Is laterality of congenital diaphragmatic hernia a reliable prognostic factor? French national cohort study.

OBJECTIVES: The objective of this study was to assess whether the laterality of congenital diaphragmatic hernia (CDH) was a prognostic factor for neonatal survival. METHODS: This was a cohort study using the French national database of the Reference Center for Diaphragmatic Hernias. The principal endpoint was survival after hospitalization in intensive care. We made a comparative study between right CDH and left CDH by univariate and multivariate analysis. Terminations and stillbirths were excluded from analyses of neonatal outcomes. RESULTS: A total of 506 CDH were included with 67 (13%) right CDH and 439 left CDH (87%). Rate of survival was 49% for right CDH and 74% for left CDH (P < .01). Multivariate analysis showed two factors significantly associated with mortality: thoracic herniation of liver (OR 2.27; IC 95% [1.07-4.76]; P = .03) and lung-to-head-ratio over under expected (OR 2.99; IC 95% [1.41-6.36]; P < .01). Side of CDH was not significantly associated with mortality (OR 1.87; IC 95% [0.61-5.51], P = .26). CONCLUSION: Rate of right CDH mortality is more important than left CDH. Nevertheless after adjusting for lung-to-head-ratio and thoracic herniation of liver, right CDH does not have a higher risk of mortality than left CDH.

Predictors of the Performance of Early Antireflux Surgery in Esophageal Atresia.

OBJECTIVE: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia. STUDY DESIGN: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia. RESULTS: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001). CONCLUSIONS: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia.

One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register.

OBJECTIVE: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. STUDY DESIGN: We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011. RESULTS: A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30-8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29-80.62] P < .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P < .001), low birth weight (P = .002), and size of the defect (P < .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25-62). Of these survivors, 71 (76%) were followed up for 1 year. CONCLUSIONS: Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.

Thoracoscopy in the management of congenital lung diseases in infancy.

BACKGROUND: This study aimed to compare the results of thoracoscopic surgery for congenital lung diseases between infants younger than 6 months and those older than 6 months at the time of surgery in terms of operation duration, surgical complications, chest tube duration, and hospital stay. METHODS: The charts of 30 thoracoscopic resections for congenital lung diseases were retrospectively reviewed. This study compared 17 children younger than 6 months (mean, 3.94 months; range, 0.37-5.7 months; group 1) with 13 children older than 6 months (mean, 12.05 months; range, 6.2-24.63 months; group 2) at the time of surgery. The median follow-up period was 9 months (range, 1-41 months). RESULTS: Lobectomy was performed in 27 cases, bilobectomy in 1 case, and nonanatomic excision in 2 cases. The mean operating time for group 1 (176±54 min) was similar to that for group 2 (160±46 min). The difference is not significant. The mean duration of chest tube drainage was similar in the two groups (4.4 days; range, 1-9 days for group 1 vs. 4.1 days; range, 3-8 days for group 2). The complications included 1 major and 10 minor complications, with no statistically significant difference between the two groups. Three surgical procedures in each group were converted. The hospital stay was not statistically different between the two groups (8 days; range, 3-20 days for group 1 vs. 6 days; range, 4-10 days for group 2). CONCLUSIONS: The study findings showed no statistically significant difference between the two groups in terms of operation time, complication rate, conversion rate, or hospital stay. Lobectomy can be safely and successfully performed by thoracoscopy even for children younger than 6 months.

Anesthetic management of pulmonary surgery in newborns and infants.

The main congenital pulmonary airways malformations in newborns and infants requiring surgery are cystic adenoid malformation, congenital lobar emphysema and bronchogenic cyst. The surgical treatment preferably via thoracoscopy is recommended within the first year of life to avoid the risk of pneumopathy. A monopulmonary ventilation is then required by the surgeon to operate the diseased lung. The anesthetic management of intraoperative mono-pulmonary ventilation in newborns and infants is always challenging for the anesthesiologist. The main objective of this study was to describe anesthetic protocol for thoracoscopy and variations of monitored parameters during a mono-pulmonary ventilation procedure in newborns and infants.

Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort.

INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. RESULTS: Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). CONCLUSION: About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. LEVELS OF EVIDENCE: Level III retrospective comparative treatment study.

[Renal transplantation in children with lower urinary tract dysfunction]. / Transplantation rénale chez l'enfant porteur d'une anomalie du bas appareil urinaire.

Lower urinary tract dysfunction can lead to renal failure, owing to chronic infection and hypertension resulting from incomplete bladder drainage. These complications can recur after grafting. We compared the outcome of renal transplantation between patients with lower urinary tract dysfunction (group A) and upper urinary tract dysfunction (group B). One hundred twenty-seven kidney transplants were performed in 118 children in our institution between November 1988 and October 2005. Thirty-four patients had urinary tract anomalies (17 in group A, 17 in group B). The disorders in group A included posterior urethral valves (11 cases), neurogenic bladder (4 cases), bladder extrophy (1 case), and the Prune-Belly syndrome (1 case). We reviewed infectious and surgical complications, patient and graft survival, and graft function based on serum creatinine levels at 1, 5 and 10 years. Statistical analysis was based on the Mann-Whitney test. In group A, 5 patients had augmented bladder, 2 had incontinent urinary conduit, and 1 was transplanted on a pre-existing cutaneous ureterostomy. In nine cases, transplantation was performed on the native bladder, with no preparation. Seven complications were noted in group A, consisting of recurrent pyelonephritis (2 cases), renal abscess (1 case), upper urinary tract dilation (3 cases), lithiasis (1 case) and urinary tract incrustation by Corynebacterium in the ureterocutaneous conduit (1 case). Three complications occurred in group B, consisting of acute pyelonephritis (2 cases) and urinary tract infection with prostatitis and epididymitis (1 case). Complications tended to be more frequent in group A, but the difference was not significant (p=0.246). Mean graft survival is 5.29 years in group A and 5.97 years in group B (p=0.76). There was no difference between the two groups as regards the serum creatinine level at 1 year (p=0.77 ; Mann-Whitney test), 5 years (p=0.81) or at the end of follow-up (p=0.75). These results suggest that renal transplantation is similarly feasible in children with upper and lower urinary tract dysfunction. Indeed, we found no significant difference between the groups in terms of patient survival or graft survival and function.

Quality of life and functional outcome of male patients with bladder-prostate rhabdomyosarcoma treated with conservative surgery and brachytherapy during childhood.

PURPOSE: The aim of this study was to report the long-term results of a conservative local treatment of male patients with bladder-prostate rhabdomyosarcoma (BPRMS) focusing on their outcome and quality of life (QoL). METHODS AND MATERIALS: From 1991 to 2007, 27 male patients were treated by a single team, according to the ongoing European protocols. Surgical procedure was partial cystectomy or partial prostatectomy or both, followed by low-dose-rate interstitial brachytherapy. Three patients died of metastases and two were excluded; 22 patients, who were long-term survivors with their bladder, received a QoL questionnaire derived from the International Workshop on BPRMS. Urodynamic studies were performed when patients had abnormal continence. RESULTS: Median age at surgery was 24 months (14 months-11 years). Median followup after surgery was 10 years (5-21 years); 18 male patients (77%) completed the questionnaire at a median age of 13 years (7-25 years); 13 considered themselves as having a normal QoL, with normal urinary continence (9 of 13) or very rare diurnal dribbling (4 of 13). Four male patients had frequent diurnal dribbling requiring protection for three of them and one was submitted to intermittent catheterism for a postoperative neurogenic bladder. Urodynamic studies were performed in 11 patients with urinary disturbance, often revealing detrusor sphincter dyssynergia. All pubertal patients considered themselves as having normal erections. Three sexually active patients reported having satisfying sex and orgasms. Two patients had normal ejaculations. CONCLUSIONS: The majority of long-term male survivors (76%) within this cohort considered themselves as having a normal QoL after the combined conservative local treatment of their BPRMS.

A new surgical approach of temporary ovarian transposition for children undergoing brachytherapy: technical assessment and dose evaluation.

PURPOSE: We developed a new technique of temporary ovarian transposition (OT) for prepubertal girls undergoing brachytherapy. The aim of this study was to describe it, assess its feasibility and safety and calculate the dose delivered to the ovary in order to prove its efficacy. METHODS: Sixteen prepubertal patients underwent temporary OT for brachytherapy at our center from March 2001 to December 2012. OT was done either by laparotomy or by laparoscopy. In all patients, the ovaries were grasped with an atraumatic forceps and mobilized above the iliac crest level as high as possible without any dissection or division of the ovarian ligaments or of the fallopian tube. They were sutured to the anterior abdominal wall by a transfixing stitch of non-dissolvable suture knotted on the outside of the patient on a pledget. RESULTS: Median age at surgery was 3 years (range: 2-9 years). The integrity of the fallopian tube was respected and not a single ligament was dissected or divided. None of the patients had intraoperative or postoperative complications. The stitches were retrieved after completion of irradiation and the ovaries in all the patients fell back into the pelvis. The calculated median radiation dose to the ovary was 1.4 Gy (range: 0.4-2.4 Gy). CONCLUSIONS: This surgical technique is simple and safe, either by laparotomy or by laparoscopy. It meets the radiation and physical constraints in prepubertal girls with vaginal or bladder RMS. However, longer follow-up is required to assess the ovarian function.

Laparoscopic total esophagogastric dissociation.

The total esophagogastric dissociation (Bianchi's procedure) is used to control the severe gastroesophageal reflux in patients after failure of the fundoplication techniques. The laparoscopic approach can be usefully performed in patients with impaired respiratory function. We report here 2 patients in whom the total esophagogastric dissociation has been entirely performed by laparoscopy. The laparoscopic examination of the proximal esojejunal anastomosis is made feasible using an intestinal clamp placed to avoid the esophageal retraction up into the posterior mediastinum. The principal complication after this surgery is the risk of internal hernia.