Publications by authors named "Yann Révillon"

34 Publications

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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http://dx.doi.org/10.1093/ejcts/ezy049DOI Listing
August 2018

Twenty-eight years of intestinal transplantation in Paris: experience of the oldest European center.

Transpl Int 2017 Feb;30(2):178-186

Pediatric Surgery, Necker-Enfants malades Hospital, Paris, France.

Our aim was to describe our achievements in pediatric intestinal transplantation (ITx) and define areas for improvement. After a period (1987-1990) of nine isolated small bowel transplants (SBTx) where only one patient survived with her graft, 110 ITx were performed on 101 children from 1994 to 2014: 60 SBTx, 45 liver-small bowel, four multivisceral (three with kidneys), and one modified multivisceral. Indications were short bowel syndrome (36), motility disorders (30), congenital enteropathies (34), and others (1). Induction treatment was introduced in 2000. Patient/graft survival with a liver-containing graft or SBTx was, respectively, 60/41% and 46/11% at 18 years. Recently, graft survival at 5/10 years was 44% and 31% for liver-containing graft and 57% and 44% for SBTx. Late graft loss occurred in 13 patients, and 7 of 10 retransplanted patients died. The main causes of death and graft loss were sepsis and rejection. Among the 55 currently living patients, 21 had a liver-containing graft, 19 a SBTx (17 after induction), and 15 were on parenteral nutrition. ITx remains a difficult procedure, and retransplantation even more so. Over the long term, graft loss was due to rejection, over-immunosuppression was not a significant problem. Multicenter studies on immunosuppression and microbiota are urgently needed.
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http://dx.doi.org/10.1111/tri.12894DOI Listing
February 2017

Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts.

World J Gastrointest Surg 2016 Jun;8(6):427-35

Mehdi Ouaissi, Department of General and Digestive Surgery, La Timone Hospital, 13009 Marseille, France.

Aim: To analyze the impact of previous cyst-enterostomy of patients underwent congenital bile duct cysts (BDC) resection.

Methods: A multicenter European retrospective study between 1974 and 2011 were conducted by the French Surgical Association. Only Todani subtypes I and IVb were included. Diagnostic imaging studies and operative and pathology reports underwent central revision. Patients with and without a previous history of cyst-enterostomy (CE) were compared.

Results: Among 243 patients with Todani types I and IVb BDC, 16 had undergone previous CE (6.5%). Patients with a prior history of CE experienced a greater incidence of preoperative cholangitis (75% vs 22.9%, P < 0.0001), had more complicated presentations (75% vs 40.5%, P = 0.007), and were more likely to have synchronous biliary cancer (31.3% vs 6.2%, P = 0.004) than patients without a prior CE. Overall morbidity (75% vs 33.5%; P < 0.0008), severe complications (43.8% vs 11.9%; P = 0.0026) and reoperation rates (37.5% vs 8.8%; P = 0.0032) were also significantly greater in patients with previous CE, and their Mayo Risk Score, during a median follow-up of 37.5 mo (range: 4-372 mo) indicated significantly more patients with fair and poor results (46.1% vs 15.6%; P = 0.0136).

Conclusion: This is the large series to show that previous CE is associated with poorer short- and long-term results after Todani types I and IVb BDC resection.
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http://dx.doi.org/10.4240/wjgs.v8.i6.427DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4919710PMC
June 2016

Congenital bile duct cyst (BDC) is a more indolent disease in children compared to adults, except for Todani type IV-A BDC: results of the European multicenter study of the French Surgical Association.

HPB (Oxford) 2016 06 3;18(6):529-39. Epub 2016 Jun 3.

Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain (UCL), Brussels, Belgium. Electronic address:

Aim: To compare clinical presentation, operative management and short- and long-term outcomes of congenital bile duct cysts (BDC) in adults with children.

Methods: Retrospective multi-institutional Association Francaise de Chirurgie study of Todani types I+IVB and IVA BDC.

Results: During the 37-year period to 2011, 33 centers included 314 patients (98 children; 216 adults). The adult population included more high-risk patients, with more active, more frequent prior treatment (47.7% vs 11.2%; p < 0.0001), more complicated presentation (50.5% vs 35.7%; p = 0.015), more synchronous biliary cancer (11.6% vs 0%; p = 0.0118) and more major surgery (23.6% vs 2%; p < 0.0001), but this latter feature was only true for type I+IVB BDC. Compared to children, the postoperative morbidity (48.1% vs 20.4%; p < 0.0001), the need for repeat procedures and the status at follow-up were worse in adults (27% vs 8.8%; p = 0.0009). However, severe postoperative morbidity and fair or poor status at follow-up were not statistically different for type IVA BDC, irrespective of patients' age. Synchronous cancer, prior HBP surgery and Todani type IVA BDC were independent predictive factors of poor or fair long-term outcome.

Conclusion: BDC is a more indolent disease in children compared to adults, except for Todani type IV-A BDC.
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http://dx.doi.org/10.1016/j.hpb.2016.04.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4913142PMC
June 2016

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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http://dx.doi.org/10.1016/j.jpedsurg.2016.02.043DOI Listing
July 2016

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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http://dx.doi.org/10.1007/s11695-015-1725-4DOI Listing
January 2016

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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http://dx.doi.org/10.1002/ppul.22891DOI Listing
May 2014

Prenatal intestinal obstruction affects the myenteric plexus and causes functional bowel impairment in fetal rat experimental model of intestinal atresia.

PLoS One 2013 8;8(5):e62292. Epub 2013 May 8.

INSERM U989, Paris, France.

Background: Intestinal atresia is a rare congenital disorder with an incidence of 3/10,000 birth. About one-third of patients have severe intestinal dysfunction after surgical repair. We examined whether prenatal gastrointestinal obstruction might effect on the myenteric plexus and account for subsequent functional disorders.

Methodology/principal Findings: We studied a rat model of surgically induced antenatal atresia, comparing intestinal samples from both sides of the obstruction and with healthy rat pups controls. Whole-mount preparations of the myenteric plexus were stained for choline acetyltransferase (ChAT) and nitric oxide synthase (nNOS). Quantitative reverse transcription PCR was used to analyze mRNAs for inflammatory markers. Functional motility and permeability analyses were performed in vitro. Phenotypic studies were also performed in 8 newborns with intestinal atresia. In the experimental model, the proportion of nNOS-immunoreactive neurons was similar in proximal and distal segments (6.7±4.6% vs 5.6±4.2%, p = 0.25), but proximal segments contained a higher proportion of ChAT-immunoreactive neurons (13.2±6.2% vs 7.5±4.3%, p = 0.005). Phenotypic changes were associated with a 100-fold lower concentration-dependent contractile response to carbachol and a 1.6-fold higher EFS-induced contractile response in proximal compared to distal segments. Transcellular (p = 0.002) but not paracellular permeability was increased. Comparison with controls showed that modifications involved not only proximal but also distal segments. Phenotypic studies in human atresia confirmed the changes in ChAT expression.

Conclusion: Experimental atresia in fetal rat induces differential myenteric plexus phenotypical as well as functional changes (motility and permeability) between the two sides of the obstruction. Delineating these changes might help to identify markers predictive of motility dysfunction and to define guidelines for post-surgical care.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0062292PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3648556PMC
December 2013

Intestinal absorption rate in children after small intestinal transplantation.

Am J Clin Nutr 2013 Apr 6;97(4):743-9. Epub 2013 Feb 6.

Department of Pediatric Gastroenterology, Hepatology, and Nutrition, National Reference Center for Rare Digestive Diseases in Children, APHP, Necker-Enfants Malades Hospital, Paris Descartes University, Paris, France.

Background: Small bowel transplantation has now become a recognized treatment of irreversible, permanent, and subtotal intestinal failure.

Objective: The aim of this study was to assess intestinal absorption at the time of weaning from parenteral nutrition in a series of children after intestinal transplantation.

Design: Twenty-four children (age range: 14-115 mo) received intestinal transplantation, together with the liver in 6 children and the colon in 16 children. Parenteral nutrition was slowly tapered while increasing enteral tube feeding. The absorption rate was measured from a 3-d stool balance analysis performed a few days after the child had weaned from parenteral nutrition to exclusive enteral tube feeding. Results were analyzed according to the resting energy expenditure (REE; Schofield formula).

Results: All children were weaned from parenteral nutrition between 31 and 85 d posttransplantation. Median intakes were as follows: energy, 107 kcal · kg(-1) · d(-1) (range: 79-168 kcal · kg(-1) · d(-1)); lipids, 39 kcal · kg(-1) · d(-1) (range: 20-70 kcal · kg(-1) · d(-1)); and nitrogen, 17 kcal · kg(-1) · d(-1) (range: 11-27 kcal · kg(-1) · d(-1)). Median daily stool output was 998 mL/d (range: 220-2025 mL/d). Median absorption rates were 88% (range: 75-96%) for energy, 82% (range: 55-98%) for lipids, and 77% (range: 61-88%) for nitrogen. The ratios for ingested energy to REE and absorbed energy to REE were 2.2 (range: 1.6-3.6) and 1.8 (range: 1.3-3.3), respectively.

Conclusion: These data indicate a suboptimal intestinal graft absorption capacity with fat malabsorption, which necessitates energy intakes of at least twice the REE.
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http://dx.doi.org/10.3945/ajcn.112.050799DOI Listing
April 2013

Successful management of congenital bronchial stenosis using an expandable stent.

J Pediatr Surg 2012 Jan;47(1):e1-4

Department of Pediatric Surgery, CHR Felix Guyon, 97405 St Denis, Reunion Island.

Congenital bronchial stenosis is a very rare cause of neonatal dyspnea. Surgical management remains challenging in small children. We report successful implantation of a bronchial stent in a 3-month-old female infant presenting with congenital right bronchial stenosis and 18 months of follow-up. Use of stents in children remains controversial because of the problem of size mismatch as the child grows. Nevertheless, expandable stent implantation could be an interesting alternative to complex surgery for localized bronchial stenosis in neonates.
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http://dx.doi.org/10.1016/j.jpedsurg.2011.09.066DOI Listing
January 2012

Bacteriologic epidemiology and empirical treatment of pediatric complicated appendicitis.

Diagn Microbiol Infect Dis 2011 Apr;69(4):376-81

Assistance Publique-Hôpitaux de Paris, Laboratoire de Microbiologie, Hôpital Necker-Enfants Malades, Paris 75015, France.

Preoperative samples in the context of complicated appendicitis (CA) are rarely collected, and there is no consensus regarding the optimal antibiotic therapy in children. To help optimize empirical preoperative treatment, we studied clinical and bacteriologic data from a prospective cohort of 93 children with CA in a French hospital. All the bacteria isolated from peritoneal fluids were identified, using phenotypic and/or molecular techniques. The most commonly recovered species were Escherichia coli (71%), Streptococcus group milleri (34%), anaerobes (20%), and Pseudomonas aeruginosa (19%). The association piperacillin-tazobactam is an accurate choice of empirical therapy as it is active against 97% of bacteria. A third-generation cephalosporin with metronidazole in association with an aminoglycoside is a good alternative. Although antibiotic use may be considered as an adjunct to surgical intervention of CA, the appropriate use of preoperative antibiotics is essential and must be constantly reevaluated according to the bacterial epidemiology.
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http://dx.doi.org/10.1016/j.diagmicrobio.2010.11.003DOI Listing
April 2011

Indications and strategies for intestinal transplantation.

J Pediatr Surg 2011 Feb;46(2):280-3

Hôpital Necker-Enfants Malades, Université René Descartes, 75015 Paris, France.

This lecture updates the pediatric surgeon on current indications, techniques, and results of intestinal transplantation in children with intestinal failure with reference to the Paris series.
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http://dx.doi.org/10.1016/j.jpedsurg.2010.11.002DOI Listing
February 2011

Loss-of-function of MYO5B is the main cause of microvillus inclusion disease: 15 novel mutations and a CaCo-2 RNAi cell model.

Hum Mutat 2010 May;31(5):544-51

Université Paris Descartes, Faculté Necker, INSERM U793, Paris, France.

Autosomal recessive microvillus inclusion disease (MVID) is characterized by an intractable diarrhea starting within the first few weeks of life. The hallmarks of MVID are a lack of microvilli on the surface of villous enterocytes, occurrence of intracellular vacuoles lined by microvilli (microvillus inclusions), and the cytoplasmic accumulation of periodic acid-Schiff (PAS)-positive vesicles in enterocytes. Recently, we identified mutations in MYO5B, encoding the unconventional type Vb myosin motor protein, in a first cohort of nine MVID patients. In this study, we identified 15 novel nonsense and missense mutations in MYO5B in 11 unrelated MVID patients. Fluorescence microscopy, Western blotting, and electron microscopy were applied to analyze the effects of MYO5B siRNA knock-down in polarized, brush border possessing CaCo-2 cells. Loss of surface microvilli, increased formation of microvillus inclusions, and subapical enrichment of PAS-positive endomembrane compartments were induced in polarized, filter-grown CaCo-2 cells, following MYO5B knock-down. Our data indicate that MYO5B mutations are a major cause of microvillus inclusion disease and that MYO5B knock-down recapitulates most of the cellular phenotype in vitro, thus independently showing loss of MYO5B function as the cause of microvillus inclusion disease.
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http://dx.doi.org/10.1002/humu.21224DOI Listing
May 2010

Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009.

Orphanet J Rare Dis 2010 Feb 3;5. Epub 2010 Feb 3.

Service d'hématologie oncologie pédiatrique, Centre de référence des histiocytoses, AP-HP Hôpital Armand Trousseau, Paris, France.

Objective: To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children.

Study Design: Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009.

Results: 21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications.

Conclusion: Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.
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http://dx.doi.org/10.1186/1750-1172-5-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2830950PMC
February 2010

Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children.

J Pediatr Surg 2008 Oct;43(10):1833-8

UFR Necker-Enfants Malades, University René Descartes Paris V, FAMA de Transplantation Intestinale, AP-HP and the National Reference Centre for Rare Digestive Diseases, Paris, France.

Background: Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy.

Methods: Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n = 8) after 10 to 144 months of total PN. All grafts included the right colon.

Results: After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children.

Conclusion: These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.
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http://dx.doi.org/10.1016/j.jpedsurg.2008.03.028DOI Listing
October 2008

Lung resection in cystic fibrosis: a survival analysis.

Pediatr Pulmonol 2008 Jan;43(1):72-6

Pediatric Pumonology Unit, University Hospital, Federal University of Minas Gerais, Belo Horizonte, Belo Horizonte, Brazil.

Lung resection may be considered for cystic fibrosis (CF) patients showing localized severe chronic atelectasis and/or bronchiectasis. Nonetheless, literature on survival after surgery is scarce. This study was carried out to assess survival time after partial lung resection. Twenty-one CF patients were operated from 1988 to 2003 and were followed until November 30th, 2004. Survival analysis was performed through Kaplan-Meier method. Mean age at resection was 8.09 years (SD 4.40 years) and two-thirds were females. Z-scores for height, weight, and body mass index as well as FEV1 values showed no statistical significance when comparing values obtained from 2 years before to 2 years after resection. Eleven years after resection, survival probability was 93.8%. Our results suggest that lobectomy or segmentectomy are safe procedures and should be considered in carefully selected patients with unilateral severe symptomatic localized and chronic persistent atelectasis and/or bronchiectasis refractory to conservative management.
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http://dx.doi.org/10.1002/ppul.20742DOI Listing
January 2008

Definitive diagnosis and surgical planning of H-type tracheoesophageal fistula in a critically ill neonate: first experience using air distension of the esophagus during high-resolution computed tomography acquisition.

J Thorac Cardiovasc Surg 2007 Apr;133(4):1116-7

Department of Pediatric Radiology, University Rene Descartes-Paris V, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.

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http://dx.doi.org/10.1016/j.jtcvs.2007.01.003DOI Listing
April 2007

Paraesophageal bronchogenic cyst: first case reports in pediatric.

Pediatr Surg Int 2006 Oct 3;22(10):849-51. Epub 2006 Aug 3.

Department of Pediatric Surgery, Hospital Necker Enfants-Malades, 149 rue de Sèvres, 75015 Paris, France.

We described for the first time a paraesophageal intra-abdominal bronchogenic cyst, first considered as hepatic tumor, in a young boy. Resection was successfully realized by laparoscopy. Abdominal bronchogenic cyst and differential diagnosis are discussed.
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http://dx.doi.org/10.1007/s00383-006-1738-0DOI Listing
October 2006

Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis.

J Pediatr Surg 2006 Jul;41(7):e13-5

Department of Pediatric Surgery, Necker Enfants-Malades Hospital, 75015 Paris, France.

Extramedullary hematopoiesis affects about 15% of the patients treated for thalassemia intermedia. Usually seen in adulthood, the most common location is the paraspinal region. Diagnosis and treatment of extramedullary hematopoiesis located in the pelvis of a young 15-year-old girl is discussed. The young age of the patient and the uncommon site of the mass first lead to the diagnosis of an ovarian dermoid cyst. Because of the clinical history and the typical feature on computed tomography scan, extramedullary hematopoiesis was concluded. A specific treatment based on blood transfusion and hydroxyurea was first proposed but remained inefficient. Surgical excision was thus successfully performed. Whereas surgery is limited to spinal cord compression in paraspinal extramedullary hematopoiesis, this observation argues for surgical treatment in symptomatic intraabdominal extramedullary hematopoiesis when medical treatment fails.
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http://dx.doi.org/10.1016/j.jpedsurg.2006.03.015DOI Listing
July 2006

Grand mal seizures: an unusual and puzzling primary presentation of ruptured hepatic hydatid cyst.

Paediatr Anaesth 2006 Jun;16(6):676-9

Department of Pediatric Anesthesia, CHU Necker Enfants Malades et Faculté René Descartes, Paris, France.

We report a case of hepatic hydatidosis where the first clinical manifestations, generalized seizures after minor head and abdominal trauma, and delayed anaphylaxis, made the primary diagnosis difficult. Severe anaphylaxis has been reported as initial presentation of quiescent hepatic hydatidosis. In endemic areas, the diagnosis must be carefully ruled out in patients experiencing abrupt anaphylactic shock of uncertain etiology. The occurrence of unexplained vascular collapse after minor abdominal trauma in a patient originating from an endemic area should prompt the diagnosis and urgent treatment should be initiated; firstly emergency management of the anaphylactic shock and later, surgical treatment of the cysts.
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http://dx.doi.org/10.1111/j.1460-9592.2005.01825.xDOI Listing
June 2006

Study of the impact of liver transplantation on the outcome of intestinal grafts in children.

Transplantation 2006 Apr;81(7):992-7

Pediatrics Department, Saint-Vincent-de-Paul Hospital, Necker-Enfants Malades Hospital, Paris, France.

Background: Successful small bowel transplantation remains a challenge due to the septic and immune content of the gut. The possible beneficial role of the liver was assessed in pediatric recipients of isolated intestinal and liver intestinal combined transplantation, receiving the same immunosuppressive therapy.

Methods: Fifteen children who underwent small bowel transplantation (seven SbTx) or combined liver-small bowel transplantation (eight LSbTx) at a single center between 1994 and 1998 were retrospectively reviewed and compared with fifteen controls (eight normal and seven appendicitis as inflammatory control). Transplant and patient survival, acute rejection episodes were analyzed and compared. Epithelial apoptotic body counts (ABC) and NF-kB (p65), Caspase-3 and Bax intestinal immunostaining from days 0 to 20 after transplantation were assessed.

Results: Graft and patient survivals at 5 years were respectively 75% and 75% in LSbTx; 43% and 57% in SbTx (NS). Histological analysis showed higher ABC in LSbTx intestinal mucosa (P = 0.05 on day 5, P < 0.01 thereafter). Immunostaining of biopsies on day 0 after reperfusion showed different expression of NF-kB, Caspase-3 and Bax on endothelial (P < 0.05 for NF-kB and Bax), mononuclear (P < 0.05 for Bax) and epithelial cells in LSbTx and SbTx.

Conclusions: Our results suggest a protective role of the liver toward intestinal transplantation even in absence of significative difference, probably due to the small number of children. Early changes in NF-kB immunostaining in the biopsies sampled on day 0, pointed to a possible beneficial effect of the liver in the very early phase following transplantation, perhaps through the differential control of ischemia-reperfusion.
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http://dx.doi.org/10.1097/01.tp.0000195899.32734.83DOI Listing
April 2006

New clinical and therapeutic perspectives in Currarino syndrome (study of 29 cases).

J Pediatr Surg 2006 Jan;41(1):126-31; discussion 126-31

Department of Pediatric Surgery, Hôpital Necker-Enfants Malades, 75015 Paris cedex 15, France.

Purpose: The aim of the study was to clearly define the anomalies that compose the Currarino syndrome (CS). We highlight the frequency of associated malformations of the spinal cord and the possibility of a communication between the presacral tumor and the spinal canal, leading to neurological complications.

Methods: We studied 29 patients with CS, including 12 familial cases; histological examination of the presacral tumor was performed, and cytogenetic and molecular biology studies of the HLXB9 locus were carried out.

Results: All except 2 patients had a sacral malformation; 23 had an anorectal anomaly and 8 had isolated chronic intestinal pseudo-obstruction. There were 20 presacral tumors, one of which was malignant. There was a communication between the presacral tumor and the spinal canal in 12 cases, and tethering of the spinal cord in 17 cases. Twenty-five patients underwent surgery with a single-stage operation for 7, on both the intestinal and the presacral malformations, and, when required, the spinal cord anomalies. Twelve patients harbored a heterozygous point mutation of the coding sequence of HLXB9 gene.

Conclusion: By accurate evaluation of the 4 main features in the CS, the correct surgical management, including neurosurgery, can be performed in a 1-stage approach.
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http://dx.doi.org/10.1016/j.jpedsurg.2005.10.053DOI Listing
January 2006

The transmanubrial approach: a new operative approach to cervicothoracic neuroblastoma in children.

Surgery 2006 Jan;139(1):109-14

Department of Pediatric Surgery, Necker Enfants-Malades Hospital, 149 rue de Sèvres, 75015 Paris, France.

Background: Cervicothoracic neuroblastoma originates from the cervical sympathetic nerves and ganglia and thus presents a problem when dissecting the vascular and nervous elements of the subclavian region. The standard operation is based on thoracotomy or dual cervicotomy/thoracotomy, but these approaches do not provide optimal control of the subclavian vessels. We report our experience in children with cervicothoracic neuroblastoma by using a technique usually performed for apical lung cancer.

Methods: Four patients with localized cervicothoracic neuroblastoma with no N-myc amplification were resected after chemotherapy by this approach. The anatomic evaluation was performed preoperatively with angio-magnetic resonance imaging. This transmanubrial approach, performed through a manubrial L-shaped transection and first costal cartilage resection, affords excellent access to the subclavian region with safe control of the vessels and nerves and exposure of the first 4 thoracic intervertebral foramina.

Results: Removal of more than 90% of the tumor was possible in all cases. The postoperative course was uneventful in 3 cases, and the fourth patient with a left-sided tumor had a transient chylothorax. No recurrence occurred with a follow-up period of 8 to 32 months.

Conclusions: The transmanubrial approach is an osteomuscular-sparing technique that seems particularly suitable for the treatment of these tumors, which require a resection that is as complete as possible to avoid postoperative chemotherapy and tumor relapse.
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http://dx.doi.org/10.1016/j.surg.2005.07.029DOI Listing
January 2006

Inflammatory myofibroblastic tumor in children: clinical review with anaplastic lymphoma kinase, Epstein-Barr virus, and human herpesvirus 8 detection analysis.

J Pediatr Surg 2005 Oct;40(10):1581-6

Department of Pediatric Surgery, Hôpital Necker-Enfants Malades, 75743 Paris Cedex 15, France.

Background/purpose: Inflammatory myofibroblastic tumor (IMT) is considered as an intermediate neoplasm that may present malignant features. Differential diagnosis with other tumor processes is sometimes difficult. Similar anaplastic lymphoma kinase (ALK) gene abnormalities as in anaplastic large cell lymphoma have been reported. Human herpesvirus 8 (HHV-8) DNA sequences have been described in adult pulmonary IMTs and Epstein-Barr virus (EBV) has been reported in splenic and hepatic IMTs, suggesting the importance of both viruses in IMT development. This article aims to evaluate ALK, EBV, and HHV-8 expression in children with IMT and to correlate our findings with clinical features.

Methods: Sixteen children (range, 1-15 years) who had surgery for IMT between 1978 and 2003 were evaluated retrospectively. Formalin-fixed, paraffin-embedded archival tissues were stained for HHV-8 and ALK with immunohistochemistry. Epstein-Barr virus was detected by in situ hybridization (EBER probes).

Results: Tumors were located in the pulmonary lobe (n = 4), urinary tract (n = 4), mesentery or bowel (n = 4), hepatic lobe (n = 1), vena cava (n = 1), spinal cord (n = 1), and soft tissue (n = 1). Five children were treated with steroids and/or antibiotics before surgery, with no substantial result. IMT was excised totally in all but 2 cases. Four patients presented aggressive IMT with recurrence or metastasis requiring new surgery. ALK was positive in 3 (18.8%) cases and EBV in 1 pulmonary and 1 bladder tumor, all of them without recurrence or metastasis. None of the cases were positive for HHV-8. All patients are now disease-free with a mean follow-up of 4.2 years.

Conclusions: Considering the present lack of efficient medical treatment, surgery should still be considered as the mainstay therapy in IMT, even in cases of recurrence or metastases. Larger multicentric studies would be necessary to understand the prognostic significance of ALK, EBV, and HHV-8 and their relationships with the origins of the tumor.
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http://dx.doi.org/10.1016/j.jpedsurg.2005.06.021DOI Listing
October 2005

Enhanced in situ expression of NF-kappaBp65 is an early marker of intestinal graft rejection in rats.

J Pediatr Surg 2005 Sep;40(9):1420-7

INSERM E-0212, Faculté Necker Enfants-Malades, 75730 Paris Cedex 15, France.

Background: Although intestinal transplantation provides a unique situation of free access to the graft because of the presence of temporary enterostomas, evaluation of local immunosuppression is still an unresolved issue and may constitute one of the causes of grafting failure.

Aims: To study in a rat model of allogeneic intestinal transplantation the expression of transcription factors involved in lymphocyte activation in situ in the graft and to identify factors reflecting the efficiency of drug immunosuppression.

Methods: Intestinal transplantation was performed in a Brown Norway (RT1n-donors)-Lewis (RT1(l)-recipients) rat strain combination. The animals were treated with tacrolimus to induce tolerance or left untreated. Syngeneic intestinal grafts and intestine from donor rats with peritonitis were used as controls. NF-kappaBp65, p-c-Jun, interleukin 2 receptor (CD25), and major histocompatibility complex class II antigen (OX-6) expression was studied in graft biopsies on days 2 and 5 by immunohistochemistry.

Results: On day 2, before the onset of histologic signs of rejection, the number of cells expressing NF-kappaBp65 in the pericryptic lamina propria was significantly higher in untreated recipients of allogeneic grafts than in the other groups (P = .009). NF-kappaBp65 expression then fell between days 2 and 5 (P = .009). Classic markers of T-cell activation (CD25 and OX-6) were expressed during rejection in the lamina propria and on crypt enterocytes, respectively. p-c-Jun expression did not differ among the 3 groups.

Conclusion: NF-kappaBp65 expression in intestinal grafts is a precocious sign of local activation during rejection and could thus serve to optimize the management of immunosuppressive therapy.
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http://dx.doi.org/10.1016/j.jpedsurg.2005.05.061DOI Listing
September 2005

[Lynch syndrome pediatric: about a case].

Ann Pathol 2004 Dec;24(6):621-3

Service d'Anatomie et de Cytologie Pathologiques, UPRES EA 219, Université Paris V, France.

A duodenal carcinoma centered on the vater ampulla is reported in a child of 15 years with Lynch syndrome. This case illustrates the problem of early diagnosis and treatment of Lynch syndrome associated tumors.
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http://dx.doi.org/10.1016/s0242-6498(04)94023-0DOI Listing
December 2004

New perspectives for children with microvillous inclusion disease: early small bowel transplantation.

Transplantation 2004 Apr;77(7):1024-8

Combined Program of Liver and Intestinal Transplantation, Hôpital Necker-Enfants Malades, Paris, France.

Background: Microvillous inclusion disease (MVID) is a congenital intestinal epithelial cell disorder leading to lifelong intestinal failure. Despite long-term total parenteral nutrition, life expectancy is extremely reduced because of metabolic or septic complications or liver failure.

Methods: Twelve patients with early-onset MVID were evaluated between 1995 and 2002 for the possibility of small bowel transplantation (SbTx). Three patients died before they could be placed on the waiting list for SbTx, and one patient is still awaiting SbTx. SbTx was contraindicated in one patient.

Results: Seven of 12 patients (six boys and one girl) underwent transplantation (three SbTxs and four combined liver-SbTxs). Actuarial survival rates were 100% and 75% in the SbTx and combined liver-SbTx groups, respectively, with a mean follow-up of 3 years (1.1-8.5 years). In contrast, the survival rate was only 40% in the subgroup of five patients who did not undergo transplantation. After transplantation, all patients were weaned from parenteral nutrition: the five patients with an additional colon graft were weaned within 36 days as opposed to the others without colonic transplant who obtained full intestinal autonomy several months after transplantation. The only two surviving patients who did not undergo SbTx remain highly dependent on total parenteral nutrition, which is complicated by repeated episodes of metabolic decompensation.

Conclusions: SbTx alone or in combination with the liver is highly successful in children with MVID, offering them a long-term perspective for the first time. Associated colon grafting markedly improves the outcome and quality of life after SbTx in patients with MVID.
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http://dx.doi.org/10.1097/01.tp.0000119163.30745.c1DOI Listing
April 2004

Intestinal transplantation.

Indian J Pediatr 2003 Sep;70(9):737-42

Combined Programme of Intestinal Transplantation Hôspital Necker-Enfants Malades, Paris, France.

Advances in immunosuppressive treatment as well as better monitoring and control of acute rejection have brought intestinal transplantation (ITx) into the realm of standard treatment for permanent intestinal failure. The results from the intestinal Transplant International Registry (www.intestinaltransplant.org) indicate that ITx is currently an acceptable clinical modality for selected patients with permanent intestinal failure. The goal of this short review is to deal with indications, clinical results and complications of ITx. Although it has been used in humans for the past two decades, very few data are available regarding graft function and its monitoring.
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http://dx.doi.org/10.1007/BF02724317DOI Listing
September 2003

Management of asymptomatic neonatal cystic adenomatoid malformations.

J Pediatr Surg 2003 Apr;38(4):548-52

Department of Pediatric Surgery, Hospital Necker-Enfants Malades, Paris Cedex, France.

Background/purpose: Although much is known about the prenatal course of cystic adenomatoid malformations (CCAM), the postnatal course of asymptomatic lesion is less well documented. The authors studied the pre- and postnatal course and treatment of asymptomatic CCAM.

Methods: The authors reviewed the files of all patients referred to Necker-Enfants Malades hospital with an antenatal diagnosis of CCAM and asymptomatic at birth.

Results: Files of 29 patients were studied. The first x-ray film was considered normal in 12 cases (41.3%). Computed tomography was normal in 4 cases and showed cystic lung malformations in the other patients. Postnatally, clinical manifestations occurred in 3 patients (10.3%). CCAM vanished in 6 cases. Surgical resection of CCAM was performed in 17 cases (58.6%). All the patients currently are asymptomatic.

Conclusions: CCAM can shrink or vanish during pregnancy and antenatal ultrasound findings are not predictive of the postnatal course. Thus, all infants with prenatal diagnosis of CCAM require postnatal evaluation. Normal radiographic findings at birth do not rule out CCAM persistence on CT. The treatment of asymptomatic CCAM is controversial. Surgery may be advocated because of the low morbidity and the prevention of late complications, above all, cancer. The surgical indications of small (<3 cm) and asymptomatic lesions should be discussed on a case-by-case basis with the parents.
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http://dx.doi.org/10.1053/jpsu.2003.50119DOI Listing
April 2003