French national reference center for chronic and malformative conditions of the esophagus (CRACMO)
The rare diseases managed by the reference center are chronic malformations and disorders of the esophagus. They are often isolated, but can sometimes be observed in syndromic associations. Associated malformations (pulmonary, digestive, ENT, cardiac, etc.) must be identified to ensure better overall management.
The Necker-Enfants malades reference center brings together all the skills and expertise needed to manage esophageal pathologies, whatever their complexity. The center also has particular expertise in the management of associated ENT, cardiac, pulmonary and orthopedic pathologies, starting in the neonatal period, thanks to the existence of an expert neonatology department on site. The Necker-Enfants malades referral center has a strong referral activity, accepting children from all over France, from overseas departments and regions, and from abroad.
The site’s medical staff (visceral surgeon, thoracic surgeon, orthopedist, neonatologist, pulmonologist, ENT specialist, gastroenterologist, oral specialist) and paramedical staff (stomatotherapist if gastrostomy, dietician, psychologist, social worker) work together to offer a multi-disciplinary care pathway and comprehensive care tailored to each child.
This reference center is affiliated to the Fimatho rare diseases healthcare network and the ERNICA European reference network (ERN) for rare Inherited and Congenital (digestive and gastrointestinal) Anomalies.
Keywords: esophageal atresia, achalasia, eosinophilic oesophagitis, feeding difficulties.
Medical
team
Responsible physician
Pr Alexandre Lapillonne
MD, PhD
Dr Véronique Rousseau
In case of emergency
On-call surgeon : +33 (0)1 71 39 68 49
Emergency department : +33 (0)1 44 49 42 90
- Esophageal atresia
> Orphanet file - Congenital esophageal stenosis
- Esophageal achalasia
> Triple A syndrome
> Idiopathic achalasia of esophagus
> Achalasia-microcephaly syndrome - Congenital tracheoesophageal fistula
- Eosinophilic esophagitis
- Therapeutic education of children with digestive and / or urinary continence disorders with digestive and / or urinary stoma and their parents
- Prevention of respiratory decompensations
- Child’s disability stress rehabilitation center
- Therapeutic education pathway for young people with Pierre Robin Syndromes or related syndromes, congenital sucking-swallowing disorders and post-traumatic oral disorders, and their parents
- Therapeutic education pathway for children and their parents in home enteral feeding techniques
- Therapeutic education for chronic respiratory failure in children treated with non-invasive ventilation
A number of research studies have been carried out directly using full or partial data from the registry, as well as nested cohort studies, such as:
- Fate at age 6 of a population of children operated on at birth for esophageal atresia
- Esophageal atresia in newborns <1500g: epidemiological study, status report on management, search for prognostic factors
- Qualitative study of the impact of the birth of a child with a rare abdominothoracic malformation on parental adjustment in the first year of life
- Frequency and characteristics of dumping syndrome after surgery for type III and IV esophageal atresia
- Comparison of endoscopic dilatation and Heller myotomy in the treatment of achalasia of the esophagus in children: a multi-center study
- Evaluation of growth and nutritional status at one year of age in patients operated on for esophageal atresia.
- Dietary orality of children born with esophageal atresia compared with orality of children born without malformation at equivalent term.
- Study of feeding disorders in children with esophageal atresia (FEED-EASY)
- Esophageal atresia and duodenal atresia: national review of the last 10 years and evaluation of their long-term gastroesophageal function
- Long-term outcome of esophageal atresia: Transomic profiles in adolescence (TransEAsome)
- Study of the effect of the time of diagnosis (antenatal vs. postnatal) on the post-traumatic reactions of parents of children operated on for esophageal atresia: DANAO
- Long-term management and follow-up of patients with congenital hiatal hernia: pediatric experience
- Coordination of the Neonatal Medicine and Intensive Care IUD – Neonatology option
2022
– Comment je fais… L’examen échographique de l’œsophage au premier trimestre [How I do… fetal esophagus sonographic assessment in the first trimester].
Codaccioni C, Bault JP, Deloison B, Gaillard A, Salomon LJ, Arthuis C.
Gynecol Obstet Fertil Senol. 2022 May;50(5):415-418.
– The role of perinatal palliative care following prenatal diagnosis of major, incurable fetal anomalies: a multicentre prospective cohort study.
de Barbeyrac C, Roth P, Noël C, et al. PALLIMAGE Study Group.
BJOG. 2022 Apr;129(5):752-759.
– Noninvasive Respiratory Support as an Alternative to Tracheostomy in Severe Laryngomalacia.
Veroul E, Amaddeo A, Leboulanger N, Gelin M, Denoyelle F, Thierry B, Fauroux B, Luscan R.
Laryngoscope. 2022. doi: 10.1002/lary.29928. Epub ahead of print.
2021
– Endoscopic diagnosis of a tracheo-esophageal fistula: Proposal for guidelines, with video. (France).
Luscan, R., Eveno, T., and Thierry, B. (2021b).
Eur Ann Otorhinolaryngol Head Neck Dis. 2021 Aug;138 Suppl 1:6-7.
– Thulium LASER for endoscopic closure of tracheoesophageal fistula in esophageal atresia’s spectrum: An appropriate tool?
Luscan, R., Simon, F., Khen Dunlop, N. et al.
J. Pediatr. Surg. (2021a). 56, 1752–1756.
2020
– Should autism spectrum disorder be considered part of CHARGE syndrome? A cross-sectional study of 46 patients.
Abadie V, Hamiaux P, Ragot S, et al.
Orphanet J Rare Dis. 2020 Jun 3;15(1):136
– Risk factors for bronchiolitis hospitalization in infants: A French nationwide retrospective cohort study over four consecutive seasons (2009-2013).
Fauroux, B., Hascoët, J.-M., Jarreau, P.-H., et al. (2020).
PloS One 15, e0229766.
– Spinal dysraphism as a new entity in V.A.C.TE.R.L syndrome, resulting in a novel acronym V.A.C.TE.R.L.S.
Amelot, A., Cretolle, C., de Saint Denis, T., Sarnacki, S., Catala, M., and Zerah, M.
Eur. J. Pediatr. (2020). 179, 1121–1129.
– Thoracoscopy Versus Thoracotomy in the Repair of Esophageal Atresia with Distal Tracheoesophageal Fistula.
Elhattab, A., Ali, L., Rousseau, V., Clermidi, P., Michelet, D., Farnoux, C., Lapillonne, A., Aly, K.A.-E., Sarnacki, S., Bonnard, A., Khen-Dunlop N.
J. Laparoendosc. Adv. Surg. Tech. (2020). A 30, 1289–1294.
2018
– ESPGHAN/ESPEN/ESPR/CSPEN guidelines on pediatric parenteral nutrition: Lipids.
Lapillonne A, Fidler Mis N, Goulet O, et al.
Clin Nutr. 2018 Dec;37(6 Pt B):2324-2336
– Management and outcome of neonates with a prenatal diagnosis of esophageal atresia type A: a population based study.
Garabedian C, Bonnard A, Rousseau V, Sfeir R; CRACMO, Blanc S, Boubnova J, Jacquier C, Irtan S, Breton A, Fouquet V, Guinot A, Lamireau T, Habonimana E, Schneider A, Elbaz F, Ranke A, Poli-Merol ML, Kalfa N, Petit T, Michel JL, Buisson P, Lirussi-Borgnon J, Lardy H, Parmentier B, Cremillieux C, Schmitt F, Borderon C, Jaby O, Pelatan C, De Vries P, Pouzac-Arnould M, Grosos C, Breaud J, Tolg C, Chaussy Y, Laplace C, Drumez EE, Michaud L, Gottrand F, Houfflin-Debarge V.
Prenat Diagn. 2018 May 8. doi: 10.1002/pd.5273. [Epub ahead of print
– Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort.
Bastard F, Bonnard A, Rousseau V, Gelas T, Michaud L, Irtan S, Piolat C, Ranke-Chrétien A, Becmeur F, Dariel A, Lamireau T, Petit T, Fouquet V, Le Mandat A, Lefebvre F, Allal H, Borgnon J, Boubnova J, Habonimana E, Panait N, Buisson P, Margaryan M, Michel JL, Gaudin J, Lardy H, Auber F, Borderon C, De Vries P, Jaby O, Fourcade L, Lecompte JF, Tolg C, Delorme B, Schmitt F, Podevin G.
J Pediatr Surg. 2018 Apr;53(4):605-609. doi: 10.1016/j.jpedsurg.2017.07.013. Epub 2017 Jul 21.
2017
– Eosinophilic esophagitis and colonic mucosal eosinophilia in Netherton syndrome.
Paluel-Marmont, C., Bellon, N., Barbet, P., Leclerc-Mercier, S., Hadj-Rabia, S., Dupont, C., and Bodemer, C.
J. Allergy Clin. Immunol. (2017). 139, 2003-2005.e1
– Prenatal diagnosis of congenital malformations for the better and for the worse.
Kermorvant-Duchemin E, Ville Y.
J Matern Fetal Neonatal Med. 2017 Jun;30(12):1402-1406.
2016
– Posttraumatic Stress Reactions in Parents of Children Esophageal Atresia.
Le Gouëz M, Alvarez L, Rousseau V, Hubert P, Abadie V, Lapillonne A, Kermorvant-Duchemin E.
PLoS One. 2016 Mar 8;11(3):e0150760. doi: 10.1371/journal.pone.0150760. eCollection 2016.
2015
– Performance of prenatal diagnosis in esophageal atresia.
Spaggiari E, Faure G, Rousseau V, Sonigo P, Millischer-Bellaiche AE, Kermorvant-Duchemin E, Muller F, Czerkiewicz I, Ville Y, Salomon LJ.
Prenat Diagn. 2015 Sep;35(9):888-93. doi: 10.1002/pd.4630. Epub 2015 Jul 6.
2011
– Prenatal diagnosis and evaluation of defect length in esophageal atresia using direct and indirect (tracheal print) signs.
Quarello E, Saada J, Desbriere R, Rousseau V, De Lagausie P, Benachi A.
Ultrasound Obstet Gynecol. 2011 Aug;38(2):225-8. doi: 10.1002/uog.8888. Epub 2011 Jun 27.
- Association française de l’atrésie de l’œsophage
In 2021, in partnership with the health insurance, the association developed a social aid simulator (governed by French law, on French territory). Its objective is to indicate for each person caring for a relative, the financial and social aid to which they can claim, according to their personal situation and the situation of the person being cared for. It can therefore be used by any person – beyond oesophageal atresia – concerned by a rare or chronic disease, a disability, etc.
Contact information
Necker-Enfants malades university hospital
> Pediatric visceral surgery department
149 rue de Sèvres
75743 PARIS Cedex 15
In Necker, the CRACMO reference center in brief …
* data valid for 2022