CHALLENGES IN PEDIATRIC REFLUX ADVICE TO THE PRACTITIONER Masqueraders of GERD Scope of the Problem Gastroesophageal reflux (GER) and gastro­ esophageal reflux disease (GERD) are common in children and may be incompletely responsive to standard medical therapy. When this clinical scenario develops, other diagnoses must be considered. Clinical experience and the advent of endoscopy have identified a number of differ- ent diseases presenting with complaints formerly thought to fall under the umbrella of gastroesopha- geal reflux disease (GERD). These “masqueraders of GERD” include: • eosinophilic esophagitis • food allergic diseases • achalasia • cyclic vomiting syndrome • rumination syndrome The importance of recognizing these conditions lies in the fact that they require therapeutic mea- sures different than those used for GERD. Eosinophilic Esophagitis (EoE) Definition: Eosinophilic esophagitis ( EoE) is characterized by upper gastrointestinal symptoms and dense esophageal eosinophilia, both of which are unre- sponsive to acid blockade. induction and maintenance treatment. Potential problems are related to non-compliance. Clinical features: In the young child, symptoms include feeding Other off label treatment is directed at reducing refusal, vomiting and abdominal pain. Whereas in allergic inflammation with corticosteroids (systemic the teenager, dysphagia and food impaction are or topically – swallowing rather than inhaling the common. A personal or family history of allergic dose delivered by a metered actuation). diseases or peripheral eosinophilia can be absent When food/foreign body impactions are encoun- but is seen in 75% of patients. Upper gastrointesti- tered endoscopic disimpaction with or without nal series may reveal isolated strictures or longitu- esophageal dilation may need to be performed. CHILDREN’S DIGESTIVE dinal narrowing as a manifestation of longstanding HEALTH & NUTRITION FOUNDATION inflammation. Proximal strictures are seen more Prognosis: often than distal strictures. Abnormal features seen The natural history of EoE is unknown. Reports to at upper endoscopy include whitish exudates, date document the presence of esophageal stric- circular rings, longitudinal furrows, and splitting of tures in children and adults with EoE. No cases of NORTH AMERICAN SOCIETY FOR the mucosa. carcinoma have been reported to date. PEDIATRIC GASTROENTEROLOGY, HEPATOLOGY AND NUTRITION Diagnosis: Food Allergies Mucosal biopsy is required to make the diagnosis www.KidsAcidReflux.org of EoE. The histological hallmark of the disease Definition: www.TeensAcidReflux.org is a markedly increased number of eosinophils Mechanisms of food allergies include both non-IgE www.CDHNF.org (>20/ HPF) found in the squamous epithelium mediated inflammation (gluten intolerance), and www.NASPGHAN.org despite at least two months of proton pump inhibi- IgE mediated reactions (peanut anaphylaxis). In CDHNF National Office tion. The gastric and duodenal mucosa are nor- some instances, mixed reactions take place (eosin- P.O. Box 6, Flourtown, PA 19031 mal. Other causes for esophageal eosinophilia ophilic gastrointestinal diseases). Symptoms are 215-233-0808 YOUR SOURCE FOR PEDIATRIC include GERD, hypereosinophilic syndrome, relieved by the removal of the triggering food and REFLUX AND GERD INFORMATION inflammatory bowel disease, parasitic infection, reappear with the ingestion of the antigen. eosinophilic gastroenteritis,food/drug allergy Educational support for The CDHNF and collagen vascular diseases. Clinical Manifestations: Pediatric GERD Education Campaign Children with food allergies masquerading as was provided by Major Sponsor Treatment: GERD may present with vomiting and abdominal TAP Pharmaceutical Products Inc. Dietary elimination of specific food allergens or pain often associated with other complaints such To order more of this resource or the use of an elemental diet (amino acid based as hives, rash, wheezing, edema, bleeding or diar- other materials from CDHNF e-mail us at formula) is very effective and can be used for both rhea. When esophageal inflammation is present, [email protected] manifestations may be related to esophageal dys- out abdominal pain, interspersed with episodes of been described who exhibit the same clinical pat- motility with dysphagia, food impaction or feeding returning to a normal state of health. tern. This occurs more often in females, but it it is refusal (see EoE above). In patients with small not considered an eating disorder. Most individu- intestinal and/or colonic inflammation, diarrhea , Clinical Features: als with regurgitation syndrome, regurgitate with bleeding or protein losing enteropathy can occur. CVS can develop in young children, typically every meal. Typically, the regurgitation is effortless around age 5 or 6 years. The vomiting is intense and within 10-20 minutes. The patient may exhibit Diagnosis: and protracted, often 6 times per hour. Vomiting halitosis or complain of a sour taste rather than The diagnosis for food allergies lies in an excel- can be associated with abdominal pain, pre- discomfort or typical peptic symptoms. lent history and physical examination. Radioim- ceding and/or accompanying the episodes of munosorbent testing (RAST) or skin prick can be vomiting. The abdominal pain may also be associ- Diagnosis: abnormal for suspected foods. An elimination trial ated with nausea. However, the abdominal pain A careful history and identification of the recurrent, with or without re-challenge should also be con- and/or nausea are not always associated with the effortless, painless regurgitations is often sufficient sidered. If vomiting is a key symptom on presenta- vomiting episodes in all patients. These “stereo- to make the diagnosis. Observation of preceding tion an upper gastrointestinal series may reveal typical” episodes can last for hours or days. The air swallowing and contraction of the abdominal mucosal thickening. Endoscopic examination can same pattern often repeats with each attack. The wall (while the lower esophagus and glottis relax) detect ulcers, polyps and inflammation. attacks usually stop abruptly and the child quickly can clinch the diagnosis without need for involved and often unnecessary investigations. If avail- Treatment: resumes normal diet and activities. The bouts can occur during intercurrent infections, menstruation able, antro-duodenal manometry will document Food elimination is the treatment of choice for the presence of the diagnostic pressure pattern food allergic diseases. or at times of excitement or stress. The impact on family life and the child’s daily functioning and confirming the creation of a “common cavity” be- Prognosis: quality of life can be significant. While both GER tween the stomach and the mouth. The prognosis of food allergic diseases depends and CVS exhibit vomiting, the vomiting in the for- Treatment: on the allergen. Cow’s milk allergy in infancy is mer is milder and more constant, not as debilitat- Behavioral treatments are usually employed. In usually outgrown by one year of age however, ing and disruptive as in CVS. infants, this involves developmental stimulation. peanut allergy can be lifelong. In adolescents and adults, teaching patients Diagnosis: diaphragmatic breathing and other behavioral Achalasia The causes of CVS are not known. There is often a techniques seems effective in some, while the ru- positive family history for migraines. mination can be stubborn and difficult to dissipate Definition: in others. Determination of any underlying psychi- Achalasia is a motor disease that leads to ab- When doing a differential diagnosis it is crucial atric disorders and directing appropriate medical normal esophageal peristalsis and a functional to exclude raised intracranial pressure from any cause. Chronic sinusitis is sometimes implicated. and psychological therapy is important in achiev- obstruction of the distal esophagus from failure of Rare conditions such as midgut volvulus, ureteral ing successful treatment. the lower sphincter to relax. pelvic obstruction and metabolic causes such as Prognosis: Clinical Manifestations: mitochondrial disease or urea cycle abnormalities The recently reported Mayo Clinic experience Typically, achalasia is a condition seen in adults can present with bouts of intermittent vomiting. suggests a favorable prognosis in most. but can present during childhood (mean age around 9 years). Symptoms include vomiting, Treatment: dysphagia, food impaction, weight loss/failure to The mainstay of CVS management is prevention of thrive, nocturnal regurgitation, chest pain, cough suffering, dehydration, and ketosis once the attack and recurrent pneumonia. begins. Benzodiazepines and ondansetron may be useful adjuncts. Prophylaxis with cyprohepta- Diagnosis: dine, amytriptyline or propanolol may be effective Esophageal manometry remains the gold standard in some patients. Recognizing the pattern of the test for the diagnosis of achalasia. Suggestive vomiting and confirming the diagnosis of CVS is abnormalities include a widened mediastinum and extremely important and provides reassurance, air-fluid level on plain chest radiograph, proximal even though the attacks might not disappear. esophageal dilation and “beaking” on the barium Management can be more focused and measures CHILDREN’S DIGESTIVE swallow study; HEALTH & NUTRITION can be taken to prevent, anticipate, or effectively FOUNDATION Treatment: treat breakthrough attacks. Mechanical dilation is accomplished by esopha- Prognosis: geal balloons or bougienage. The injection of the Cyclic Vomiting Syndrome resolves in most chil- LES with Botulinum toxin temporarily relieves tonic NORTH AMERICAN SOCIETY FOR dren and many children will go on to develop PEDIATRIC GASTROENTEROLOGY, contraction. Esophageal myotomy is a permanent HEPATOLOGY AND NUTRITION more typical migraines by adolescence. solution but carries GERD as a potential complica- tion so fundoplication may be performed prophy- Rumination Syndrome www.KidsAcidReflux.org lactically. www.TeensAcidReflux.org Definition: www.CDHNF.org Prognosis: Rumination Syndrome consists of chronic regurgi- www.NASPGHAN.org Patients with achalasia can redevelop obstructive tation of partially digested food either ejected or symptoms even after effective treatment. This can CDHNF National Office re-swallowed. P.O. Box 6, Flourtown, PA 19031 be on the basis of recurrent achalasia or acid-re- 215-233-0808 lated stricture. Clinical Features: YOUR SOURCE FOR PEDIATRIC Rumination syndrome has long been observed in REFLUX AND GERD INFORMATION Cyclic Vomiting Syndrome infants and young children with developmental delays or in those institutionalized or understimu- Educational support for The CDHNF Definition: lated. In this setting, it has been considered a Pediatric GERD Education Campaign was provided by Major Sponsor Cyclic Vomiting Syndrome (CVS) is characterized form of self-stimulation. However, more recently, TAP Pharmaceutical Products Inc. by recurring bouts of intense vomiting, with or with- otherwise normal adolescents and adults have