Mirna Haddad

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.

Management of choledochal cyst: Evolution with antenatal diagnosis and laparoscopic approach.

BACKGROUND/AIM: Laparoscopic excision of a choledochal cyst (CC) with hepaticojejunostomy gained a wide acceptance in the treatment of children even in neonatal period. Although, the use of prenatal diagnostic techniques causes a significant increase in antenatal diagnosis of CC, the time of surgical intervention during infancy is still controversial. A retrospective study was performed to evaluate the results of laparoscopic management of CC with special emphasis on antenatal diagnosis and treatment, and to compare the results with open procedure. MATERIALS AND METHODS: The patients who were diagnosed with choledochal cyst and underwent either open or laparoscopic hepaticojejunostomy in two centres, hopital d’enfant de La Timone from Marseille and hopital Robert Debre from Paris between November 2000 and December 2009 were included in the study. The data obtained from medical reports were evaluated for sex, time of antenatal diagnosis, age at time of operation, operative time, time of postoperation. RESULTS: A total of 19 hepaticojejunostomy were performed, including 10 open procedures (group A), and 9 laparoscopic procedures (group B, 4 were diagnosed prenatally, without conversion to open procedure). There were 3 boys and 16 girls, ranging in age from 2 weeks to 16 years. Patients in the group A were older than patients in the group B. The mean hospital stay and time to return of bowel fuction was longer in the group B. there were 60% of pre-operative complications in group A versus 22% in group B. There was one postoperative complications in group B (biliary leakage nedeed redo surgery). No significant differences were found between different parameters except for operative time (288.56 min in the group B versus 206 min in the group A. (p = 0.041)). CONCLUSIONS: Our initial experience indicates that the laparoscopic approach in infancy is technically feasible, safe, and effective, with a low morbidity and a comparable outcome to the open approach. Therefore, we propose a laparoscopic approach for antenatally diagnosed CC as early as possible, before the onset of complications.

Congenital pouch colon: antenatal diagnosis and video-assisted management

Congenital pouch colon (CPC) is an unusual abnormality associating a pouch-like dilatation of a shortened colon with an anorectal malformation (ARM). There are few reports of CPC in Europe, a contrario it represents up to 15% of ARM in India. Coloplasty and excision are described in the surgical management. This report describes a new case of CPC. This is the first reported case with a prenatal clinical presentation as an hypoechogenic abdominal image at ultrasound and a video-assisted management.

Intestinal loop lengthening: early treatment of vanishing bowel

Bianchi’s procedure experience for short bowel syndrome in children is positive. This technique is generally performed after the first year of life. Here the authors propose a case of gastroschisis with prenatal spontaneous closure of abdominal defect and vanishing gut presenting as intestinal atresia, absence of ileo-cecal valve, and residual short intestinal dilatation, treated by early gut lengthening (ILP).

Long-Term Efficacy of Young-Dees Bladder Neck Reconstruction: Role of the Associated Bladder Neck Injection for the Treatment of Children With Urinary Incontinence

OBJECTIVE To compare the long-term efficacy of Young-Dees bladder neck reconstruction (YDBNR) alone versus YDBNR plus bladder neck injection (BNI) in patients with urinary incontinence caused by urethral sphincter insufficiency. PATIENTS AND METHODS Between 1987 and 2006, we assessed the continence rates obtained with YDBNR and BNI as a supplementary treatment for persistent outlet insufficiency in patients with neurogenic bladder (group 1, n = 35) and bladder exstrophy (group 2, n = 20). Median postoperative follow-up was 16 years (range: 5-29). RESULTS A total of 55 children (23 males and 32 females) underwent YDBNR at the median age of 7.6 years (range: 1.9-17.25). Only 10 patients (18%) were considered continent after the isolated YDBNR; 17% (n = 6/35) from group 1 and 20% (n = 4/20) from group 2 (p = 1). Because of unsatisfactory results after YDBNR, 81.8% (n = 45/55) received BNI. Fifteen patients (33%) became continent with an average of 2.29 injections (±1.1); 44.8% (n = 13/29) from group 1 and 12.5% (n = 2/16) from group 2. A significant difference was found on comparing the social continence rate attained with YDBNR plus BNI between patients from group 1 and group 2 (54% vs 30%, P = .04). The difference between males and females in terms of continence rates was not statistically significant. CONCLUSION Long-term results of YDBNR are modest. BNI does increase the results of YDBNR, especially in patients with neurogenic bladder.

Family History is Underestimated in Children with Isolated Hypospadias: A French Multicenter Report of 88 Families

Purpose: While familial forms of complex disorders/differences of sex development have been widely reported, data regarding isolated hypospadias are sparse and a family history is thought to be less frequent. We aimed to determine the frequency of hypospadias in families of boys with hypospadias, to establish whether these familial forms exhibit a particular phenotype and to evaluate the prevalence of genetic defects of the main candidate genes. Materials and Methods: A total of 395 boys with hypospadias were prospectively screened for a family history with a standardized questionnaire, extensive clinical description, family tree and sequencing of AR, SF1, SRD5A2 and MAMLD1. Results: Family history of hypospadias was more frequent than expected (88 patients, 22.3%). In 17 instances (19.3%) familial hypospadias cases were multiple. Familial hypospadias was related to the paternal side in 59.1% of cases, consisting of the father himself (30.7%) as well as paternal uncles and cousins. Premature birth, assisted reproductive techniques, other congenital abnormalities and growth retardation were not more frequent in familial hypospadias than in sporadic cases. The severity of phenotype was similar in both groups. The results of genetic analysis combined with previous data on androgen receptor sequencing revealed that familial cases more frequently tend to demonstrate genetic defects than sporadic cases (5.68% vs 1.63%, p = 0.048). Conclusions: Familial forms of hypospadias are far more frequent than previously reported. Even minor and isolated forms justify a full clinical investigation of the family history. Detecting these hereditary forms may help to determine the underlying genetic defects, and may improve followup and counseling of these patients.

Laparoscopic nephrectomy of a cross-fused ectopic kidney in a child with hypertension

OBJECTIVE We report a case of laparoscopic nephrectomy of a cross-fused ectopic kidney in a 4-year-old girl with renal hypertension and Fanconi anemia. MATERIALS AND METHODS We performed a transperitoneal laparoscopy. Ectopic kidney resection was done after dissection of the pathological kidney and after clamping vessels, using an ultrasonic device. Hospitalization time was 4 days. RESULTS At 6 months, blood pressure was normalized and the patient showed an adequate growth curve. CONCLUSION The transperitoneal route is very effective when a nephrectomy is necessary. It offers perfect exposure with limited risk of complications.

Hypospadias : Que dire aux parents ?

La decouverte d’un hypospadias en salle de naissance necessite une information des parents. Le pediatre doit savoir repondre a leurs nombreuses interrogations concernant notamment l’avenir fonctionnel et les corrections chirurgicales eventuellement necessaires.

SFCP-P38 – Chirurgie viscérale – Résultats de la toxine botulique dans l’hypertonie sphinctérienne résiduelle de la maladie de Hirschsprung

Objectifs L’hypertonie sphincterienne (HS) post operatoire dans la maladie de Hirschsprung (MH) est frequente. Elle peut entrainer constipation severe et/ou enterocolites iteratives. Nous avons evalue au cours d’une etude retrospective clinique les resultats de l’injection intra-sphincterienne de la toxine botulique (TB) sur cette hypertonie. Sujets De 2004 a 2007,13 enfants operes d’une MH (2 Duhamel, 4 Swenson et 7 Swensons transanaux) et presentant des troubles digestifs (groupe 1 : enterocolites iteratives, groupe 2 : constipation severe avec fuites fecales) associes a une HS clinique (toucher rectal) ou manometrique ont beneficie d’une injection intra-sphincterienne de TB. La TB est injectee a 4UI/Kg en 4 quadrants, a la partie superieure du sphincter externe sous anesthesie generale, apres reperage par neurostimulation. Les resultants ont ete evalues selon des criteres cliniques : ballonnement, consistence et odeurs des selles, fuites fecales, apparition de selles spontanees, et acquisition de la proprete. Resultats Le groupe 1 (recul moyen : 25 mois) reunit 8 enfants (2 MH pancoliques), avec un âge moyen a la premiere injection de 4 ans (1- 8 ans). Apres 2 injections de TB, aucun n’a plus presente d’enterocolites, la moitie d’entre eux necessitaient encore une aide a l’exoneration. Cinq d’entre eux ont necessite de 1 a 4 injections supplementaires (delai moyen entre 2 injections : 6 mois). Au final, ces 8 enfants ont acquis la proprete avec des selles spontanees de consistance normale. Le groupe 2 (recul moyen : 15 mois) reunit 5 enfants avec un âge moyen a la premiere injection de 8,5 ans (2,5-15 ans). Apres la premiere injection, 4 enfants sur 5 n’ont plus presente de fuites et ont obtenu des selles spontanees. Des reinjections ont ete necessaires pour 3 d’entre eux. Un enfant necessite encore un support medical. Aucune complication n’a ete notee. Conclusion L’injection de TB semble une technique efficace et peu invasive pour lever l’hypertonie sphincterienne residuelle apres cure de MH. La TB a permis une amelioration des symptomes cliniques et un allegement du traitement medicamenteux au seul prix d’une repetition de ces injections. Une etude prospective manometrique est en cours.

SFCP-024 – Urologie – La neuromodulation sacrée dans le traitement des troubles de la continence chez l’enfant

Objectifs La neuromodulation sacree (NMS) a montre son efficacite dans le traitement des troubles refractaires urinaires et digestifs chez l’adulte. Nous presentons les resultats preliminaires d’une etude en « cross over », prospective et multicentrique pour evaluer l’efficacite de la NMS dans la prise en charge de l’incontinence urinaire et fecale de l’enfant. Methodes L’etude porte sur des enfants âges entre 5 et 18 ans presentant une incontinence urinaire secondaire a une vessie neurologique associee ou non a une incontinence fecale. Les patients ayant une agenesie sacree > 50 % ont ete exclus. Le consentement eclaire et les criteres de sortie de l’etude ont ete definis selon la declaration d’Helsinki et en accord avec la loi Huriet. Apres un reperage de la 3eme racine sacree sous anesthesie generale, les patients ont ete implantes au niveau de S3. Chaque patient est traite successivement par NMS puis par son traitement conventionnel ou inversement (randomisation). Un examen clinique, des calendriers mictionnel et fecal, une evaluation urodynamiqueet rectomanometrique sont faits tous les 3 mois. Resultats A ce jour, 35 patients ont ete inclus. 19 sont arrives a la fin de l’etude et sont evalues cliniquement.12 garcons et 7 filles d’âge moyen de 12.5 ans. Les etiologies principales sont : spina bifida et agenesie sacree. L’incontinence etait mixte (urinaire et fecale) chez 11 patients, urinaire isolee chez 5 et fecale isolee chez 3.3 patients (2 mixtes et 1 urinaire) n’ont pas ete evalues (1 perdu de vue, 1 pour infection du materiel et 1 pour des problemes respiratoires lies a sa maladie). Parmi les 16 patients evalues apres NMS : 3 sont un succes (1 disparition des fuites urinaires et 1 normalite du transit sur 9 incontinences mixtes, 1 disparition des fuites fecales sur 3 incontinences fecales), 9 ont ete ameliores au niveau de la sensation vesicale et/ou du transit intestinal (6/9 mixtes, 2/4 urinaires et 1/3 fecales), 4 sont des echecs (1/9 mixtes, 2/4 urinaires et 1/3 fecales). Conclusions La NMS a un effet benefique sur la vessie et surtout sur le dysfonctionnement intestinal. Ces resultats cliniques ne sont que preliminaires. Une analyse a terme et une validation statistique seront necessaires pour confirmer l’efficacite de cette approche.