Virginie Fouquet

Esophageal atresia: Data from a national cohort

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

Pancreatic resections for solid or cystic pancreatic masses in children.

Objectives: The aim of the study was to assess the diagnosis and management of solid pancreatic neoplasm in children and the type of surgical treatment, focusing on short- and long-term outcomes. Methods: We retrospectively reviewed the charts of all children who had undergone pancreatic resection for suspicion of pancreatic tumor in Kremlin Bicêtre Hospital, Paris, between 1986 and 2008. We studied the symptoms at diagnosis, the type of surgery, and the short- and long-term morbidity and mortality. Results: Of 18 patients identified, there were 7 pseudopapillary tumors, 3 neuroblastomas, 2 rhabdomyosarcomas, 1 acinar cell carcinoma, 1 endocrine cell carcinoma, 1 renal angiomyolipoma, and 3 pancreatic cysts. Symptoms at diagnosis were abdominal trauma, abdominal mass, and jaundice. Operative procedures were duodenopancreatectomy (11), mid-pancreatic resections (2), splenopancreatectomy (2), distal pancreatectomy (1), and tumorectomy (2). There were no deaths related to surgery. The postoperative morbidity rate was 45%, including 2 cases of fistula (11%) occurring after a mid-pancreatic resection and a pancreaticoduodenectomy. The median follow-up was 4.2 years (range 2–11). There was no diabetes mellitus, but there was 1 case of fat diet intolerance requiring pancreatic enzyme substitution. All of the children had a growth curve within normal limits. Conclusions: In this experience, pancreatic resections have proven to be a safe and efficient procedure, with low long-term morbidity, for the treatment of tumoral and selected nontumoral pancreatic masses.

Prenatal and postnatal Ciliated Hepatic Foregut Cysts in infants.

PURPOSE Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC. METHODS We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis. RESULTS Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery. Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia. CONCLUSION In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.

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

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

Hepatic Portocholecystostomy: 97 Cases From a Single Institution

Objectives: Approximately 20% of cases of biliary atresia do not involve the gallbladder, the cystic duct, and the common bile duct. In these cases, a hepatoportocholecystostomy (HPC) may be performed instead of the classical hepatoportoenterostomy (HPE). Methods: We reviewed our cohort to investigate the efficacy of HPC and the associated surgical complications and clinical problems. Results: From 1984 to 2009, 97 patients underwent HPC in our institution. In the first 6 months of the postoperative period no patient presented with bacterial cholangitis. Nine patients had bile leakage, and 1 patient had a gallbladder obstruction. For these 10 patients, HPC was modified in HPE. Jaundice clearance was maintained after HPE, but 4 of the patients presented 1 or more cholangitis episodes. After 6 months, there were no cases of cholangitis recorded, 2 patients presented bile leakage and 4 patients experienced gallbladder obstruction. After 5 years of follow-up, 46% of the patients were jaundice free with their native liver and 29.4% were transplanted. Conclusions: In patients with biliary atresia with patent extrahepatic bile ducts, hepatoportocholecystomy is a good surgical technique that can prevent cholangitis. These results are counterbalanced by specific surgical complications that need to be known and looked for in the postoperative period.

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

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

First liver transplantation for biliary atresia in children: The hidden effects of non-centralization

The aim of our study was to determine the impact of initial orientation for medical and surgical care of children with BA on procedures and outcomes of the first LT. We retrospectively analyzed charts of children with BA who underwent first LT between 2006 and 2015. Patients were divided into two groups for comparison: a single‐center management group (from diagnosis to transplantation) and a secondarily referred group (children referred after failure of KP). We focused analysis on disease severity at transplantation, blood transfusion, and overall survival. One hundred and eighty‐five children were included. The median delay between pretransplant check‐up and transplantation was shorter in patients secondarily referred. A severe undernutrition was observed in 23.7% of children secondarily referred compared to 11.1% in children with a single‐center management (P = .024). At transplantation, INR and factor V level were higher in single‐center group patients (respectively, 67% vs 55%, P < .001 and 61% vs 49%, P = .002). The total of red blood cell and fresh frozen plasma administrated during procedure was two times higher in patients secondarily referred. Finally, patients with a single‐center management had a higher overall 12 months of survival rate (92.1% vs 83.1%, P = .033). In a country without low‐density population issues, the authors advocate an early referring to transplant center to further improving LT outcomes in children with BA.

SFCP CO-73 - Tumeurs rhabdoïdes du foie : étude observationnelle de 5 cas pédiatriques

Objectifs Les tumeurs rhabdoides hepatiques (TRH) sont rares et agressives, survenant essentiellement chez les enfants de moins de 1 an. L’objectif de cette etude est de decrire les traitements recus et la survie de patients avec TRH. Patients et methode Il s’agit d’une etude unicentrique retrospective de 5 patients traites pour TRH entre le 1 er janvier 2007 et le 31 decembre 2013. Les variables etudiees ont ete l’âge au diagnostic, la presence de metastases, le traitement recu et la survie globale. Resultats L’âge median au diagnostic etait de 6 mois (4–23). Dans tous les cas, le diagnostic a ete histologique avec absence d’expression du gene INI1. Aucun n’enfant n’etait metastatique au diagnostic. Tous les patients ont eu de la chimiotherapie et de la chirurgie. Un seul a eu de la radiotherapie. Les 3 patients (60%) vivants ont tous recu de la doxorubicine et ont eu une chirurgie moins de 3 mois apres le diagnostic. Le recul median est de 9 mois (9–80). Conclusions La survie serait amelioree par une chimiotherapie intensive contenant de la doxorubicine et une chirurgie precoce.

SFCP CO-51 - Prise en charge unicentrique de 60 hernies de coupole diaphragmatique

Objectifs Etudier la correlation entre l’image antenatale et le devenir clinique des patients ayant une hernie de coupole diaphragmatique et analyser les resultats a court et moyen terme. Materiels et methodes Etude retrospective de 60 enfants de 2010 a 2013. Nous avons etudie le LHR prenatal, l’utilisation d’un plug tracheal, la technique chirurgicale, la duree d’hospitalisation, les complications et la survie globale. Resultats Le diagnostic antenatal a ete fait chez 49 enfants. Selon le LHR, 21 (43%) formes favorables, 15 (30%) intermediaires et 13 (27%) severes. Seize enfants ont eu un plug. La chirurgie a ete realisee chez 46 (77%) enfants, 13 ont eu un patch. La duree d’hospitalisation mediane etait de 25 jours (7–132). Trois patients sont rentres a domicile avec une nutrition enterale et 2 avec une oxygenotherapie temporaires. Sept patients ont eu une occlusion sur bride et 3 une recidive. Aucun geste de gastrostomie ou anti-reflux n’a ete pratique. Vingt (33%) enfants sont decedes: 9 (45%) formes severes, 8 (40%) intermediaires et 3 (15%) legeres. Le recul median est de 23,5 mois (4–47). Conclusions Le LHR semble etre un bon facteur pronostic. Meme si la mortalite reste severe, les complications associees a une faible morbidite permettent une qualite de vie satisfaisante.

SFCP CO-74 - Chirurgie des métastases hépatiques des néphroblastomes : expérience monocentrique

Introduction Les metastases hepatiques de nephroblastome (MHN) sont rares et leur prise en charge est specialisee. Materiel et methodes Etude monocentrique retrospective (1993–2010) incluant les patients (pts) ayant une chirurgie hepatique pour MHN. Resultats Dix pts ont ete identifies : 4 pts avec metastases synchrones, 6 pts metachrones (delai moyen de rechute de 23 mois [1–123]). Toutes touchaient le lobe hepatique droit. Elles etaient multiples 4 fois et unique 6 fois. Une atteinte pulmonaire etait associee chez 5 pts (dont les 4 synchrones). Localement, dans 40% des cas le stade de la tumeur etait I, II dans 40%, III dans 20%. Elle etait a droite chez 6 pts. Le geste chirurgical etait une chirurgie reglee pour 6 pts et une resection atypique pour 4. Un patient a necessite la pose d’un packing. Il n’y a pas eu de mortalite peroperatoire. La resection a ete microscopiquement complete pour 8 patients. Une chirurgie pulmonaire a ete faite chez 4 pts. 3 patients ont presente une rechute hepatique avec un delai moyen de 5 mois. Parmi eux, 2 sont decedes. La survie globale est de 80% dans cette serie. Conclusion Le pronostic des enfants ayant une resection complete des MHN persistant apres chimiotherapie est bon. Une prise en charge chirurgicale agressive est necessaire.