NASPGHAN EA/TEF Guidelines

• Acid suppression is recommended for all EA patients in the neonatal period
•  Long term consequences of acid suppression on microbiota, and risk for GI and resp infections, must be considered, however; risk of GER-associated complications are still believed to outweigh those risks
•  Complications of GER in EA are Peptic esophagitis, Barrett, Anastomotic strictures (AS)
• PPI should be first line therapy
  
• It is recommended that GER be systematically treated for prevention of peptic complications and anastomotic stricture up to the first year of life or longer, depending on persistence of GERD
•  Recent studies suggest GERD can be more prevalent after 1yr of age
• pH monitoring
• a.       Can evaluate severity and association of acid reflux in patients with EA
• b.       Can evaluate and correlate non-acid reflux with symptoms in selected patients (symptomatic on PPI, on continuous feeding, with extra-digestive symptoms, ALTE, GER symptoms with normal pH-probe and endoscopy) Since there is no medication for non-acid reflux, this testing would be done if considering fundoplication
• Endoscopy with biopsies is mandatory for routine monitoring of GERD in EA patients
• a.       At least 4 biopsies in each quadrant, 1cm above the Z line
• b.       Consider EGD in post-fundoplication patients because recurrence of GER and peptic esophagitis is possible
•  Monitor for GER (EGD or pH) at the time of discontinuation of anti-acid treatment and during long term follow up
• Routine endoscopy in asymptomatic EA patients is recommended to detect early esophagitis (with the opportunity for subsequent intervention) before the development of late complications of strictures, Barrett esophagus, and cancer. Surveillance endoscopy should occur at the following 3 times in childhood:
• a.       At the time of discontinuation of PPI
• b.       Before the age of 10
• c.       Once at the time of transition to adulthood
• Fundoplication
• a.       Severe esophageal dysmotility predisposes EA patients to post-fundoplication complications
• b.      Fundoplication should be considered in patients with
• i.      Recurrent anastomotic strictures, especially in long-gap EA
• ii.      Poorly controlled GERD despite maximal PPI therapy
• iii.      Long term dependency on trans-pyloric feeding
• iv.      Cyanotic Spells
• Prior to fundoplication patient should have:
• i.      Barium contrast study (Upper GI Series)
• ii.      24hr pH-metry or pH-MII testing
• iii.      Endoscopy with 4 quadrant biopsies above, at, and below level of anastomosis
• Symptoms of aspiration during swallowing may be identical to GER symptoms in young children
• a.       Extra-esophageal manifestations of reflux and dysmotility:
• i.      Cough
•  ii.      Wheezing
  
• iii.      Dyspnea
• iv.      Bronchitis
•  v.      Recurrent infections
•  vi.      Bronchiectasis
  
• vii.      Restrictive lung disease
•  viii.      Obstructive lung disease
•  ix.      Tracheomalacia
•  x.      PNA
• b. GI causes of pulm Sx:
•  i.      Aspiration
• ii.      Anastomotic strictures
• iii.      Impaired esophageal motility
• iv.      CES
• v.      GERD
• vi.      Recurrent or missed fistulae
• vii.      EoE
• viii.      Pooling over fundoplication
• c.  Non-GI causes: 
  
• vocal cord paralysis
• laryngeal clefts
• vascular rings
• aspiration during swallowing
• Patients with EA should be evaluated regularly by a multidisciplinary team (pulm & ENT) regardless of symptoms
• a.       Anatomic abnormalities (laryngeal cleft, vocal cord paralysis, missed or recurrent fistulae, anastomotic stricture, congenital stenosis, vascular ring) should be ruled out in EA patients with respiratory symptoms
• b.       If pH-metry or pH-MII is performed, symptom correlation during reflux testing, rather than total reflux burden is the most important indicator of reflux-associated symptoms
• Acid suppression should be used with caution in patients with extra-esophageal manifestation of reflux
• a.       More studies are needed to determine the impact of acid suppression, fundoplication and transpyloric feeding on extra-esophageal symptoms
• b.       Prolonged acid suppression use has been associated with: an increased risk of respiratory infections (pneumonia, upper respiratory tract infections, pharyngitis), which may exacerbate the underlying lung disease
• “Cyanotic Spells”
• a.       The etiology of life-threatening events is multifactorial and merits a multidisciplinary diagnostic evaluation before surgical intervention
• b.       Anatomic issues (strictures, recurrent or missed fistulae, congenital esophageal stenosis, vascular rings, laryngeal clefts) and aspiration need to be excluded in children with ALTE
• Dysphagia should be suspected in patients with EA who present with:
• a.       Food aversion
• b.       Food impaction
• c.       Difficulty in swallowing
• d.       Odynophagia
• e.       Choking
• f.        Cough
• g.       Pneumonia
• h.       Alteration in eating habits
• i.         Vomiting
• j.         Malnutrition.
• EA patients with dysphagia, should undergo evaluation with:
• a.       Upper GI contrast study
• b.       EGD/Esophagoscopy with biopsies
• Esophageal manometry can characterize esophageal motility patterns in EA patients with dysphagia (impact on clinical outcomes TBD)
• Tailor treatment of dysphagia to underlying mechanisms. Treatment might include:
• a.       Feeding adaptation
• b.       Treatment of esophagitis (peptic, eosinophilic, or infectious) and inlet patch
• c.       Prokinetics
• d.       Treatment of stricture, stenosis, mucosal bridge, or anastomotic diverticulum
• e.       Surgical repair of vascular anomaly
• f.        Gastrostomy tube feeding
• g.       Esophageal replacement
• h.       Dilation of fundoplication
• In EA patients with post-fundoplication dysphagia, Obtain:
• a.       Contrast study to rule out mechanical complications
• b.       EGD with biopsy and, if inconclusive, high-resolution manometry w/wo impedance
• Tailor management of post-fundoplication dysphagia to the underlying mechanism(s)
• a.       Feeding adaptation
• b.       Treatment of esophagitis (peptic, eosinophilic, or infectious)
• c.       Prokinetics
• d.       Balloon dilation of the wrap
• e.       Botulinum toxin to the LES
• f.        Gastrostomy tube feeding
• g.       Surgical revision of the fundoplication
• h.       Gastrostomy
• i.         Esophageal replacement
•  Congenital vascular malformations should be excluded by chest CT or MR angiography. Even though congenital vascular malformations are usually asymptomatic, they may be the underlying etiology for dysphagia, dyspnea, or cyanosis, by causing external compression on the esophagus and/or trachea
• No data are available on the most efficacious methods of avoiding feeding disorders in children with EA. However, the committee recommends a multidisciplinary approach to prevent and treat feeding difficulties
• Intensive early enteral and oral nutrition intervention and advances in neonatal care and surgery have reduced the risk of long term malnutrition in children with EA; however, other associated comorbidities may increase this risk
• Anastomotic strictures are considered to be clinically significant if there is relative esophageal narrowing at the level of the anastomosis (by contrast and/or endoscopy) with significant functional impairment and associated symptoms
• There is no evidence that routine screening and dilation is superior to evaluation and treatment in symptomatic patients.
  
• We recommend that Anastomotic strictures (AS) be excluded in symptomatic children, and those children who are unable to achieve feeding milestones.
• Diagnose AS via contrast study or endoscopy
• Treat anastomotic strictures with dilation
• a.       Performed under General Anesthesia and tracheal intubation
• b.       Use of guidewire to insert the chosen dilator (balloon vs Savary-Gilliard semi-rigid) through the stricture under endoscopic or fluoroscopic control
•  i.      No studies have compared balloon vs semi rigid dilator for Tx of AS
• 3 or more clinically relevant stricture relapses constitute recurrent stricture
• Potential Adjuvant treatments for the management of recurrent strictures in EA patients:
• a.       Intralesional and/or systemic steroids
• b.       Topical mitomycin C
• c.       Stents
• d.       Endoscopic knife
• If Congenital Esophageal Stenosis (CES) is suspected:
• a.       Esophagram followed by
• b.       Endoscopy to confirm diagnosis and r/o other pathology
• Endoscopic dilation is the first line treatment of Congenital Esophageal Stenosis (CES)
• EoE needs to be excluded in all EA patients (all ages) with dysphagia, reflux sx, coughing, choking, or recurrent strictures that are refractory to PPI, before proceeding to anti-reflux surgery
• Multiple esophageal biopsies, both proximal and distal to the anastomosis are required for the diagnosis of EoE.
• Management of EA patients with EoE should follow consensus recommendations for treatment of EoE in the general population
• Transition to adulthood
• a.       Dysphagia and symptoms of GER continue into adulthood in EA patients and are more frequent in EA survivors than in the general population
• b.       The incidence of esophagitis and esophageal gastric and intestinal metaplasia (Barrett) is increased in adults with EA as compared with the general population
• c.       Current studies show no increase incidence of esophageal cancer (adenocarcinoma, squamous cell carcinoma) in adults with EA, however, esophageal cancer remains a concern
• We recommend transition of young adults from pediatric care to an adult physician with expertise in EA (general practitioner, surgeon, gastroenterologist, pulmonologist, or any informed specialist aware of the specificities of the care of adults operated for EA)
• We recommend regular clinical follow up in every adult patient with EA, with special reference to presence of dysphagia, GER, respiratory symptoms and anemia with:
• a.       Routine endoscopy (with biopsies in 4 quadrants at gastroesophageal junction and anastomotic site) at time of transition into adulthood and every 5 to 10 years
• b.       Additional endoscopy if new or worsening symptoms develop
• c.       In presence of Barrett as per consensus recommendations
• Although GI and respiratory symptoms and associated comorbidities (esophageal replacement and congenital anomalies) may negatively impact HRQoL, no evidence currently shows that the overall HRQoL is impaired in children and adults with EA compared with the general population. We recommend long-term medical and psychosocial support for these patients and families.

Notes:

-GER is associated with complications in neonates and infants undergoing surgery for EA

-It is suggested that GER is a major factor for recurrent anastomotic strictures (AS)

-Pulmonary complications associated with GER include:

                --persistent atelectasis

                --aspiration PNA

                --Asthma/increased airway reactivity

                --Chronic lung disease with bronchiectasis

                --Worsened tracheomalacia

-In EA/TEF patients who present with Cyanotic Episode/BRUE, it should not be considered “unexplained” and patient should be evaluated as per these guidelines

-Esophagitis and dysmotility are often found in EA patients

-Gold standard for diagnosing GERD is pH monitoring: pH probe testing, pH-impedance (pH-MII), and wireless pH testing

References:

https://naspghan.org/files/documents/pdfs/position-papers/ESPGHAN_NASPGHAN_Guidelines_for_the_Evaluation_and.19%20(1).pdf