The Management of Cyanotic Spells in Children with Oesophageal Atresia

Congenital esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) are common congenital anomalies. Respiratory and GI complications occur frequently, and may persist lifelong. Late complications of EA/TEF include tracheomalacia, a recurrence of the TEF, esophageal stricture, and gastroesophageal reflux. These complications may lead to a brassy or honking-type cough, dysphagia, recurrent pneumonia, obstructive and restrictive ventilatory defects, and airway hyperreactivity. Aspiration should be excluded in children and adults with a history of EA/TEF who present with respiratory symptoms and/or recurrent lower respiratory infections, to prevent chronic pulmonary disease.

To assess tolerance and early results of Dumon silicone stents inserted in patients with nonneoplastic airway obstruction. Tracheobronchial stenting for palliative or curative restoration of airway narrowing has been evaluated in a retrospective study. Tertiary-referral teaching hospital. Between May 1991 and September 1994, 64 patients with a mean age of 52 years had endobronchial silicone stent insertion for benign tracheal stenosis (82% secondary to intubation or tracheostomy injury). Lesions were pure fibrous stenosis in 25 patients and fibroinflammatory stenosis in 38. Prostheses were used for temporary stenting of the airway during 18 months in 48 patients in whom cure was expected and as a procedure for palliation in the remaining 15 patients. In all cases, the Dumon tracheobronchial stent was implanted with the rigid bronchoscope under general anesthesia. Five patients died (four from unrelated causes); one was due to hypersecretion and airway obstruction at the time of an emergency tracheostomy 20 days after stent insertion. Complications included migration of prostheses in 11 (17.5%) patients, granuloma formation in 4 (6.3%) patients, and airway obstruction due to heavy secretion in 4 (6.3%). In 48 patients who received silicone stents with curative expectations, removal of the device was accomplished in 21 patients. Therapy proved successful in 17 patients with a mean follow-up of 259+/- 173 days and stenosis recurred in 4. In 16 patients, stents still remain for a mean period of 364+/-119 days. In the series of 15 patients in whom silicon stents were implanted for palliation, prostheses were placed permanently in 11 with a mean follow-up of 486+/-260 days. In the remaining four patients with tracheostomy, silicone stents were used after inability to expand the upper limb of the T-tube (two patients) or placed above the tracheostomy stoma to maintain laryngotracheal patency and preserve phonation when a T-tube was poorly tolerated (two patients). Silicone tracheobronchial stents are effective in the maintenance of airway patency and are associated with good tolerance and infrequent complications that are rarely life-threatening.

The objective of this study was to describe the incidence of complications in children with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) at a tertiary pediatric hospital and to identify predictive factors for their occurrence. A retrospective chart review of 110 patients born in or transferred to Sydney Children's Hospital with EA/TEF between January 1999 and December 2010 was done. Univariate and multivariate regression analyses were performed to identify predictive factors for the occurrence of complications in these children. From univariate analysis, early esophageal stricture formation was more likely in children with 'long-gap' EA (odds ratio [OR] = 16.32). Patients with early strictures were more likely to develop chest infections (OR = 3.33). Patients with severe tracheomalacia were more likely to experience 'cyanotic/dying' (OR = 180) and undergo aortopexy (OR = 549). Patients who had gastroesophageal reflux disease were significantly more likely to require fundoplication (OR = 10.83) and undergo aortopexy (OR = 6.417). From multivariate analysis, 'long-gap' EA was a significant predictive factor for late esophageal stricture formation (P = 0.007) and for gastrostomy insertion (P = 0.001). Reflux was a significant predictive factor for requiring fundoplication (P = 0.007) and gastrostomy (P = 0.002). Gastrostomy insertion (P = 0.000) was a significant predictive factor for undergoing fundoplication. Having a prior fundoplication (P = 0.001) was a significant predictive factor for undergoing a subsequent aortopexy. Predictive factors for the occurrence of complications post EA/TEF repair were identified in this large single centre pediatric study.