Initial experience with ACT™ periurethral adjustable balloons to treat urinary incontinence due to intrinsic sphincter deficiency in the pediatric population.

PURPOSE: To present our initial experience with periurethral adjustable continence therapy (ACT™) for urinary incontinence due to intrinsic sphincter deficiency (ISD) in children. METHODS: This is an approved prospective non-randomized pilot study (NCT03351634) aiming to treat children born with spinal dysraphism (SD) or exstrophy epispadias complex (EEC) with ACT™. Endpoints were patient-reported changes in daily pad count, 24-h Pad test and complications. RESULTS: Since April 2018, 13 children (six girls, seven boys) were implanted at the median age of 12 years (5-16). The etiology of incontinence was neurogenic ISD (7/13, 54%) and EEC (6/13, 46%). After ACT™ implantation, continence (no pad or 1 security pad/day) was achieved in 9(69%) patients (5/7 SD, 4/6 EEC). Additionally, two (15%) patients had a significant improvement (decreasing Pad test from 1049 to 310 g at 3 months). One patient (7%) had no improvement. Results were stable at 21 months (6-43) of follow-up. Mean final balloon volume was 2.89 ml (± 0.85) with a median of 3 fillings to obtain continence. We had four revisions due to cutaneous port erosion (n = 3) and balloon migration (n = 1) and two definitive explantations. PinQ score was significantly improved (47 vs 40.5 with balloon, p = ns). Neither degradation of the upper urinary tract nor cystomanometric changes have been observed at 6 and 12 months postoperatively. CONCLUSION: Urinary incontinence due to ISD owing to EEC or SD can be successfully treated with ACT™ periurethral balloons. Given the minimal invasiveness of this therapy, it might be a first-line option treatment in children with complex stress urinary incontinence.

Neurostimulation-guided Anal Intrasphincteric Botulinum Toxin Injection in Children With Hirschsprung Disease.

OBJECTIVES: In Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients. METHODS: In this monocenter cohort study, 15 HD patients with postoperative functional intestinal obstructive symptoms received neurostimulation-guided anal intrasphincteric BT injections. Short-, medium-, and long-term effects were evaluated. The Bristol stool form scale was used to assess stool consistency, and the Jorge-Wexner (JW) score to assess fecal continence. RESULTS: The median age at first injection was 4 years. In the short-term, a significant improvement in stool consistency was noted in 12 of 14 patients (P = 0.0001) and JW score decreased for 14 of 15 patients (P = 0.001). In the medium-term, JW score significantly decreased for all patients (P = 0.0001), with an improvement of 50% or more for 10 patients (66.7%). In the long term, 83.3% of patients had normal stool consistency and JW score was <3 for all. Recurrent enterocolitis decreased from 86.7% to 8.3%. A complete resolution of all symptoms without further medication was observed in 66.7% of patients in the long term. CONCLUSIONS: Intrasphincteric BT injection was a safe, effective, and durable option for the management of postoperative functional intestinal obstructive symptoms in HD. The use of neurostimulator guidance for specific delivery of BT to muscular fibers of nonrelaxing anal sphincter complex takes into consideration the variability of patient's anatomy secondary to curative surgery.

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.

What is the outcome of paediatric gastrocystoplasty when the patients reach adulthood?

OBJECTIVE: To document the long-term outcomes of paediatric augmentation gastrocystoplasty (AGC) in terms of preservation of renal function and maintenance of dryness, and to analyse the rate of complications. PATIENTS AND METHODS: The medical records of children who had undergone AGC between 1992 and 2000 (minimum time interval of 15 years) were reviewed retrospectively. The following data were collected: age at surgery, the cause of bladder dysfunction, functioning of the AGC, any complications, and the long-term outcome of the patients. All of the patients were re-contacted by telephone. RESULTS: A total of 11 AGCs were carried out between 1992 and 2000, at a median (range) age of 11 (6.5-14) years. The diagnosis of patients undergoing AGC included myelomeningocele (four), bladder exstrophy (four), posterior urethral valves (one), irradiated bladder (one), and Prune Belly syndrome (one). The median [interquartile range (IQR)] follow-up was 17 (15-19.5) years. Renal function was preserved or improved in seven of the 11 patients and nine patients were dry after AGC. Seven of the 11 patients reported symptoms linked to haematuria-dysuria syndrome, which was resistant to treatment in one case and requiring excision of the gastric patch. Three of the 11 patients developed a tumour on the gastric graft after a median (range) delay of 20 (11-22) years after the initial procedure. All had gastric adenocarcinoma of which two were metastatic at the time of diagnosis requiring pelvectomy with pelvic lymph node dissection and adjuvant chemotherapy. Seven of the 11 patients underwent excision of the gastric patch after a median (IQR) time of 11 (8.5-20.5) years. CONCLUSIONS: Our long-term data confirmed that most patients undergoing AGC had preservation of their renal function and were continent. However, long-term, AGC was associated with a significant risk of malignant transformation and a high rate of surgical re-intervention involving removal of the gastric patch. These results question the use of this technique for bladder augmentation, irrespective of the indication. We highlight the importance of strict endoscopic follow-up of all patients already having undergone an AGC and the need to inform and educated patients about tumour-related symptoms.

Laparoscopic cystojejunostomy for type I cystic biliary atresia in children.

PURPOSE: The use of laparoscopy in the treatment of biliary atresia (BA) is still debated. We report our strategy using laparoscopy in type I cystic BA. MATERIALS AND METHODS: We reviewed the records of patients treated for BA from 2002-2013. When the diagnosis was suspected, an ultrasound was performed. If it showed a cyst > 5 mm in the hilum with no patent gallbladder, we performed an initial explorative laparoscopy. In the case of a patent biliary tree above the cyst, a laparoscopic cystojejunostomy was performed. In cases of absent communication (type III), conversion and portoenterostomy were performed. Pre and postoperative data and overall survival rate with the native liver were reviewed. RESULTS: Forty-four children were treated for BA. Six presented with a cystic form diagnosed by US. Three children had type I BA; three had type III BA. No postoperative complications were noted. Median follow-up was 62.2 months (22.7-93.5). One patient died of a cardiac malformation. The five remaining patients are alive with their native liver. Of the 38 treated for noncystic BA, 16 were transplanted. CONCLUSION: We confirmed the prognosis of cystic BA, which is less severe than noncystic BA. Our strategy using laparoscopy allowed for the confirmation and qualification of the type of BA. In type I, complete treatment by laparoscopy has been performed safely.

Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition.

UNLABELLED: In adults, congenital pulmonary malformations are candidates for surgery due to symptoms. A pre-natal diagnosis is simple and effective, and allows an early thoracoscopic surgical treatment. A retrospective study was performed to assess management in two different populations of adults and children to define the best strategy. SUBJECTS AND METHODS: Pulmonary malformations followed at the University Hospital from 2000 to 2012 were reviewed. Clinical history, malformation site, duration of hospitalisation, complications and pathology examinations were collected. RESULTS: A total of 52 cases (33 children, 19 adults) were identified. In children, 28 asymptomatic cases were diagnosed pre-natally and 5 during the neonatal period due to infections. Surgery was performed on the children between the ages of 2 and 6 months. Nineteen adults underwent surgery, 16 because of symptoms and 3 adults for anomalies mimicking tumours. The mean age within the adult group was 42.5 years. In children, there was one thoracotomy and 32 thoracoscopies, with 7 conversions for difficult exposure, dissection of vascular pedicles, bleeding or bronchial injury. In the adults, there were 15 thoracotomies and 4 thoracoscopies, with one conversion. Post-operative complications in the adults were twice as frequent than in children. The mean time of the children's hospitalisation was 7.75 days versus 7.16 days for the adults. Pathological examinations showed in the children: 7 sequestrations, 18 congenital cystic pulmonary malformations (CPAM), 8 CPAM associated sequestrations; in adults: 16 sequestrations, 3 intra-pulmonary cysts. CONCLUSION: Early thoracoscopic surgery allows pulmonary parenchyma conservation with pulmonary development, reduces respiratory and infectious complications, eliminates a false positive cancer diagnosis later in life and decreases risks of thoracic parietal deformation.

Bronchogenic cyst: Clinical course from antenatal diagnosis to postnatal thoracoscopic resection.

PURPOSE: The purpose of this study was to describe an approach to surgical management of bronchogenic cysts based on the natural course observed from the time of antenatal screening to surgical resection in patients treated at our institution and reported in the literature. MATERIALS AND METHODS: We retrospectively reviewed the clinical features of all children presenting bronchogenic cyst diagnosed antenatally from 2007 to 2010. A total of six children were included. RESULTS: Antenatal diagnosis was accurate in 62.5% of cases. In the first year of life, the size of the cyst remained stable in four patients, doubled in one, and increased 30% within six months in one. The indication for surgery was emphysema of the left bronchus in two patients and rapid growth in two patients. One patient is still awaiting surgery. CONCLUSION: Bronchogenic cysts grow slowly in the first months of life, but growth is exponential even in the absence of complications. We recommend complete resection before the age of two years to prevent infectious complications and facilitate 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.

Acute effect of a dual ETA-ETB receptor antagonist on pulmonary arterial vasculature in preterm lamb fetuses with surgically induced diaphragmatic hernia.

PURPOSE: To study the effects of tezosentan, a dual ETA and ETB receptor antagonist on the cardiopulmonary profile in a fetal lamb model of CDH in utero. METHODS: A diaphragmatic hernia was surgically created at day 75 of gestation. During 45 min of tezosentan perfusion (1 mg/kg), hemodynamic parameters (pulmonary and aortic pressures, left pulmonary and aortic flows, left auricle pressure, heart rate) were measured at day 135 of gestation. Age-matched fetal lambs served as control animals. Secondarily, parietal tension of vessels rings of pulmonary arteries was assessed in organ baths under increasing concentration of tezosentan. RESULTS: In CDH group, under perfusion of tezosentan, pulmonary artery pressure decreased from 45.8 ± 4.1 to 37.6 ± 5.9 mmHg (P < 0.05). Pulmonary artery flow and pulmonary vascular resistance remained constant. In control group, pulmonary artery flow increased from 153.9 ± 15.8 to 233.4 ± 26 ml/min (P < 0.05). Pulmonary artery pressure did not vary. Subsequently calculated pulmonary vascular resistance decreased. In organ bath, no significant relaxation was observed. CONCLUSION: In this fetal lamb model of CDH, tezosentan decreased pulmonary artery pressure but did not modify pulmonary blood flow. Endothelin may play a role in the regulation of pulmonary vascular tone in utero.

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.

Intralobar sequestration associated with cystic adenomatoid malformation: diagnostic and thoracoscopic pitfalls.

BACKGROUND: Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association. METHODS: Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors' institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively. RESULTS: In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case. CONCLUSIONS: Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun.

Congenital lung malformation: evaluation of prenatal and postnatal radiological findings.

BACKGROUND AND OBJECTIVE: This study evaluated the accuracy of prenatal MRI and postnatal CT imaging in the identification of congenital cystic adenomatoid malformation and bronchopulmonary sequestration by comparison with histological analysis. METHODS: Over a 3-year period, 15 patients with lung malformations diagnosed prenatally by ultrasound were referred for prenatal MRI, and all were investigated postnatally by chest CT. All asymptomatic newborns with unresolved lesions underwent elective surgery by thoracoscopy. All surgical specimens were analysed histologically. RESULTS: Among the 15 patients with an abnormality diagnosed by ultrasound, prenatal MRI findings differed from the final histological diagnosis with respect to extent (n = 3), type of lesion (n = 1) and aberrant vessel identification (n = 4). Postnatal chest CT failed to visualize the aberrant vessel in one patient. Complete regression of the lesion was noted in two patients with bronchopulmonary sequestration, and in one patient with congenital cystic adenomatoid malformation and was confirmed by CT. Elective thoracoscopic lobectomy of the affected lobe was performed for 12 patients. Two conversions to thoracotomy were required. All operated patients had an uneventful hospital course. CONCLUSIONS: Prenatal MRI is less accurate than postnatal CT scan, which remains the most reliable diagnostic modality to specify the location and extent and kind of lesions.

Laparoscopic partial splenectomy: indications and results of a multicenter retrospective study.

INTRODUCTION: Partial splenectomy (PS) in children is a surgical option in haematological diseases and focal splenic tumours. The aim of this study was to describe the feasibility and the results of laparoscopic partial splenectomy in children in these two indications by a multicentric retrospective study. METHODS: The authors reviewed the files of all children who underwent laparoscopic PS between March 2002 and September 2006 in two paediatric surgical centers. The data of 11 children were collected and included clinical presentation, age, gender, radiographic examinations, surgical procedure, need for blood transfusion and early complications. RESULTS: From March 2002 to September 2006, laparoscopic PS had been performed on 11 children (6 boys, 5 girls) aged 23 months to 11 years (mean 7, 9). Four children had splenic focal tumours and seven had haematological diseases: six hereditary spherocytosis (HS) and one hemoglobinosis E. During the surgical procedure for haematological diseases 75-80% of the splenic tissue was removed. When PS was performed for focal splenic tumours, the splenic remnant was around 70%. No preoperative complications occurred (no bleeding, no diaphragmatic injury). Neither preoperative nor conversion was necessary. One postoperative complication occurred (left pleural effusion) but required no further treatment. The mean hospital stay was 7.7 days (range from 3 days to 10 days). No infectious postoperative complications occurred; the mean follow up was 21.1 months (range 3-52 months). CONCLUSION: Laparoscopic partial splenectomy is feasible and safe in children with hypersplenism or focal splenic tumours. Partial splenectomy is a good way to prevent postsplenectomy infections by preservation of the immune role of spleen in children with haematological diseases. This technique performed for focal splenic tumours allows the surgeon to choose the size of the splenic remnant.

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.

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.

Is Hypospadias Associated with Prenatal Exposure to Endocrine Disruptors? A French Collaborative Controlled Study of a Cohort of 300 Consecutive Children Without Genetic Defect.

BACKGROUND: Numerous studies have focused on the association between endocrine-disrupting chemicals (EDCs) and hypospadias. Phenotype variability, the absence of representative comparison groups and concomitant genetic testing prevent any definitive conclusions. OBJECTIVE: To identify the role of occupational and environmental exposures to EDCs in nongenetic isolated hypospadias. DESIGN, SETTING, AND PARTICIPANTS: A total of 408 consecutive children with isolated hypospadias and 302 normal boys were prospectively included (2009-2014) in a multi-institutional study in the south of France, the area of the country with the highest prevalence of hypospadias surgery. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: In patients without AR, SRD5A2, and MAMLD1 mutations, parental occupational and professional exposures to EDCs were evaluated based on European questionnaire QLK4-1999-01422 and a validated job-exposure matrix for EDCs. Environmental exposure was estimated using the zip code, the type of surrounding hazards, and distance from these hazards. Multivariate analysis was performed. RESULTS: Fetal exposure to EDCs around the window of genital differentiation was more frequent in the case of hypospadias (40.00% vs 17.55%, odds ratio 3.13, 95% confidence interval 2.11-4.65). The substances were paints/solvents/adhesives (16.0%), detergents (11.0%), pesticides (9.0%), cosmetics (5.6%), and industrial chemicals (4.0%). Jobs with exposure were more frequent in mothers of hypospadiac boys (19.73% vs 10.26%, p=0.0019), especially cleaners, hairdressers, beauticians, and laboratory workers. Paternal job exposure was more frequent in the cases of hypospadias (40.13% vs 27.48%, p=0.02). Industrial areas, incinerators, and waste areas were more frequent within a 3-km radius for mothers of hypospadiac boys (13.29% vs. 6.64%, p<0.00005). Association of occupational and environmental exposures increases this risk. CONCLUSIONS: This multicenter prospective controlled study with a homogeneous cohort of hypospadiac boys without genetic defects strongly suggests that EDCs are a risk factor for hypospadias through occupational and environmental exposure during fetal life. The association of various types of exposures may increase this risk. PATIENT SUMMARY: Our multi-institutional study showed that parental professional, occupational, and environmental exposures to chemical products increase the risk of hypospadias in children.

Repair of a type IV laryngotracheoesophageal cleft with cardiopulmonary bypass.

Laryngotracheoesophageal clefts (LTECs) are rare congenital defects of variable severity depending on the extent of malformation. Management of a complete LTEC represents a major surgical and anesthetic challenge. The main problems are achieving adequate operative exposure and maintaining ventilatory support during and after the operation. We describe correction of a type IV LTEC extending down to the carina in an infant who had respiratory distress at birth. Surgical repair was achieved in a single stage by an anterior sternotomy approach on the 11th day of life. The procedure was facilitated by cardiopulmonary bypass. After the operation, the infant was intubated, mechanically ventilated, and sedated. Nissen fundoplication and gastrostomy were carried out on the 11th postoperative day. The child was extubated on the 12th postoperative day. The rationale for this method and its advantages in comparison with other surgical approaches are discussed.

[Vesicoureteric reflux in children: long-term results of endoscopic treatment by Macroplastique injection]. / Reflux vésico-urétéral chez l'enfant: résultats à long terme du traitement endoscopique par injection de Macroplastique.

OBJECTIVE: To evaluate the long-term efficacy (from the fifth year after injection and up to seven years) of endoscopic treatment by Macroplastique implant for vesicoureteric reflux (VUR) in children, sometimes associated with urological malformations or renal complications. The secondary objective was to evaluate the local tolerance of the Macroplastique implant and the development of any local (haematuria, stones, ureteric stasis) or distant complications. MATERIAL AND METHODS: Retrospective analysis of the medical files of 389 patients, representing a total of 590 refluxing ureters treated by Macroplastique injection in eight different centres. RESULTS: With a follow-up of 5 to 7 years, reflux had not recurred in 309 of the 389 cases, i.e. 79.4% of cases and no case of ureteric stricture was observed. CONCLUSION: The good short-term results in terms of correction of reflux by Macroplastique injection and the very low morbidity are maintained with time. It therefore appears legitimate to propose endoscopic treatment of VUR by first-line Macroplastique injection instead of surgical ureteric reimplantation.

Value of diagnosis imaging in the evaluation of the severity of histological lesions in duplex systems.

OBJECTIVE: In order to determine the effectiveness of imagery in the assessment of the severity of histological lesions in duplex systems in children we compared histology results from heminephrectomies with diagnosis imaging findings [renal ultrasound (US), scintigraphy, unenhanced and contrast-enhanced magnetic resonance imagery (MRI)]. MATERIALS AND METHODS: Between 2007 and 2013, 34 children with duplex system underwent surgery. The results from US (n = 34), dimer captosuccinic acid scintigraphy (n = 23) and MRI (n = 16) were compared with histological data. Five histological lesions were found (chronic interstitial inflammation, interstitial fibrosis, tubular atrophy, glomerulosclerosis and dysplasia) and categorized as severe (>25%) or moderate (≤ 25%). RESULTS: Severe histological lesions were found in 76.5% and moderate lesions in 23.5%. Radiological features were compared with histological results. In US, severe parenchymal thinning was associated with chronic interstitial inflammatory. The absence of parenchymal enhancement and/or severe cortical thinning in MR urography (MRU) was significantly associated with interstitial fibrosis. All poorly functioning poles were associated with severe histological lesions (p = 0.091), but not to a specific category of lesions. CONCLUSIONS: MRI sensibility was excellent (90%) in the diagnosis of poorly functioning pole. Severe thinning on US and minimal pole function on MRU can be used to predict the severity of histological lesions.