G1A. INFANT REGURGITATION

Regurgitation is the involuntary return of previously swallowed food or secretions into or out of the mouth. Regurgitation is distinguished from vomiting, which is defined by a central nervous system reflex involving both autonomic and skeletal muscles in which gastric contents are forcefully expelled through the mouth because of coordinated movements of the small bowel, stomach, esophagus, and diaphragm. Regurgitation, vomiting, and rumination are examples of gastroesophageal reflux. When the latter causes or contributes to tissue damage or inflammation (e.g. esophagitis, obstructive apnea, reactive airway disease, pulmonary aspiration, or failure to thrive), it is called gastroesophageal reflux disease.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

Prematurity, developmental delay, and congenital abnormalities of the oropharynx, chest, lungs, central nervous system, or gastrointestinal tract are considered risk factors for gastroesophageal reflux disease. Accompanying systemic conditions (milk allergy) may be suspected when eczema is present; an abnormal neurological examination should prompt further investigation. A diagnosis of gastroesophageal reflux disease should be evoked in the presence of failure to thrive, hematemesis, occult blood in the stool, anemia, or refusal to eat. However, chronic regurgitation, early satiety, refusal of food, and excessive crying, sometimes leading to failure to thrive, may be the physical consequences of emotional distress.9

TREATMENT

Since infant regurgitation is a transient problem, possibly due in part to the immaturity of gastrointestinal motility, treatment goals are to provide effective reassurance and symptom relief. Symptoms often improve with prone positioning after meals,10 formula thickened with cereal,11 smaller volume feedings, and drugs that improve motility such as cisapride.12

G1B. INFANT RUMINATION SYNDROME

Rumination is a rare disorder characterized by the voluntary, habitual regurgitation of stomach contents into the mouth for self-stimulation.13

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

Observation of the ruminative act is all that is needed to make the diagnosis. This might be difficult as rumination may cease as soon as the infant notices the observer. Parents will not provide the information spontaneously, but when asked about the possibility of stereotypical behavior, they will recognize it. The infant who ruminates may not retain enough nutrients and may develop potentially lethal malnutrition, a complication that seldom, if ever, occurs in older ruminators.13 Sensory and/or emotional deprivation characterize the child with rumination. This explains why the disorder occurs in institutionalized children and infants in intensive care units, as well as in normal infants from emotionally distant mothers.

TREATMENT

Treatment efforts should be directed toward caregivers as well as the child. In infant rumination, the relationship between the infant and caregiver is devoid of enjoyment. The goal of therapy is to provide a nurturing environment and comforting care to the infant, and to help the mother change her feelings towards herself and her infant. This can be achieved by improving her ability to recognize and respond sensitively to her infant’s physical and emotional needs. In mentally handicapped children, providing a nurturing caregiver may not be sufficient; behavioral therapy involving positive reinforcement and possibly aversive techniques may be necessary.14

G1C. CYCLIC VOMITING SYNDROME

Cyclic vomiting syndrome consists of recurrent, stereotypical episodes of intense nausea and vomiting lasting hours to days, which are separated by symptom-free intervals. The frequency of episodes averages 12 per year (range 1–70) and intervals between attacks may be fairly regular or sporadic. Episodes tend to begin at the same time of day, usually during the night or early morning. The duration of episodes tends to be the same in each patient over months or years.15 Once vomiting begins, it reaches its highest intensity during the first hours.16

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

Typically, cyclic vomiting occurs in a child aged 2–7 years (with a possible range from infancy to mid-life), who is free of vomiting between episodes. However, like their family members, these children often complain of migraines, motion sickness, and other functional bowel disorders. During vomiting episodes, accompanying signs and symptoms include pallor, weakness, increased salivation, abdominal pain, intolerance to noise, light or odors, headache, diarrhea, fever, tachychardia, hypertension, skin blotching, and leukocytosis. A triggering factor (emotions, infection) can be identified in 80% of cases. Differential diagnosis should include brain stem tumors, obstructive uropathy, peptic disease, recurrent pancreatitis, intermittent bowel obstruction, chronic intestinal pseudo-obstruction, and familial dysautonomia. Among metabolic and endocrine diseases, those which may mimic cyclic vomiting include pheochromocytoma, adrenal insufficiency, diabetes mellitus, urea cycle enzyme deficiency, medium-chain acyl coenzyme A dehydrogenase deficiency, proprionic acidemia, and porphyria.

TREATMENT

Emotional conditions which trigger episodes should be identified and treated. When episodes are frequent and severe, prophylactic daily treatment with cyproheptadine, amitriptyline, erythromycin, phenobarbital, sumatripan, or propanolol may succeed in reducing frequency or eliminating episodes.17-19 In some children with a recognizable prodrome, oral medications such as ondansetron, erythromycin, and ibuprofen may be useful prior to onset of nausea. In patients whose episodes cannot be prevented, it may be helpful to begin, as early as possible, an oral acid inhibiting drug to protect esophageal mucosa and dental enamel, and lorazepam for its anxiolytic, sedative, and anti-emetic effects. Intravenous ondansetron, granisetron, diphenhydramine, and chlorpromazine may also be helpful and will sometimes interrupt the crisis. The presence of electrolyte deficits, hypertension, and/or inappropriate secretion of antidiuretic hormone should be detected and treated.

G2A. FUNCTIONAL DYSPEPSIA

Dyspepsia refers to pain or discomfort centered in the upper abdomen (see also functional dyspepsia in adults). Discomfort may be characterized by fullness, early satiety, bloating, belching, queasiness, nausea, retching, or vomiting. These symptoms typically include a component of upper abdominal distress.24

DIAGNOSTIC CRITERIA

Functional dyspepsia has not been rigorously defined in a pediatric population. Therefore we have adopted the adult diagnostic criteria for use in children.

The concept of subdividing functional dyspepsia based on distinctive features may be useful. These divisions seem especially relevant to those proposing to study this FGID.

G2A1. ULCER-LIKE DYSPEPSIA

G2A2. DYSMOTILITY-LIKE DYSPEPSIA

G2A3. UNSPECIFIED (NON-SPECIFIC) DYSPEPSIA

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

In children mature enough to provide an accurate pain history, the clinician conducts an interview which includes queries about dietary, psychological, and social factors. The clinician determines whether symptoms are likely to represent mucosal disease (esophagitis, gastritis, duodenitis, ulcer). Demographic and familial data should raise suspicion of Helicobacter pyloriinfection.25 Endoscopy will confirm or eliminate these diagnoses. A previous episode of viral infection may suggest post-viral gastroparesis.26

Measuring serum amylase, lipase, and aminotransferase concentrations, as well as performing an abdominal ultrasound, aid in assessing the presence of pancreatic, liver, or biliary disease. In functional dyspepsia, physical examination and growth are normal and there are no signs of inflammatory bowel disease.

TREATMENT

There are no controlled treatment trials for functional dyspepsia in children. Medication and food known to aggravate symptoms should be discontinued. Histamine receptor antagonists, proton pump inhibitors, sucralfate, and low dose tricyclic antidepressants have been used. Prokinetic drugs such as cisapride may be helpful for feelings of fullness and metoclopramide may alleviate nausea. If psychological stress aggravates symptoms, the clinician and family should collaborate on a plan to reduce the stress.

G2B. IRRITABLE BOWEL SYNDROME

In IBS (see also criteria for irritable bowel syndrome in adults) abdominal discomfort or pain is associated with defecation or a change in bowel habit, with features of disordered defecation. Ten to 20% of adolescentsand adults have symptoms consistent with IBS.22

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

A history which fits the Rome criteria for a diagnosis of IBS, accompanied by a normal physical examination and growth, is consistent with a diagnosis of childhood IBS. A nutritional history, assessing for adequacy of dietary fiber in those with constipation, as well as ingestion of sugars such as sorbitol and fructose in those with diarrhea, is often useful. Factors alerting the clinician to the possibility of disease include nocturnal pain or diarrhea, weight loss, rectal bleeding, fever, arthritis, delayed puberty, and a family history of inflammatory bowel disease. Limited laboratory screening for disease is frequently reassuring to the clinician, patient, and family. Screening may include a complete blood count and erythrocyte sedimentation rate, stool studies for enteric bacterial pathogens and parasites, and breath hydrogen testing, or a trial of a milk-free diet for lactose malabsorption. During the initial visits, and after establishing rapport with the patient and parents, the clinician inquires about the psychosocial history of the child and family. In patients with intractable symptoms endoscopic evaluation of the colon may be indicated (e.g., colonoscopy and biopsy). If the terminal ileum is not intubated, it may be imaged with small bowel series or white blood cell technetium scan. IBS may occur concurrently in patients with inflammatory bowel disease.

TREATMENT

Once there is a confident diagnosis of IBS, treatment goals are to provide effective reassurance and symptom relief. The presence and severity of the pain should be acknowledged. The clinician must educate and reassure the child and family that although IBS causes discomfort, it is not a serious disease. A review of the current understanding of IBS and the exacerbating effects of stress and anxiety on the problem helps the child and family to understand why the pain occurs.27 Psychosocial difficulties and triggering events for symptoms should be identified and tackled.

Drug therapy plays an adjunctive role in treatment. Tricyclic antidepressants such as imipramine or amitriptyline in low doses improved symptoms in adults with IBS in controlled, blinded studies.28 ,29 These drugs have been used for decades for chronic visceral pain by pain management specialists, but there are only anecdotal reports concerning their use in children with chronic abdominal pain. As amitriptyline has greater sedative and anticholinergic effects than imipramine, amitriptyline may be a better choice for children who wake up at night with pain, or for children with a diarrheal component to their disorder. Imipramine may be a better choice than amitriptyline for children with constipation; at the low doses recommended for chronic visceral pain, chronic constipation is rarely exacerbated. Anticholinergic medications such as dicyclomine, hyoscine, mebeverine, and octylonium have been used for their antispasmodic properties. There are no well designed studies confirming efficacy. In those with constipation, increased dietary fiber (recommended daily fiber intake = age (years) + 5 g), milk of magnesia, or mineral oil may be a helpful adjunct.

G2C. FUNCTIONAL ABDOMINAL PAIN

It should be noted that the term “functional abdominal pain” is not a substitute for Appley’s “recurrent abdominal pain of childhood.” In some children with functional abdominal pain, the symptoms do not meet the diagnostic criteria for IBS or functional dyspepsia. The pain is usually periumbilical and does not relate to any specific activity; it may keep the patient from sleeping but rarely awakes the patient from sleep. Some of these children are perfectionists, whereas others often have unrecognized learning difficulties; generally, however, the patients or their parents often have high expectations of achievement.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

The child may also complain of headache, light-headedness, dizziness, nausea (without vomiting), and fatigue. Psychological factors which should be examined include anxiety and/or depression in the child and family, somatization, school phobia, separation anxiety, and secondary gains for illness behavior. Physical examination, growth, and laboratory tests are normal (urinalysis, erythrocyte sedimentation rate, complete blood count, stool examinations for occult blood, leucocytes, ova and parasites, blood chemistries, abdominal ultrasound, and breath hydrogen testing). When symptoms are intractable, further evaluation for organic disease may become necessary, using radiography and endoscopy.

TREATMENT

Effective reassurance and an explanation for how symptoms occur in the absence of laboratory abnormalities (see earlier) help to establish a therapeutic alliance between the family and the clinician. Psychological support for personal, familial, and school difficulties should be offered when necessary. The best guarantee for success consists of accompanying the children through difficult periods of adjustment and reevaluation whenever symptoms change. The use of a diary to record symptoms, thoughts, and feelings helps the child become involved in his own healing process.

G2D. ABDOMINAL MIGRAINE

Abdominal migraine is a paroxysmal disorder affecting about 2% of children and characterized by acute, incapacitating, non-colicky, midline abdominal pain that lasts for hours and is accompanied by pallor and anorexia. Associated features include personal and family history of migraine headache.30 ,31

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

When accompanied by a history of migraine headaches, the diagnosis is straightforward; otherwise it should remain presumptive. All other causes of intermittent severe abdominal pain should be considered, including obstructive uropathy, intermittent bowel obstruction, recurrent pancreatitis, biliary tract disease, intracranial space-occupying lesions, familial Mediterranean fever, and metabolic disorders. The diagnosis of abdominal migraine is further supported by a favorable response to medications used prophylactically for migraine.

TREATMENT

Pizotifen, a serotonin receptor antagonist unavailable in the United States, provides effective prophylaxis.32Cyproheptadine is effective in some patients with migraine headaches and cyclic vomiting syndrome18 and might also be helpful in the prophylaxis of abdominal migraine.

G2E. AEROPHAGIA

Aerophagia consists of excessive air swallowing causing progressive abdominal distension.34 Abdominal discomfort is often such that children limit their food intake. This explains why the committee decided to include aerophagia among the abdominal pain disorders.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

Aerophagia often goes unnoticed by parents. The physician will observe repeated audible swallows, and inquire about anorexia, abdominal pain, excessive flatus, and excessive burping. The symptoms and abdominal distension resolve during sleep.33Aerophagia may be confused with gastroesophageal reflux disease (because of noises in the throat), and with generalized motility disorders such as chronic intestinal pseudo-obstruction or Hirschsprung’s disease (because of associated abdominal distension). A normal physical examination and growth history help exclude disease. Breath hydrogen testing may be useful. It is important to ask about stressful life events because anxiety is a frequent cause of excessive swallowing.

TREATMENT

Treatment consists of effective reassurance and explanation of the symptom for the parents and child. Often the clinician can help the child become aware of swallows during the visit. Excessive use of chewing gum or carbonated beverages should be discouraged. Stress and anxiety problems should be promptly addressed.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

The clinician queries about causes of chronic diarrhea, including enteric infections, ingestion of laxatives, urinary tract infection, and antibiotics. A diet history assesses overfeeding, excessive fruit juice or sorbitol consumption, excessive carbohydrate ingestion with low fat intake, and food allergens. In the absence of failure to thrive, a malabsorption syndrome is unexpected.

TREATMENT

It is important to avoid restrictive diets which may induce caloric deprivation.34 In functional diarrhea, a meal fails to interrupt the migrating motor complex.35 Children recover spontaneously, and usually no treatment is necessary. Effective reassurance for parents is important. A daily diet and defecation diary helps to reassure them that specific dietary items are not responsible for the symptom.

G4A. INFANT DYSCHEZIA

Infants with dyschezia strain and scream during prolonged attempts to defecate. This behavior persists for up to 20 minutes, until soft or liquid stools pass. This may be repeated several times a day and occurs during the first few months of life. Symptoms resolve spontaneously in a few weeks.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

We speculated that this disorder occurs when neonates fail to coordinate increased intraabdominal pressure with relaxation of the pelvic floor. Thus, the complete history (including diet), physical examination (including rectal examination to exclude anorectal abnormalities), and chart of the infant’s growth are normal.

TREATMENT

Parents are reassured by a systematic physical examination completed in their presence. The disorder will resolve when coordination between increased abdominal pressure and relaxation of the pelvic floor is acquired. To avoid perpetuation of dyschezia, maneuvers of rectal stimulation, which produce artificial and potentially noxious sensory experiences, should be discouraged.

G4B. FUNCTIONAL CONSTIPATION

Defecation disorder represents the chief complaint in 3% of pediatric outpatient visits and 10–25% of pediatric gastroenterology visits. Few reports distinguish between functional constipation and functional fecal retention, so the frequency of functional constipation is unknown.39 Children with cerebral palsy often develop functional constipation.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

In most cases, a thorough history and physical examination are sufficient to diagnose functional constipation. Delayed passage of meconium in a full term newborn raises the suspicion of Hirschsprung’s disease.40 In a few healthy breast fed infants there may be weeks between bowel movements but the stools are soft.41 The transition from breast to formula feedings is often associated with the appearance of functional constipation. However infants on formula feedings and children who pass stools at intervals greater than a week apart and fail to thrive are likely to have an enteric neuromuscular, anatomic, or metabolic disease. Ingestion of medication causing constipation needs to be excluded.

At physical examination, inspection of the back and spine and verification of deep tendon and cremasteric reflexes help to exclude occult spinal dysraphism. At least one anorectal examination is performed allowing evaluation of anatomy, tone, and masses. Laboratory and radiologic examinations are not warranted in the absence of poor weight gain, persistent abdominal distension, fever, Down syndrome, bilious vomiting, or any abnormal physical findings mentioned above. Functional constipation is important because it may predispose to the development of functional fecal retention.

TREATMENT

In infants, fruit juices which contain fructose and sorbitol such as prune and pear increase stool water. Barley, corn syrup, lactulose, and sorbitol can also be used, but mineral oil is not recommended.42 When solid food is introduced provision of adequate amounts of liquids and fibers is encouraged (age in years +5 = number of grams of dietary fiber per day).

G4C. FUNCTIONAL FECAL RETENTION

Functional fecal retention is the most common cause of constipation and fecal soiling in children. It consists of repetitive attempts to avoid defecation because of fears associated with defecation.43 Consequently, a fecal mass accumulates in the rectum.

 Accompanying symptoms may include fecal soiling, irritability, abdominal cramps, decreased appetite, and/or early satiety. The accompanying symptoms disappear immediately following passage of a large stool.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

Physical examination allows assessment of rectal fecal mass, which is judged for height above the pelvic brim and consistency with bimanual palpation on either side of the rectus sheath. In these children, who fear painful defecation, the necessity of a rectal examination remains debatable. A neurological examination excludes occult spinal dysraphism as the cause of retentive or non-retentive fecal soiling. Anorectal manometry, anal sphincter ultrasound, or radiography is unnecessary.44 The child’s behavior during the interview may assist in making the diagnosis. These children appear as if they are unaware and unconcerned, but in reality they are ashamed and feel isolated.

TREATMENT

Incontinence occurs when stool seeps around the fecal mass and leaks out. Therefore, the clinician, child, and parent should agree on a plan for evacuating the rectal fecal mass, often using daily oral mineral oil. Many toddlers with functional fecal retention are frightened of any anal manipulation, and find enemas particularly scary. Other experts view the fecal mass as an obstruction, and favor enemas and stimulant laxatives to expedite its expulsion. They argue that early passage of the obstructing mass will give the child immediate relief, and provide the confidence necessary for continuing toilet learning. In toddlers, toilet training will not proceed smoothly until the child’s fear of painful defecation resolves. Long term maintenance using stool softeners, including mineral oil, lactulose, and colonic lavage solutions assures a prolonged period of painless defecation until the disorder resolves.

G4D. FUNCTIONAL NON-RETENTIVE FECAL SOILING

Functional non-retentive soiling may be a manifestation of an emotional disturbance in a school-aged child. Soiling episodes may have a relationship to the presence of a specific person (e.g., a parent) or time of day, and may represent impulsive action triggered by unconscious anger.45

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

Most children with non-retentive fecal soiling have daily bowel movements and often have complete stool evacuation in their undergarments. There are few complaints of associated constipation and no fecal mass is found upon physical or xray examination.

TREATMENT

The goal is to help parents acknowledge the absence of organic disease and accept a referral to a mental health professional for the treatment of emotional problems.

(1)  Persistent or recurrent pain or discomfort centered in the upper abdomen (above the umbilicus); and

(2)  No evidence (including at upper endoscopy) that organic disease is likely to explain the symptoms; and

(3)  No evidence that dyspepsia is exclusively relieved by defecation or associated with the onset of a change in stool frequency or stool form.