Increased Risk of Eosinophilic Esophagitis in Esophageal Atresia Patients

Briefly noted: U Krishnan et al. Analysis of eosinophilic esophagitis in children with repaired congenital esophageal atresia. JACI 2018. Published online Oct 24, 2018.

This retrospective, single-center study examined 4 eosinophilic esophagitis (EoE) study cohorts and identified EoE in 20 of 110 patients (18%) who had surgically-repaired esophageal atresia.

This association has been seen previously: . 2014 Dec 21; 20(47): 18038–18043.  This case study stated ” We are suggesting that EoE is a frequent concomitant problem in patients with history of congenital esophageal deformities, and for this reason any of these patients with refractory reflux symptoms or dysphagia (with or without anastomotic stricture) may benefit from an endoscopic evaluation with biopsies to rule out EoE.”

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Pain in Children with Severe Neurologic Impairment

A recent commentary (JM Hauer JAMA Pediatrics; 2018. doi: 10.1001/jamapediatrics.2018.1531) addresses a common misconception regarding children with severe neurologic impairment (SNI):

“we don’t think she experiences pain”

She notes that literature since 2002 has challenged this assumption and that this is addressed in a new AAP clinical report as well (Hauer J, Houtrow AJ. Pediatrics 2017; 139: e20171002).

Key points:

  • Children with SNI may have moaning, grimacing, changes in tone/body position in reaction to pain and treatment can make them comfortable.
  • “We can never prove that such a child does not feel pain…When parents of children with hydranencephaly were asked whether their child felt pain, 96% indicated yes.”
  • Pain can trigger changes in catecholamines, cortisol and stress hormones.  “These considerations suggest that untreated chronic pain is more harmful to the well-being of children with SNI than is treatment used for pain.”
  • Sometimes no source for pain is identified.  This may be related to a CNS etiology (alteration of CNS) and may benefit from treatment.
  • “It is time to do away with the question of whether these children feel pain and focus on how we as individuals” identify/consider pain

My take: Reframing this issue is important; pain can occur in children with SNI.  At the same time, we have to be careful that some “palliative” measures could paradoxically prolong suffering in some children.

Related blog post: Suffering


Declining Role of Fundoplication in Esophageal Atresia, Too

A recent study (SA Pellegrino et al. J Pediatr 2018; 198: 60-6) examines the likelihood of redo fundoplication for esophageal atresia (EA) in comparison to redo fundoplication for other indications.

Key findings:

  • Among all EA cases, n=344 (1994-2013), 85 underwent fundoplication (single-center study from Melbourne)
  • Rates of fundoplication were declining over study period: there was a 37% drop from 2010-2013 compared to 1994-97.
  • Overall, 767 patients had a fundoplication (n=682 without EA).  The rates of redo fundoplication were similar 11/85 compared with 53/682, despite the fact that EA patients had earlier surgery with median ages of 7.2 months versus 23 months, respectively.

Factors leading to fewer fundoplications:

  • Improving medical therapies, including proton pump inhibitors and use of jejunal feedings
  • Awareness that fundoplication may not be curative and is associated with significant morbidity

Anecdotally, I have had some EA patients whose lives were transformed positively by fundoplication, though many are difficult operations due to anatomic factors like small gastric volumes and pulmonary issues.  Careful selection and surgical expertise are essential to good outcomes.

My take: The authors note that “this study challenges the assertion that fundoplicaiton is less successful in patients with EA.”

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Gibbs Gardens

Esophageal Atresia and Barrett’s Esophagus

Briefly noted: FWT Vergouwe et al. Clin Gastroenterol Hepatol 2018; 16: 513-21.

In this prospective study of adult patients with esophageal atresia (EA) with 151 participants, 6.6% had Barrett’s Esophagus (BE); squamous cell cancer (SCC) was identified in 0.7% (youngest at 42 years).  The authors note that the prevalence of BE and SCC were ~4-fold and ~100-fold higher, respectively, compared to the general population.

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Creek near Chattahoochee River

Guideline Links: Infant Cholestasis and Esophageal Atresia-Tracheoesophageal fistula

One way that I use this blog is to use the search function for previous posts with useful links.  For example, I know if I search “foreign” that I will come across a post that has a summary as well as a link to the NASPGHAN recommendations on Foreign Bodies (Foreign Bodies in Children -Expert Guidance).

This post has two links :

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Guidelines for Esophageal Atresia-Tracheoesophageal Fistula

From a NASPGHAN-ESPGHAN working group of surgeons and pediatric gastroenterologists. a recent guideline (U Krishan et al JPGN 2016; 63: 550-70) regarding esophageal atresia (EA) and tracheoesophageal fistula (TEF) has made 36 specific statements and graded the evidence for these recommendations.


  • Low level evidence: The authors recommended starting PPIs in the neonatal period and continuing them “up to the first year of life or longer, depending on persistence of GERD.”
  • High level evidence: pH monitoring is useful in evaluating the severity of acid reflux in patients with EA.  Low level evidence: pH-impedance monitoring is useful to evaluate and correlate non-acid reflux in selected patients.
  • High level evidence: “Endoscopy with biopsies is mandatory for routine monitoring of GERD in patients with EA.” The authors recommend evaluation (pH, impedance, and/or endoscopy) at time of stopping anti-acid treatment and during long-term follow-up. Specifically,  with low level of evidence, the authors advocate for one endoscopy after stopping PPI therapy, one before age 10 years, and one at transition to adulthood.”
  • High level evidence: Fundoplication has accepted role, despite dysmotility, in patients with recurrent anastomotic strictures and poorly controlled GERD despite maximal PPI therapy. Full evaluation prior to fundoplication is recommended.

Other Statements:

  • Low level evidence: the authors recommend regular multidisciplinary evaluation, the authors note that symptoms of aspiration during swallowing may be identical to GERD symptoms, and evaluation of dysphagia with EGD/UGI.
  • High level evidence: ENT evaluation may disclose other anatomic abnormalities
  • Low level evidence: The authors recommend that anastomotic strictures be excluded in symptomatic children and that there is no “evidence that routine screening and dilatation” is necessary in asymptomatic patients.
  • Low level evidence: Management of EA patients with eosinophilic esophagitis (EoE) should follow the treatment of EoE in non-EA patients.
  • High level evidence: Incidence of Barrett’s esophagus is increased in adults with EA.
  • No level evidence: Potential treatments for recurrent strictures: steroids, mitomycin C, stents, and endoscopic knife.

While some these recommendations noted above are based on low level evidence, it is worthwhile for experts to provide their opinions.  At the same time, there are some completely useless statements included in the guidelines, like those that suggest tailoring the treatment to the underlying problem.  For example: “Statement 19: We recommend tailoring management of post-fundoplication dysphagia to the underlying mechanism(s).”  Another useless statement: “Statement 21: 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.” (very low level evidence).  In my view, shortening the number of recommendations, mainly by eliminating the useless ones, would have been helpful.

My take: Despite my view that about 20% of the recommendations are useless (Statements 17, 19, 21, 25, 26A & 26B, 31, and 34), the overall guideline is helpful and it is still worthwhile for experts to provide their recommendations.

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Rockland Harbor Lighthouse

Rockland Harbor Lighthouse

Endoscopic Surveillance after Esophageal Atresia: Low Yield In Pediatrics

A recent study of patients who had undergone repair of esophageal atresia (Koivusalo et al. JPGN 2016; 62: 562-66) confirms that “routine endoscopic surveillance had limited benefit and seems unnecessary” before 15 years of age.

A retrospective review of pediatric patients with esophageal atresia (EA) from 1980-2014) identified 209 patients with 616 biopsies.  60 patients had undergone antireflux surgery. Key findings:

  • Gr I esophagitis was noted in 37%, Gr II or III in 16%.
  • Gastric metaplasia was found in 17% and reached a prevalence of 15% by age 15 years.
  • Only 9% of patients with gastric metaplasia and 32% of patients with gr II esophagitis were symptomatic.
  • No cases of dysplasia or cancer were identified.

My take: Conditions, including esophageal atresia, that predisposed to chronic reflux esophagitis increase the risk of esophageal malignancy; however, this risk remains very low in childhood.  Therefore, surveillance for asymptomatic children is not needed prior to age 15 years.

Related blog postNever quite right | gutsandgrowth

Vik Muniz recreation of Mary Cassatt painting

Vik Muniz recreation of Mary Cassatt painting

Up close, one sees this painting is actually a collage of images

Up close, one sees this painting is actually a collage of images

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.