Delayed primary anastomosis in poor-risk patients with esophageal atresia associated with tracheoesophageal fistula

A 32+4-week-preterm neonate was operated on day 1 for esophageal atresia with tracheoesophageal fistula with a tight primary anastomosis and closure of a tracheoesophageal fistula. Postoperatively, he needed ventilation for 6 days. Post-extubation, he needed continuous positive airway pressure support for increased work of breathing, increasing oxygen requirement, and respiratory acidosis when respiratory support was weaned. Further, during the post-operative period, he had right hemidiaphragmatic paresis and acute lifethreatening events requiring manual high-pressure, non-invasive positive pressure ventilation resuscitation. These complications were considered, and a computed tomography assisted by three-dimensional reconstruction was performed. This revealed a congenital tracheal diverticulum and severe tracheomalacia which was confirmed with microlaryngoscopy and bronchoscopy. The presentation and the diagnostic dilemma surrounding this rare diagnosis are discussed in this case report.

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Transabdominal closure of tracheoesophageal fistula: A new procedure for the management of poor-risk esophageal atresia with tracheoesophageal fistula

Journal of Pediatric Surgery ◽

10.1016/s0022-3468(86)80375-x ◽

1986 ◽

Vol 21 (9) ◽

pp. 812-814 ◽

Cited By ~ 9

Author(s):

Shuhei Ogita ◽

Kazuaki Tokiwa ◽

Toshio Takahashi

Keyword(s):

Esophageal Atresia ◽

Tracheoesophageal Fistula ◽

Poor Risk

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ERNICA Consensus Conference on the Management of Patients with Esophageal Atresia and Tracheoesophageal Fistula: Follow-up and Framework

European Journal of Pediatric Surgery ◽

10.1055/s-0039-3400284 ◽

2019 ◽

Vol 30 (06) ◽

pp. 475-482 ◽

Cited By ~ 3

Author(s):

Carmen Dingemann ◽

Simon Eaton ◽

Gunnar Aksnes ◽

Pietro Bagolan ◽

Kate M. Cross ◽

...

Keyword(s):

Esophageal Atresia ◽

Tracheoesophageal Fistula ◽

Primary Anastomosis ◽

Consensus Conference ◽

Reference Network ◽

Pediatric Gastroenterology ◽

Treatment Protocols ◽

The North ◽

Consensus Statements

Abstract Introduction Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF. Materials and Methods The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9. Results Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items. Conclusion Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

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Efficacy of a standardized tube weaning program in pediatric patients with feeding difficulties after successful repair of their esophageal atresia/tracheoesophageal fistula

European Journal of Pediatrics ◽

10.1007/s00431-020-03673-w ◽

2020 ◽

Vol 179 (11) ◽

pp. 1729-1737

Author(s):

Sabine Marinschek ◽

Karoline Pahsini ◽

Victor Aguiriano-Moser ◽

Marion Russell ◽

Barbara Plecko ◽

...

Keyword(s):

Esophageal Atresia ◽

Tracheoesophageal Fistula ◽

Primary Anastomosis ◽

Oral Feeding ◽

Feeding Problems ◽

Feeding Difficulties ◽

Long Gap ◽

Tube Weaning ◽

Tube Dependency ◽

Graz Model

Abstract Children born with esophageal atresia (EA) might suffer from significant oral feeding problems which could evolve into tube dependency. The primary aim of the study was to define the outcome of tube weaning in children after successful EA repair and to compare outcomes in children with short gap/TEF (tracheoesophageal fistula) and long-gap EA. Data of 64 children (28 with short-gap EA/TEF with primary anastomosis and 36 with long-gap EA with delayed surgical repair) who participated in a standardized tube weaning program based on the “Graz model of tube weaning” (in/outpatients in an intensive 3-week program, online coaching (Netcoaching) only, or a combined 2-week intensive onsite followed by online treatment “Eating School”) from 2009 to 2019 was evaluated. Sixty-one patients completed the program by transitioning to exclusive oral intake (95.3%). Three children (4.7%) were left partially weaned at the time of discharge. No significant differences could be found between short gap/TEF and long-gap EA group regarding outcomes. Conclusions: The study’s findings support the efficacy of tube weaning based on the published “Graz model of tube weaning” for children born with EA/TEF and indicate the necessity of specialized tube weaning programs for these patients. What is Known:• Children with esophageal atresia/tracheoesophageal fistula often suffer from feeding problems and tube dependency.• Different tube weaning programs and outcomes have been published, but not specifically for children with EA. What is New:• Evaluation of a large sample of children referred for tube weaning after EA repair.• Most children with EA can be weaned off their feeding tubes successfully after attending a specialized tube weaning program.

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