Sacral neuromodulation in patients with congenital faecal incontinence. Special issues and review of the literature.

BACKGROUND: The aim of the present study was to evaluate the effectiveness of sacral neuromodulation (SNM) as a treatment for congenital faecal incontinence (FI). METHODS: A retrospective study was conducted on patients with congenital FI who had SNM surgery at our institution between October 2005 and June 2013. An initial percutaneous nerve evaluation was performed, and patients with an improvement of more than 50% in their symptoms had permanently implants for SNM treatment. RESULTS: There were 4 patients who received a permanent implant. Mean duration of follow-up was 67.5 months (range 45-135 months). At last follow-up, 2 patients maintained significant improvement with SNM, 1 was explanted after 4 years of treatment due to infection but remained asymptomatic and SNM failed in the remaining patient who went on to graciloplasty. CONCLUSIONS: SNM may be of value for treating FI in patients with anorectal malformations.

Evaluation of transcutaneous electrical posterior tibial nerve stimulation for the treatment of fecal and urinary leaks in children: preliminary results.

OBJECTIVE: To examine the effectiveness of posterior tibial nerve stimulation (PTNS) for the treatment of fecal and urinary incontinence in children with malformations of the bowel or neurological pathologies. INTRODUCTION: Treatment of fecal and urinary leaks, in cases of congenital malformations remains a challenge. Recent studies in adults have shown the effectiveness of PTNS. METHOD: Eight children: 4 with anorectal malformations, 3 with neurological causes (1 medullary lipoma, 1 Arnold Chiari malformation, 1 sacrococcygeal teratoma) and 1 with Hirschsprung's disease presenting with serious anal incontinence, despite extensive bowel management during at least 2 years, were treated with PTNS. Six children had associated urinary leaks. Jorge-Wexner score for defecation and Schurch score for urine were used before treatment and after the second and sixth months of stimulation. RESULTS: After six months, five patients had no more fecal leakage, two patients were improved and one did not respond. Five out of the 6 patients with urinary leaks were continent at 6 months. CONCLUSION: PTNS is a noninvasive technique and painless modality which seems to be effective for the treatment of fecal and urinary leaks in children even with congenital digestive pathologies or neurological malformations. These results will be confirmed in a prospective study.

International Children's Continence Society's recommendations for initial diagnostic evaluation and follow-up in congenital neuropathic bladder and bowel dysfunction in children.

PURPOSE: The objective of this ICCS standardization document is to report the initial diagnostic evaluation and subsequent work-up of children with neuropathic bladder dysfunction. MATERIALS AND METHODS: Due to a paucity of level I or level II, "levels of evidence" publications, these recommendations are actually a compilation of best practices because they seem to be effective and reliable, although not with any control. RESULTS: Throughout the document, the emphasis is on promoting early, comprehensive evaluation of lower urinary tract function that is thorough but with a minimum of unnecessary testing. This includes what tests to order, when to order them and what to do with the results. Some of the recommendations may not be practical in various worldwide locations but the suggested testing should be considered the ideal approach to completely diagnosing and then promulgating treatments based on the full knowledge of the condition and its effect on urinary tract function. Once the findings are delineated, those lower urinary tract patterns of dysfunction that put the kidneys at risk for deterioration, that are barriers to attaining eventual continence, and that have long-term consequence to the lower urinary track can be obviated by specific management recommendations. The indications and timing of investigations to achieve these objectives are clearly defined in each diagnostic category and during follow-up. RECOMMENDATIONS: This document should be used as a basis for appropriate evaluation and timely surveillance of the various neuro-urologic conditions that affect children.

International Children's Continence Society's recommendations for therapeutic intervention in congenital neuropathic bladder and bowel dysfunction in children.

PURPOSE: We present a consensus view of members of the International Children's Continence Society on the therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. MATERIAL AND METHODS: Discussions were held by a group of pediatric urologists and gastroenterologists appointed by the board. The following draft review document was open to all the ICCS members via the ICCS web site. Feedback was considered by the core authors and by agreement, amendments were made as necessary. 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 therapeutic intervention in congenital neuropatic bladder and bowel dysfunction in children. RESULTS: Guidelines on pharmalogical and surgical intervention are presented. First the multiple modalities for intervention that do not involve surgical reconstruction are summarized concerning pharmacological agents, medical devices, and neuromodulation. The non-surgical intervention is promoted before undertaking major surgery. Indicators for non-surgical treatments depend on issues related to intravesical pressure, upper urinary tract status, prevalence of urinary tract infections, and the degree of incontinence. The optimal age for treatment of incontinence is also addressed. This is followed by a survey of specific treatments such as anticholinergics, botulinum-A toxin, antibiotics, and catheters. Neuromodulation of the bladder via intravesical electrical stimulation, sacral nerve stimulation, transcutaneous stimulation, and biofeedback is scrutinized. Then follows surgical intervention, which should be tailored to each individual, based on careful consideration of urodynamic findings, medical history, age, and presence of other disability. Treatments mentioned are: urethral dilation, vesicostomy, bladder, augmentation, fascial sling, artificial urinary sphincters, and bladder neck reconstruction and are summarized with regards to success rates and complications. Finally, the treatment on neuropathic bowel dysfunction with rectal suppositories irrigation and transrectal stimulation are scrutinized.

The relationship between etiology, symptom severity and indications of surgery in cases of anal incontinence: a 25-year analysis of 1,046 patients at a tertiary coloproctology practice.

BACKGROUND: The etiology of anal incontinence (AI) is often multifactorial. There is little data on the relationship between the etiology of AI, symptom severity, and the need for surgery. The aim of our study was to investigate this association in a large number of unselected patients with AI referred to a tertiary specialist coloproctological practice. METHODS: Patients with AI seen at our unit between 1983 and 2008 were analyzed. The main etiologies were categorized as congenital, traumatic, neurologic, idiopathic, post-operative, post-obstetric, secondary to rectal prolapse, or inflammatory bowel disease. The severity of AI was graded using the validated Pescatori incontinence scale. RESULTS: Overall, 1,046 patients were studied. The AI score was higher in patients with congenital (4.7 ± 1.1), traumatic (4.6 ± 1.4), and neurological (4.4 ± 1.2) incontinence. Surgical treatment was indicated in 214 cases (20.5%). Patients with AI related to trauma and congenital anomalies required surgery in 43.5 and 31.4% of cases, respectively, a percentage significantly higher than that for patients with other etiologies (P = 0.002). Prolapse-related AI usually responded to correction of the prolapse. CONCLUSIONS: Patients with congenital, traumatic, and neurological AI tend to have greater symptom severity. Traumatic, rectal prolapse-related, and congenital AI cases more often require surgery.

Antegrade continence enema in the treatment of congenital fecal incontinence beyond childhood.

PURPOSE: The role of antegrade continence enema for the treatment of congenital fecal incontinence in adult patients remains unclear. MATERIALS: Twenty-seven patients, median age 19 (range, 17-43) years, with congenital fecal incontinence underwent surgery for antegrade continence enema and were prospectively followed up for functional outcome after a median of 25 (range, 3-117) months. RESULTS: The diagnoses included myelodysplasia (n = 14), anorectal malformations (n = 6), and others (n = 7). Antegrade continence enema conduits included appendicostomy (n = 22) and cecal (n = 2), ileal (n = 2), and sigmoid (n = 1) tubes. Thirteen (48 percent) patients had complications. Eighteen (66 percent) patients became fully continent, six (23 percent) had minor, and three (11 percent) major soiling. Antegrade continence enema became unnecessary in three patients (11 percent). Treatment with antegrade continence enema failed in three cases. Of the 21 patients who continued with antegrade continence enema, 16 (76 percent) are fully continent, and bowel function and quality of life was improved in 15 (71 percent) and 13 (62 percent) patients, respectively. The scores of convenience (1 = easy, 5 = difficult) and overall satisfaction (1 = poor, 10 = excellent) were median 2 (range, 1-4) and 8 (range, 3-10). CONCLUSIONS: Despite numerous complications and occasional treatment failures, 90 percent of adult patients with congenital fecal incontinence benefited from antegrade continence enema.

Evaluation of outcome of anorectal anomaly in childhood: the role of anorectal manometry and endosonography.

The aim of this study was to evaluate role of anorectal manometry (ARM) and anal endosonography (ES) in assessment of the internal anal sphincter (IAS) quality on continence outcome following repair of anorectal anomalies (ARA). We devised a scoring system to evaluate the quality of the IAS based on ARM and ES and correlated the scores with clinical outcome, using a modified Wingfield score (MWS) for faecal continence. We also assessed the implication of megarectum and neuropathy on faecal continence. Of 54 children studied, 34 had high ARA and 20 had low ARA. Children with high ARA had poor sphincters on ES and ARM, and also poor faecal continence compared to those with low ARA. The presence of megarectum and neuropathy was associated with uniformly poor outcome irrespective of the IAS quality. The correlations between MWS on one hand, and ES and ARM scores for IAS on the other hand were weak in the whole study group, ES r = 0.27, P < 0.04, and ARM r = 0.39, P < 0.004. However, the correlations were strong in those who had isolated ARA without megarectum or neuropathy, ES r = 0.51, P < 0.02 and ARM r = 0.55, P < 0.01, respectively. In conclusion, the ARM and ES are valuable in evaluation of continence outcome in children after surgery for ARA and those with good quality IAS had better faecal continence. The IAS is a vital component in functional outcome in absence of neuropathy and megarectum.

Use of a Monti channel for administration of antegrade continence enemas.

PURPOSE: Success with Malone antegrade continence enemas (MACE) requires reliable access to the colon and a customized enema regimen. Use of the appendix in situ provides a natural and well-vascularized conduit. When the appendix is absent or inadequate, alternative techniques are required. We report our experience using Monti channels to administer antegrade continence enemas. MATERIALS AND METHODS: Of the 106 MACE procedures performed in 53 months a Monti-MACE was created in 13. Indications for use of ileum, technique, ease of catheterization and incidence of complications were reviewed. RESULTS: Mean followup was 21.7 months. Two patients had transient difficulty advancing the catheter into the cecum. Stoma revisions were required for critical stenosis in 2 patients (15.4%). Stoma leakage occurred in 1 patient after multiple stoma revisions. CONCLUSIONS: The Monti-MACE provides continent access to the colon for antegrade enemas. The rate of stomal revision is only slightly higher than that reported for appendicocecostomy. While use of the appendix in situ remains our preference, reconfigured ileum is a reliable substitute in patients without a suitable appendix.

[Fecal incontinence: therapy of congenital sphincter dysplasia in adulthood--a case report]. / Stuhlinkontinenz: Therapie der kongenitalen Sphinkterdysplasie im Erwachsenenalter--ein Fallbeispiel.

The dynamic graciloplasty has gained acceptance in the therapy of intractable fecal incontinence. With a success-rate of 60 to 80%, the dynamic graciloplasty is a good alternative towards a permanent colostomy for individual cases. Furthermore, adults suffering from congenital anal atresia may be well treated by this therapy as described in this case. Following surgery, an accurate follow-up is inevitable in these patients, including training of neosphincter control. After 8 to 12 weeks the training-process of the neosphincter-control should be finished. At this point of time the patient will have obtained defecation-control and should be able to execute voluntary defecations.

Sacral agenesis and caudal spinal cord malformations.

Thirty-three children and one adult with sacral agenesis (SA) were studied by computed tomographic myelography and/or magnetic resonance imaging and were monitored for a mean period of 4.7 years. Four children had the OEIS (concurrent omphalocele, cloacal exstrophy, imperforate anus, and spinal deformities) complex, and three others had VATER (vertebral abnormality, anal imperforation, tracheoesophageal fistula, and renal-radial anomalies) syndrome. All patients shared some of the characteristic features of SA, namely, a short, intergluteal cleft, flattened buttocks, narrow hips, distal leg atrophy, and talipes deformities. Neurologically, lumbosacral sensation was much better preserved than the motor functions, and urinary and bowel symptoms were universal. The level of the vertebral aplasia was correlated with the motor but not with the sensory level. The important neuroimaging findings of SA were as follows: 1) 12 patients (35%) had nonstenotic, tapered narrowing of the caudal bony canal, and 2 patients had hyperostosis indenting the caudal thecal sac; 2) 16 patients (47%) had nonstenotic, tapered narrowing and shortening of the dural sac, but 3 patients (9%) had true, symptomatic dural stenosis, in which the cauda equina was severely constricted by a pencil-sized caudal dural sac; 3) the coni could be divided into those ending above the L1 vertebral body (Group 1, 14 patients) and those ending below L1 (Group 2, 20 patients). Thirteen of 14 Group 1 coni were club or wedge-shaped, terminating abruptly at T11 or T12, as if the normal tip was missing. All 20 Group 2 coni were tethered: 13 were tethered by a thick filum; 2 were extremely elongated and had a terminal hydromyelia; 3 were terminal myelocystoceles; and 2 were tethered by a transitional lipoma. High blunt coni were highly correlated with high (severe) sacral malformations (sacrum ending at S1), but low-lying tethered coni were highly correlated with low sacral malformations (S2 or lower pieces present).(ABSTRACT TRUNCATED AT 400 WORDS)

[The value of CT scanning in ano-rectal malformations]. / Apport de l'étude scanographique dans les malformations ano-rectales.

Eighteen patients with ano-rectal malformation were studied by computered tomographie (1 low malformation, 9 intermediates, 6 high malformations and 2 cloacale malformations). There is no indication of this technic during neo-natal period. Computered tomographie is more important for analysis of the sequel because the appreciation of the pull through digestive segment is better. However, perineal muscles are more difficult to evalue, because there is not objective criterion.