Pathogenesis of apparent life‐threatening events in infants with esophageal atresia

Abstract

Many infants with a repaired esophageal atresia (EA) undergo fundoplication, aortopexy, or glossopexy because the mechanisms most responsible for airway obstruction and/or apparent life-threatening event (AO/ALTE) syndrome are considered to be gastroesophageal reflux (GER), tracheal compression (TC), or obstructive apnea, respectively. In the present study, we investigated whether these mechanisms are independent or interrelated. We developed a database of 120 consecutive patients with EA treated by the senior author between 1967-2002. We studied the clinical manifestations of patients with a cervical esophagostomy and/or blind lower esophageal stump, which ruled out TC and/or proximal esophageal GER as a mechanism for AO/ALTE. Of 25 neonates who underwent section/ligation of lower tracheo-esophageal fistula and/or feeding gastrostomy, 10 critically ill neonates died. Of 15 survivors, 9 infants had a feeding gastrostomy without an esophagostomy. Of these, 6 infants presented one or more episodes of AO, and 8 presented ALTE with or without AO. Subsequently, 5 of the 9 infants underwent an esophagostomy. Eventually, 11 infants had a feeding gastrostomy with an esophagostomy. Of the latter, 5 infants presented one or more episodes of AO, and 6 presented ALTE without AO. In conclusion, oral feeding, proximal esophageal GER, and TC are not essential for AO/ALTE syndrome to occur. They are probably factors which offer evidence of an underlying problem with control of upper airway patency.

A prospective comparative evaluation of persistent respiratory morbidity in esophageal atresia and congenital diaphragmatic hernia survivors

“…32 Less frequent are RTIs, wheezing, and cough; these findings mainly are derived from studies using a cross-sectional design. [33][34][35][36] showed that 41% of adolescents after repair of EA still had respiratory symptoms, and 52% had ever had pneumonia or wheezing.…”

Asymmetric sweating and flushing in infants with esophageal atresia

“…One or more of these dysautonomic features are found in nearly all patients with esophageal atresia [1][2][3], suggesting that autonomic disturbances and esophageal atresia may be manifestations of maldevelopment of cephalic neural crest derivates (neurocristopathy) [3]. This theory is supported by the findings that about three fourths of patients with esophageal atresia, such as the present patients with asymmetric sweating and/or flushing, present a neural crest related cardiovascular anomaly [11] and an asymmetric facial defect [3].…”

“…We reviewed the hospital records of patients consecutively admitted between January 2003 and December 2007 to update the database used in a previous study [2]. In our database, flushing was not coded.…”

“…Hyperthermia was diagnosed in those infants who presented recurrent episodes of high body temperature without clinical or laboratory evidence of infection [1][2][3]. Apparent life-threatening episode (ALTE) was defined as respiratory arrest which prompted resuscitation maneuvers [1][2][3].…”

Current Controversies in the Surgical Treatment of Esophageal Atresia

“…Patients may suffer apneic spells (72,73) or barking cough (74) during infancy and childhood, frequent vomiting (75), repeated pneumonia (69,76,77) and/or chronic respiratory tract disease (78) that are considered as sequelae of the esophageal malformation but that might be related directly to the GER. Recurrent anastomotic strictures are indeed related to GER and become manageable after effective anti-reflux treatment (79).…”