Authors Accepted Manuscript

Gastroesophageal reflux disease in children

Douglas C. Barnhart

www.elsevier.com/locate/sempedsurg

PII: S1055-8586(16)30025-7
DOI: http://dx.doi.org/10.1053/j.sempedsurg.2016.05.009
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To appear in: Seminars in Pediatric Surgery
Cite this article as: Douglas C. Barnhart, Gastroesophageal reflux disease in
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http://dx.doi.org/10.1053/j.sempedsurg.2016.05.009
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#4

Gastroesophageal Reflux Disease in Children

Douglas C. Barnhart, MD, MSPH

Douglas C. Barnhart, MD, MSPH

Professor of Surgery, University of Utah
Primary Children's Hospital
Pediatric Surgery - Suite 3800
100 N Mario Capecchi Drive
Salt Lake City, UT
(801) 662-2950
Email: Douglas.Barnhart@imail2.org

Keywords: Fundoplication, GERD, gastroesophageal reflux, gastrostomy, neurological

impairment, gastrojejunostomy
Abstract

Despite the frequency with which antireflux procedures are performed, decisions about

gastroesophageal reflux disease treatment remain challenging. Several factors contribute to the

difficulties in managing gastroesophageal reflux. First, the distinction between physiologic and

pathologic gastroesophageal reflux (gastroesophageal reflux disease, GERD) is not always clear.

Second, measures of the extent of gastroesophageal reflux often poorly correlate to symptoms

or other complications attributed to reflux in infants and children. A third challenge is that the

outcome of antireflux procedures, predominately fundoplications, are relatively poorly

characterized. All of these factors contribute to difficulty in knowing when to recommend anti-

reflux surgery. One of the manifestations of the uncertainties surrounding GERD is the high

degree of variability in the utilization of pediatric antireflux procedures throughout the United

States.

Pediatric surgeons are frequently consulted for GERD and fundoplication, uncertainties

notwithstanding. Although retrospective series and anecdotal observations support

fundoplication in some patients, there are many important questions for which sufficient high-

quality data to provide a clear answer is lacking. In spite of this, surgeons need to provide

guidance to patients and families while awaiting the development of improved evidence to aid

in these recommendations. The purpose of this article is to define what is known and what is

uncertain, with an emphasis on the most recent evidence.

Definitions & Natural History

The definition of gastroesophageal reflux (GER) is involuntary retrograde passage of gastric

contents into the esophagus with or without regurgitation or vomiting. In infancy, the incidence

of GER is approximately 75%. Persistence and severity of symptoms distinguish GER from

gastroesophageal reflux disease (GERD). However, the distinction is often blurred. Many studies

document the natural history of GER in infants, with a peak incidence at 4-5 months of age.1

The rate of cessation of symptoms by one year of age in the literature ranges between 80 - 95%

of cases.24 There are identifiable high-risk groups for GERD, including those with prematurity,

esophageal atresia, congenital diaphragmatic hernia, neurologic impairment, obesity, and

specific genetic disorders.1

Physiology

Normally, the lower esophageal sphincter (LES) maintains a resting tone of about 12-25 mm Hg.

The LES normally relaxes briefly when there is a peristaltic wave. There are also longer duration

LES relaxations that last 5 30 seconds, during which the pressure in the esophagus is the same

as that in the stomach. These are called transient lower esophageal sphincter relaxations

(TLESRs), and they are the physiological cause of GER. TLESRs are independent of swallowing

and of relatively longer duration than the relaxation triggered by a swallow.2,5 They may not be

clinically noticeable if reflux is in the lower esophagus and volume clearance and peristalsis are

prompt. The LES tone in infants with GER is not different from normal but they do have more

TLESRs and/or TLESRs of longer duration.6

Clinical Presentation

Clinical presentations vary with age, and the symptoms of GERD are familiar to pediatric

surgeons. Clinical symptoms in infants are generally less reliable indicators of pathologic GERD

and correlate poorly with pH monitoring results and endoscopy and endoscopic biopsy

findings.6 There is no symptom or constellation of symptoms that are diagnostic of GERD or

that predict response to therapy in infants and toddlers. Although apnea and sudden infant

death syndrome are a significant cause of neonatal mortality, there is little evidence of a cause

and effect relationship between GER/GERD and infant apnea.7

Effortless vomiting, epigastric pain and dyspepsia, anemia, failure to thrive, and strictures may

be the presentation of GERD in older children. Airway problems such as laryngeal irritation,

chronic cough, or recurrent pneumonia may be seen. GERD and reactive airway disease are

both common, and may occur in the same patients (they are correlated). However, the degree

to which GERD causes or exacerbates asthma is a subject of controversy. Recent publications

have downplayed the impact of GERD on asthma control.810

Diagnostic evaluation

A 2013 review of the literature found poor data and low levels of evidence for the accuracy of

diagnostic tests for GERD in children.7 Routine upper GI radiography (UGI) in the evaluation of

GERD has been shown to have a low yield,11,12 and the 2009 consensus recommendations from
the North American and European societies, discussed in detail below, state that routine UGI is

not indicated for the diagnosis of GERD: the false-positive rate is high due to non-pathologic

reflux, and episodes of reflux are often infrequent, brief and easily missed.13 UGI may be used

to rule out other anatomic abnormalities mimicking GERD, but the yield is low if obtained for

screening purposes.11

More invasive studies include continuous pH-monitoring via a probe in the distal esophagus.

The pH study has historically been the most accurate test for GERD; a variety of scoring systems

are used, but the common elements include the number of significant pH drops (below 4 and

longer than 15 seconds duration), time required for the pH to return to normal (clearance), and

the number of reflux episodes with a clearance time of more than 5 minutes (longer episodes).

Other parameters may be measured to attempt to differentiate normal patients from those

with GERD. The correlation between clinical symptoms and reflux is often poor, even if the pH

score is abnormal. In addition, the test is expensive (requiring hospitalization in children),

invasive, and often impractical or impossible in complex patients. pH studies do not, of course,

demonstrate alkaline or neutral reflux, and the advent of multichannel intraluminal impedance

(MII) studies addresses this failing. There is some evidence that combined MII/pH studies may

better correlate with duration of GERD symptoms and provide prognostic information. 1416 In

older children with respiratory and laryngeal problems, addition of MII to standard pH studies

can increase the sensitivity of detecting GERD correlation with symptoms.1618 Combined

MII/pH studies are widely used, but as yet high quality evidence for the sensitivity, specificity

and utility in many clinical scenarios is lacking.19

Pediatric Gastroesophageal Reflux Clinical Practice Guidelines

The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

(NASPHGAN) along with its European counterpart ESPGHAN (European Society for Pediatric

Gastroenterology, Hepatology and Nutrition) published clinical practice guidelines in 2009.13

This manuscript provides an extensive review of available evidence on gastroesophageal reflux

in children and infants. It is important for pediatric surgeons to be aware of its contents, since it

is a consensus document from two large pediatric gastroenterology societies and provides a

systematic assessment of the evidence. The review is quite extensive, and the details of its

conclusions are beyond the scope of this article. However, a brief summary will be valuable for

surgeons.

To create the guidelines, nine pediatric gastroenterologists and two pediatric epidemiologists

were selected from the two participating societies. They then performed a systematic review of

the available literature. This review included 377 articles related to therapy and 195 additional

articles related to etiology, diagnosis and prognosis. Articles were evaluated using Oxford

Center for Evidence-Based Medicine Levels of Evidence and Oxford Grades of

Recommendation.20 All recommendations were based on consensus, and feedback from

society members was considered prior to finalization of the recommendations. It is reasonable

to believe that these recommendations represent the best evidence in 2009.

As mentioned, the extent of these recommendations is beyond the scope of this article but it is

worth mentioning items that are included in the guidelines. These include a discussion of

definitions and mechanisms. Discussion of diagnostic methods included history and physical

examination, esophageal pH monitoring, combined MII, motility studies, endoscopy and biopsy,

barium contrast radiography, nuclear scinitgraphy, esophageal and gastric ultrasonography

tests on fluids from the ear, lung and esophagus, and empiric trials of acid suppression.

Treatment modalities considered included lifestyle changes, pharmacologic therapies

(histamine2 receptor [H-2] antagonists, proton pump inhibitors, and prokinetic agents) and

surgical therapy. While it is of particular interest to surgeons, the discussion of surgical therapy

is relatively limited. Guidelines were then provided for the evaluation and management of

specific patient populations with suspected GERD. Finally, groups at high risk for

gastroesophageal reflux disease were considered. The manuscript begins with an initial three-

page synopsis of all of these areas. This provides an effective summary review, and all surgeons

are encouraged to read at least the synopsis.

These guidelines provide an effective starting point for a consideration of how to approach

gastroesophageal reflux disease in infants and children. Unfortunately, like many such

guidelines, recommendations are limited by the lack of high quality evidence. The clinician is

therefore left with many situations in which no sound guidance can be provided. This is
particularly the case with regards to surgical treatment of reflux. Much of the discussion in the

guidelines regarding surgical treatment of GERD is extrapolated from adult data.

Medical Treatment (briefly)

Thickened feedings have long been a recommended treatment for GERD. However, the efficacy

of this approach is limited; some have suggested that this has been driven in part by the baby

food industry. A review of 14 randomized controlled trials (RCTs) found that thickeners

significantly increased the percentage of infants with no regurgitation and slightly reduced the

daily frequency of regurgitation and vomiting, and increased weight gain per day.21 There was

no effect on many of the severity of reflux parameters. The authors concluded that thickeners

were only moderately effective.

Positional therapy is another longstanding treatment for GERD. A recent review from the UK

evaluated seven RCTs, with data on nine positions. They concluded that positional therapy

should not be used in sleeping infants (due to the risk of SIDS, the supine position is strongly

recommended even though GER is diminished when prone).22 Tummy time in the awake and

supervised infant is permissible, but positional therapy overall was not recommended. Another

recent RCT suggested a mild benefit (decreased emesis) with left lateral positioning. 23
Pharmacologic treatment for GERD primarily consists of prokinetic or motility agents and acid-

reduction therapy. Probably the most widely used H2-blocker is ranitidine, although little

benefit from one agent over another has been identified.22 A 2014 Cochrane review found

moderate evidence to support the use of PPIs (proton pump inhibitors), particularly in infants.

PPIs and histamine antagonists demonstrated moderate improvement in symptom scores, pH

indices and endoscopic/histological appearances in children with GER and GERD, but there was

a lack of data regarding their relative efficacy. 24

Surgical

Indications for operation

The definition of failure of medical management of GERD and the indications for fundoplication

are quite vague, reflected in the huge variation (75-fold) identified among childrens hospitals in

regard to rates of fundoplication.25

There has been a recent trend away from pediatric fundoplication,26,27 which is reflected in the

literature. The reasons for the shift are unclear: poor evidence or lack of good evidence

comparing fundoplication to the alternatives, better medical management with proton-pump

inhibitors28, advent of interventional radiologically placed gastrojejunostomy tubes 29, and

better understanding of the natural history of GER3 may all play a role. A 2014 review of 36

studies over the past 15 years found that the level and quality of the evidence supporting

laparoscopic fundoplication was extremely poor.30,31 A 2015 Cochrane meta-analysis concluded,

There is considerable uncertainty in the balance of benefits versus harms of laparoscopic

fundoplication compared to long-term medical treatment with proton pump inhibitors.32

Technical Aspects of Fundoplication:

New Insights from Randomized Controlled Trials and Administrative Database Studies

There have been four relatively recent RCTs addressing technical aspects of fundoplication.

Since RCTs are infrequent in pediatric surgery, they merit detailed discussion. Two trials

compared open to laparoscopic Nissen fundoplication.33,34 Another study was a single center

comparison of the efficacy of two types of laparoscopic fundoplication, Nissen versus Thal.35

The other RCT examined the effect of esophageal mobilization on postoperative transhiatal

migration of the wrap and the need for re-operation.36

Fyhn and colleagues in Norway conducted a two center RCT comparing open and laparoscopic

Nissen fundoplications.33 The primary endpoint was recurrence, defined by both symptoms

consistent with gastroesophageal reflux and objective evidence - with either a reflux index (RI)

greater than 4% on pH monitoring, or herniation of the wrap on UGI. Structured follow-up

included pH probe monitoring and a UGI study six months postoperatively. Telephone

interviews were also subsequently performed. They enrolled 87 children over a 6-year period,

with a median duration of follow-up of four years. There was a significantly higher rate of

recurrence in those undergoing laparoscopic fundoplication (37% vs. 7%, OR 2.5 95% CI 1.6-

16.6). However, it is important to note that the esophagus was circumferentially mobilized in all
cases. As discussed subsequently, this technique was shown in a different RCT to be associated

with a higher rate of recurrence.36 In patients with no evidence of recurrence, parental

assessment of the childs general well-being was similar between the two groups.

A second RCT comparing laparoscopic to open Nissen fundoplication was published in 2015.34

This study was focused on children under age two years of age and examined postoperative

length of stay as the primary outcome measure. Thirty-nine patients were enrolled over a

seven-year period. This study found that laparoscopic Nissen fundoplications were associated

with longer operative times (173 minutes versus 91 minutes). Length of hospital stay was not

significantly different, but surprisingly trended towards being longer in the laparoscopic group

(6 days versus 4 days). There was a 23% conversion rate from laparoscopic to open

fundoplication. It is difficult to know how generalizable this study is for several reasons. The

overall postoperative length of stay and operative times are longer the reported in

retrospective cohort studies.37,38 The conversion rate is also relatively high. Some of these data

may therefore represent the institutional learning curve; a possibility that the authors

hypothesize in their discussion.

Two large administrative database studies also examined whether there was a difference

between laparoscopic and open Nissen fundoplication. Both of these studies were published in

2011 and while they suffer from the typical limitations of administrative database studies, they

are of value. In particular they allow one to possibly detect an increased rate of rare but
serious events as well as allowing a broad survey of resource utilization. Rhee et al reviewed

twenty years of state inpatient databases to compare open and laparoscopic fundoplication.39

This study analyzed the records of 33,355 children who undergone fundoplication: 5,392 were

done laparoscopically. With this this large dataset they were not able to detect any increased

rate of complications with the laparoscopic approach, and in fact decubitus ulcers and sepsis

were increased in the open group. In-hospital mortality, length of stay and charges were less in

the laparoscopic group. Some caution should be used in interpreting these results given the

long period included in the study, potentially causing generalized secular trends in care to be

misattributed to a change in operative technique.

Fox et al. analyzed 7,083 fundoplication admissions from the PHIS database from 2005 to 2008.

56% of these were done laparoscopically. The laparoscopic approach was associated with

lower costs and a decreased rate of infectious and other surgical complications. There number

of fundoplications during the study period was stable, although the proportion done

laparoscopically increased.40

Multiple retrospective cohort studies have sought to examine potential outcome differences

between partial and complete fundoplications.41,42 Each of these has been plagued by similar

problems including probable patient selection bias. These have not shown consistent

differences in outcome between the techniques on systematic review.43,44 In 2011, Kubiak et al

reported an RCT with 175 patients enrolled over a 9-year period.35 These patients were
randomized to undergo either laparoscopic Thal or Nissen fundoplication. The primary endpoint

was failure, indicated by recurrence of symptoms severe enough to require revision surgery.

Secondary outcome measures included symptoms severe enough to prompt reintroduction of

medications. Other postoperative complications were also summarized. All of the operations

were performed by a single surgeon. The overall median follow-up was 30 months, with a

failure rate of 11%. Thal fundoplication was associated with a significantly higher reoperation

rate than Nissen fundoplication (15.9% vs 5.9%). The median time of reoperation was 16

months after the initial fundoplication. Only one of the 16 children requiring revision was

neurologically normal. Nissen fundoplication was associated with an increased need for

postoperative dilatation. However, an esophageal dilator was not placed at the time of

fundoplication. No difference was identified in regard to reinstitution of anti-reflux

medications.

The most common serious postoperative complication after laparoscopic Nissen fundoplication

is recurrent GERD due to transhiatal herniation of the wrap. A retrospective cohort study

suggested that minimization of esophageal dissection and esophagocrural fixation sutures

could reduce this complication.45 A two-center RCT compared extensive esophageal

mobilization with minimal esophageal mobilization during laparoscopic Nissen fundoplication.

The primary endpoint was transhiatal migration of the wrap at one year as demonstrated by

UGI contrast study. Reoperation and GERD symptoms and dysphagia were also assessed. 177

patients were randomized over a 29-month period. The study was stopped early because

interim analysis showed a significantly increased incidence of transhiatal herniation in the

maximal dissection group at one year (30% vs. 7.8%). The concomitant revision surgery rate

was also significantly increased (18.4% vs. 3.3%). There was no difference in other reflux

complications between the treatment groups. Subsequently, one of the two centers performed

a long term follow-up of their patients.46 82 of the patients were contacted for long-term

follow-up with a median of 6.5 years. There was an increase in the rate of herniation noted in

both groups but the minimal dissection group remained threefold less. Only one additional

patient in each group required reoperation, for a total reoperation rate of 30% in the maximal

dissection group and 8% in the minimal dissection group.

Postoperative complications

The overall recurrence rate for symptomatic reflux after fundoplication varies in the literature,

ranging widely, but is generally around 5-15%. A 2013 series of over 2,000 laparoscopic Nissen

fundoplications identified a primary failure rate of 4.6%, increased in those under 6 months of

age; the most common causes of wrap failure overall were hiatal hernia and slippage of the

wrap.38 Higher fundoplication failure rate in younger children were reported by other authors

as well,47,48 but conflicting reviews have found that complication rates after fundoplication in

small children or infants did not differ from that of older children.37,49
Specific Patient Populations

Children with Neurological Impairment

Neurologically impaired (NI) children account for nearly half of the fundoplication procedures

performed,26 although the number of antireflux procedures overall, as well as the percent

performed in NI children appear to be declining.27,50 Infants and children with NI are at

significantly increased risk of pathologic gastroesophageal reflux,51,52 as are children who have

specific neurologic conditions, such as Down syndrome or Cornelia de Lange.53 The increased

rate of pathologic GER is multifactorial. First, children with NI often have decreased

gastrointestinal motility, with delayed gastric emptying and poor esophageal clearance. This

results in an increased tendency for reflux to occur and for the episodes to be longer-lasting.

They also frequently have a increased gag reflex, and symptoms are often exacerbated by

constipation. Secondary factors such as prolonged supine positioning and spasticity may also

aggravate GER. Importantly, many of these children have abnormal swallowing and inadequate

ability to protect their airway. Therefore, GER can result in aspiration pneumonias. Due to this

tendency to aspirate, quantitatively normal amounts of reflux become pathologic due to the

consequences.

Proton pump inhibitor therapy is effective at symptom control and healing and treatment of

esophagitis.54 However, acid suppression has not been demonstrated to be effective in

decreasing the volume or frequency of reflux, or in controlling pulmonary complications.

Gastrostomy tube placement is frequently required in these children, and has been implicated
in increasing the risk of pathologic GER.55,56 It is therefore common for fundoplication to be

performed at the time of gastrostomy tube placement in NI children. In a study of 42 childrens

hospitals, the performance of concomitant fundoplication at the time of gastrostomy

placement was highly variable between hospitals, ranging from a few percent to almost 80%.25

Additionally, NI infants and children appear to be at increased risk of postoperative

complications including recurrent gastroesophageal reflux.35,48,57 Other studies have not

identified neurologic impairment as a risk factor, however.58,59

There has been a trend towards the use of gastrojejunostomy (GJ) tubes for NI children and

away from fundoplication, 29 although one has not been shown to be superior to the other. 60

Use of GJ tubes and drip feeding regimens may trade one set of complications for another - one

comparative review found an increase in small bowel obstruction and intussusception in the GJ

group (28%), versus a 36.8% rate of retching, 12.7% incidence of dysphagia, and an 11%

reoperation rate in the (open) fundoplication and gastrostomy group.29 Another study

demonstrated a higher minor complication rate in the GJ group.61 GJ tubes require frequent ED

and clinic visits for mechanical problems (dislodgement, breakage, blockage) the reported

rates vary from 1.68 to 2.75 visits/year.29,62,63 An alarming incidence of perforation and major

morbidity/mortality has been reported with GJ tubes in small infants.62,63 A seven-fold increase

in the incidence of perforation was noted for infants < 6 months old or < 6 kg in one of the

reports, involving 142 infants treated at a tertiary care childrens hospital.63

There has recently been an increased focus on assessing the effectiveness of anti-reflux

procedures in NI children. In 2011, researchers from the Hospital for Sick Children in Toronto

published a structured qualitative study designed to characterize the impact of fundoplication

and gastrostomy tube placement on well-being and quality of life in NI children.31 This study

included in-depth interviews of parents of children with severe NI who had undergone either

fundoplication or gastrojejunal feeding tube placement. Caregivers had constant concerns

about their childs health including pulmonary disease, vomiting, gastroesophageal reflux,

feeding, infections, and pain. They reported that these health issues were difficult to anticipate,

of high intensity and urgency, and caused a sense of social isolation. All parents identified a

significant impact on the family as a whole. However, the parents noted that fundoplication

appeared to help their children in multiple domains including feeding tolerance, feeding

duration and caregiving intensity. While this was a qualitative study and does not provide

definitive evidence of the benefits of fundoplication, it does suggest important outcome

measures for future studies.

Additionally, a recent prospective cohort study including children with NI demonstrated that

the majority of parents felt that their childs overall condition was improved with Nissen

fundoplication. The parents of only 2 of 46 NI children would not have chosen fundoplication

again.59
Three large database studies have investigated whether or not Nissen fundoplication decreases

the rate of reflux-related hospitalizations in children with NI. Goldin et al examined Washington

state data,51 which included 1,142 patients who underwent antireflux procedures. Both

neurologically normal and impaired children were included. Three age groups were analyzed (1

year, 1-3 years, and 4-19 years). Both of the two younger age groups had decreased rates of

reflux-related hospitalizations in both neurologically normal and in NI children. In contrast,

older children with NI actually had an increased rate of admission after an anti-reflux

procedure.

A similar study based on the Pediatric Health Information System (PHIS) database examined the

impact of fundoplication on reflux-related admissions in children with NI.64 This study used data

from 42 childrens hospitals and included 6,716 children with NI who underwent

fundoplication. The cohort was followed for one year. Similar to Goldin et al, the authors found

a significant reduction in reflux-related admissions in NI children who underwent fundoplication

in the younger age groups (< 36 months). A reduction in reflux-related hospitalizations was not

demonstrated in older age groups. Most admissions were driven by pulmonary events. A

second PHIS-based study examined whether concomitant fundoplication at the time of

gastrostomy decreased the rate of reflux-related admissions in infants with NI.25 This cohort

included 2,759 infants who underwent GT placement only, and 1,404 who underwent

concomitant fundoplication. Despite propensity score matching, there was no reduction in

reflux-related hospitalizations in the fundoplication group. It is possible that this study failed to

detect a treatment effect due to an inability to adequately control for clinical factors that
prompted selective use of fundoplication, such as preoperative tolerance of nasogastric

feedings.

When considered as a whole, these studies suggest that young children with NI who undergo

fundoplication have decreased reflux complications severe enough to prompt hospitalization.

This effect was not strong enough to demonstrate when considering all infants with NI

undergoing gastrostomy placement. Additionally the effect of decreasing hospital admissions

was not seen in older children. The effectiveness of fundoplication in improving the quality of

life of children with neurological impairment and that of their caregivers needs to be studied in

a more systematic fashion. Case series suggest improved quality of life after fundoplication but

these studies are not methodologically rigorous. Characterization of the benefits and risks of

this common procedure is imperative, given the significant burden of morbidity suffered by

these children.

Children with Congenital Diaphragmatic Hernia

Infants born with congenital diaphragmatic hernia (CDH) frequently have more severe GER.

This is likely due to the altered esophagophrenic attachments and disruption of the angle of His.

A large discharge database study showed that 18% of infants with CDH underwent

fundoplication during the initial admission.65 There are no similar long-term large cohorts to

describe the prevalence of GERD requiring operation in CDH patients.

Single center studies do provide insights into the extent of GERD associated with CDH. Two

studies provide sufficient detail to merit specific consideration. Koivusalo et al reported a series

of 33 patients from Finland who underwent CDH repair.66 While not a particularly large series, it

provided longitudinal follow-up with scheduled assessments of GERD symptoms at 6 months, 1

year, 3 years, 5 years and 10 years. Routine endoscopy and pH monitoring was performed at

one year. Significant GERD (defined as moderate esophagitis, RI greater than 10% or need for

fundoplication), was present in 27% at 1 year and increased to 42% at 3 years. Only 15%

required fundoplication. Additionally, symptoms seemed to attenuate with time.

Kawahara and colleagues provided additional insight into GERD after CDH repair with a detailed

evaluation of 52 CDH survivors.67 All of these children underwent pH monitoring and 16

underwent videomanometry. Monitoring of pH was done during early infancy (at a median of

six weeks of age). The RI was less than 10% in 58% of infants and none required treatment for

GERD. Thirteen children (25%) had an RI of 10-20%; three were treated with H-2 blockers. Only

one child underwent fundoplication (RI 28%). Videomanometry showed LES tones ranging from

15 to 35 mm Hg. Swallow-induced peristalsis and LES relaxation was demonstrated in all but

one child (who had an RI of 44%). This study supports a conservative approach to GERD after

CDH repair; most children were successfully managed without fundoplication. Importantly, they

observed that many patients symptoms decreased by three years of age.

In contrast to this conservative approach, surgeons in Germany performed an RCT to evaluate

concomitant fundoplication at the time of CDH repair.68 79 patients were randomized to either

CDH repair with concomitant Thal fundoplication or CDH repair only. Interestingly, families

were blinded to the type of operation until the child was two years of age. Standardized GER

questionnaires were completed by families. At the time of discharge, the CDH repair-only group

had more GER symptoms and this difference persisted at six months of age. However,

symptoms improved over time and by 24 months the groups were equal, with 20% having GER

symptoms. Children who underwent the Thal fundoplication were more likely to have a

reoperation for GER than the control was to undergo an initial procedure (22 % vs. 7%). There

were no differences in growth detected between the groups. The evidence from this study

argues against routine fundoplication at the time of CDH repair and encourages watchful

waiting, as symptoms improved with time.

Esophageal Atresia

Patients with esophageal atresia constitute an at risk group for GERD. These children are

particularly problematic since they may have small stomachs (microgastria) from the lack of

stimulatory secretions in utero, poor esophageal motility with reduced ability to clear refluxate

or secretions from the esophagus, and disordered anatomy from the anatomic defect (short

esophagus, abnormal angle of His). A complete wrap may decrease reflux but exacerbate

swallowing problems. For these reasons, many authors have favored partial wrap

fundoplications (Thal, Toupet) in these children.42,69,70 A comparison of 47 children with prior

esophageal atresia repair who had a partial wrap (n=31) or a 360-degree wrap (n=16), found

that long-term symptom and medication free recovery was more common (52% versus 13%) in

the partial wrap group.42

Conclusion

The evaluation and management of esophagogastric reflux in children is a complex and at times

controversial topic. Although there is a surfeit of literature and extensive clinical experience

over many decades, a surprisingly, and disappointingly, small amount of high-level evidence is

available to guide the clinician. Recent years have provided an increasing number of high-

quality randomized studies and some long-term clinical and quality of life studies. There have

also been substantive improvements in the diagnostic and pharmacologic armamentarium and

refinement of the surgical techniques available.

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