Multicentric evaluation of the adherence to Peristeen® transanal irrigation system in children.

BACKGROUND: Since 2009 in France, the Peristeen® transanal irrigation (TAI) device has represented an alternative treatment of faecal incontinence (FI). OBJECTIVE: The primary objective of this study was to assess the mid-term adherence to TAI in paediatric patients. The secondary objective was to identify factors determining TAI continuation. METHODS: This observational study conducted in 5 French paediatric centres prospectively reviewed from March to May 2012 all children educated in TAI for at least 9months. RESULTS: We included 149 children (mean [SD] age 10.6 [4.1] years) educated in TAI. Children mainly had neurogenic disorders (52.3%) or congenital malformations (30.9%). The main symptoms motivating TAI initiation were recurring faecaloma (59.7%) and daily FI (65.1%). At last follow-up (mean 14 [7.4] months), 129 (86.6%) children continued the TAI procedure, independent of pathology or age. The main motivation was resolution of FI and/or constipation (77.3%). In total, 107 (82.9%) children fulfilled the initial therapeutic contract established with their healthcare professional before TAI initiation was met. Twenty children had stopped the TAI when they answered the questionnaire, at a mean duration of 16 (8.4) months. The reasons were mainly "lack of motivation" (45%), "poor tolerance" (35%), "difficulties" performing the procedure (35%) and "inefficacy" (30%). Factors related to continuation were performing at least one TAI procedure under a nurse's supervision during the initial training and prescribing TAI at a daily frequency (P=0.014 and P=0.04). Continuing constipation treatment after the training session was a factor in discontinuation (P=0.024). CONCLUSION: This study reports a very high mid-term adherence to TAI in a paediatric cohort, provided that the training is pragmatic, personalized and repeated.

Long-term renal outcome in infants with congenital lower urinary tract obstruction.

INTRODUCTION: Congenital Lower Urinary Tract Obstructions (LUTO) is a heterogeneous group of diseases in which urine elimination is obstructed at the level of bladder neck or urethra. The aim of the study is to evaluate the long-term renal outcome of patients suffering of congenital LUTO. PATIENTS AND METHOD: We retrospectively reviewed patients with congenital LUTO. All patients had at least 1 year follow-up. Data on surgery, renal imaging and Schwartz estimate creatinine clearance were collected. Incidence of Chronic Renal Disease (CRD) is presented with Kaplan-Meier method. RESULTS: 40 patients were included, 23 patients with Posterior Urethral Valve (PUVs) and 17 patients with other aetiologies: anterior urethral valve (2), urethral atresia (2), urethral stenosis (2), cloacal malformations (2), obstructive ureterocele (1), bladder trigone malformation (1) and neonatal bladder-sphincter dysfunction without neurological abnormalities (7). Incidence of CRD at age 10 years was 37% in congenital LUTO, 42% in PUVs and 30% in other aetiologies, and was significantly higher in PUVs (P=0.032). Renal prognosis was significantly worsened by discover of retentional bladder wall changes in initial cystoscopy, and by loss of parenchymal differentiation or cortical microcysts in first ultrasonography. The use of urinary diversion was significantly higher in LUTO of other aetiologies. CONCLUSION: A high incidence of CRD is observed in patients with congenital LUTO, significantly higher in patients with PUV. LUTO of other aetiologies require step by step surgical management and higher use of urinary diversion. Precise initial evaluation in cystoscopy and ultrasonography is required and participate to evaluate future renal outcome. LEVEL OF INCIDENCE: 4.

[Does prenatal diagnosis modify neonatal management and early outcome of children with esophageal atresia type III?]. / Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l'œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.

Quality of life and continence in patients with spina bifida.

PURPOSE: Spina bifida (SB) is the most common congenital cause of incontinence in childhood. This study attempts to determine the relationships between urinary/faecal incontinence, methods of management, and Health Related Quality of Life (HRQoL) in people with SB. PATIENTS AND METHOD: A total of 460 patients (300 adults and 160 adolescents) from six centres in France have taken part in this cross-sectional study. Clinical outcome measures included walking ability, urinary/faecal continence, and medical management. HRQoL was assessed using the SF36 in adults and the VSP in adolescents and their parents. Univariate and multivariate analysis was used to determine the relationships between clinical parameters and HRQoL. RESULTS: HRQoL were significantly lower than in the general population. Adult women had significantly lower scores than men, and adolescent females had significantly lower scores for psychological well being. We did not found strong relationship between incontinence and HRQoL in this population. Moreover patients surgically managed for urinary/fecal incontinence did not show significantly higher scores of HRQoL. CONCLUSION: Using generic HRQoL measures, urinary/faecal incontinence and their medical management may not play a determinant role in HRQoL of persons with SB. However many other factors affect HRQoL in these patients. A longitudinal study design is recommended to assess whether incontinence management is associated with improved HRQoL.

A multicentre study of the management of disorders of defecation in patients with spina bifida.

Patients with spinal dysraphism may have severe constipation and faecal incontinence. The impact of antegrade colonic enema (ACE) in the management of patients with spina bifida (SB) is analysed. In a multicentre cross-sectional study, constipation, faecal incontinence and faecal management were described. Cases surgically treated were identified. Data were collected from 423 patients, of whom 230 did not use any manoeuvre or laxatives to assist evacuation. Conventional treatment was used in 193 patients, including digital extraction in 39%, retrograde enema in 21% and oral laxatives in 52%. For intractable constipation and overflow of faecal incontinence, 47 patients were treated with ACE, of whom 41 used the method at a mean time of interview of 4.1 +/- 1.9 years after ACE operation; six abandoned ACE for conventional management. With ACE, faecal continence was significantly improved compared with conventional management, and neither retrograde rectal enema nor digital extraction were required. The conduit was fashioned to the right colon in 32 cases and to the left colon in nine cases. This study provides information on a multicentre experience in bowel management in SB patients. Whatever the technique used, ACE has improved faecal status compared with conventional therapy.

A multicenter evaluation of urinary incontinence management and outcome in spina bifida.

PURPOSE: We describe urinary continence management and outcome in patients with spina bifida to identify the procedures that are most successful. MATERIALS AND METHODS: In a multicenter retrospective cohort study medical charts were studied. At the same time in a cross-sectional survey sociodemographic characteristics, orthopedic features and urinary continence were described based on the frequency of leakage from the viewpoint of patients or close relatives using a Likert scale of 5 items, namely 1-leakage permanent to 5-leakage never. RESULTS: A total of 421 patients were included, of whom 191 (45%) had been medically treated with a normal voiding pattern according to the patient viewpoint in 21%, clean intermittent catheterization in 61% and no specific bladder emptying method in 18%. The mean leakage score +/- SD was 2.74 +/- 1.55. On the other hand, 230 patients (55%) were surgically treated. Except for 23 patients who underwent noncontinent urinary diversion 207 were considered for treatment and continence description. The mean leakage score was 3.45 +/- 1.60. An artificial urinary sphincter in male and females, and a sling or Kropp technique in females were satisfactory when bladder enlargement was not required. In cases of bladder augmentation without continent diversion an artificial urinary sphincter in males and a bladder neck sling or cinch, Kropp and Young-Dees procedures in females have provided the best results. In cases of bladder enlargement with continent urinary diversion bladder neck closure or a wrap have provided the best results whatever the patient sex. CONCLUSIONS: Many factors may influence the choice of a technique, such as patient sex, bladder characteristics or orthopedic conditions. However, since to our knowledge no randomized, controlled study has been yet performed, definitive conclusions on the best way to achieve urinary continence in patients with spina bifida cannot be established.

Single percutaneous access for endoscopic extraction of lithiasis after intestinocystoplasty.

PURPOSE: Bladder-stone formation is a common problem following augmentation cystoplasty. Urological management is controversial. We describe a minimally invasive technique using electrohydraulic vesicolithotripsy via a single percutaneous access. MATERIAL AND METHODS: Between 1998 and 2004, 10 percutaneous extractions of bladder calculi were performed in seven patients with bladder augmentation. Prior bladder enlargement was performed for neuropathic bladder dysfunction in four cases and bladder exstrophy in three cases. The procedure involved percutaneous placement of a 10- or 12-mm laparoscopic trocar, after which the airtightness valve of the trocar was removed. A rigid nephroscope allowed direct visualization of calculi. Stones were fragmented with electrohydraulic lithotripsy when necessary. Removal of the fragments was by grasping forceps or basket. Vigorous, warmed-saline bladder irrigation eliminated residual fragments. RESULTS: The technique was successful in all cases, resulting in controlled bladder-stone-free status and no surgical complications. The only complication was hypothermia linked to prolonged operation time, inadequate warmed-saline irrigation and lack of waterproof sheets. CONCLUSIONS: Percutaneous vesicolithotripsy was highly successful and easy to perform, without being restricted by size and number of calculi. The continence mechanism of a reconstructed urethra or Mitrofanoff conduit should not be compromised. As recurrent stone formation is expected in children with augmented bladder, this technique appears to be suitable for bladder calculi removal during the long-term follow-up of multioperated patients.

Comparative study of the Yang-Monti channel and appendix for continent diversion in the Mitrofanoff and Malone principles.

PURPOSE: Continent conduits for Mitrofanoff and Malone procedures are widely used for incontinence management. Because the appendix is not always suitable or available, Monti proposed a transversely retubularized short segment of ileum. We present our experience and compare outcomes according to the type of conduit. MATERIALS AND METHODS: In a retrospective study from 1988 to 2003 case notes were reviewed specifying conduit characteristics, underlying disease, overall surgical management of incontinence and subsequent complications. Comparison was based on followup, details of complications and treatments. RESULTS: In 32 men and 14 women with a total of 65 conduits (Mitrofanoff in 41 and Malone procedure in 24) the condition was spina bifida in 29, bladder exstrophy in 5 and sacral agenesia in 3. Other cases included various origins. Mean age at operation was 14.2 years (range 1 to 31). Mean followup was 5.3 years (range 1 to 14.7). Appendix was used in 23 cases and a Yang-Monti channel was performed in 18 (ileum in 7 and sigmoid in 11) for urinary diversion. The Malone procedure was performed using appendix in 11 cases and a Yang-Monti tube in 13. At followup 61 conduits (93.8%) were in use and 4 (6.2%) were abandoned. Complications were cutaneous stenosis, kink blockage and stomal leakage. No significant differences were observed between the types and purposes of the conduit. CONCLUSIONS: With constant availability and various possibilities of construction the Yang-Monti channel is recommended for continent diversion creation when appendix does not look suitable or is already in use or absent.

Dynamic MR imaging of bladder haemangioma.

Haemangioma is a rare benign mesenchymal tumour of the bladder. We report a case of bladder haemangioma and describe the enhancement kinetics observed on MR images after gadolinium bolus administration, which is similar to that reported in liver haemangioma.

Bowel management with antegrade colonic enema using a Malone or a Monti conduit--clinical results.

During this last decade, major surgical improvements have been proposed for the treatment of patients suffering from faecal incontinence or intractable constipation. Between 1996 and 1999, 12 patients mainly presenting with spina bifida were operated for antegrade colonic enema (ACE). In seven cases, a Malone procedure was performed. In 5 cases we used a Monti plasty to create a continent catheterisable conduit. In 4 cases the neoconduit was placed between the transverse colon and the umbilicus. The mean follow-up was 15 months. Bowel control and continence were obtained in 11 cases, postoperatively. One early complete stenosis of the cutaneous stoma occurred, after which the patient did not wish to carry on. Another patient stopped using his ACE stoma after several months because he was able to manage his bowel evacuation voluntarily without requiring an enema. The other ten patients have greatly improved their quality of life. Complications included principally stomal stenosis which can be regularly dilated by the patient himself using the Hegar dilator. Patients with umbilical stoma have a result comparable to patients with Malone procedure. Our series shows that ACE can be performed without an appendix using a Monti plasty. Implantation of the continent conduit in the caecum or in the transverse colon does not modify the overall results of ACE.

[Surgical management of urinary and fecal incontinence in neurological sphincter disorders of children and adolescents]. / Prise en charge chirurgicale de l'incontinence urinaire et fécale dans les troubles sphinctériens neurologiques de l'enfant et de l'adolescent.

The management of urinary and fecal incontinence in children and adolescents with neurogenic disorders related to congenital or acquired conditions was frequently considered to be of secondary importance compared with orthopaedic complications. The improvement of artificial urinary sphincter and continent urinary diversion techniques allowed to establish for each case an individual plan considering overall, abilities and voiding dysfunction as well as renal complications. Antegrade colonic enema has greatly improved the quality of life of children with fecal incontinence or intractable constipation. Surgical management requires a large contribution by the patients and their closest relatives and complete information about goals, advantages and obligations of surgical management. Surgical principles for bladder augmentation, bladder neck reconstruction and continent urinary diversion are presented and discussed according to data in the recent literature and the author's clinical experience.

Colonic stenosis after hemolytic-uremic syndrome.

Colonic necrosis and intestinal perforation are the usual surgical complications of hemolytic syndrome. We present a case of right transverse colon stricture following hemolytic-uremic syndrome.

[Inguinal hernia in the infant]. / Hernie inguinale de l'enfant.

The inguinal hernia repair account among surgical procedures the most frequently performed in children. It represents a flawless example of the paediatric specificity of a common reputed pathology. Its diagnosis especially in the young children can be delicate justifying thus a systematic tracking during the clinic examination. The pathology of the vaginalis processus is varied and the associated anomalies recognition especially the presence of an undescended testis has to be detected. Inguinal incarceration is often inaugural in infants. Consequences concern in the first place the testicular blood supply before to interest the vitality of the incarcerated intestine whose ischemic suffering will be more delayed. Systemic and early herniotomy has become recommended because of improvement of anesthetic and surgical safety in all ages. Risks of abstention today exceed these of a precocious operation with all respects of surgical and anesthetic criteria required according to the age of the child. Despite inguinal hernia repair is one of the operations on basis in general surgery, its realization in children requires to be warned of the specificity of this act. The dissection of the spermatic cord exposes to a traumatic damage of vas deferens which must be avoided by an appropriate technique.

[Congenital skin defect and fetus papyraceus. A case]. / Absence cutanée congénitale et foetus papyracé. Une observation.

BACKGROUND: Congenital skin defect is an uncommon condition. The term of "congenital skin aplasia" should be avoided since the origin may not necessarily be congenital malformation. The scalp is involved in 80 p. 100 of the cases. CASE REPORT: We observed a congenital skin defect located exclusively on the trunk. The infant lacked wide areas of skin in symmetrical star-like configurations. DISCUSSION: The notion of a twin expulsed after 4 months gestation suggested the diagnosis of congenital skin defect and fetus papyraceus or group V congenital skin aplasia in the Frieden classification as recalled. This diagnosis must not be overlooked as the prognosis is good. Closure with atrophic skin usually occurs within a few weeks. The etiopathogenesis remains obscure.

A follow-up study of pre-natally detected primary vesico-ureteric reflux: a review of 61 patients.

OBJECTIVE: To describe the pre- and post-natal characteristics of primary vesico-ureteric reflux (VUR) in a retrospective study of babies presenting with VUR suspected from pre-natal ultrasonography. PATIENTS AND METHODS: Between 1984 and 1994. 61 children (41 boys and 20 girls) with primary VUR were followed for 2 years after the pre-natal detection of urinary tract anomalies. Patients with VUR secondary to infravesical obstruction and duplex systems were excluded. The mean age at ante-natal diagnosis was 28.4 weeks of gestation; in 37 the reflux was bilateral and 98 refluxing units were reviewed. RESULTS: According to the international classification of VUR, 8% were grade 1, 32% grade 2, 38% grade 3, 16% grade 4 and 6% grade 5. Twelve patients (22 refluxing units) underwent ureteric re-implantation. Six kidneys showed renal scars on isotope renography and two nephrectomies were carried out in patients < 2 years old: in 40 patients (64 renal units) the VUR resolved spontaneously. Of the latter, eight were grade 1, 20 grade 2, 27 grade 3 and nine grade 4: seven patients (10 refluxing units) are still being followed and awaiting a decision on treatment. CONCLUSION: This study confirms the predominance of boys in those with ante-natally suspected VUR. The spontaneous resolution during the first 2 years of life was apparent in most cases, even in those with severe reflux (grade 3-5).

[Nephronogenic nephroma: a benign kidney tumor in adults. Two case reports]. / Le néphrome néphronogène: tumeur bénigne du rein de l'adulte. A propos de 2 cas.

The authors report 2 cases of a benign and uncommon type of renal tumour in adult, the nephronogenic nephroma. The pathology is discussed and compared to other groups of solid renal tumour. The confusion with malign tumour is usual. According to radiological investigations and pathological analysis, aspects of surgical treatment are presented and discussed.