Esophageal atresia, choanal atresia, and dysautonomia

Cozzi, Francesco; Myers, N. A.; Madonna, L.; Drago, S; Fiocca, G; Piacenti, S.; Pierro, Agostino

doi:10.1016/0022-3468(91)90704-w

Cited by 14 publications

(10 citation statements)

References 20 publications

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“…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].…”

Section: Discussionmentioning

confidence: 72%

“…The diagnosis of hyperhidrosis was made when the infant's bed sheet or clothes were wet from dripping sweat [1][2][3]. 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].…”

Section: Methodsmentioning

confidence: 99%

“…We previously reported that another infant with esophageal atresia presented recurrent episodes of profuse sweating of the right side of the face [1].…”

mentioning

confidence: 99%

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Asymmetric sweating and flushing in infants with esophageal atresia

Cozzi

Mele

Totonelli

et al. 2009

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 72%

Section: Methodsmentioning

confidence: 99%

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Asymmetric sweating and flushing in infants with esophageal atresia

Cozzi

Mele

Totonelli

et al. 2009

Journal of Pediatric Surgery

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“…During the ventilatory period characterised by severe glossoptotic pharyngeal obstruction we found: (1) a further decrease in the breathing rate; (2) increasing obstructed inspiratory efforts (equivalent of Muller's manoeuvre) often associated with stridor; (3) increasing obstructed expiratory efforts (equivalent of Valsalva's manoeuvre); and (4) retarded expiratory flow often associated with audible grunting. These findings are similar to those observed in children and adults with sleepapnoea syndrome.…”

Section: Discussionmentioning

confidence: 88%

Assessment of pulmonary mechanics and breathing patterns during posturally induced glossoptosis in infants.

Cozzi

Bonanni

Cozzi

et al. 1996

Archives of Disease in Childhood

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Respiratory mechanics were studied in nine infants with glossoptosis-apnoea syndrome to determine whether glossoptosis may account for signs of both inspiratory and expiratory airway obstruction. Airflow, oesophageal pressure, inspiratory and expiratory time (Ti and Te), and inspiratory and expiratory resistance (R. and R,) were measured before and during ventilatory phases characterised by glossoptotic pharyngeal obstruction, induced by turning the infants onto their backs. In addition, an attempt was made to correlate the abnormalities in pulmonary mechanics with the clinical features. During partial glossoptotic pharyngeal obstruction, a significant increase was observed in Te and RK and variable changes in Ti and R,. During severe obstruction, the infants displayed obstructed inspiratory efforts often associated with stridor, as well as obstructed expiratory efforts often associated with audible grunting and retarded expiratory flow pattern. The expiratory grunt was loudest over the neck and mimicked bronchospasm over the chest. These findings indicate that glossoptotic pharyngeal obstruction induces functional airway obstruction which may affect both inspiration and expiration. Expiratory airway obstruction seems, at least in part, to be due to active braking of expiratory flow. (Arch Dis Child 1996;74:512-516)

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“…10 In these infants, different types of mechanical airway obstruction, by increasing the negative intrathoracic pressure due to increased inspiratory efforts, offer evidence of an underlying dysautonomic control of upper airway dilating muscles, responsible for a collapse of soft pharyngeal airways. [10][11][12] Support for this concept comes from the good results obtained in a few infants with repaired EA and AO/ALTE syndrome treated by glossopexy, which stabilizes the upper airway by avoiding, to a certain extent, the falling back of the tongue. 13,14 At present, in infants with EA, there is still controversy as to whether overactive laryngeal chemoreflex, intrathoracic TC, and dysautonomic control of pharyngeal airway patency are independent or interrelated pathogenic factors.…”

Section: Introductionmentioning

confidence: 99%

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

Cozzi

Zani

Conforti

et al. 2006

Pediatric Pulmonology

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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.

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Esophageal atresia, choanal atresia, and dysautonomia

Cited by 14 publications

References 20 publications

Asymmetric sweating and flushing in infants with esophageal atresia

Asymmetric sweating and flushing in infants with esophageal atresia

Assessment of pulmonary mechanics and breathing patterns during posturally induced glossoptosis in infants.

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

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