Oral Presentation - 32
Key factors linked to the development of restrictive lung syndrome in children following surgery for type III esophageal atresia.
J Goulin*, T Brigly**, F Bastard*, A Bonnard***, V Rousseau****, T Gelas*****, A Guinot******, E Habonimana*******, A Breton********, A Ranke*********, I Talon**********, S Irtan***********, T Lamireau************, C Jacquier*************, F Elbaz**************, N Kalfa***************, N Panait****************, V Fouquet*****************, H Lardy******************, A Scalabre*******************, P Buisson********************, M Margaryan*********************, F Auber**********************, C Grosos***********************, C Borderon************************, C Tölg*************************, G Podevin*, R Sfeir**************************, F Gottrand**************************, F Schmitt*
*Angers university Hospital Centre
**Laval Regional Hospital Centre
***AP-HP Robert Debré Hospital
****AP-HP Necker Sick Children Hospital
*****Lyon University Hospital Centre
******Nantes University Hospital Centre
*******Rennes University Hospital Centre
********Toulouse University Hospital Centre
*********Nancy University Hospital Centre
**********Hautepierre University Hospital of Strasbourg
***********AP-HP Armand Trousseau Hospital
************Bordeaux University Hospital Centre
*************Grenoble University Hospital Centre
**************Rouen University Hospital Centre
***************Montpellier University Hospital Centre
****************AP-HM University Hospital Centre Marseille
*****************AP-HP Bicêtre Hospital
******************Tours University Hospital Centre
*******************Saint Etienne University Hospital Centre
********************Amiens University Hospital Centre
*********************Le Mans Regional Hospital
**********************Besançon University Hospital Centre
***********************Limoges University Hospital Centre
************************Clermont-Ferrant University Hospital Centre
*************************Martinique University Hospital Centre
**************************Lille University Hospital
Purpose: To evaluate the incidence of restrictive lung syndrome in children after type III esophageal atresia (EA) surgery and to identify potential associated factors.
Method: After local institution review board approval, a multicenter retrospective study was conducted using data from 216 patients with type III EA included in a population-based registry between 2008 and 2013. Restrictive lung syndrome was defined based on the Z-score of forced vital capacity measured during pulmonary function tests conducted between 6 and 9 years of age. (ClinicalTrial NCT04136795)
Results: At follow-up (7.5 ± 1.5 years), 58 (27%) had pure restrictive syndrome and 137 (63%) had normal respiratory function. Patients with restrictive syndrome were more often premature (52% vs. 30%, p = 0.0005) and had more associated cardiac malformations (36% vs. 12%, p = 0.0003). Surgically, these patients more frequently required delayed anastomosis (8.6% vs. 1.5%, p = 0.03), gastrostomy (17% vs. 1.5%, p = 0.0001), or other thoracic surgeries (17% vs. 5%, p = 0.01). At age 6, they had a lower body mass index and more respiratory history (85% vs. 63%, p = 0.002) but no significant increase in thoracic wall sequelae (73% vs. 69%, p = 0.84). Multivariate analysis showed that restrictive syndrome was associated with Caucasian origins (Odds ratio OR = 4.3 [1.2 – 15.4]), a history of tracheomalacia (OR 4.06 [1.62-10.20]), cardiac anomalies (OR 5.75 [1.95-16.95]), and gastroesophageal reflux (OR 3.06 [1.26-7.41]). On the contrary, neither surgical factors nor chest-wall sequelae were associated with a restrictive pattern.
Conclusion: These findings suggest that restrictive lung syndrome affects more than a quater of children with type III EA and is linked to patient-associated anomalies rather than surgical or health care-associated factors.