Recommendations are made for the standardization of nomenclature describing duplex systems, ectopic ureters and ureteroceles. The elimination of some terms and redefinition of others are proposed in the hope to eliminate the ambiguity and confusion that exist currently.

A series of 238 hydronephrotic kidneys in 219 children is reported. The condition was more common in the male than in the female subject and occurred more frequently on the left side. It was often bilateral, especially in infants, with an abdominal mass as the common presenting feature. Loin or abdominal pain was the most frequent complaint in older children. In some cases hydronephrosis presented as a ruptured kidney following trauma. Only 1 patient was hypertensive. The lesion was asymptomatic in 18 cases and the incidence of urinary infection was low. Dismembered pyeloureteroplasty was the procedure of choice for reconstruction. Preliminary nephrostomy was used rarely and nephrectomy was done in 10 per cent of the kidneys. Of the 7 reoperations 4 were for persistent obstruction and 3 were because stones had formed after the pyeloplasty. The late results, assessed clinically and radiologically, have been entirely satisfactory. Many kidneys of initially doubtful value showed useful improvement after reconstructive operation and no secondary nephrectomies were performed. The only death in the series occurred 2 1/2 years postoperatively and was unrelated to the urinary tract.

Peak retrograde flow can serve as a valuable tool in predicting persistent, progressive and new onset asymmetry. Varicoceles associated with a peak retrograde flow of 38 cm per second or greater and 20% or greater asymmetry should be considered for varicocelectomy at initial presentation. Patients with peak retrograde flow greater than 30 cm per second need to be monitored carefully. Those with peak retrograde flow less than 30 cm per second are less likely to require surgery.

Valve ablation alone without urodynamic followup is inappropriate. Proactive management must have a significant role. The vast majority of temporarily diverted cases do not result in a fibrotic, noncompliant bladder. Surgeons who strongly favor diversion should be comfortable with such an approach, although in most cases if diversion is considered, those same patients often can be treated just as adequately with proactive urodynamics and anticholinergic therapy without the required surgery for undiversion. With great anticipation, we look forward to studies that will determine if growth factor inhibitors or angiotensin converting enzyme inhibitors will have a role in preventing or reversing the histological changes of the valve bladder.

No abstract

On objective urodynamic assessment pediatric nonneurogenic voiding dysfunction can essentially be divided into 4 specific conditions. These conditions have distinct urodynamic features that distinguish them from each other, as opposed to their clinical features (particularly lower urinary tract symptoms), which frequently overlap and are not as defining as they are often presumed to be.

Objective: To identify the molecular basis for prenatally suspected cases of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) (MIM 249210) in 3 independent families with clinical and radiographic evidence of MMIHS. Methods: Whole-exome sequencing (WES) and Sanger sequencing of the ACTG2 gene. Results: We identified a novel heterozygous de novo missense variant in ACTG2 c.770G>A (p.Arg257His) encoding γ-2 smooth muscle actin (ACTG2) in 2 siblings with MMIHS, suggesting gonadal mosaicism of one of the parents. Two additional de novo missense variants (p.Arg257Cys and p.Arg178His) in ACTG2 were identified in 2 additional MMHIS patients. All of our patients had evidence of fetal megacystis and a normal or slightly increased amniotic fluid volume. Additional findings included bilateral renal hydronephrosis, an enlarged fetal stomach, and transient dilated bowel loops. ACTG2 immunostaining of the intestinal tissue showed an altered muscularis propria, a markedly thinned longitudinal muscle layer, and a reduced amount and abnormal distribution of ACTG2. Conclusion: Our study demonstrates that de novo mutations in ACTG2 are a cause of fetal megacystis in MMIHS and that gonadal mosaicism may be present in a subset of cases. These findings have implications for the counseling of families with a diagnosis of fetal megacystis with a preserved amniotic fluid volume and associated gastrointestinal findings.

Noninvasive uroflowmetry with simultaneous electromyography offers an excellent alternative to invasive urodynamics to diagnose 4 urodynamically defined conditions. It identifies the most appropriate therapy for the specific condition and objectively monitors the treatment response.