case 2 1960 is presented. described in association with DS (3-25). We report three cases of DS associated with posterior urethral valves (PUV) followed at a single institution. We have also reviewed the renal and urological anomalies in patients with DS reported in the literature since 1960.
TL;DR: The ultrasonic ureteric jet study is a non-invasive method of measuring the distance between the midline of the bladder and the UREteric orifice (MOD) as discussed by the authors.
Abstract: Summary: The ultrasonic ureteric jet study is a non-invasive method of measuring the distance between the midline of the bladder and the ureteric orifice (MOD). Statistical comparison with the results of single cycle voiding cystourethrograms (VCU) indicates that a normal MOD measurement on the ureteric jet study is a good predictor of the absence of vesico-ureteric reflux (VUR). For ureters of children less than 4 years of age, a MOD ≤8mm has a negative predictive value of 83% (95% confidence interval; 74-93%). For ureters of children aged 4-10 years, a MOD ≤9mm has a negative predictive value of 91% (95% confidence interval; 86–97%). the ureteric jet study has the potential to identify children who do not have reflux, thus sparing them an unpleasant, invasive procedure.
TL;DR: The success rate of 100% in antireflux surgery enables the policy to perform early operative interventions in infants with high grade reflux to continue.
Abstract: BACKGROUND Due to the advancement of prenatal and postnatal ultrasound screening in addition to better understanding by pediatricians, more infants have been detected to have vesicoureteal reflux. There are still debates, however, about their management whether to take conservative or surgical treatment. METHODS We analysed clinical courses of 51 children with primary reflux undergoing antireflux surgery when they were under 1 year. RESULTS A total of 41 infants (80%) presented with febrile urinary tract infections. The first UTI was seen as early as 7th day after birth, with the average age of 2 months. Seven infants had hydronephrosis detected by prenatal ultrasound, and 2 were identified by neonatal screening ultrasonography. Sixty-eight of 86 reflux ureters (79%) showed high grade reflux (grade IV approximately V). All the other 18 ureters with mild to moderate grade of reflux except one accompanied with contralateral high grade reflux. One ureter with grade II reflux was associated with contralateral obstructed meganreter. Renal parenchymal abnormalities, either with scarring and/or small kidney, were identified in 64 kidneys (74%) at or before the reimplantation. The operation was done as early as 1 month-old with the average age of 5.6 months. Among the children, 24 (47%) underwent anti-reflux surgery before 6 month-old. Smallest infant weighted 2500 gr at the time of operation. Reimplantation was done by the Cohen transverse advancement technique in 75 ureters (87%). The modified Politano-Leadbetter method in 6 with Hutche's diverticulum, and the Glenn-anderson advancement method in 5 with moderate to mild reflux. Two dilated ureters were treated with folding of the intravesical segment. Follow-up voiding cystourethrogram of 49 children at 6 months revealed no evidences of persistent reflux. There were no patients with obstruction of reimplantation except one with slight upper-tract dilatation. One infant revealed new low grade contralateral reflux, and he is being observed. Two children presented pyelonephritis after the operation. CONCLUSION Our success rate of 100% in antireflux surgery enables us to continue our policy to perform early operative interventions in infants with high grade reflux. In addition to the meticulous and careful technique, the conditions for successful results include; 1) A through checkup of the lower tracts by VCG and endoscopy. 2) Find the better operative instruments for the small bladder. 3) Do not hesitate to perform the ureteral tapering. 4) Make good use of operative loupe.
TL;DR: Early detection of urinary dysfunction through clinical symptoms and appropriate urodynamic studies, with institution of bladder training and anticholinergic medication can improve the patients' voiding patterns, both medically and socially.