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.

[Type 1 neurofibromatosis: Onset of two tumors before the age of 5years]. / Neurofibromatose de type 1 : survenue de deux tumeurs avant l'âge de 5 ans.

Neurofibromatosis type 1 (NF1) is a frequent autosomal dominant genetic disorder that predisposes to the development of benign and malignant tumors. Mutation of the NF1 gene affects the RAS-MAPK signaling pathway and leads to a dysfunction in cell proliferation and induces tumor development. Epidemiology of cancer in children with NF1 is very different from the general pediatric population, which requires regular and specific monitoring. Neurofibroma is the most frequent benign tumor. It can be very invalidating depending on the size and location of the tumor. Currently, there is no specific treatment for these tumors. The most frequent malignancies in children with NF1 are leukemias, rhabdomyosarcomas, malignant peripheral nerve sheath tumors and gliomas. The treatment of these tumors should consider the risk of second cancers induced by radio- and chemotherapy. We report on the case of a 5-year-old boy with NF1 developing two tumors.

[Epidemiology and management of rare paediatric tumours within the framework of the French Society for Children Cancer]. / Tumeurs rares en pédiatrie : particularités épidémiologiques et organisation de leur prise en charge dans le cadre de la Société française des cancers et des leucémies de l'enfant et de l'adolescent (SFCE).

Less than 1% of cancer occurs in children. With the progress made by national and international cooperative groups 75% of them are actually cured. However some entities have an incidence so weak that we can't actually establish standardized therapeutics guidelines. To improve our knowledge on these rare tumours a national organisation become necessary as well as an international collaboration. A French rare tumour committee was created within the French Society for Children Cancer (SFCE). Others European countries have such organisation. The objectives of these tasks groups are to enhance our knowledge of the real incidence of these rare tumours, their evolution, and to propose therapeutic recommendations for each of them. This article focuses on the specific French organization for rare tumours treatment. It also describes the draft for the creation of a new data base for prospective registry of clinical, therapeutics and follow up data. To provide a better understanding of these pathologies, the "Bulletin du Cancer's" editorial board decided to regularly publish an update on a rare paediatric tumour in a specific section.

[Child pleuropulmonary blastoma]. / Pleuropneumoblastome de l'enfant.

Pleuropulmonary blastoma is an extremely rare and aggressive thoracic tumour seen exclusively in children. The initial symptoms are respiratory and non specific and the chest X-ray done in that context reveals a thoracic mass. The chest CT scan then leads to the diagnosis of a cystic, mixt or solid mass. The diagnosis will be affirmed on anatomopathology of the tumour or biopsy. There are three different histological types: type I, cystic, type II, cystic and solid and type III, solid exclusively. Type I is less aggressive and its treatment is essentially surgical. Types II and III are highly aggressive and require surgery associated to polychemotherapy. In all cases, surgery is essential and should be the most complete possible. Extension and follow-up exams will include bone scan and brain imagery searching for metastasis as well as abdominal ultrasound searching associated renal lesions because 30% of pleuropulmonary blastoma are part of a familial predisposition syndrome.

[Granulomatous pulmonary involvement preceding diagnosis of Crohn disease: a pediatric case report]. / Atteinte granulomateuse pulmonaire précédant le diagnostic de maladie de Crohn: à propos d'un cas pédiatrique.

Crohn disease (CD) is a chronic bowel disorder that may affect many other organs such as the eyes, hepatobiliary system, skin, and joints. Pulmonary involvement in association with CD is a classic but uncommon manifestation. It can be primitive with granulomas or secondary to treatments. We report on the case of a teenager in whom the onset of CD was dominated by respiratory symptoms. Because of this presentation, we also suspected opportunistic infections such as tuberculosis and other granulomatous pulmonary diseases such as sarcoidosis or hypersensitivity pneumonitis.

Sacral neuromodulation in children with urinary and fecal incontinence: a multicenter, open label, randomized, crossover study.

PURPOSE: The clinical benefit of sacral neuromodulation is unclear due to the paucity of randomized trial data. The purpose of this study was to evaluate sacral neuromodulation for management of urinary and fecal incontinence in a pediatric population. MATERIALS AND METHODS: This multicenter, open label, randomized, crossover study included children older than 5 years. After trial stimulation of the S3 root a neuromodulator (InterStim) was implanted on the S3 foramen. Clinical examinations, voiding and bowel diaries, and urodynamic and manometric evaluations were performed at the beginning (t1) and end (t2) of the first period, and at the beginning (t3) and end (t4) of the second period. RESULTS: A total of 33 patients (24 boys) with a mean +/- SD age of 12.22 +/- 5.09 years were randomized. Etiologies were mainly of neurological origin. Incontinence was mixed urinary and fecal in 19 cases, urinary only in 9 and fecal only in 5. Cystometric bladder capacity increased during sacral neuromodulation (delta +24.27 ml vs -37.45 ml, p = 0.01). There was no significant change in other urodynamic or manometric parameters. Overall positive response rate was more than 75% for urinary (81%) and bowel (78%) function. Crossover analysis indicated that sacral neuromodulation is more effective than conservative treatment for both types of incontinence (p = 0.001). CONCLUSIONS: In a pediatric population sacral neuromodulation is effective for bladder and bowel dysfunction and should be considered before irreversible surgery.

[Acquired non hypertrophic pyloric stenosis in children]. / Sténose acquise non hypertrophique du pylore chez l'enfant.

UNLABELLED: Pediatric non hypertrophic pyloric stenosis (NHPS) are uncommon. Their causes and treatments are debated. MATERIAL AND METHOD: Retrospective review of all cases of NHPS from 3 pediatric surgery services during the period 1984-2002. RESULTS: Six children, aged 17 months to 15 years, underwent surgery for NHPS. Clinical symptoms, food vomiting and loss of weight, were present for several weeks before the diagnosis of NHPS was made. The diagnosis was peptic stenosis in 3 cases and has not been established in 3 cases. Search for Helicobacter pylori was negative in all cases. Failure of specific medical treatment and endoscopic dilatations led to pyloric resection in 3 cases and pyloroplasty in 3 cases. Post operative course was uneventful with normal oral feeding and normalisation of weight status. Histologic data were aspecific. No recurrence was observed. DISCUSSION: We discuss the origin of the pyloric stenosis, regarding clinical, operative and pathological data: were the stenosis the cause or consequence of peptic ulcer? Peptic disease is always advocated, but difficult to prove and may be excessively incriminated. Late symptomatic congenital and acquired idiopathic pyloric stenosis should be recalled. In all cases of proved pyloric stenosis, after failure of medical and endoscopic treatment, a simple surgical procedure (pyloroplasty) associated with medical treatment seems to be effective. CONCLUSION: The diagnosis of NHPS should be suspected in a child with food vomiting and loss of weight if his age is not concordant with hypertrophic pyloric stenosis. Upper gastro-intestinal series and endoscopy are diagnostic. The precise cause of the stenosis is more difficult to asses. When the medical treatment fails, a pyloroplasty is usually curative.

Interest of laparoscopy in polysplenia syndrome.

Polysplenia syndrome (PS) is usually discovered in symptomatic patients in association with congenital heart disease or biliary atresia. Asymptomatic patients can present associated anomalies of the digestive tract such as intestinal malrotation and gastric or splenic malfixation. We report a case of PS presenting as a left flank mass shown to be an accessory spleen by denatured red blood cell scintigraphy. Upper gastrointestinal tract studies showed intestinal malrotation. Laparoscopic exploration confirmed intestinal malrotation and showed the absence of fixation of the accessory spleen. We performed a laparoscopic Ladd's procedure followed by fixation of the accessory spleen and resection of Meckel's diverticulum through a short left-flank incision.

Laparoscope-assisted appendectomy in children: the two-trocar technique.

UNLABELLED: The conventional cannulae position in laparoscopic appendectomy does not permit good visualization of the appendix, especially if it is in a retrocaecal or pelvic position. In children, the smaller dimension of the peritoneal cavity aggravates this problem. METHOD: In order to optimize appendix visualization, we propose a modification of the cannulae position associated with extraabdominal appendectomy. Two trocars are inserted, one in the umbilicus under direct vision and one in the midline above the pubic symphysis. The telescope is inserted in the second cannula. The appendix is delivered via the umbilical trocar incision and a conventional appendectomy is performed. RESULTS: During a one-year period, 38 appendectomies were performed. In 29 cases (76.3 %) the technique was successful. In 9 patients (4 with peritonitis, 4 with a retrocaecal and one with a pelvic appendix) a third trocar was necessary to complete the operation. We had two conversions (5.2 %), one to open surgery and one to intraabdominal laparoscopic appendectomy. Mean operation time was 19 min. There were two infectious complications (one parietal and one intraabdominal). CONCLUSIONS: The excellent visualization permitted considerable time gain. Infectious complications were also low. This method is suggested as an alternative procedure for appendectomy, especially in children, because of their favorable anatomy.

Noninvasive monitoring of citrate, acetate, lactate, and renal medullary osmolyte excretion in urine as biomarkers of exposure to ischemic reperfusion injury.

Injury during the transplant process affects the alloantigen-dependent factors and the alloantigen-independent processes of "chronic" rejection. Consequently, the determination of reliable parameters for the assessment of ischemic damage is essential for the prediction of renal changes after ischemia/reperfusion injury. The aim of this study was to assess the ability of (1)H NMR spectroscopy to predict the early graft dysfunction in an ischemia/reperfusion model after preservation in two standard preservation solutions, Euro-Collins (EC) and University of Wisconsin (UW). The second aim was to specify the role of the UW solution in preventing renal medullary injury. Urine and plasma samples from three experimental groups were examined during 2 weeks: control group (n = 5), EC group (cold flushed and 48-h cold storage of kidney in EC and autotransplantation, n = 12), and UW group (cold flushed and 48-h cold storage of kidney in UW and autotransplantation; n = 12). We also examined these kidneys 30-40 min after implantation and on the sacrifice day. Creatinine clearance was significantly reduced in the EC group during the second week. Fractional excretion of sodium and urine N-acetyl-beta-d-glucosaminidase activity were improved but not significantly different in the preserved groups. Urinary concentrations of the alpha-class glutathione S-transferase were significantly greater in the EC group during the first week after transplantation. The most relevant resonances for evaluating renal function after transplantation determined by (1)H NMR spectroscopy were those arising from citrate, dimethylamine (DMA), lactate, and acetate in urine and trimethylamine-N-oxide (TMAO) in urine and plasma. These findings suggest that graft dysfunction is associated with damage to the renal medulla determined by TMAO release in urine and plasma associated with DMA and acetate excretion. Citrate is also a urinary marker that can discriminate kidneys with a favorable evolution. Our results suggest that (1)H NMR spectroscopy is an efficient technique for detecting ischemic damage when accurate and precise data on graft injury is required. In addition, this study outlines the specific impact of the UW solution against injury to the renal medulla.