Yves Homsy

alpha-Adrenergic blockade in children with neuropathic and nonneuropathic voiding dysfunction.

PURPOSE Inadequate bladder emptying is a common urinary dysfunction in children. The role of alpha-blockers for managing bladder outlet obstruction remains relatively unexplored in children. Because of the well established impact of alpha-blocker therapy in men, we investigated its use for treating inadequate bladder emptying in the pediatric population. MATERIALS AND METHODS We treated 17 children 3 to 15 years old with documented poor bladder emptying of various etiologies, including dysfunctional voiding, the Hinman syndrome, the lazy bladder syndrome, posterior urethral valves, myelomeningocele and the prune-belly syndrome, using the alpha-1 adrenergic receptor antagonist, doxazosin. The initial dose of 0.5 to 1.0 mg. nightly was increased according to patient response and as tolerated. Patients were followed weekly to monthly by symptomatic history, and urine flow and/or post-void residual urine volume measurement. Two patients with neurogenic bladder were also followed with cystometrography and leak point pressure determination. RESULTS Bladder symptomatology and/or emptying improved in 14 patients (82%). Ten patients had decreased post-void residual urine during treatment and in 3 uroflowmetry showed increased maximum flow. Two patients with neuropathic bladder secondary to myelomeningocele had decreased leak point pressure on alpha-blocker therapy and in 2 with a history of posterior urethral valves new onset bilateral hydronephrosis completely resolved. Only 1 patient had mild postural hypotension, which resolved with dose reduction. CONCLUSIONS Selective alpha-blocker therapy seems to be well tolerated in children and appears effective for improving bladder emptying in various pediatric voiding disorders at short-term followup. Long-term followup and further investigation are warranted to validate the potential role of alpha-blocker therapy in pediatric urinary dysfunction.

Primary vesicoureteral reflux: review of current concepts

Abstract. The well-known association between vesicoureteral reflux and urinary tract infection is the basis for pathophysiological and therapeutic implications which have dominated the literature on the subject for the last 2 decades. We critically review the following issues: (1) does urinary tract infection cause reflux? (2) does reflux predispose to infection? (3) does reflux predispose to pyelonephritis? (4) does reflux predispose to a renal parenchymal scar? (5) does long-term urinary antibiotic prophylaxis prevent renal damage in patients with reflux? We conclude that none of the reviewed issues have been rigorously proven or validated and that the role of vesicoureteral reflux in urinary tract infections needs to be redefined through well-designed, multicenter, prospective, randomized, controlled studies using state of the art renal imaging techniques.

THE PRENATAL DIAGNOSIS OF CLOACAL EXSTROPHY

PURPOSE We assess and clarify diagnostic features for making the prenatal diagnosis of cloacal exstrophy. MATERIALS AND METHODS We evaluated 9 patients born with cloacal exstrophy at our institutions (2 prospectively and 7 retrospectively) for diagnostic features on prenatal ultrasound studies. We also thoroughly reviewed the literature on 13 previous prenatally diagnosed cloacal exstrophy cases. Diagnostic criteria were assessed by combining the findings in our patients and those in previous reports. RESULTS Of the 22 patients with prenatal ultrasound studies and cloacal exstrophy whom we analyzed 1 of our 9 and 2 in the literature had a cloacal membrane that persisted at 22 weeks of gestation. Major ultrasound criteria for diagnosing cloacal exstrophy prenatally are nonvisualization of the bladder, a large midline infraumbilical anterior wall defect or cystic anterior wall structure (persistent cloacal membrane), omphalocele and lumbosacral anomalies. Seven less frequent or minor criteria include lower extremity defects, renal anomalies, ascites, widened pubic arches, a narrow thorax, hydrocephalus and 1 umbilical artery. CONCLUSIONS We propose major and minor criteria to assist in the prenatal diagnosis of cloacal exstrophy. Despite these major and minor criteria the certainty of establishing a prenatal diagnosis remains challenging. Persistence of the cloacal membrane beyond the first trimester in 1 patient was an exception to the classic concept of cloacal exstrophy embryogenesis. An accurate prenatal diagnosis requires validation of these criteria by further correlation of prenatal and postnatal observations.

WILMS TUMOR AND MULTICYSTIC DYSPLASTIC KIDNEY DISEASE

PURPOSE There is ongoing controversy concerning the management of multicystic dysplastic kidney disease, particularly with regard to the potential for malignant transformation. Our report fuels the debate by adding the 2 youngest patients in whom malignancy was present from birth or developed subsequently. MATERIALS AND METHODS Two well documented cases of malignancy associated with multicystic dysplastic kidney disease are presented in 2 female infants (5 and 3 months old). The 5-month-old female infant was followed for multicystic dysplastic kidney disease and had no evidence of tumor either antenatally or at birth. The 3-month-old presented with hypertension and interventricular septal defect. A renal tumor was present on initial ultrasound. RESULTS Even though malignant degeneration is rare in multicystic dysplastic kidney disease, 9 cases have been reported in the literature so far. Of these cases 3 were Wilms tumor, 5 were renal cell carcinomas and 1 mesothelioma. CONCLUSIONS Our 2 cases lend support to the surgical management of multicystic dysplastic kidney disease, particularly as nephrectomy can now be performed in a day surgery setting with minimal morbidity. Only the risks of coexisting malignancy and possible malignant degeneration transformation are specifically addressed in this article. Other complications of multicystic dysplastic kidney disease such as hypertension, infection, abdominal pain, hematuria and persistent dysplastic renal tissue despite ultrasonographic resolution of multicystic dysplastic kidney disease are additional risk factors to be considered. A recommendation for nephrectomy in all cases of multicystic dysplastic kidney disease cannot be based only on these 2 cases. Several other factors must be weighed before making that decision.

Successful in utero endoscopic ablation of posterior urethral valves: a new dimension in fetal urology

Fetal lower urinary tract obstructive uropathy, when associated with oligohydramnios, is usually associated with a poor outcome. We present a case of successful in utero endoscopic ablation of posterior urethral valves in which the infant survived the neonatal period without evidence of renal dysplasia. The role, indications, and potential benefits of this novel technique are discussed.

Management of functional constipation in children with lower urinary tract symptoms: report from the Standardization Committee of the International Children's Continence Society

PURPOSE We present a consensus view of members of the International Childrens Continence Society (ICCS) together with pediatric gastroenterologists, experts in the field of functional gastrointestinal disorders, on the management of functional constipation in children with lower urinary tract symptoms. MATERIALS AND METHODS Discussions were held by the board of the ICCS and a multidisciplinary core group of authors was appointed. The draft document review process was open to all ICCS members via the website. Feedback was considered by the core authors and, by agreement, amendments were made as necessary. RESULTS Guidelines on the assessment, and pharmacological and nonpharmacological management of functional constipation in children with lower urinary tract symptoms are outlined. CONCLUSIONS The final document is not a systematic literature review. It includes relevant research when available, as well as expert opinion on the current understanding of functional constipation in children with lower urinary tract symptoms. The document is intended to be clinically useful in primary, secondary and tertiary care settings.

UROLITHIASIS IN THE LOW BIRTH WEIGHT INFANT: THE ROLE AND EFFICACY OF EXTRACORPOREAL SHOCK WAVE LITHOTRIPSY

PURPOSE Nephrolithiasis in preterm infants rarely requires surgical management. When it persists despite conservative therapy, treatment options are not clearly defined. We report a single institutional experience with extracorporeal shock wave lithotripsy (ESWL)* for the treatment of these small infants. MATERIALS AND METHODS We treated 8 infants (mean age 13 months) with a history of prematurity and 9 persistent stones with a Dornier HM3 lithotriptor between 1996 and 1999. Mean weight was 7,700 gm. Of the infants 7 had been treated with furosemide for bronchopulmonary dysplasia and 1 presented with multiple anatomical abnormalities. Gantry modification with a wooden platform and polystyrene foam positioning was used for lung and visceral protection. Ureteral stents were placed in 5 patients before ESWL. Renal ultrasonography was performed before, and 2 and a mean of 8 weeks after ESWL. Stone risk factors in our population were investigated through a multispecialty approach. RESULTS Average stone burden was 47.9 mm.2. A total of 9 sessions of ESWL were required for complete fragmentation of the 9 renal stones. A mean total of 2,100 shocks at a mean 16.1 kV. were administered. One patient with bilateral stones was treated in 2 separate sessions after a 4-week interval. No repeat ESWL sessions or other surgical interventions were required in any patient. Renal ultrasonography demonstrated no post-ESWL morphological changes. Practices leading to a higher incidence of neonatal nephrolithiasis at our institution were also identified. CONCLUSIONS ESWL is effective treatment for nephrolithiasis in small infants. Short-term safety has been established but continued long-term functional followup is essential. Multifactorial etiologies of nephrolithiasis must be identified and modified promptly in the care of preterm infants.

LONG-TERM METABOLIC ADVANTAGES OF A GASTROINTESTINAL COMPOSITE URINARY RESERVOIR

PURPOSE We investigated the long-term metabolic impact of gastrointestinal composite reservoirs. MATERIALS AND METHODS Nine patients underwent construction of a gastroileal (7) or gastrocolonic (2) reservoir for continent urinary diversion. Four cases of metabolic acidosis were converted from a preexisting conduit and the other 5 patients had diversion for either preexisting metabolic acidosis or the short bowel syndrome. All were reconstructed using a medium sized gastric segment (8 x 4 cm.) from the greater curvature of the stomach. The anti-incontinence segment was constructed from a tapered and reimplanted ileal segment. All patients underwent preoperative and postoperative measurements of serum pH, serum electrolytes, and urinalysis. Serum gastrin was measured in all patients postoperatively. Followup from surgery ranged from 47 to 61 months (mean 54.4). RESULTS All 9 patients demonstrated electrolyte neutrality in serum on long-term followup. Postoperative serum pH (mean 7.40) was significantly different (p < 0.01) from preoperative serum pH (mean 7.36) and serum bicarbonate was also significantly different (p < 0.01) preoperatively versus postoperatively (mean 22.3 versus 25.14). Urine pH values were not significantly different throughout the study. One patient with mildly acidic urinary pH (6.0 to 6.5) had ulcerative skin changes at the stoma site. Three patients had elevated serum gastrin levels on short-term followup but all patients had normal serum gastrin levels on long-term followup. One patient, with persistent alkaline urine, had urolithiasis and symptomatic urinary tract infections. CONCLUSIONS Our results demonstrate that a composite urinary reservoir constructed using gastric and intestinal segments achieved serum electrolyte neutrality on long-term followup. These results indicate a long-term metabolic advantage over other intestinal reservoirs associated with hyperchloremic metabolic acidosis and may be beneficial in patients compromised by either preexisting metabolic acidosis or the short bowel syndrome.

Primary Vesicoureteral Reflux: Review of Current Concepts

The well-known association between vesicoureteral reflux and urinary tract infection is the basis for pathophysiological and therapeutic implications which have dominated the literature on the subject for the last 2 decades. We critically review the following issues: (1) does urinary tract infection cause reflux? (2) does reflux predispose to infection? (3) does reflux predispose to pyelonephritis? (4) does reflux predispose to a renal parenchymal scar? (5) does long-term urinary antibiotic prophylaxis prevent renal damage in patients with reflux? We conclude that none of the reviewed issues have been rigorously proven or validated and that the role of vesicoureteral reflux in urinary tract infections needs to be redefined through well-designed, multicenter, prospective, randomized, controlled studies using state of the art renal imaging techniques.

Management of functional nonretentive fecal incontinence in children: Recommendations from the International Children's Continence Society

BACKGROUND Fecal incontinence (FI) in children is frequently encountered in pediatric practice, and often occurs in combination with urinary incontinence. In most cases, FI is constipation-associated, but in 20% of children presenting with FI, no constipation or other underlying cause can be found - these children suffer from functional nonretentive fecal incontinence (FNRFI). OBJECTIVE To summarize the evidence-based recommendations of the International Childrens Continence Society for the evaluation and management of children with FNRFI. RECOMMENDATIONS Functional nonretentive fecal incontinence is a clinical diagnosis based on medical history and physical examination. Except for determining colonic transit time, additional investigations are seldom indicated in the workup of FNRFI. Treatment should consist of education, a nonaccusatory approach, and a toileting program encompassing a daily bowel diary and a reward system. Special attention should be paid to psychosocial or behavioral problems, since these frequently occur in affected children. Functional nonretentive fecal incontinence is often difficult to treat, requiring prolonged therapies with incremental improvement on treatment and frequent relapses.