Hubert Lardy

Esophageal atresia: Data from a national cohort

PURPOSE A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan. METHODS All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report. RESULTS Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted. CONCLUSIONS Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.

Multicentric assessment of the safety of neonatal videosurgery

BackgroundComplex procedures for managing congenital abnormalities are reported to be feasible. However, neonatal videosurgery involves very specific physiologic constraints. This study evaluated the safety and complication rate of videosurgery during the first month of life and sought to determine both the risk factors of perioperative complications and the most recent trends in practice.MethodsFrom 1993 to 2005, 218 neonates (mean age, 16 days; weight, 3,386 g) from seven European university hospitals were enrolled in a retrospective study. The surgical indications for laparoscopy (n = 204) and thoracoscopy (n = 14) were congenital abnormalities or exploratory procedures.ResultsOf the 16 surgical incidents that occurred (7.5%), mainly before 2001, 11 were minor (parietal hematoma, eventration). Three neonates had repeat surgery for incomplete treatment of pyloric stenosis. In two cases, the incidents were more threatening (duodenal wound, diaphragmatic artery injury), but without further consequences. No mortality is reported. The 26 anesthetic incidents (12%) that occurred during insufflation included desaturation (<80% despite 100% oxygen ventilation) (n = 8), transient hypotension requiring vascular expansion (n = 7), hypercapnia (>45 mmHg) (n = 5), hypothermia (<34.9°C) (n = 4), and metabolic acidosis (n = 2). The insufflation had to be stopped in 7% of the cases (transiently in 9 cases, definitively in 6 cases). The significant risk factors for an incident (p < 0.05) were young age of the patient, low body temperature, thoracic insufflation, high pressure and flow of insufflation, and length of surgery.ConclusionDespite advances in miniaturizing of instruments and growth in surgeons’ experience, the morbidity of neonatal videosurgery is not negligible. A profile of the patient at risk for an insufflation-related incident emerged from this study and may help in the selection of neonates who will benefit most from these techniques in conditions of maximal safety.

Congenital diaphragmatic hernia: an evaluation of risk factors for failure of thoracoscopic primary repair in neonates

PURPOSE Publications aiming to prove the feasibility and safety of thoracoscopic CDH-repair in neonates grow in numbers. Some teams use selection criteria, but none have proven statistical evidence. The aim of this study is to detect risk factors for failure of thoracoscopic primary closure of CDH in neonates. METHODS In 8 centers performing minimal access surgery (MAS), complete prenatal, postnatal, and operative data were evaluated for a retrospective study concerning patients with thoracoscopic congenital diaphragmatic hernia (CDH) repair. Most of the selection criteria and risk factors mentioned in the literature were analyzed. Two groups were defined: Group A - neonates who tolerated thoracoscopic primary repair, and Group B - neonates who required conversion or presented with major complications after thoracoscopic repair. Univariate and multivariate logistic regressions were used to compare these two groups. RESULTS From 2006 to 2010, thoracoscopy was performed in 40 neonates: Group A consisting of 28 neonates, and Group B 9 patients. Three patients were excluded because of insufficient data or major associated malformations. Significant statistical differences were found in Group B for postnatal PaCO2 >60 mmHg, need of iNO during postnatal stabilization, intrathoracic position of the stomach, pulmonary hypertension signs on the postnatal cardiac ultrasound, and preoperative OI >3.0. On multivariate analysis, only an OI >3.0 was significantly associated with conversion or major post-operative complication of thoracoscopic primary repair. CONCLUSION CDH can be safely repaired in the neonatal period by thoracoscopy. The limiting factor for thoracoscopic CDH repair is PPHN. The best preoperative indicator for PPHN is OI. Prospective studies are nonetheless necessary to prove the effectiveness of using these risk factors as selection criteria to help design surgical management protocols for neonates presenting CDH.

Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype

BACKGROUND There is a wide difference of opinion between the medical-surgical community and advocacy group regarding Disorders of Sexual Development (DSD) secondary to congenital adrenal hyperplasia (CAH) being ranked in the intersex category. This rupture is even more evident when the issue of genitoplasty is brought up. For physicians it is obvious and unequivocal that a person with CAH and an XX karyotype has a female gender identity, whereas associations tend to rank persons with CAH in the intersex category and advocate holding-off on surgical management. MATERIAL/METHODS A retrospective case study vs. control group, spanning over 40years, included 21 patients who were treated in 3 different centers. Each patient and their parents were contacted independently and interviewed regarding interpersonal relationships, psychological impact of genitoplasty, gender identity and opinion on optimal care management for this disorder. Three couples controls (parent-child) per CAH patients were used and matched according to age, sex assigned at birth and ethnic origin. RESULTS Sex assigned at birth seemed to concord with the gender identity perceived by the patients in 85.7% of cases. In fact, 89.7% of patients and 100% of parents felt that feminizing genitoplasty should be performed within the first year of life. There is however a significant difference compared to controls who felt that surgical management should occur later on in life. No difference was highlighted during childhood regarding parents-child relationships or social integration. However, during adolescence, the parents-child relationship tended to be significantly more painful for the CAH group. Integrating their parenting role was significantly harder for patients in the CAH-DSD group. In the population of CAH-DSD patients who had genitoplasty the level of sexual fulfillment was not lower to the one reported by the control group. CONCLUSION Female sex assignment seems legitimate according to this study and the development of gender identity in these patients matches the sex assigned at birth. Resolving early on the adequacy of the genital anatomy with the sex assigned is promoted by patients as well as their parents. Proper psychomotor development and sexual satisfaction underline the absence of complications related to the surgical technique and the relevance of early surgical management.

How to successfully implement a robotic pediatric surgery program: lessons learned after 96 procedures.

BackgroundBoth our teams were the first to implement pediatric robotic surgery in France. The aim of this study was to define the key points we brought to light so other pediatric teams that want to set up a robotic surgery program will benefit.MethodsWe reviewed the medical records of all children who underwent robotic surgery between Nov 2007 and June 2011 in both departments, including patient data, installation and changes, operative time, hospital stay, intraoperative complications, and postoperative outcome. The department’s internal organization, the organization within the hospital complex, and cost were evaluated.ResultsA total of 96 procedures were evaluated. There were 38 girls and 56 boys with average age at surgery of 7.6 years (range, 0.7–18 years) and average weight of 26 kg (range, 6–77 kg). Thirty-six patients had general surgery, 57 patients urologic surgery, and 1 thoracic surgery. Overall average operative time was 189 min (range, 70–550 min), and average hospital stay was 6.4 days (range, 2–24 days). The procedures of 3 patients were converted. Median follow-up was 18 months (range, 0.5–43 months). Robotic surgical procedure had an extra cost of €1934 compared to conventional open surgery.ConclusionsOur experience was similar to the findings described in the literature for feasibility, security, and patient outcomes; we had an overall operative success rate of 97 %. Three main actors are concerned in the implementation of a robotic pediatric surgery program: surgeons and anesthetists, nurses, and the administration. The surgeon is at the starting point with motivation for minimally invasive surgery without laparoscopic constraints. We found that it was possible to implement a long-lasting robotic surgery program with comparable quality of care.

Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia

OBJECTIVE Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA. STUDY DESIGN We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year. RESULTS Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P < .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P < .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P < .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P < .001). CONCLUSION Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers.

Assessment of paediatric thoracic robotic surgery

OBJECTIVES Many studies have reported that robotic-assisted surgery is safe and feasible for paediatric cases. However, very few paediatric thoracic robotic cases have been described. The aim of this study was to share our preliminary experience with robot-assisted thoracic surgery (RATS). METHODS We reviewed our first, consecutive thoracic robotic procedures between January 2008 and December 2013. Data describing the perioperative and intraoperative periods were prospectively collected in two surgical paediatric centres and then retrospectively analysed. Operation time, completion rate, length of hospitalization and postoperative complications were compared with thoracoscopic results in the literature. RESULTS Eleven patients were operated on with the robot, and this included operations for oesophageal atresia (3), mediastinal cyst (4), diaphragmatic hernia (2), oesophagoplasty (1) and oesophageal myotomy (1). The mean age at surgery was 72 (range 0-204) months, and the mean weight was 24.4 (range 3.0-51.5) kg. Three of the operations were converted to thoracotomies. The total operation time was 190 (120-310) min, and the average length of hospital stay was 13 (3-35) days. RATS offers similar advantages to thoracoscopy for mediastinal cyst excision in patients weighing more than 20 kg. Appropriate patient positioning and trocar placement were necessary for neonatal patients and thereby resulted in longer preparation times. Despite cautious adjustments, technical feasibility was reduced for low-weight patients. CONCLUSIONS These data support mediastinal cyst excision as a suitable indication for larger children. Currently, there is a lack of evidence that lower weight children, and particularly neonates, are good candidates for RATS.

The position of a written document in preoperative information for pediatric surgery: A randomized controlled trial on parental anxiety, knowledge, and satisfaction

INTRODUCTION Preoperative information is a legal and ethical obligation. Very little studies have evaluated the preoperative information method in pediatrics. Having a child operated on is stressful for the parents. Improving information is a way to lower their anxiety. Our study aims to measure the impact of a leaflet, which supports spoken information on parental anxiety, the comprehension-memorization of the information and their satisfaction. MATERIALS & METHODS Prospective study including 178 patients of outpatient surgery, randomized in two groups: spoken information versus spoken information supported by a leaflet, which is then handed out to the parents. The messages were identical: physiopathology, risks without treatment, surgical technique and its possible complications, description of the hospitalization day, and postoperative care. Parental evaluation was made with self-questionnaires after the preoperative consultation, then on the day of surgery. At each moment we evaluated the level of anxiety, satisfaction of information quality and the comprehension-memorization of the data. RESULTS Written information significantly improves the scores of comprehension-memorization, parental satisfaction and significantly decreases the level of anxiety. CONCLUSION Significant impact of the written document as communication support in pediatric surgery, validating the method and encouraging it to be generalized to other pediatric surgery acts. LEVEL OF EVIDENCE Level I. TYPE OF STUDY Prognosis study.

What prenatal ultrasound features are predictable of complex or vanishing gastroschisis? A retrospective study

To evaluate prenatal ultrasound parameters as prognostic factors for complex and vanishing gastroschisis.

Partial nephrectomy for small children: Robot-assisted versus open surgery

To compare the outcomes of robot‐assisted heminephrectomy for duplex kidney in children with those of open heminephrectomy.