Publications by authors named "Naziha Khen-Dunlop"

42 Publications

Long-Term Results of Laparoscopic Adjustable Gastric Banding in French adolescents: The Utmost Importance of Follow-Up.

J Pediatr Gastroenterol Nutr 2021 Jun;72(6):906-911

Hôpital Universitaire Necker - Enfants Malades, Department of Pediatric Gastroenterology-Hepatology-Nutrition.

Objectives: To investigate the long-term follow-up (FU) and effectivity of laparoscopic adjustable gastric banding (LAGB) in a French adolescent cohort.

Methods: We retrospectively analyzed the results of LAGB at our institution. We collected information on FU, adjustable gastric banding (AGB) status, weight-related parameters, and comorbidity at multiple timepoints.

Results: Fifty-six patients (77% female) with a mean age of 16.5 years and a mean body mass index (BMI) of 45 kg/m2 underwent LAGB over a period of 12 years. The mean postpediatric FU was 23 months. FU decreased progressively from 96% at 3 years to 54% and 29% at 6 and 9 years, respectively. The loss to FU was 39% at last contact. AGB was removed in 17 patients (30%) and 12 patients (21%) underwent a second bariatric procedure. Mean BMI decreased by 11 kg/m2 at last contact (P < 0.001). The prevalence of most comorbidities also decreased significantly after 3 years. The mean excess weight loss (to reach a BMI of 25 kg/m2) was 47% during the first year postsurgery and further increased to 55% at last contact.

Conclusion: Overall, AGB resulted in significant weight loss; however, the increase in heterogeneity suggests that LAGB is more effective in some individuals than in others in the long-term. This study confirmed that LAGB is a valuable bariatric procedure in adolescents, either as a long term-term efficient or bridging method that would be replaced at the time of transition to adult care. The importance of a standardized long-term follow-up should always be emphasized.
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June 2021

Thoracoscopic surgery for congenital lung malformations: Does previous infection really matter?

J Pediatr Surg 2021 Jan 27. Epub 2021 Jan 27.

Department of Pediatric Surgery, AP-HP, Necker-Enfants malades Hospital, Paris, France; Université de Paris, Paris, France.

Background/ Purpose: Elective resection of congenital lung malformations (CLMs) is still debatable. The two main risks are malignant transformation and recurrent pulmonary infections. Our study aimed to assess the effect of previous pulmonary infection on the intraoperative and postoperative courses of thoracoscopic surgery for CLMs.

Methods: This is a retrospective study including all thoracoscopic lung resections for CLMs between 2010 and 2019. Ninety patients were included. There was a history of previous pulmonary infection in 28 patients (group A) and no such history in 62 patients (group B).

Results: The median age at operation for group A was 20.4 months (IQR:14.9-41.4) versus 15.1 months (IQR:9.7-20.8) in group B (p = 0.006). There were 10 conversions (35.7%) in group A and 8 (12.9%) in group B (p = 0.02). The operative time was significantly shorter in group B (p<0.002). In group A, 32.1% of patients experienced postoperative fever versus 11.3% of group B (p = 0.03), with higher antibiotics requirement (28.6% versus 6.5% respectively, p = 0.007). However, no significant differences were found in terms of postoperative complications (p = 0.99).

Conclusion: Earlier intervention for CLMs before the development of pulmonary infection carries higher chances for the success of the thoracoscopic approach with shorter operative time and more uneventful postoperative courses.
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January 2021

Thoracoscopic Management of Pediatric Patients with Congenital Lung Malformations: Results of a European Multicenter Survey.

J Laparoendosc Adv Surg Tech A 2021 Mar 11;31(3):355-362. Epub 2021 Jan 11.

Pediatric Surgery Unit, "Federico II" University of Naples, Naples, Italy.

This study aimed to report a European multi-institutional experience about thoracoscopic management of children with congenital lung malformations. The records of 102 patients (49 girls and 53 boys) with median age at surgery of 1 year (range 6 months-1.5 years), who underwent thoracoscopic lobectomy in five European Pediatric Surgery units, were retrospectively collected. Indications for surgery included congenital pulmonary airway malformation (CPAM) ( = 47), intra- and extralobar pulmonary sequestration ( = 34), hybrid lesion (CPAM/intralobar sequestration) ( = 2), severe bronchiectasis ( = 9), congenital lobar emphysema ( = 8), and others ( = 2). The condition was asymptomatic in 77/102 (75.5%), whereas symptoms such as recurrent pneumonia and/or respiratory distress were present in 25/102 (24.5%). Surgical procedures included 18 upper, 20 middle, and 64 lower lobe resections. No conversions to open were reported. A 3 mm sealing device and 5 mm stapler were adopted in the last 48/102 patients (47%). The median operative time was 92.2 minutes (range 74-141). The median operative time significantly decreased in patients in whom the vessel division and bronchial sealing were performed using sealing devices (75.5 minutes) compared with suture ligations (118.9 minutes) ( = .001). The median hospital stay was 3.7 days (range 2-6.2). Three/102 patients (2.9%) developed postoperative complications, including air leakage requiring pleural drainage ( = 1) (Clavien IIIb) and respiratory infection ( = 2) (Clavien II). A reoperation was required in one patient with residual pleuropulmonary blastoma (0.9%). All symptomatic patients reported resolution of symptoms postoperatively. Thoracoscopic lobectomy is a safe and effective procedure with excellent cosmetic outcome, in expert hands. Based upon our experience, we strongly recommend surgery in patients with congenital lung malformations by the first year of life, to reduce the risk of infection and make the procedure technically easier, despite the small patients' size. Surgeon's experience and use of miniaturized instruments and sealing devices remain key factors for successful outcome.
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March 2021

Robotic lobectomy in children with severe bronchiectasis: A worthwhile new technology.

J Pediatr Surg 2020 Nov 12. Epub 2020 Nov 12.

Service de Chirurgie Pédiatrique Viscérale, Hôpital Necker-Enfants malades, Paris, France; Université de Paris, Paris, France. Electronic address:

Background/purpose: Lobectomy is required in children affected by non-responsive, symptomatic, localized bronchiectasis, but inflammation makes thoracoscopy challenging. We present the first published series of robotic-assisted pulmonary lobectomy in children with bronchiectasis.

Methods: Retrospective analysis of all consecutive patients who underwent pulmonary lobectomy for severe localized bronchiectasis (2014-2019) via thoracoscopic versus robotic lobectomy. Four 5 mm ports were used for thoracoscopy; a four-arm approach was used for robotic surgery (Da Vinci Surgical Xi System, Intuitive Surgical, California).

Results: Eighteen children were operated (robotic resection, n = 7; thoracoscopy, n = 11) with infected congenital pulmonary malformation, primary ciliary dyskinesia, and post-viral infection. There were no conversions to open surgery with robotic surgery, but five with thoracoscopy. Total operative time was significantly longer with robotic versus thoracoscopic surgery (mean 247 ± 50 versus 152 ± 57 min, p = 0.008). There were no significant differences in perioperative complications, length of thoracic drainage, or total length of stay (mean 7 ± 2 versus 8 ± 3 days, respectively). No blood transfusions were required. Two thoracoscopic patients had a type-3 postoperative complication.

Conclusions: Pediatric robotic lung lobectomy is feasible and safe, with excellent visualization and bi-manual hand-wrist dissection - useful properties in difficult cases of infectious pathologies. However, instrumentation dimensions limit use in smaller thoraxes.
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November 2020

Thulium LASER for endoscopic closure of tracheoesophageal fistula in esophageal atresia's spectrum: An appropriate tool?

J Pediatr Surg 2020 Oct 19. Epub 2020 Oct 19.

Pediatric Otolaryngology Department, Hôpital Necker-Enfants-Malades, APHP.Centre - Université de Paris, F-75015, Paris, France; Université de Paris, UFR Médecine Paris Centre, F-75006, Paris, France; Université de Paris, Human Immunology, Pathophysiology and Immunotherapy, Division Stem cell Biotechnologies, INSERM UMR976, F-75010, Paris, France.

Purpose: To report our experience with endoscopic Thulium LASER for treatment of recurrent TEF after EA surgery, and for H-Type fistulas.

Methods: A retrospective chart review of consecutive patients undergoing standardized endoscopic closure as first line therapy of recurrent tracheoesophageal fistula (RTEF) and H-type fistula using Thulium LASER, from 2013 to 2019, in a pediatric tertiary care center. Control endoscopic procedure was systematically performed. If persistence of the TEF was noted an external approach was performed. Patient demographics, medical history, symptoms, TEF type, treatment modalities, complications and outcomes were collected.

Results: Eleven patients with tracheoesophageal fistula were included: six RTEF after primary repair of esophageal atresia and five H-type fistulas. The average age at endoscopic treatment was 19months (SD 23months, range 13days-63months). Closure of the fistula after single endoscopic procedure with Thulium LASER was obtained in 3 RTEF (50%) and 1 H-type fistula (20%). Six patients with failure of endoscopic treatment were cured after a single external procedure without any complications. One child, treated for H-type fistula, presented a severe complication of Thulium LASER treatment. Median follow-up after last repair was 24months (range: 14-72months). All fistulas were successfully treated.

Conclusions: In H-Type fistula, success rate of Thulium LASER is only 20% and thus should not be used. In contrast, in RTEF, success rate of 50% is achieved, avoiding as many open procedures, and Thulium LASER could be considered as first line treatment. In any case, open surgery is safe and efficient and can be considered as a first-line treatment for H-type fistulas, and as a salvage treatment for endoscopic treatment failures.

Levels Of Evidence: Level IV.
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October 2020

Thoracoscopy Versus Thoracotomy in the Repair of Esophageal Atresia with Distal Tracheoesophageal Fistula.

J Laparoendosc Adv Surg Tech A 2020 Dec 5;30(12):1289-1294. Epub 2020 Nov 5.

Department of Pediatric Surgery, AP-HP, Hopital Necker-Enfants Malades, Paris, France.

Thoracoscopic repair of esophageal atresia (EA) is gaining popularity, but it is a highly technically demanding procedure. The aim of our study is to evaluate our outcomes in the management of type C EA comparing the thoracoscopic and the open (thoracotomy) approaches. This is a retrospective bicentric study of two major pediatric surgery centers, reviewing all the patients operated for EA with distal tracheoesophageal fistula. Only patients who underwent primary anastomosis were included. From 2008 to 2018, 187 patients were included. Forty-seven patients were operated thoracoscopically (TS group) and 140 by the open approach (TT group). Mean gestational age was 38 ± 2.4 weeks in TS group and 36.4 ± 3.3 weeks in TT group ( = .005) with a mean birth weight of 2785 ± 654 g and 2404.9 ± 651 g in TS and TT groups, respectively ( = .003). The mean operative time was 127.6 ± 35 minutes in TS group and 105.7 ± 23 minutes in TT group ( = .0005). The mean postoperative ventilation time and the mean length of stay were significantly shorter in the thoracoscopic group ( = .004 and  < .0001, respectively). The incidence of anastomotic leak was 8.9% in TS group versus 16.4% in TT group ( = .33). Anastomotic stenosis occurred in 33.3% of TS group and in 22.4% of TT group ( = .17). Surgical outcome of thoracoscopic repair of EA is comparable to the open repair with no higher complication rate with the expected skeletal and cosmetic benefits. However, possible bias regarding prematurity, weight at surgery, and associated anomalies must be taken into consideration.
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December 2020

Assessment of BOLD response in the fetal lung.

Eur Radiol 2021 May 30;31(5):3090-3097. Epub 2020 Oct 30.

EA FETUS and LUMIERE PLATEFORM - Faculte Paris Descartes, Paris, France.

Objective: Assessment of lung development and maturity is of utmost importance in prenatal counseling. Blood oxygen level-dependent (BOLD) effect MRI was developed for functional evaluations of organs. To date, no data are available in fetal lungs and nothing is known about the existence of a BOLD effect in the lungs. The aim of our study was to evaluate if a BOLD response could be detected in fetal lungs.

Materials And Methods: From January 2014 to December 2016, 38 healthy pregnant women were prospectively enrolled. After a routine scan on a 1.5-T MRI device (normoxic period), maternal hyperoxia was induced for 5 min before the BOLD sequence (hyperoxic period). R2* was evaluated by fitting average intensity of the signal, both for normoxic (norm) and hyperoxic (hyper) periods.

Results: A significant BOLD response was observed after maternal hyperoxia in the lungs with a mean R2* decrease of 12.1 ± 2.5% (p < 0.001), in line with the placenta response with a mean R2* decrease of 19.2 ± 5.9% (p < 0.0001), confirming appropriate oxygen uptake. Conversely, no significant BOLD effect was observed for the brain nor the liver with a mean ∆R2* of 3.6 ± 3.1% (p = 0.64) and 2.8 ± 3.7% (p = 0.23).

Conclusion: This study shows for the first time in human that a BOLD response can be observed in the normal fetal lung despite its prenatal "non-functional status." If confirmed in congenital lung and chest malformations, this property could be used in addition to the lung volume for a better prediction of postnatal respiratory status.

Key Points: • Blood oxygen level-dependent (BOLD) effect MRI was developed for functional evaluations of organs and could have interesting implications for the fetal organs. • Assessment of lung development is of utmost importance in prenatal counseling, but to date no data are available in fetal lungs. • BOLD response can be observed in the normal fetal lung opening the way to studies on fetus with pathological lungs.
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May 2021

Ultrasound detected prenatal hyperechoic lung lesions and concordance with postnatal findings: A common aspect for multiple diagnoses.

Prenat Diagn 2021 Feb 5;41(3):323-331. Epub 2020 Nov 5.

Department of Pediatric Surgery, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.

Objective: Hyperechoic lung lesions are largely detected prenatally but their underlying etiology is still poorly defined. The aim of the study was to determine the concordance between pre and postnatal diagnosis of hyperechoic lung lesions.

Methods: Prenatal ultrasound (US) evaluation was performed by a fetal medicine specialist. Postnatal diagnosis was based on CT-scan. Pre- and postnatal features were retrieved from medical charts.

Results: Seventy five patients were included from January 2009 to December 2018. Main prenatal diagnoses were bronchopulmonary sequestrations (BPS) (n = 24%-32%), pulmonary cystic malformations (PCM) (n = 19%-25%), congenital lobar emphysemas (CLE) (n = 15%-20%). Mediastinal shift was observed in 18 cases (24%). The prenatal detection of a systemic arterial supply had a diagnostic accuracy of 90%, while the prenatal detection of a cystic component had a diagnostic accuracy of 76.5%. All 16 neonates with prenatal isolated mediastinal shift were asymptomatic at birth. Seven neonates showed respiratory distress that was not predicted prenatally.

Conclusions: Hyperechoic lung malformations reflect a heterogeneous group of lesions with a good concordance for bronchopulmonary sequestration, but not a satisfying prediction for cystic lesions.
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February 2021

Management of COVID-19-Positive Pediatric Patients Undergoing Minimally Invasive Surgical Procedures: Systematic Review and Recommendations of the Board of European Society of Pediatric Endoscopic Surgeons.

Front Pediatr 2020 7;8:259. Epub 2020 May 7.

Department of Pediatric Surgery, Queen Fabiola Children's Hospital, University of Brussels, Brussels, Belgium.

Hospital response to the COVID-19 outbreak has involved the cancellation of elective, deferrable surgeries throughout Europe in order to ensure capacity for emergent surgery and a selection of elective but non-deferrable surgeries. The purpose of this document is to propose technical strategies to assist the pediatric surgeons to minimize the potential aerosolization of viral particles in COVID-19 patients undergoing urgent or emergent surgical treatment using laparoscopic approaches, based on the currently available literature. The situation and recommendations are subject to change with emerging information. The Scientific Committee and the Board of the European Society of Pediatric Endoscopic Surgeons gathered together in order to address the issue of minimally invasive surgery during this COVID-19 pandemic. A systematic search through PubMed, Embase, and World Wide Web of the terms "COVID-19," "Coronavirus," and "SARS-CoV-2" matched with "pneumoperitoneum," "laparoscopy," "thoracoscopy," "retroperitoneoscopy," and "surgery" was performed. Non-English language papers were excluded. A PRISMA report was performed. Criticalities were identified and a consensus was achieved over a number of key aspects. We identified 121 documents. A total of 11 full-text documents were assessed to address all concerns related to the adoption of minimally invasive surgery. All aspect of pediatric minimally invasive surgery, including elective surgery, urgent surgery, laparoscopy, thoracoscopy, retroperitoneoscopy, and pneumoperitoneum creation and maintainance were extensively addressed through systematic review. A consensus regarding urgent laparoscopic procedures, setting and operation techniques was obtained within the Committee and the Board. The ESPES proposes the following recommendations in case minimally invasive surgery is needed in a COVID-19 positive pediatric patients: (1) consider conservative treatment whenever safely possible, (2) dedicate a theater, columns and reusable laparoscopic instrumentation to COVID-19 pediatric patients, (3) prefer disposable instrumentation and cables, (4) use low CO insufflation pressures, (5) use low power electrocautery, (6) prefer closed-systems CO insufflation and desufflation systems, and (7) avoid leaks through ports. These recommendations are subject to change with emerging information and might be amended in the near future.
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May 2020

Congenital cystic adenomatoid malformations of the lung: an epithelial transcriptomic approach.

Respir Res 2020 Feb 4;21(1):43. Epub 2020 Feb 4.

Service de Pneumologie et d'Allergologie Pédiatriques, AP-HP, Hôpital Universitaire Necker-Enfants Malades, 75743 Cedex 15, Paris, France.

Background: The pathophysiology of congenital cystic adenomatoid malformations (CCAM) of the lung remains poorly understood.

Aim: This study aimed to identify more precisely the molecular mechanisms limited to a compartment of lung tissue, through a transcriptomic analysis of the epithelium of macrocystic forms.

Methods: Tissue fragments displaying CCAM were obtained during planned surgical resections. Epithelial mRNA was obtained from cystic and normal areas after laser capture microdissection (LCM). Transcriptomic analyses were performed and the results were confirmed by RT-PCR and immunohistochemistry in independent samples.

Results: After controlling for RNA quality, we analysed the transcriptomes of six cystic areas and five control areas. In total, 393 transcripts were differentially expressed in the epithelium, between CCAM and control areas. The most highly redundant genes involved in biological functions and signalling pathways differentially expressed between CCAM and control epithelium included TGFB2, TGFBR1, and MAP 2 K1. These genes were considered particularly relevant as they have been implicated in branching morphogenesis. RT-qPCR analysis confirmed in independent samples that TGFBR1 was more strongly expressed in CCAM than in control tissues (p < 0.03). Immunohistochemistry analysis showed TGFBR1 (p = 0.0007) and TGFB2 (p < 0.02) levels to be significantly higher in the epithelium of CCAM than in that of control tissues.

Conclusions: This compartmentalised transcriptomic analysis of the epithelium of macrocystic lung malformations identified a dysregulation of TGFB signalling at the mRNA and protein levels, suggesting a possible role of this pathway in CCAM pathogenesis.

Trial Registration: Identifier: NCT01732185.
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February 2020

Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT).

Laryngoscope 2020 04 15;130(4):E243-E251. Epub 2019 May 15.

Airway Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.

Objective: Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs.

Methods: Patients <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event-free survival were considered.

Results: Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event-free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029).

Conclusion: The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments.

Level Of Evidence: NA Laryngoscope, 130:E243-E251, 2020.
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April 2020

Surgery is not superior to dilation for the management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome: a multicenter comparative observational study in 131 patients.

Am J Obstet Gynecol 2018 09 21;219(3):281.e1-281.e9. Epub 2018 Jul 21.

Department of Pediatric Endocrinology, Gynecology, and Diabetology, Assistance Publique - Hôpitaux de Paris, Hôpital Necker, Paris, France; Université Paris Descartes, Paris, France.

Background: Vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome can be managed either by various surgeries or dilation. The choice still depends on surgeon's preferences rather than on quality comparative studies and validated protocols.

Objective: We sought to compare dilation and surgical management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome, in terms of quality of life, anatomical results, and complications in a large multicenter population.

Study Design: Our multicenter study included 131 patients >18 years, at least 1 year after completing vaginal agenesis management. All had an independent gynecological evaluation including a standardized pelvic exam, and completed the World Health Organization Quality of Life instrument (general quality of life) as well as the Female Sexual Function Index and Female Sexual Distress Scale-Revised (sexual quality of life) scales. Groups were: surgery (N = 84), dilation therapy (N = 26), and intercourse (N = 20). One patient was secondarily excluded because of incomplete surgical data. For statistics, data were compared using analysis of variance, Student, Kruskal-Wallis, Wilcoxon, and Student exact test.

Results: Mean age was 26.5 ± 5.5 years at inclusion. In all groups, World Health Organization Quality of Life scores were not different between patients and the general population except for lower psychosocial health and social relationship scores (which were not different between groups). Global Female Sexual Function Index scores were significantly lower in the surgery and dilation therapy groups (median 26 range [2.8-34.8] and 24.7 [2.6-34.4], respectively) than the intercourse group (30.2 [7.8-34.8], P = .044), which had a higher score only in the satisfaction dimension (P = .004). However, the scores in the other dimensions of Female Sexual Function Index were not different between groups. The Female Sexual Distress Scale-Revised median scores were, respectively, 17 [0-52], 20 [0-47], and 10 [10-40] in the surgery, dilation therapy, and intercourse groups (P = .38), with sexual distress in 71% of patients. Median vaginal depth was shorter in dilatation therapy group (9.6 cm [5.5-12]) compared to surgery group (11 cm [6-15]) and intercourse group (11 cm [6-12.5]) (P = .039), but remained within normal ranges. One bias in the surgery group was the high number of sigmoid vaginoplasties (57/84, 68%), but no differences were observed between surgeries. Only 4 patients achieved vaginas <6.5 cm. Delay between management and first intercourse was 6 months (not significant). Seventy patients (53%) had dyspareunia (not significant), and 17 patients all from the surgery group had an abnormal pelvic exam. In the surgery group, 34 patients (40.5%) had complications, requiring 20 secondary surgeries in 17 patients, and 35 (42%) needed postoperative dilation. In the dilation therapy group, 13 (50%) needed maintenance dilation.

Conclusion: Surgery is not superior to therapeutic or intercourse dilation, bears complications, and should therefore be only a second-line treatment. Psychological counseling is mandatory at diagnosis and during therapeutic management.
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September 2018

Early surgical management for giant omphalocele: Results and prognostic factors.

J Pediatr Surg 2018 Oct 24;53(10):1908-1913. Epub 2018 May 24.

Department of Paediatric Surgery, Hôpital Necker-Enfants Malades, APHP, Paris, France. Electronic address:

Objectives: Giant omphalocele often represents a major surgical challenge and is reported with high mortality and morbidity rates. The aim of this study was to assess the outcome of neonates with giant omphalocele managed with early operative surgical treatment, and subsequently to identify possible factors that could alter the prognosis.

Methods: We reviewed the medical records of 29 consecutive newborns with prenatally diagnosed giant omphalocele. In these cases one of two procedures had been performed: either staged closure after silo, or immediate closure with a synthetic patch. The cases were separated into 2 groups: Isolated giant omphalocele (IO group) and giant omphalocele associated with malformation (NIO group).

Results: Infants in the IO group had a lower size of the omphalocele (p<0,001), a shorter hospital stay (95 days [45-915] vs. 41.5 days [10-110] p= 0, 02), and a shorter median ventilation length (10 days [1-33] vs. 27, 5 [6-65] p = 0, 05). In the NIO group, 5 cases displayed a significantly more difficult course than the others. They were compared to the remaining cases for prenatal and anatomic features. Four factors associated with greater morbidity were identified: CONCLUSIONS: Isolated omphalocele, even containing the whole liver, has a very good prognosis with early surgical treatment. Without associated anomalies, 95% of giant omphaloceles can be discharged with a median of 41.5 days in hospital. However, associated anomalies (especially cardiopathies) may burden the prognosis and should be both carefully assessed during pregnancy and taken into account in parental information.

Type Of Study: Retrospective Study LEVEL OF EVIDENCE: Level I.
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October 2018

Hemodynamic Shock Caused by Tension Pneumoperitoneum in a 5-Year-Old Girl.

Pediatr Emerg Care 2018 Jun;34(6):e102-e103

From the Departments of Pediatric Emergency.

Tension pneumoperitoneum is a relatively rare occurrence in the pediatric population. However, tension pneumoperitoneum is associated with significant morbidity and mortality if it is not promptly diagnosed and treated. A 5-year-old girl was admitted to emergency department with abdominal pain. She was in shock, and the radiograph film of the abdomen revealed a voluminous tension pneumoperitoneum. Aggressive fluid challenges were performed in intensive care followed by urgent laparotomy. Primary abdominal compartment due to trapped gas caused a decreased venous return and visceral perfusion. In the absence of hemodynamic improvement after vascular filling, needle decompression was performed before surgery.
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June 2018

Bronchopulmonary sequestrations in a paediatric centre: ongoing practices and debated management.

Eur J Cardiothorac Surg 2018 08;54(2):246-251

Deparmtent of Pediatric Surgery, AP-HP, Hopital Necker-Enfants malades, Paris, France.

Objectives: Bronchopulmonary sequestration (BPS) is the second most common congenital lung malformation, with an estimated incidence ranging from 0.15% to 1.8%. Surgical treatment is elective in patients with symptoms, but the management of asymptomatic patients remains controversial.

Methods: We retrospectively reviewed the medical records of 99 patients treated for BPS in our institution from January 2000 to December 2015. BPS was diagnosed prenatally in 86 (87%) cases. Management throughout this 16-year period was based on 3 interventions: resection by open surgery, resection by thoracoscopy and embolization.

Results: Among the 86 patients with a prenatal diagnosis of BPS, 14% had symptoms at birth and 10% had delayed symptoms at a median delay of 8 months (4.5-42 months). For the other 13 patients, symptoms occurred at a median age of 34 months (range 3-96 months). Embolization of the feeding vessel was performed in 46 patients with 6 secondary surgical resections (13%). A total of 59 patients were operated on: 23 cases by open surgery and 36 cases by thoracoscopy. The mean hospitalization stay was significantly longer for open surgery: 4.8 ± 1.3 days vs 4.1 ±1.5 days, respectively (P = 0.03). Differences in hospitalization stay were also found between asymptomatic and symptomatic patients: 3.5 ± 1.2 vs 5.1 ±1.6 days, respectively (P = 0.002). Two of the operated patients died.

Conclusions: When surgery is chosen, thoracoscopy appears to be a valuable procedure. A better understanding of the natural history of BPS is still needed to define the optimal management and the respective roles of surgery, embolization or non-interventional follow-up.
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August 2018

Diaphragmatic function in infants and children with congenital diaphragmatic hernia: a cross-sectional study.

Eur J Cardiothorac Surg 2018 04;53(4):740-747

Pediatric Noninvasive Ventilation and Sleep Unit, AP-HP, Hôpital Necker-Enfants Malades, Paris, France.

Objectives: Few studies have evaluated long-term diaphragmatic function in congenital diaphragmatic hernia (CDH). The aim of our cross-sectional study was to assess diaphragmatic function in infants and young children with CDH after surgical repair.

Methods: All the patients with CDH repair followed in our centre between February 2014 and January 2016 were enrolled. Patients with a postnatal diagnosis after 1 month of life were excluded. Breathing pattern and diaphragmatic function were assessed using esophageal and gastric (Pgas) pressure recording after surgery, or at 1 or 5 years of age.

Results: Twenty-eight patients (24 left-sided CDH, 6 with diaphragmatic patch) were included. Twelve patients were assessed before hospital discharge (Y0), 6 around the age of 1 year (Y1) and 10 around the age of 5 years (Y5). Mean antenatal estimated pulmonary volume (VLA) was 42 ± 10% (n = 23). Diaphragmatic strength, assessed by transdiaphragmatic pressure during crying/sniff, was low at Y0 (47 ± 18 cmH2O, n = 12) and within normality at Y5 (81 ± 15 cmH2O, n = 7). Diaphragmatic dysfunction, assessed by Pgas during crying/sniff, was present at Y0 (-58 ± 22 cmH2O, n = 12) and Y1 (-53 ± 36 cmH2O, n = 5) and still present at Y5 (3 ± 9 cmH2O, n = 7) but to a lesser extent. The diaphragmatic tension time index (TTdi), which estimates diaphragmatic endurance, was high at Y0 (0.10 ± 0.04, n = 11) and within normality at Y5 (0.03 ± 0.01, n = 6). VLA correlated with neonatal TTdi (r = -0.961, P < 0.001).

Conclusions: Infants with CDH have diaphragmatic dysfunction in the neonatal period, which correlates with VLa and normalizes with age. Future longitudinal studies should assess the role of CDH side, size of diaphragmatic defect and patch repair.
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April 2018

Segmental volvulus in the neonate: A particular clinical entity.

J Pediatr Surg 2017 Mar 13;52(3):454-457. Epub 2016 Oct 13.

AP-HP, Hôpital Necker-Enfants malades, Service de Chirurgie Pédiatrique Viscérale, Paris, France; Université Paris Descartes, Paris, France.

Background: Complete intestinal volvulus is mainly related to congenital anomalies of the so-called intestinal malrotation, whereas segmental volvulus appears as a distinct entity, mostly observed during the perinatal period. Because these two situations are still lumped together, the aim of this study was to describe the particular condition of neonatal segmental volvulus.

Study Design: We analyzed the circumstances of diagnosis and management of 17 consecutives neonates operated for segmental volvulus more than a 10-year period in a single institution. During the same period, 19 cases of neonatal complete midgut volvulus were operated.

Results: Prenatal US exam anomalies were observed in 16/17 (94%) of segmental volvulus, significantly more frequently than in complete volvulus (p=0.003). Intestinal malposition was described peroperatively in all cases of complete volvulus, but also in 4/17 segmental volvulus (23%). Intestinal resection was performed in 88% of segmental volvulus when only one extensive intestinal necrosis was observed in complete volvulus. Parenteral nutrition was required in all patients with segmental volvulus with a median duration of 50days (range 5-251).

Conclusion: Segmental volvulus occurs mainly prenatally and leads to fetal ultrasound anomalies. This situation, despite a limited length of intestinal loss, is associated to significant postnatal morbidity.

Type Of The Study: Treatment study.

Level Of Evidence: Level IV.
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March 2017

Respiratory Morbidity in Infants Born With a Congenital Lung Malformation.

Pediatrics 2017 Mar 15;139(3). Epub 2017 Feb 15.

Pneumologie Pédiatrique,

Background And Objectives: The actual frequency of respiratory symptoms related to congenital pulmonary malformations (CPMs) remains undetermined. The goal of this study was to prospectively evaluate the respiratory symptoms occurring in infants with prenatally diagnosed CPMs, identify factors associated with the occurrence of these symptoms, and evaluate their resolution after surgery.

Methods: Infectious and noninfectious respiratory symptoms were prospectively collected in a French multicenter cohort of children with CPMs.

Results: Eighty-five children were followed up to the mean age of 2.1 ± 0.4 years. Six children (7%) underwent surgery during the first 28 days of life. Of the 79 remaining children, 33 (42%) had respiratory symptoms during infancy before any surgery. Wheezing was the dominant symptom (24 of 79 [30%]), and only 1 infant had documented infection of the cystic lobe. Symptoms were more frequent in children with noncystic CPMs, prenatally ( = .01) or postnatally ( < .03), and with postnatally hyperlucent CPMs ( < .01). Sixty-six children underwent surgery during the follow-up period, and 40% of them displayed symptoms after the intervention. Six children had documented pneumonia during the postoperative period. At the end of the follow-up, pectus excavatum was observed in 10 children, significantly associated with thoracotomy ( < .02) or with surgery before the age of 6 months ( < .002).

Conclusions: CPMs are frequently associated with wheezing episodes. Surgery had no significant impact on these symptoms but was associated with a paradoxical increase in pulmonary infections, as well as an increased risk of pectus excavatum after thoracotomy.
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March 2017

Risk of Readmission for Wheezing during Infancy in Children with Congenital Diaphragmatic Hernia.

PLoS One 2016 12;11(5):e0155556. Epub 2016 May 12.

Pneumologie Pédiatrique, Necker, AP-HP, Paris, France, Centre de Référence des Maladies Respiratoires Rares, Paris, France.

Rationale: Congenital diaphragmatic hernia (CDH) is associated with a high incidence of respiratory problems, even after initial hospital discharge. These problems are likely to lead to re-hospitalization during infancy, although actual frequency of readmissions is unknown.

Objective: We aimed to determine the rate of hospitalization for wheezing in infants with CDH between the time of initial discharge and 24 months of age, and to identify factors associated with readmission.

Methods: Data about infants with CDH born in three French reference tertiary centers between January 2009 and March 2013 who were alive at hospital discharge, were extracted from a prospective national database.

Results: Ninety-two children were identified, and 86 were included in the analysis. In total, 116 wheezing episodes requiring a doctor's visit occurred in 50 infants (58%) before 24 months of age. Twenty-two children (26%) were readmitted at least once for wheezing exacerbations. RSV was present in 6 of 15 (40%) of children with available nasal samples at first readmission, and 1 of 5 (20%) at second readmission. Thoracic herniation of the liver, low gestational age, longer initial hospitalization, need for oxygen therapy at home, and eczema were all significantly associated with readmission for wheezing exacerbations. Fifty-three infants (62%) received palivizumab prophylaxis, but there was no association with the overall rate of readmission for wheezing exacerbations or RSV-related hospitalization.

Conclusions: The rate of readmission for wheezing among infants with CDH is high, and significantly influenced by several prenatal and neonatal factors. Palivizumab prophylaxis was not associated with the rate of readmission.
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July 2017

Comparative results of gastric banding in adolescents and young adults.

J Pediatr Surg 2016 Jul 28;51(7):1122-5. Epub 2016 Feb 28.

AP-HP, Hôpital Necker-Enfants malades, Service de Chirurgie Pédiatrique Viscérale, Paris, France; Université Paris Descartes, Paris, France. Electronic address:

Background/purpose: Obesity has become a major public health priority. Because of disappointing results obtained with dietary and medical programs, bariatric surgery has been offered to adolescents, although this practice remains controversial. Our aim was to evaluate laparoscopic adjustable gastric banding (LAGB) in adolescents at 2-year follow-up.

Material And Methods: This prospective study, from 2008 to 2013, compared results between adolescent patients and young adult controls. The LAGB technique and the follow-up program were similar. Weight loss and comorbid disease were analyzed.

Results: Thirty-six adolescents (mean age at surgery=16.7±1.3years) were operated on and compared to 53 young adults (mean age at surgery=21.7±1.9years). The mean weight and BMI at surgery were 124.4±20.7 and 43.9±5.5kg/m(2), respectively. Among the adolescents, none were diabetic or hypertensive. The mean glycated hemoglobin was 5.6±1.2%. In four cases (11%) dyslipidemia was observed. There was no significant difference between the two groups in terms of initial preoperative weight or BMI. The absolute BMI values at 6, 12 and 24months after surgery were comparable between adolescents and young adults: 38.7 vs 39.8, 36.0 vs 37.6 and 33.5 vs 36.1kg/m(2), respectively. The excess weight loss was higher in adolescents at 12 and 24months: 48.6 vs 37.6% (p=0.03); and 62.3 vs 45.5% (p=0.02). During this period, insulin resistance and dyslipidemia decreased similarly in both groups.

Conclusion: Provided there is careful selection of patients and a supportive multidisciplinary team, satisfying results can be obtained after LAGB in adolescents, comparable to those obtained in young adults at 2-year follow-up.
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July 2016

Congenital diaphragmatic hernia: does gestational age at diagnosis matter when evaluating morbidity and mortality?

Am J Obstet Gynecol 2015 Oct 10;213(4):535.e1-7. Epub 2015 Jun 10.

Université Paris-Sud, Le Kremlin-Bicêtre, France; Centre de Référence des Maladies Rares, Hernie de Coupole Diaphragmatique, France; Service de Gynécologie-Obstétrique, Assistance Publique-Hôpitaux de Paris, Hôpital Antoine Béclère, Clamart, France.

Objective: The objective of the investigation was to study the relationship between gestational age at diagnosis and mortality and morbidity in fetuses with an isolated congenital diaphragmatic hernia.

Study Design: Between January 2008 and November 2013, 377 live births with isolated congenital diaphragmatic hernia diagnosed antenatally at a known gestational age were recorded in the database of the French National Center for Rare Diseases. The primary outcome studied was mortality estimated at 28 days and at 6 months. The secondary outcome was morbidity evaluated by pulmonary arterial hypertension at 48 hours, oxygen therapy dependence at 28 days, oral disorders, enteral feeding, and prosthetic patch repair. Analyses were adjusted for the main factors of congenital diaphragmatic hernia severity (side of the hernia, thoracic herniation of the liver, gestational age at birth, lung-to-head ratio, and prenatal treatment by tracheal occlusion.

Results: Mortality rates at 28 days decreased significantly (P < .001) when gestational age at diagnosis increased: 61.1%, 39.2%, and 10.4% for a diagnosis in the first, second, and third trimester, respectively. Adjusted odds ratios were 3.12 [95% confidence interval, 1.86-5.25] and 0.35 [95% confidence interval, 0.18-0.66] for a diagnosis in the first and third trimesters, respectively, compared with a diagnosis in the second trimester. Similarly, morbidity decreased significantly when gestational age at diagnosis increased, and the trend remained significant after adjustment for the main factors of congenital diaphragmatic hernia severity (P < .001).

Conclusion: Gestational age at diagnosis is an independent predictor of postnatal prognosis for children presenting an isolated congenital diaphragmatic hernia and should be taken into account when estimating postnatal morbidity and mortality.
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October 2015

Primordial Influence of Post-operative Compliance on Weight Loss After Adolescent Laparoscopic Adjustable Gastric Banding.

Obes Surg 2016 Jan;26(1):98-104

AP-HP, Hôpital Necker-Enfants Malades, Service de Chirurgie Pédiatrique Viscérale, 149 rue de Sevres, 75015, Paris, France.

Background: Accumulating evidence suggests that the benefits seen in adult bariatric surgery can be reproduced in adolescents. In contrast with North America, bariatric surgery in adolescents is still not well accepted in Europe and indications and protocols have still to be formulated.

Methods: This prospective study tested the gastric banding procedure in 49 patients operated in a single French institution since 2008. The mean age at surgery was 16.2 ± 0.9 years with a weight of 118.8 ± 22.3 kg and body mass index of 42.5 ± 5.9 kg/m(2).

Results: At 6, 12 and 24 months after surgery, weight was 103.7 ± 20.8 kg, 98.7 ± 21 kg and 93.6 ± 19.3 kg, respectively (p < 0.001), corresponding to excess weight loss (EWL) of 31.6 ± 17.2 %, 41.8 ± 21.4 % and 59.1 ± 24.9 % (p < 0.001), respectively. Multivariate analysis showed that the number of consultations per year was the only variable significantly associated to weight loss. Metabolic disorders were corrected, with a decreased prevalence of insulin resistance from 100 to 17 % and normalisation of homeostasis model assessment-insulin resistance (HOMA-IR) at 24 months (2.09 ± 0.95). Band-related complications were five slippages, one psychological intolerance and two ports repositioning. Six patients (12 %) had the device explanted. The death of a patient was an exceptionally severe adverse event.

Conclusion: Given frequent follow-up support by a multidisciplinary team, laparoscopic adjustable gastric banding (LAGB) surgery in adolescent results in sustained weight loss. However, even exceptional, potentially serious complications are possible and long-term follow-up is needed to evaluate the risk/benefit ratio at 5 or 10 years after LAGB surgery.
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January 2016

Results from the French National Esophageal Atresia register: one-year outcome.

Orphanet J Rare Dis 2014 Dec 11;9:206. Epub 2014 Dec 11.

Reference Center for Congenital Esophageal Anomalies, University Hospital Lille, Avenue Eugène Avinée, Lille, 59037, France.

Background: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA).

Methods: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database.

Results: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007).

Conclusions: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.
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December 2014

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

Am J Obstet Gynecol 2015 Mar 28;212(3):340.e1-7. Epub 2014 Sep 28.

Department of Obstetrics, Jeanne de Flandre Hospital, CHRU Lille, France.

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.
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March 2015

Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations.

Pediatrics 2014 May;133(5):e1285-91

Pneumologie Pédiatrique, Necker, AP-HP, France; Centre de Référence des Maladies Respiratoires Rares, Paris, France; Université Paris-Descartes, Paris, France;

Background And Objective: Congenital pulmonary malformations (CPM) are mostly recognized on prenatal ultrasound scans. In a minority of cases, they may impair breathing at birth. The factors predictive of neonatal respiratory distress are not well defined, but an understanding of these factors is essential for decisions concerning the need for the delivery to take place in a tertiary care center. The aim of this study was to identify potential predictors of respiratory distress in neonates with CPM.

Methods: We selected cases of prenatal diagnosis of hyperechoic and/or cystic lung lesions from RespiRare, the French prospective multicenter registry for liveborn children with rare respiratory diseases (2008-2013). Prenatal parameters were correlated with neonatal respiratory outcome.

Results: Data were analyzed for 89 children, 22 (25%) of whom had abnormal breathing at birth. Severe respiratory distress, requiring oxygen supplementation or ventilatory support, was observed in 12 neonates (13%). Respiratory distress at birth was significantly associated with the following prenatal parameters: mediastinal shift (P = .0003), polyhydramnios (P = .05), ascites (P = .0005), maximum prenatal malformation area (P = .001), and maximum congenital pulmonary malformation volume ratio (CVR) (P = .001). Severe respiratory distress, requiring oxygen at birth, was best predicted by polyhydramnios, ascites, or a CVR >0.84.

Conclusions: CVR >0.84, polyhydramnios, and ascites increased the risk of respiratory complications at birth in fetuses with CPM, and especially of severe respiratory distress, requiring oxygen supplementation or more intensive intervention. In such situations, the delivery should take place in a tertiary care center.
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May 2014

Characteristics and management of congenital esophageal stenosis: findings from a multicenter study.

Orphanet J Rare Dis 2013 Dec 1;8:186. Epub 2013 Dec 1.

Reference Centre for Congenital and Malformative Esophageal Diseases, Lille, France.

Background: Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.

Methods: Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.

Results: Over 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27).

Conclusions: CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.
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December 2013

Extralobar pulmonary sequestration with combined gastric and intradiaphragmatic locations.

Pediatr Pulmonol 2014 May 10;49(5):512-4. Epub 2013 Sep 10.

AP-HP, Pediatric Surgery Department, Necker-Enfants Malades Hospital, Paris, France.

Extralobar pulmonary sequestration is a congenital lung malformation characterized by a non-functional lung segment with systemic feeding vessel. Over 90% of sequestrations are found in the thorax with less than 10% located in the abdomen. We present an unusual case of intra abdominal pulmonary sequestration, located suprarenally, adherent to both the stomach and the diaphragm. The malformation was surgically excised via laparoscopy in the second year of life, when no evidence of regression was found on follow up imaging. On the occasion of this description, the spectrum of bronchopulmonary foregut malformation is discussed.
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May 2014

FGF10 Signaling differences between type I pleuropulmonary blastoma and congenital cystic adenomatoid malformation.

Orphanet J Rare Dis 2013 Sep 3;8:130. Epub 2013 Sep 3.

AP-HP, Hôpital Necker-Enfants Malades, service de Pneumologie Pédiatrique, Centre de Référence pour les Maladies Respiratoires Rares de l'Enfant, Paris, France.

Background: Type I pleuropulmonary blastoma (PPB) and congenital cystic adenomatoid malformation of the lung (CCAM) are cystic lung diseases of childhood. Their clinical and radiological presentations are often similar, and pathologic discrimination remains difficult in many cases. As a consequence, type I PPB and CCAM are frequently confused, leading to delayed adequate management for type I PPB. Recent studies have suggested a role for fibroblast growth factor (FGF) 10 signal pathway in CCAM pathogenesis. The objective of our study was to determine whether FGF10 signaling differs between CCAM and type I PPB.

Methods: Immunohistochemical studies were performed for expression of FGF10, its receptor FGFR2b, and its inhibitor sonic hedgehog (SHH) in focal type I PPB (n=6), CCAM type I (n=7), CCAM type II (n=7), and control lungs (n=5).

Results: FGF10, FGFR2b, and SHH expressions differed markedly between type I PPB and both types of CCAM. Type I and type II CCAM cystic walls expressed FGF10, FGFR2b, and SHH, whereas staining was absent or poor in type I PBB cystic walls. Expression of FGF10, FGFR2b, and SHH did not differ between CCAM cystic walls and control airway walls.

Conclusions: These findings show that immunohistochemistry with FGF10, FGFR2b, or SHH could be useful in differentiating CCAM from type I PPB, when a child presents with a focal cystic lung lesion. The absence of strong expression of FGF10, FGFR2b, and/or SHH makes the diagnosis of CCAM very doubtful.
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September 2013

Specific biochemical amniotic fluid pattern of fetal isolated esophageal atresia.

Pediatr Res 2013 Nov 11;74(5):601-5. Epub 2013 Sep 11.

Department of Pediatric Surgery, Robert Debré Hospital, AP-HP, University of Paris VII, Paris, France.

Background: Perinatal care of esophageal atresia (EA) may be improved by prenatal diagnosis. Ultrasound findings (polyhydramnios and/or nonvisualization of fetal stomach) lead to a detection rate of ~50%. An amniotic fluid (AF) biochemical pattern characterized by high total protein, γ-glutamyl transpeptidase (GGTP), and normal l-leucine-aminopeptidase (AMP) leads to a 100% detection rate. The aim of this study was to explain this specific pattern.

Methods: On the basis of enzyme activities assay, the following four objectives were sought: (i) comparing AF markers between EA and other digestive tract atresias, (ii) determining local GGTP synthesis in the esophagus (immunohistobiochemistry), (iii) determining the presence of a specific AF-AMP activity inhibitor, and (iv) comparing AF-AMP and AF-GGTP half-lives.

Results: The AF-EA pattern was similar to that observed in upper duodenal atresia (above the Oddi sphincter). No local synthesis of GGTP was observed in the esophagus. No AF-AMP activity inhibitor was found. AF-GGTP had a longer half-life than AF-AMP.

Conclusion: Due to the swallowing anomaly observed in EA, GGTP and AMP values physiologically observed at 18 wk will decrease on the basis of the half-lives of markers, with a flat slope for GGTP and a sharp slope for AMP, therefore explaining the differences observed in the AF-EA pattern.
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November 2013

Esophageal atresia: data from a national cohort.

J Pediatr Surg 2013 Aug;48(8):1664-9

Reference Center for Congenital Oesophageal Anomalies, University Hospital Lille, France.

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.
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August 2013