[Anorectal malformations]. / Malformations ano-rectales.

Anorectal malformations (ARM) are the result of an abnormal development of the terminal part of the digestive tract interesting anus and/or rectum that occur early between the sixth and tenth week of embryonic development. They carry a malformation spectrum of severity depending on the level of disruption of the anorectal canal and of the associated caudal malformations (sacrum and spine). ARM are associated in over half the cases with other malformations that can be integrated in some cases in known syndromes. If surgical treatment to restore anatomy as normal as possible is indispensable, post-operative care is essential for these patients whose defecation mechanisms are altered, to reach if not continence, at least a socially acceptable cleanliness.

Outcome of continence procedures after failed endoscopic treatment with dextranomer-based implants (DEFLUX®).

PURPOSE: To evaluate outcome of further continence procedures after failure of endoscopic injections of dextranomer-based bulking agent. MATERIALS AND METHODS: From 1997, 89 children (3-18 years) and one young adult were treated for incontinence with 145 endoscopic injections of dextranomer. On evaluation, each patient was classified as: dry, significantly improved, or treatment failure. Eighty-five patients had at least 12 months of follow up. Out of 34 (40%) treatment failures, 24 patients had a subsequent bladder neck procedure: artificial urinary sphincter (7), bladder neck plasty (9), bladder neck closure (1), fascial sling (3). Six patients had further endoscopic treatment (including 2 after bladder neck plasty). RESULTS: At surgery, Deflux(®) paste was easily identified with minimal surrounding tissue inflammatory reaction. Artificial urinary sphincter: all 7 dry; bladder neck plasty: 7 dry, 1 improved and 1 still incontinent; 1 bladder neck closure patient dry; fascial sling: 1 significantly improved and 2 dry. Repeated endoscopic treatment: 1 dry, 1 improved and 4 failed. Eleven of the 21 (52%) patients who are either dry or improved have voiding abilities. CONCLUSION: Endoscopic injections with dextranomer do not adversely affect the outcome of further surgical procedures. Repeated injections after a previous endoscopic treatment failure have a low success rate.

Vesicoureteral reflux and elimination disorders.

Two kinds of elimination disorders can be associated with Vesico Ureteral Reflux (VUR): pure bladder elimination disorders or combination of bladder and bowel elimination disorders. An elimination disorder is always a factor which worsens the prognosis of VUR, as it increases the risk of infectious complications and thus presents a threat for the upper urinary tract. Regarding pure bladder elimination disorders, a chronic urine residue is observed in four clinical situations: the syndrome megacystis-mega ureter; the mega bladder without mega ureter, but with VUR; high grade massive VUR without a mega bladder; organic obstructions of the urethra (such as posterior urethral valves.). VUR associated with urine and fecal elimination disorders cover functional pelvi perineal dyscoordination, bladder sphincter dysynergia, disturbances of visceral motricity and anal sphincter function. The most characteristic type is represented by the neuropathic detrusor-sphincter dysfunction; also enter in this category neurogenic non-neurogenic bladders (Hinman's syndrome); However the vast majority of urine and fecal elimination disorders is represented by non neuropathic perineal dyscoordination associating at various degrees: voiding postponement, lack of sphincter relaxation during micturation, interrupted voiding, and constipation. The diagnosis of elimination disorders associated with VUR is based on non invasive investigations such as anamnesis and drinking/voiding chart in children and adolescents, and "four observation test" in infants. Ultrasound and uroflowmetry are also useful tools. Invasive investigations include mainly voiding cystourethrography and urodynamics, ideally combined in video urodynamic studies. The management of urinary and intestinal elimination disorders is based on the prevention of infections, the suppression of the post voiding residual urine and the treatment of an associated constipation. If surgical treatment of VUR is needed, it must be associated to the management of elimination disorders in the peri operative period. In many instances, an appropriate treatment of elimination disorders often leads to the VUR resolution.

Primary monosymptomatic nocturnal enuresis in children and adolescents.

Nocturnal enuresis (NE) is one of the most frequent paediatric pathologies. The prevalence of primary nocturnal enuresis (PNE) is around 9% in children between 5 and 10 years of age and about 40% of them have one or more episodes per week. Still for too long, PNE has not been recognised as a pathological condition, particularly by the medical community; as a consequence, there was no specific education at medical school, and a poor involvement by the practitioners. Enuretic children have a sense of social difference and isolation; some of them do express a low self-esteem. Also, self-esteem is improved by the management NE even if this management fails to cure the condition. Primary monosymptomatic nocturnal enuresis (PMNE) is an heterogeneous condition for which various causative factors have been identified such as: nocturnal polyuria, sleep disturbances, reduced bladder capacity or bladder dysfunction, upper airway obstruction. The positive diagnosis of PMNE is based on a complete questionnaire and a careful physical examination. A drinking and voiding chart is an essential non-invasive tool: first, to collect information about the initial drinking and voiding habits of the child, then to reassess the accuracy of the diagnosis. Only motivated patients should receive a specific treatment for their NE and the treatment should be proposed based on the type of PMNE. PMNE associated with nocturnal polyuria should be treated with desmopressin, which reduces nighttime urine production. For PMNE with a reduced bladder capacity alarms should be the first-line treatment. Oxybutinin, a drug with anticholinergic properties, is not theoretically indicated for the treatment of PMNE except for a very small subgroup of patients who have an overactive bladder only during sleep. In cases refractory to monotherapy, NE is probably the result of an association of different physiopathological factors (e.g. both a nocturnal polyuria together with a small bladder capacity) some of them are still unknown. In these patients, a combination of treatments may be more effective than monotherapy. Various combination therapies can be proposed to improve the cure rates.