Thirty-six clinical questions addressing the diagnosis, treatment, and prognosis of the common GI complications in patients with EA were formulated. Questions on the diagnosis, and treatment of gastroesophageal reflux, management of "cyanotic spells," etiology, investigation and management of dysphagia, feeding difficulties, anastomotic strictures, congenital esophageal stenosis in EA patients were addressed. The importance of excluding eosinophilic esophagitis and associated GI anomalies in symptomatic patients with EA is discussed as is the quality of life of these patients and the importance of a systematic transition of care to adulthood. A systematic literature search was performed from inception to March 2014 using Embase, MEDLINE, the Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Clinical Trials, and PsychInfo databases. The approach of the Grading of Recommendations Assessment, Development and Evaluation was applied to evaluate outcomes. During 2 consensus meetings, all recommendations were discussed and finalized. The group members voted on each recommendation, using the nominal voting technique. Expert opinion was used where no randomized controlled trials were available to support the recommendation.
Eosinophilic esophagitis (EoE) has only rarely been reported in esophageal atresia (EA) patients. A retrospective case analysis of all EA patients born at our center between January 1999 and April 2012 was performed. A total of 113 of patients were identified; 10 patients were excluded as a result of inadequate data. Eighteen patients (17%) were diagnosed with EoE. The average number of eosinophilis was 30/high-power field (HPF) (19/HPF-80/HPF). The median age for diagnosis of EoE was 1 year and 6 months (8 months-8 years and 7 months). Children with EoE had a significantly greater incidence of reflux symptoms, dysphagia, tracheomalacia, and 'hypoxic spells' (P < 0.05). EoE patients also underwent significantly more surgery including fundoplication and aortopexy when compared with those without EoE (P < 0.0001). Although the incidence of gastrostomy was greater in the EoE group (33% vs. 13%), this was not statistically significant. Half of the EoE patients had a coexisting atopic condition at time of diagnosis. The commonest condition was asthma 7/18 (38%) followed by specific food allergy 6/18 (33%). EoE was treated in 11 patients with either swallowed fluticasone or budesonide slurry. All improved clinically. Histologically, five had complete resolution and six had partial improvement. Six children with EoE were treated with acid suppression alone. All improved clinically, and 5/6 had subsequent histological resolution. One child who received acid suppression and an exclusion diet also improved. Seven patients (38%) had an esophageal stricture at time of EoE diagnosis. Five were dilated at time of the initial endoscopy, prior to the diagnosis of EoE being available. Two patients had resolution of their strictures on medical treatment of their EoE alone and did not require further dilatation. EoE was seen in 17% of children with EA in this study. EoE should be considered in EA patients with persistent symptoms on standard reflux treatment, increasing dysphagia, and recurrent strictures.
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.
In young children with CF, GOR is primarily acidic and proximal migration is common. Physiotherapy in the head-down position does not appear to exacerbate GOR. The impact of GOR on lung disease remains to be elucidated.
Poor growth and inability to manage age-appropriate textures are often present in children with EA, particularly in the younger years. This highlights the need for early intervention in a specialist multidisciplinary EA clinic in which dietetics and speech pathology are available.
PEGs had a significantly higher early complication rate than LAPGs and the only major complications occurred in the PEG group. PEGs also had a higher overall complication rate than LAPGs, although the difference was not statistically significant. Both PEGs and LAPGs were significantly superior to OG in terms of overall complication rates.
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