Esophagus! & Nutritional/GI Outcomes with Esophageal Atresia

In 8th grade, our English teacher would say ‘Esophagus, recite that poem.’  He would say this to everybody.  It is possible that the flask that he carried could have influenced his word choice.

That anecdote came to mind as reading a recent article (K Birketvedt et al. J Pediatr 2020; 218: 130-7) which showed that a large number of adolescents with history of esophageal disease (esophageal atresia [EA]) had a high likelihood of suboptimal nutritional parameters. In this study with 68 pediatric subjects, extensive investigations including 4-day diet records, blood tests, pH measuring, EAT (eating assessment test) questionnaire, and interviews. Median age at follow-up was 16 years.

Key findings:

  • Median height-for-age Z score was -0.6. 10 patients (15%) were considered stunted (height-for-age Z score <-2).
  • More than two-thirds reported symptoms of dysphagia (EAT score ≥3) and avoided specific foods.
  • 48 (71%) had suboptimal energy intake on diet records –intake below age-appropriate recommendations.  Many had low vitamin D and iron intake.
  • 13 of 68 patients had VACTERL association and 17 (25%) had congenital heart disease

My take: If your esophagus is not working right, it definitely can impair one’s nutritional status.  Some patients have other reasons (other comorbidities) that could influence these reported results.

As for the anecdote, I still remember some of the poems we had to recite.  The one I remember best:

To An Athlete Dying Young  A. E. Housman – 1859-1936 

The time you won your town the race
We chaired you through the market-place; 
Man and boy stood cheering by, 
And home we brought you shoulder-high.  

To-day, the road all runners come,    
Shoulder-high we bring you home,  
And set you at your threshold down,  
Townsman of a stiller town.  

Smart lad, to slip betimes away  
From fields where glory does not stay, 
And early though the laurel grows  
It withers quicker than the rose.  

Eyes the shady night has shut  
Cannot see the record cut,  
And silence sounds no worse than cheers 
After earth has stopped the ears:  

Now you will not swell the rout  
Of lads that wore their honours out,  
Runners whom renown outran  
And the name died before the man. 

So set, before its echoes fade,  
The fleet foot on the sill of shade,  
And hold to the low lintel up  
The still-defended challenge-cup.  

And round that early-laurelled head
Will flock to gaze the strengthless dead,  
And find unwithered on its curls  
The garland briefer than a girl’s.

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How Bad is Reflux in Children with Esophageal Atresia?

A recent retrospective study (FWT Vergouwe et al. JPGN 2019; 69: 515-22) with 57 children with esophageal atresia (EA) found most children have a normal reflux index.

This study, analyzing data between 2012-2017, reviewed all 24-hour pH-impedance (MII) studies in children at ≤18 months and 8 year olds with EA.  “All children with EA born in our hospital are offered a 24-hour pH-MII study at the age of 0.5 years and 8 years.”  In this institution, PPI treatment is given for at least 6 months after surgery. Of the 57 in the cohort, 20 had completed pH-MII at <18 months of age and 32 at age 8 years.

Key findings:

  • In children ≤18 months of age, median reflux index was 2.6% (abnormal in 2), median number of retrograde boluses was 61 (62% nonacid, 58% mixed)
  • In the older cohort (~8 years of age), median reflux index was 0.3% (abnormal in 4) and median number of retrograde boluses was 21 (64% nonacid, 75% mixed)
  • Overall, 10 of 57 children (17.5%) had GERD with reflux index >7% (n=6) or positive SI/SAP (n=4).  The authors note that much higher rates of GERD have been found in prior studies.  If they included children with fundoplication who were considered as having GERD (prior to fundoplication), then the GERD rate was 32%.

My take: This study showed that reflux in this cohort of children with EA was similar to the general population and likely indicates that a substantial portion of patients with EA do not need indefinite PPI therapy.  In children with more complex EA, PPI therapy is likely to be more beneficial.

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Recent (November 4th) GI-Related Tweets:

Esophageal Disorders: POEM in Kids, Mitomycin C for Refractory Strictures

At our recent national meeting, Dr. Peter Kahrilas indicated that POEM (Per-oral Endoscopic Myotomy) was now the treatment of choice for most adults with achalasia (#NASPGAN19 Postgraduate Course -Part 3).

A Chone et al (JPGN 2019; 69: 523-7) provide recent multicenter retrospective data on POEM in the pediatric age group (mean age 14 years), n=117.

Key findings:

  • Clinical success, defined as Eckardt score ≤3 during followup, was achieved in 90.6% of cases. The Eckardt score was >3 in 5 (4.3%) and data was missing in 6 (5.1%)
  • Adverse events included 1 case with significant bleeding, 2 cases of aspiration pneumonia (related to anesthesia), 1 esopleural fistula (managed endoscopically), and 6 mild AEs (4 mucosomtomies, 2 subcutaneous emphysema)

Additional related blog posts:

D Ley et al (JPGN 2019; 69: 528-32) provide retrospective data on 39 patients, median age 19 months, with refractory esophageal strictures which were treated with mitomycin C.  The authors considered mitomycin C after a minimum of two previous dilatations.

Key findings:

  • Etiology: The majority had strictures/stenosis associated with esophageal atresia (n=25) followed by caustic ingestion in 9.
  • Number of stenosis: The majority (n=35) had a single stenosis.
  • In 26 patients (67%), topical application of mitomycin C was considered a success based on a reduction in the number of dilatations.  In this group, the number of dilatations dropped from 102 to 17 over a comparable period.
  • 16 (41%) never required further dilatation following mitomycin C application

My take: This study provides some of the best evidence that mitomycin C may be helpful.  Long-term followup and more studies are needed.

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Lincoln Park, Chicago

Esophagitis in Pediatric Esophageal Atresia

A recent study (JL Yasuda et al. JPGN 2019; 69: 163-70) shows that esophagitis is common with and without proton pump inhibitor (PPI) therapy in children with esophageal atresia (EA).

Background: This study encompassed 310 patients (34% long gap EA) and 576 endoscopies (median age 3.7 years)

Key findings:

  • Erosive esophagitis was found in 8.7% of patients.
  • 15.2% of patients had esophagitis with >15 eos/hpf; 49% of patients had ≥1 eos/hpf (histologic eosinophilia)
  • 87% of endoscopies were preceded by acid suppression therapy; being on acid suppression reduced the odds for abnormal esophageal biopsy (P=0.011).
  • Histologic esophagitis was “highly prevalent even with high rates of acid suppressive medications use.”
  • For example, among those receiving PPI monotherapy, 150 had normal biopsy and 136 had abnormal biopsy.  Among those off all acid suppression, 30 had normal biopsy and 45 had abnormal biopsy.
  • For erosive esophagitis, this occurred in 12 on PPI and was not present in 274 on PPI therapy. Among those off all acid suppression, 4 had erosive esophagitis and 70 did not.
  • Presence or integrity of fundoplication was not significantly associated with esophagitis.

While this is a large study, the findings have several limitations. This is a single center retrospective study and this center attracts highly complex cases of EA.

My take: In addition to fairly high rates of erosive esophagitis and eosinophilic esophagitis, this study shows a high incidence of microscopic esophagitis, the significance of this is unclear.   This study supports the current recommendations of 3 endoscopies in childhood and perhaps more frequent surveillance in those with more complex EA.

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Sign in Hood River, OR

 

How Long Should Be PPIs Be Used in Patients with Esophageal Atresia?

A recent study (FR Grunder et al. JPGN 2019; 69: 45-51) examines the use of proton pump inhibitor use after surgical repair of esophageal atresia; this involved a longitudinal cohort (n=73) with prospectively collected data over 11 years.

Background: While PPIs have been used for long-term treatment due to the high frequency of reflux and concerns regarding anastomotic strictures, the authors note that data on long-term outcomes/natural history and benefits/risks of this approach are lacking.

Key findings:

  • 48% of patients had PPIs discontinued at followup.
  • Among the 43 with PPI discontinuation, 40 had endoscopy results available.  Histologic abnormalities were noted in 8 (19%) which was lower than in the group receiving ongoing PPI use (n=19, 63%).. These 8 patients had PPI restarted.
  • Among patients unable to discontinue PPI therapy, there was a higher rate of prior anti-reflux surgical procedure, 27% compared to 5% who had anti-reflux procedure among group who were able to discontinue PPI therapy.
  • Patients more likely to remain on PPIs more frequently had a prior anastomotic leak and/or moderate to severe tracheomalacia.
  • The authors state that among patients receiving PPIs, there was more frequent recurrent pneumonia as well as more frequent use of inhaled beta-adrenergic agonists and steroids. However, this was not shown to be a causal association.  It is unclear whether these patients had more severe esophageal dysfunction or whether PPI use contributed to this outcome.

In their discussion, the authors note that PPIs have not been shown to reduce the rate of anastomotic strictures.  They argue that “PPI could be used more selectively in the following: in children with long-gap EA or anastomotic tension or anastomotic leak; after a first dilatation for anastomotic stricture rather than systematically, given the lack of preventive effect of PPI; and in children whose esophagoscopy demonstrates peptic esophagitis, eosinophilic esophagitis, or gastric metaplasia.”

My take: The authors are probably right that a large fraction of EA patients may not need long-term PPI use.  Selecting which patients will benefit will remain a challenge. Published guidelines recommend monitoring for GERD complications in EA, especially after stopping PPIs.

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University of Virginia

 

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