GERD is defined as a backward flow of stomach contents
up the esophagus and sometimes into the mouth. The stomach contains hydrochloric acid,
but is protected from acid injury by a mucosal lining. The
esophagus, throat, nose, and lungs have no such protection. As result, with
GERD, damage to the above structures can occur. It will worsen with
repeated exposures. The refluxate can cause tissue edema, ulcerations, granulation, glottic scarring,
especially of the posterior larynx, and ultimately airway compromise.
The refluxate can be a result of micro- or macro-aspiration, both causing
chemical injury, including chemical pneumonitis. The infant laryngeal epithelium
is thin and is particularly susceptible to caustic chemical injury.
Contributing to infant GERD is a short intra-abdominal esophagus and
immature lower esophageal sphincter (LES), which acts as a valve at the lower
end of the stomach. LES opens with swallowing, allows passage of stomach
contents into the small intestine and then closes. In the normal, healthy
infant, the LES pressure increases when sleeping. Also, there is a decreased
frequency of swallowing. In neurologically impaired infants this physiology
often is disturbed, making them predisposed to GERD. Premature infants also are
at higher risk for developing GERD.
The incidence of GERD is significant. Fifty percent of newborns have
reflux as a result of an immature LES. The majority of these infants have no
complications, with GERD resolving spontaneously by 1 year of age. However in
approximately 3% of infants with GERD, the problem can persist. It can adversely
affect development, quality of life, and overall health of the
infant.
Symptoms and associated conditions
The symptoms of GERD are myriad and they can be atypical. They can
include spitting up, vomiting, constant or sudden crying, colic, irritability,
pain, frequent hiccups, excessive drooling, chronic cough, hoarseness, and
halitosis. The infant may have poor sleep habits and frequent waking. Arching of
neck and back during or after eating (Sandifer syndrome) is associated with
GERD, but may sometimes be confused with seizures. Other less common symptoms
include refusing food or accepting only a few bites.
Anemia, recurrent ear infections, and sinusitis also may signal GERD.
Other conditions associated with GERD include sleep apnea, chronic bronchitis
and pneumonia, asthma, subglottic stenosis, and laryngomalacia. GERD can be
aggravated by laryngomalacia as a result of increased negative intrathoracic
pressure during inspiration in severe laryngomalacia patients. Also, the
presence of GERD can aggravate the laryngomalacia.
In addition to airway compromise, erosion of dental enamel may occur as a
complication of GERD. Reflux also may be a significant cause of failure to
thrive, and may contribute to the sudden infant death syndrome
(SIDS).
In some situations, during evaluation for other conditions, such as
failure to thrive, esophagoscopy with biopsy may reveal the presence of "silent"
GERD. In these cases, reflux is occurring, but without symptoms. The infant
appears comfortable and there is no emesis, but the refluxate is getting
swallowed. Potentially, silent GERD can be more damaging, since burns occur
while acid goes up and comes down.
It is important to be aware of another entity known as eosinophilic
esophagitis (EE), which can mimic GERD. It is found in approximately 10% to 15%
of children undergoing endoscopy for GERD symptoms. EE is an eosinophilic
inflammatory infiltration of the esophagus and does not respond to acid
suppression treatment. Treatment includes elimination diets or amino acid-based
formula. Occasionally steroids are required.
Evaluation
Obtaining a thorough, detailed history is critical. Although numerous
tests can be performed to evaluate and diagnose GERD, they can be uncomfortable,
expensive, and cumbersome. Often when GERD is suspected, pediatricians opt to
start with empiric therapy and follow closely the patient's response to
treatment. Testing for a definitive diagnosis usually is performed in more
severe cases that require hospitalization.
A pH probe over 24 hours will determine the degree and frequency of
reflux. This study is considered to be the gold standard for GERD evaluation.
Reflux medications must be discontinued for 48 hours before the test to obtain
the most reliable results. This test is often required prior to consideration
for a possible surgical treatment option.
Unfortunately, there is very little correlation between the clinical
symptoms and the presence of esophagitis. A barium swallow will show anatomical
abnormalities, but there is high incidence of false positives and negatives.
Direct endoscopy and biopsy can occasionally be helpful. There is poor
correlation between biopsy and endoscopic findings, however. Biopsies can be
positive despite a normal appearance of the esophageal mucosa. Esophageal biopsy
may be used to rule out silent GERD in patients with associated conditions, as
in some cases with persistent middle ear effusion requiring tube
insertion.
One useful method to measure the severity of reflux present is with the
Reflux Findings Score (see Table 1), which is based on clinical and exam
findings at laryngosocopy (see examples in Fig. 1, 2) and bronchoscopy. The
score quantifies evidence of subglottic edema, ventricular obliteration,
arytenoidal erythema/hyperemia, vocal fold edema, diffuse laryngeal edema,
posterior commissure hypertrophy, granuloma/granulation tissue, and thick
endolaryngeal mucus. A score of 11 or above is considered as evidence of reflux.
The highest score of 26 indicates severe GERD.
FIGURE 1: Laryngoscopy finding showing severe hemorrhagic and edematous
mucosal changes of the supraglottic and glottic area with airway compromise as
an effect of reflux.
FIGURE 2: Laryngoscopy finding showing erythema on both vocal cords with
small granuloma on left anterior cord as an effect of reflux.
Nuclear medicine GER/gastric emptying is often helpful as well. Tc-99
sulfur colloid mixed in 5 ounces of formula is administered. Sequential 1-minute
images are obtained over 1 hour. This study measures frequency of reflux
episodes over 1 hour and percentage of gastric emptying.
Treatment
There are numerous treatments for GERD. Simple lifestyle changes include
keeping the infant upright and motionless for half hour after feeding. If the
infant is still breast-feeding, the mother should avoid dairy, soy, wheat, nuts,
spicy or acidic foods, chocolate, carbonated beverages, and peppermint.
Thickening formulas with rice helps to keep the food down and reduce emesis.
Small and more frequent meals also are helpful. The infant should sleep propped
up and not be fed before sleeping. The full stomach will increase stomach
pressure on the LES. Pacifier use also may be considered, since it can result in
increased saliva production. Saliva is alkaline and may help neutralize refluxed
acid.
Medications include histamine H2 receptor antagonists (acid blockers),
such as ranitidine or cimetidine. Histamine binds to H2 receptors located in the
parietal cells of the stomach lining, which results in acid production. H2
receptor antagonists can block the acid production.
The next family of drugs is the proton pump inhibitors, such as
lansoprazole, omeprazole, iansoprazole. These drugs affect the K+/H+ATPase
system in parietal cells that produce acid. They can decrease acid production by
as much as 90%. This medication must be given half hour before meals.
Improvement is seen in approximately 2 weeks after the start of treatment
with either medication type. Acid blockers usually are selected as first-line
treatment due to cost considerations. Proton pump inhibitors can be tried if
acid blockers are ineffective.
Finally, the last group includes the prokinetic/ motility agents, such as
metoclopramide. They increase the speed of stomach contents emptying. They
stimulate and coordinate esophageal, gastric, pyloric valve and small intestine
peristalsis. They also increase LES tone and gastric contractions.
Unfortunately, they can have significant adverse effects, including involuntary
muscle spasms (tardive dyskinesia). These agents usually are reserved for more
critically ill children who are hospitalized. The best treatment is likely a
combination of proton pump inhibitors and prokinetic agents.
Surgical options are considered only for GERD that has proven resistant
to aggressive, maximal medical treatment. The most commonly performed procedure
is the Nissen fundoplication. The upper portion of the stomach (fundus) is
wrapped around the lower portion of the esophagus. The procedure creates a more
closed LES to prevent reflux, but still allows passage of food. It can be
performed through open surgery, through endoscopy, and most recently
robotically. The length of stay is about equal.
Nissen fundoplication is considered to be the most effective treatment
for GERD, but it is not a cure and it carries the highest risk. It should be
combined with G-tube placement when aspiration is a concern. The success rate is
about 57% to 92%. The incidence of complications is approximately 2.3% to 45%,
including complications associated with the general anesthesia, bleeding from
spleen and gastric vessels, esophageal tears with resultant leakage, pneumonia,
and mediastinitis. Slippage of wrap can occur resulting in failure, pain, or
dysphagia. Small bowel obstruction also has been reported.
Support for the family
Although the majority of infants with GERD grow out of it without
complications, the condition can cause overwhelming stress and exhaustion for
the family. The infants are constantly upset, usually with piercing screams, and
often are inconsolable. The family tends to experience feelings of guilt,
sorrow, or disappointment. Parents may develop a sense of isolation, since these
babies are frequently disruptive to people around them. The treatment of GERD is
expensive, resulting in financial pressures. There are also extensive time
demands with office visits, diagnostic testing and administration of
medications. Sometimes anger is expressed towards the treating physician. These
families require much support, understanding, and compassion from their health
care providers.
FOR FURTHER READING
[1.] Sears W, Sears M. Breastfeeding the baby with gastroesophageal
reflux. In: The Baby Book: Everything You Need to Know About Your Baby – from
Birth to Age Two. Boston: Little, Brown; 1993.
[2.] Davenport M, Davenport T. Making Life
Better for a Child with Acid Reflux. Church Hill, MD: SportWork, Inc; 2006.
[3.] Burns D, Shah N. 100 Questions & Answers About Gastroesophageal
Reflux Disease (GERD): A Lahey Clinic Guide. Boston: Jones and Bartlett; 2007.
[4.] MacLean R, McNeil J. Life on the Reflux Roller Coaster:
Gastroesophageal Reflux Disease in Infants and Children. Baltimore: PublishAmerica;
2003.
[5.] Silva AB. Airway manifestations of pediatric gastroesophageal reflux
disease. In: Wetmore R, Muntz H, McGill T, et al. eds. Pediatric Otolaryngology:
Principles and Practice Pathways. New
York: Thieme; 2000:619-634. |