II60 Gut 1999;45(Suppl II):II60–II68

Childhood functional gastrointestinal disorders Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from

A Rasquin-Weber, P E Hyman, S Cucchiara, D R Fleisher, J S Hyams, P J Milla, A Staiano

Abstract provided clinicians with a method for standard- Co-Chair, Committee on Childhood This is the first attempt at defining izing their manner of defining clinical disor- Functional criteria for functional gastrointestinal dis- ders, and have allowed researchers from Gastrointestinal orders (FGIDs) in infancy, childhood, and various fields to study the physiology and treat- Disorders, adolescence. The decision-making proc- ment of the same disorders from diVerent Multinational Working ess was as for adults and consisted of points of view. Publication of the Rome Teams to Develop diagnostic criteria resulted in an explosion of Criteria for Functional arriving at consensus, based on clinical Disorders (Rome II), experience. This paper is intended to be a clinical research, contributed to an improved Professor of quick reference. The classification system understanding of FGIDs, and provided clini- Pediatrics, selected diVers from the one used in the cians with a positive approach to treating 3 University of adult population in that it is organized patients. Montreal, according to main complaints instead of It was perceived as a privilege by our working Montreal, Canada team to be oVered the challenge of defining A Rasquin-Weber being organ-targeted. Because the child is still developing, some disorders such as diagnostic criteria according to the Rome Chair, Committee on toddler’s (or functional di- criteria for the pediatric population. We Childhood Functional arrhea) are linked to certain physiologic believed that a collaboration with our adult Gastrointestinal stages; others may result from behavioral gastroenterology colleagues would increase our Disorders, responses to sphincter function acquisi- understanding of FGIDs and provide a basis Multinational Working for longitudinal studies on the origins and evo- Teams to Develop tion such as fecal retention; others will Criteria for Functional only be recognizable after the child is cog- lution of these disorders. Disorders (Rome II), nitively mature enough to report the Childhood FGIDs include a variable combi- Associate Clinical symptoms (e.g., dyspepsia). Infant regur- nation of often age-dependent, chronic, or Professor of gitation, rumination, and cyclic recurrent symptoms not explained by struc- Pediatrics, tural or biochemical abnormalities. As the child University of constitute the vomiting disorders. Ab- dominal pain disorders are classified as: is programmed to develop, it is not surprising California at Los that some functional disorders which occur Angeles, functional dyspepsia, irritable bowel syn- during childhood accompany normal develop- Orange County, CA, drome (IBS), functional , ment (e.g., infant regurgitation or toddler’s USA abdominal migraine, and aerophagia. P E Hyman diarrhea), or may be triggered by age appropri- Disorders of include: infant ate but maladaptive behavioral responses to

dyschezia, functional , func- http://gut.bmj.com/ Associate Professor of internal or external stimuli (e.g., functional tional fecal retention, and functional non- Pediatrics, fecal retention often results from painful University Frederico II, retentive fecal soiling. Some disorders, defecation and/or coercive toilet training). The Naples, Italy such as IBS and dyspepsia and functional diagnosis of some childhood FGIDs depends S Cucchiara abdominal pain, are exact replications of on the child’s ability to report symptoms. Thus the adult criteria because there are Associate Professor of some disorders, such as irritable bowel syn- Child Health, enough data to confirm that they repre- drome (IBS), are not described in children University of Missouri, sent specific and similar disorders in

below a certain age. This does not preclude its on September 27, 2021 by guest. Protected copyright. Columbia, MO, USA pediatrics. Other disorders not included D R Fleisher existence in younger children; rather, preschool in the pediatric classification, such as children are unable to report the necessary functional biliary disorders, do occur in Professor and Vice details for a diagnosis. Therefore, instead of Chair of Pediatrics, children; however, existing data are insuf- classifying disorders according to target organs University of ficient to warrant including them at the (as in the adult population), we divided pediat- Connecticut School of present time. For these disorders, it is Medicine, ric disorders according to main complaints suggested that, for the time being, clini- reported by children or their parents. For Hartford, CT, USA cians refer to the criteria established for JSHyams example, aerophagia was classified within the adult population. pediatric disorders as presenting as abdominal Reader in Pediatric (Gut 1999;45(Suppl II):II60–II68) pain, whereas in adults, the disorder was classi- Gastroenterology, fied as belonging to the -related University of London, Keywords: infant vomiting; cyclic vomiting syndrome; London, UK functional dyspepsia in children; irritable bowel group of symptoms. P J Milla syndrome in children; functional abdominal pain in The working team agreed that some infants children; functional diarrhea in children; functional inherit a temperament characterized in part by Associate Professor of constipation in children; Rome II gastrointestinal reactivity to stress, which Pediatrics, constitutes a genetic susceptibility to FGIDs. University Frederico Indeed, temperament-sensitive reactivity in II, School of Medicine, The pediatric working team met for the first infants has already been suggested in associ- Naples, Italy time in Rome in September 1997, seven years A Staiano ation with three other biological systems after the first paper classifying diagnostic crite- (cardiovascular, neuroendocrine, and Correspondence to: ria for functional gastrointestinal disorders immunologic).4 Conversely, our committee Andree Rasquin-Weber, MD, (FGIDs) in the adult population was Gastrointestinal Division, published.1 These disorders were further de- Hopital Ste Justine, 3175 St Abbreviations used in this paper: FGID, functional Catherine Road, Montreal, fined in a document, now referred to as the gastrointestinal disorder; IBS, irritable bowel Quebec H3T 1C5, Canada. Rome criteria for FGIDs.2 These criteria have syndrome; RAP, recurrent abdominal pain. Childhood functional gastrointestinal disorders II61

Table 1 Functional gastrointestinal disorders functional , functional ,

functional , and proctalgia fugax. Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from A. Esophageal disorders Functional biliary disorders were not described A1. Globus A2. Rumination syndrome because documentation and experience with A3. Functional chest pain of presumed esophageal origin gall bladder and sphincter of Oddi dysfunction A4. Functional heartburn A5. Functional dysphagia in children are insuYcient. Eventually, it was A6. Unspecified functional esophageal disorder agreed that the co-occurrence of FGIDs and B. Gastroduodenal disorders organic disease in the same child often goes B1. Functional dyspepsia B1a. Ulcer-like dyspepsia unrecognized. Thus, for example, the presence B1b. Dysmotility-like dyspepsia of IBS often leads to overtreatment in adoles- B1c. Unspecified (non-specific) dyspepsia cents with inflammatory bowel disease. B2. Aerophagia B3. Functional vomiting C. Bowel disorders C1. Irritable bowel syndrome G1. Vomiting C2. Functional abdominal G1a. Infant regurgitation C3. Functional constipation C4. Functional diarrhea Regurgitation is the involuntary return of pre- C5. Unspecified functional bowel disorder viously swallowed food or secretions into or out D. Functional abdominal pain of the mouth. Regurgitation is distinguished D1. Functional abdominal pain syndrome D2. Unspecified functional abdominal pain from vomiting, which is defined by a central E. Biliary disorders nervous system reflex involving both auto- E1. Gall bladder dysfunction nomic and skeletal muscles in which gastric E2. Sphincter of Oddi dysfunction F. Anorectal disorders contents are forcefully expelled through the F1. Functional mouth because of coordinated movements of F2. Functional anorectal pain the small bowel, , esophagus, and dia- F2a. Levator ani syndrome F2b. Proctalgia fugax phragm. Regurgitation, vomiting, and rumina- F3. Pelvic floor dyssynergia tion are examples of gastroesophageal reflux. G. Functional pediatric disorders When the latter causes or contributes to tissue G1. Vomiting G1a. Infant regurgitation damage or inflammation (e.g. esophagitis, G1b. Infant rumination syndrome obstructive , reactive airway disease, pul- G1c. Cyclic vomiting syndrome G2. Abdominal pain monary aspiration, or failure to thrive), it is G2a. Functional dyspepsia called gastroesophageal reflux disease. G2b. Irritable bowel syndrome G2c. Functional abdominal pain G2d. Abdominal migraine DIAGNOSTIC CRITERIA G2e. Aerophagia G3. Functional diarrhea (1) Regurgitation two or more times per G4. Disorders of defecation day for three or more weeks; G4a. Infant dyschezia G4b. Functional constipation (2) There is no retching, hematemesis, G4c. Functional fecal retention aspiration, apnea, failure to thrive, or

G4d. Non-retentive fecal soiling abnormal posturing; http://gut.bmj.com/ (3) The infant must be 1–12 months of age and otherwise healthy; members were convinced that environmental (4) There is no evidence of metabolic, factors during early life also play a role in the gastrointestinal, or central nervous sys- development of FGIDs. Firstly, plasticity of the tem disease to explain the symptom. neonatal brain allows early life events to program physiologic responses to stress during infancy, and these responses may be perpetu- CLINICAL AND DIAGNOSTIC RECOMMENDATIONS on September 27, 2021 by guest. Protected copyright. ated into adulthood.5 Secondly, children learn Prematurity, developmental delay, and con- illness-related attitudes and behaviors from genital abnormalities of the oropharynx, chest, their parents and caretakers. Health care utili- lungs, central nervous system, or gastro- zation by children closely resembles that of intestinal tract are considered risk factors for their parents.6 Thus, not only should treatment gastroesophageal reflux disease. Accompany- for children include their parents, but the fam- ing systemic conditions (milk allergy) may be ily should be taught about the role that psycho- suspected when eczema is present; an abnor- social factors play in the development and per- mal neurological examination should prompt petuation of FGIDs. further investigation. A diagnosis of gastro- This paper summarizes the diagnostic crite- esophageal reflux disease should be evoked in ria for pediatric FGIDs, and is intended to be a the presence of failure to thrive, hematemesis, quick reference (table 1). Therefore, only clini- occult blood in the stool, anemia, or refusal to cal, diagnostic, and treatment recommenda- eat. However, chronic regurgitation, early sati- tions are included. The reader is referred to ety, refusal of food, and excessive crying, other publications for detailed clinical, epide- sometimes leading to failure to thrive, may be miologic, physiologic, and psychological the physical consequences of emotional considerations.78 For certain disorders (e.g., distress.9 IBS and functional dyspepsia), criteria in the adult population were replicated exactly for the TREATMENT pediatric population because they seemed to Since infant regurgitation is a transient prob- apply equally well to children. FGIDs other lem, possibly due in part to the immaturity of than those mentioned in this article occur dur- gastrointestinal motility, treatment goals are to ing childhood, but were defined adequately by provide eVective reassurance and symptom the adult criteria. These include globus, relief. Symptoms often improve with prone II62 Rasquin-Weber, Hyman, Cucchiara, et al

positioning after meals,10 formula thickened G1c. Cyclic vomiting syndrome 11 with cereal, smaller volume feedings, and Cyclic vomiting syndrome consists of recur- Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from drugs that improve motility such as cisapride.12 rent, stereotypical episodes of intense and vomiting lasting hours to days, which are separated by symptom-free intervals. The G1b. Infant rumination syndrome frequency of episodes averages 12 per year Rumination is a rare disorder characterized by (range 1–70) and intervals between attacks the voluntary, habitual regurgitation of stom- may be fairly regular or sporadic. Episodes tend ach contents into the mouth for to begin at the same time of day, usually during self-stimulation.13 the night or early morning. The duration of episodes tends to be the same in each patient 15 DIAGNOSTIC CRITERIA over months or years. Once vomiting begins, (1) Infant rumination syndrome is defined it reaches its highest intensity during the first by at least three months of stereotypical hours.16 behavior beginning with repetitive contrac- tions of the abdominal muscles, diaphragm, DIAGNOSTIC CRITERIA and tongue, and culminating in regurgita- (1) A history of three or more periods of tion of gastric contents into the mouth intense, acute nausea, and unremitting which is either expectorated or rechewed vomiting lasting hours to days, with and reswallowed, and three or more of the intervening symptom-free intervals, following: lasting weeks to months. (a) Onset between three and eight months (2) There is no metabolic, gastrointestinal of age; or central nervous system structural or (b) Does not respond to management for biochemical disease. gastroesophageal reflux disease, anti- cholinergic drugs, hand restraints, for- mula changes, and gavage or gastros- CLINICAL AND DIAGNOSTIC RECOMMENDATIONS tomy feedings; Typically, cyclic vomiting occurs in a child aged (c) Unaccompanied by signs of nausea or 2–7 years (with a possible range from infancy distress; and/or to mid-life), who is free of vomiting between (d) Does not occur during sleep and when episodes. However, like their family members, the infant is interacting with individuals these children often complain of migraines, in the environment. motion sickness, and other functional bowel disorders. During vomiting episodes, accompa- nying include pallor, CLINICAL AND DIAGNOSTIC RECOMMENDATIONS weakness, increased salivation, abdominal Observation of the ruminative act is all that is pain, intolerance to noise, light or odors, head- needed to make the diagnosis. This might be ache, diarrhea, fever, tachychardia, hyper- http://gut.bmj.com/ diYcult as rumination may cease as soon as tension, skin blotching, and leukocytosis. A the infant notices the observer. Parents will triggering factor (emotions, infection) can be not provide the information spontaneously, identified in 80% of cases. DiVerential diagno- but when asked about the possibility of stere- sis should include brain stem tumors, obstruc- otypical behavior, they will recognize it. The tive uropathy, peptic disease, recurrent pan- infant who ruminates may not retain enough creatitis, intermittent bowel obstruction, nutrients and may develop potentially lethal chronic intestinal pseudo-obstruction, and malnutrition, a complication that seldom, if familial dysautonomia. Among metabolic and on September 27, 2021 by guest. Protected copyright. 13 ever, occurs in older ruminators. Sensory endocrine diseases, those which may mimic and/or emotional deprivation characterize the cyclic vomiting include pheochromocytoma, child with rumination. This explains why the adrenal insuYciency, diabetes mellitus, urea disorder occurs in institutionalized children cycle enzyme deficiency, medium-chain acyl and infants in intensive care units, as well coenzyme A dehydrogenase deficiency, propri- as in normal infants from emotionally distant onic acidemia, and porphyria. mothers. TREATMENT TREATMENT Emotional conditions which trigger episodes Treatment eVorts should be directed toward should be identified and treated. When epi- caregivers as well as the child. In infant sodes are frequent and severe, prophylactic rumination, the relationship between the infant daily treatment with cyproheptadine, ami- and caregiver is devoid of enjoyment. The goal triptyline, erythromycin, phenobarbital, su- of therapy is to provide a nurturing environ- matripan, or propanolol may succeed in reduc- ment and comforting care to the infant, and to ing frequency or eliminating episodes.17–19 In help the mother change her feelings towards some children with a recognizable prodrome, herself and her infant. This can be achieved by oral medications such as ondansetron, erythro- improving her ability to recognize and respond mycin, and ibuprofen may be useful prior to sensitively to her infant’s physical and emo- onset of nausea. In patients whose episodes tional needs. In mentally handicapped chil- cannot be prevented, it may be helpful to dren, providing a nurturing caregiver may not begin, as early as possible, an oral acid inhibit- be suYcient; behavioral therapy involving posi- ing drug to protect esophageal mucosa and tive reinforcement and possibly aversive tech- dental enamel, and lorazepam for its anxiolytic, niques may be necessary.14 sedative, and anti-emetic eVects. Intravenous Childhood functional gastrointestinal disorders II63

ondansetron, granisetron, diphenhydramine, The concept of subdividing functional dys- and chlorpromazine may also be helpful and pepsia based on distinctive features may be Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from will sometimes interrupt the crisis. The pres- useful. These divisions seem especially relevant ence of electrolyte deficits, hypertension, to those proposing to study this FGID. and/or inappropriate secretion of antidiuretic hormone should be detected and treated. G2a1. Ulcer-like dyspepsia G2. Abdominal pain Pain centered in the upper is the The pediatric working team achieved consen- predominant (most bothersome) symptom. sus and chose not to include infant colic20 and recurrent abdominal pain (RAP) of childhood21 as FGIDs. In the case of colic, there is no evi- dence that the gastrointestinal tract is G2a2. Dysmotility-like dyspepsia involved.20 In the case of RAP, three reasons were invoked to exclude it: (1) Appley’s criteria An unpleasant or troublesome non-painful are too general; (2) symptoms in school-aged sensation (discomfort) centered in the children with chronic or recurrent abdominal upper abdomen is the predominant symp- pain often meet the Rome criteria for func- tom; this sensation may be characterized by tional dyspepsia, IBS, or functional abdominal or associated with upper abdominal full- pain in adults.22 It seemed appropriate to apply ness, early satiety, bloating, or nausea. the most specific diagnostic category to a symptomatic child, so that we may learn more about the natural history of these FGIDs with childhood onset, and consider novel interven- G2a3. Unspecified (non-specific) tion strategies for children who meet diagnostic dyspepsia criteria for these conditions; and (3) there is a growing body of evidence to suggest that func- Symptomatic patients whose symptoms do tional abdominal pain is often associated with not fulfil the criteria for either ulcer-like or visceral hyperalgesia, a reduced threshold for dysmotility-like dyspepsia. pain related to biochemical changes in the aVerent neurons of the enteric and central CLINICAL AND DIAGNOSTIC RECOMMENDATIONS nervous systems.23 Despite the fact that evi- In children mature enough to provide an accu- dence for visceral hyperalgesia in children with rate pain history, the clinician conducts an functional abdominal pain remains prelimi- interview which includes queries about dietary, nary, we are currently using this theory to psychological, and social factors. The clinician explain the pathogenesis of pain, in order to determines whether symptoms are likely to alleviate parents’ feelings of guilt and concern represent mucosal disease (esophagitis, gastri- that the problem is psychological. tis, duodenitis, ulcer). Demographic and famil- http://gut.bmj.com/ ial data should raise suspicion of Helicobacter pylori infection.25 Endoscopy will confirm or G2a. Functional dyspepsia eliminate these diagnoses. A previous episode Dyspepsia refers to pain or discomfort cen- of viral infection may suggest post-viral tered in the upper abdomen (see also func- gastroparesis.26 tional dyspepsia in adults). Discomfort may be Measuring serum amylase, lipase, and amino- characterized by fullness, early satiety, bloat- transferase concentrations, as well as performing ing, belching, queasiness, nausea, retching, or an abdominal ultrasound, aid in assessing the on September 27, 2021 by guest. Protected copyright. vomiting. These symptoms typically include a presence of pancreatic, , or biliary disease. 24 component of upper abdominal distress. In functional dyspepsia, physical examination and growth are normal and there are no signs of DIAGNOSTIC CRITERIA inflammatory bowel disease. Functional dyspepsia has not been rigorously defined in a pediatric population. Therefore we TREATMENT have adopted the adult diagnostic criteria for There are no controlled treatment trials for use in children. functional dyspepsia in children. Medication and food known to aggravate symptoms should In children mature enough to provide an be discontinued. Histamine receptor antago- accurate pain history, at least 12 weeks, nists, proton pump inhibitors, sucralfate, and which need not be consecutive, within the low dose tricyclic antidepressants have been preceding 12 months of: used. Prokinetic drugs such as cisapride may be (1) Persistent or recurrent pain or discom- helpful for feelings of fullness and metoclopra- fort centered in the upper abdomen mide may alleviate nausea. If psychological (above the umbilicus); and stress aggravates symptoms, the clinician and (2) No evidence (including at upper endos- family should collaborate on a plan to reduce copy) that organic disease is likely to the stress. explain the symptoms; and (3) No evidence that dyspepsia is exclu- sively relieved by defecation or associ- G2b. Irritable bowel syndrome ated with the onset of a change in stool In IBS (see also criteria for irritable bowel frequency or stool form. syndrome in adults) abdominal discomfort or pain is associated with defecation or a change II64 Rasquin-Weber, Hyman, Cucchiara, et al

in bowel habit, with features of disordered ance and symptom relief. The presence and

defecation. Ten to 20% of adolescentsand severity of the pain should be acknowledged. Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from adults have symptoms consistent with IBS.22 The clinician must educate and reassure the child and family that although IBS causes dis- DIAGNOSTIC CRITERIA comfort, it is not a serious disease. A review of In children old enough to provide an the current understanding of IBS and the accurate pain history, at least 12 weeks, exacerbating eVects of stress and on the which need not be consecutive, in the problem helps the child and family to under- preceding 12 months of; stand why the pain occurs.27 Psychosocial diY- (1) Abdominal discomfort or pain that has culties and triggering events for symptoms two out of three features: should be identified and tackled. (a) Relieved with defecation; and/or Drug therapy plays an adjunctive role in (b) Onset associated with a change in treatment. Tricyclic antidepressants such as frequency of stool; and/or imipramine or amitriptyline in low doses im- (c) Onset associated with a change in proved symptoms in adults with IBS in control- form (appearance) of stool; and led, blinded studies.28 29 These drugs have been (2) There are no structural or metabolic used for decades for chronic visceral pain by abnormalities to explain the symptoms. pain management specialists, but there are only anecdotal reports concerning their use in children with chronic abdominal pain. As The following symptoms cumulatively support amitriptyline has greater sedative and anti- the diagnosis of IBS: cholinergic eVects than imipramine, amitriptyl- + Abnormal stool frequency (for research ine may be a better choice for children who wake purposes “abnormal” may be defined as up at night with pain, or for children with a greater than three bowel movements per day diarrheal component to their disorder. Imi- and less than three bowel movements per pramine may be a better choice than amitriptyl- week); ine for children with constipation; at the low + abnormal stool form (lumpy/hard or loose/ doses recommended for chronic visceral pain, watery stool); chronic constipation is rarely exacerbated. Anti- + abnormal stool passage (straining, urgency, cholinergic medications such as dicyclomine, or feeling of incomplete evacuation); hyoscine, mebeverine, and octylonium have + passage of mucus; been used for their antispasmodic properties. + bloating or feeling of . There are no well designed studies confirming eYcacy. In those with constipation, increased CLINICAL AND DIAGNOSTIC RECOMMENDATIONS dietary fiber (recommended daily fiber intake = A history which fits the Rome criteria for a age (years) + 5 g), milk of magnesia, or mineral diagnosis of IBS, accompanied by a normal oil may be a helpful adjunct.

physical examination and growth, is consistent http://gut.bmj.com/ with a diagnosis of childhood IBS. A nutri- G2c. Functional abdominal pain tional history, assessing for adequacy of dietary It should be noted that the term “functional fiber in those with constipation, as well as abdominal pain” is not a substitute for Appley’s ingestion of sugars such as sorbitol and “recurrent abdominal pain of childhood.” In fructose in those with diarrhea, is often useful. some children with functional abdominal pain, Factors alerting the clinician to the possibility the symptoms do not meet the diagnostic of disease include nocturnal pain or diarrhea, criteria for IBS or functional dyspepsia. The weight loss, rectal bleeding, fever, arthritis, pain is usually periumbilical and does not on September 27, 2021 by guest. Protected copyright. delayed puberty, and a family history of relate to any specific activity; it may keep the inflammatory bowel disease. Limited labora- patient from sleeping but rarely awakes the tory screening for disease is frequently reassur- patient from sleep. Some of these children are ing to the clinician, patient, and family. Screen- perfectionists, whereas others often have un- ing may include a complete blood count and recognized learning diYculties; generally, how- erythrocyte sedimentation rate, stool studies ever, the patients or their parents often have for enteric bacterial pathogens and parasites, high expectations of achievement. and breath hydrogen testing, or a trial of a milk-free diet for lactose malabsorption. Dur- DIAGNOSTIC CRITERIA ing the initial visits, and after establishing rap- At least 12 weeks of: port with the patient and parents, the clinician (1) Continuous or nearly continuous ab- inquires about the psychosocial history of the dominal pain in a school-aged child or child and family. In patients with intractable adolescent; and symptoms endoscopic evaluation of the colon (2) No or only occasional relation of pain may be indicated (e.g., colonoscopy and with physiological events (e.g., eating, biopsy). If the terminal ileum is not intubated, menses, or defecation); and it may be imaged with small bowel series or (3) Some loss of daily functioning; and white blood cell technetium scan. IBS may (4) The pain is not feigned (e.g., malinger- occur concurrently in patients with inflamma- ing); and tory bowel disease. (5) The patient has insuYcient criteria for other functional gastrointestinal disor- TREATMENT ders that would explain the abdominal Once there is a confident diagnosis of IBS, pain. treatment goals are to provide eVective reassur- Childhood functional gastrointestinal disorders II65

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS other causes of intermittent severe abdominal

The child may also complain of headache, pain should be considered, including obstruc- Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from light-headedness, dizziness, nausea (without tive uropathy, intermittent bowel obstruction, vomiting), and fatigue. Psychological factors recurrent pancreatitis, biliary tract disease, which should be examined include anxiety intracranial space-occupying lesions, familial and/or depression in the child and family, Mediterranean fever, and metabolic disorders. somatization, school phobia, separation anxi- The diagnosis of abdominal migraine is further ety, and secondary gains for illness behavior. supported by a favorable response to medica- Physical examination, growth, and laboratory tions used prophylactically for migraine. tests are normal (urinalysis, erythrocyte sedi- mentation rate, complete blood count, stool TREATMENT examinations for occult blood, leucocytes, ova Pizotifen, a serotonin receptor antagonist una- and parasites, blood chemistries, abdominal vailable in the United States, provides eVective ultrasound, and breath hydrogen testing). prophylaxis.32 Cyproheptadine is eVective in When symptoms are intractable, further evalu- some patients with migraine headaches and ation for organic disease may become neces- cyclic vomiting syndrome18 and might also be sary, using radiography and endoscopy. helpful in the prophylaxis of abdominal mi- graine. TREATMENT EVective reassurance and an explanation for G2e. Aerophagia how symptoms occur in the absence of labora- Aerophagia consists of excessive air swallowing tory abnormalities (see earlier) help to estab- causing progressive abdominal distension.34 lish a therapeutic alliance between the family Abdominal discomfort is often such that and the clinician. Psychological support for children limit their food intake. This explains personal, familial, and school diYculties why the committee decided to include aero- should be oVered when necessary. The best phagia among the abdominal pain disorders. guarantee for success consists of accompanying the children through diYcult periods of adjust- DIAGNOSTIC CRITERIA ment and reevaluation whenever symptoms At least 12 weeks, which need not be change. The use of a diary to record symptoms, consecutive, in the preceding 12 months of thoughts, and feelings helps the child become two or more of the following signs and involved in his own healing process. symptoms: (1) Air swallowing; and G2d. Abdominal migraine (2) Abdominal distension due to intralumi- Abdominal migraine is a paroxysmal disorder nal air; and aVecting about 2% of children and character- (3) Repetitive belching and/or increased ized by acute, incapacitating, non-colicky, mid- flatus.

line abdominal pain that lasts for hours and is http://gut.bmj.com/ accompanied by pallor and anorexia. Associ- CLINICAL AND DIAGNOSTIC RECOMMENDATIONS ated features include personal and family Aerophagia often goes unnoticed by parents. history of migraine headache.30 31 The physician will observe repeated audible swallows, and inquire about anorexia, abdomi- DIAGNOSTIC CRITERIA nal pain, excessive flatus, and excessive . (1) In the preceding 12 months, three or The symptoms and abdominal distension resolve during sleep.33 Aerophagia may be con- more paroxysmal episodes of intense, on September 27, 2021 by guest. Protected copyright. acute midline abdominal pain lasting fused with gastroesophageal reflux disease two hours to several days, with interven- (because of noises in the throat), and with gen- ing symptom-free intervals lasting eralized motility disorders such as chronic weeks to months; and intestinal pseudo-obstruction or Hirsch- (2) Evidence of metabolic, gastrointestinal, sprung’s disease (because of associated abdomi- and central nervous system structural or nal distension). A normal physical examination biochemical diseases is absent; and and growth history help exclude disease. Breath (3) Two of the following features: hydrogen testing may be useful. It is important (a) Headache during episodes; to ask about stressful life events because anxiety (b) Photophobia during episodes; is a frequent cause of excessive swallowing. (c) Family history of migraine; (d) Headache confined to one side only; TREATMENT (e) An aura or warning period consist- Treatment consists of eVective reassurance and ing of either visual disturbances explanation of the symptom for the parents and (e.g., blurred or restricted vision), child. Often the clinician can help the child sensory symptoms (e.g., numbness become aware of swallows during the visit. or tingling), or motor abnormalities Excessive use of chewing gum or carbonated (e.g., slurred speech, inability to beverages should be discouraged. Stress and speak, paralysis). anxiety problems should be promptly addressed. G3. Functional diarrhea (also called CLINICAL AND DIAGNOSTIC RECOMMENDATIONS toddler’s diarrhea, chronic non-specific When accompanied by a history of migraine diarrhea, irritable colon of childhood) headaches, the diagnosis is straightforward; Functional diarrhea is defined by daily painless otherwise it should remain presumptive. All recurrent passage of three or more large, II66 Rasquin-Weber, Hyman, Cucchiara, et al

unformed stools, for four or more weeks, with diet), physical examination (including rectal

onset in infancy or preschool years. There is no examination to exclude anorectal abnormali- Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from evidence of failure to thrive if the diet has ties), and chart of the infant’s growth are nor- adequate calories. The symptom resolves mal. spontaneously by school age.34 TREATMENT Parents are reassured by a systematic physical DIAGNOSTIC CRITERIA examination completed in their presence. The For more than four weeks, daily painless, disorder will resolve when coordination be- recurrent passage of three or more large, tween increased abdominal pressure and re- unformed stools, in addition to all these laxation of the pelvic floor is acquired. To avoid characteristics: perpetuation of dyschezia, maneuvers of rectal (1) Onset of symptoms begins between six stimulation, which produce artificial and po- and 36 months of age; tentially noxious sensory experiences, should (2) Passage of stools occurs during waking be discouraged. hours; and (3) There is no failure to thrive if caloric G4b. Functional constipation intake is adequate. Defecation disorder represents the chief com- plaint in 3% of pediatric outpatient visits and CLINICAL AND DIAGNOSTIC RECOMMENDATIONS 10–25% of pediatric gastroenterology visits. The clinician queries about causes of chronic Few reports distinguish between functional diarrhea, including enteric infections, ingestion constipation and functional fecal retention, so of laxatives, urinary tract infection, and the frequency of functional constipation is antibiotics. A diet history assesses overfeeding, unknown.39 Children with cerebral palsy often excessive fruit juice or sorbitol consumption, develop functional constipation. excessive carbohydrate ingestion with low fat

intake, and food allergens. In the absence of DIAGNOSTIC CRITERIA failure to thrive, a malabsorption syndrome is In infants and preschool children, at least unexpected. two weeks of: (1) Scybalous, pebble-like, hard stools for a TREATMENT majority of stools; or It is important to avoid restrictive diets which (2) Firm stools two or less times/week; and 34 may induce caloric deprivation. In functional (3) There is no evidence of structural, diarrhea, a meal fails to interrupt the migrating endocrine, or metabolic disease. motor complex.35 Children recover spontane- ously, and usually no treatment is necessary. EVective reassurance for parents is important. CLINICAL AND DIAGNOSTIC RECOMMENDATIONS

A daily diet and defecation diary helps to reas- In most cases, a thorough history and physical http://gut.bmj.com/ sure them that specific dietary items are not examination are suYcient to diagnose func- responsible for the symptom. tional constipation. Delayed passage of meco- nium in a full term newborn raises the suspicion of Hirschsprung’s disease.40 In a few G4. Disorders of defecation healthy breast fed infants there may be weeks Frequency of defecation in healthy infants and between bowel movements but the stools are children varies with age.36 Complaints related soft.41 The transition from breast to formula

to defecatory problems are responsible for 25% on September 27, 2021 by guest. Protected copyright. feedings is often associated with the appear- of outpatient visits to pediatric gastro- ance of functional constipation. However enterologists.37 38 infants on formula feedings and children who pass stools at intervals greater than a week G4a. Infant dyschezia apart and fail to thrive are likely to have an Infants with dyschezia strain and scream enteric neuromuscular, anatomic, or metabolic during prolonged attempts to defecate. This disease. Ingestion of medication causing con- behavior persists for up to 20 minutes, until stipation needs to be excluded. soft or liquid stools pass. This may be repeated At physical examination, inspection of the several times a day and occurs during the first back and spine and verification of deep tendon few months of life. Symptoms resolve sponta- and cremasteric reflexes help to exclude occult neously in a few weeks. spinal dysraphism. At least one anorectal exam- ination is performed allowing evaluation of anatomy, tone, and masses. Laboratory and DIAGNOSTIC CRITERIA At least 10 minutes of straining and crying radiologic examinations are not warranted in before successful passage of soft stools in an the absence of poor weight gain, persistent otherwise healthy infant less than six abdominal distension, fever, Down syndrome, months of age. bilious vomiting, or any abnormal physical findings mentioned above. Functional constipa- tion is important because it may predispose to CLINICAL AND DIAGNOSTIC RECOMMENDATIONS the development of functional fecal retention. We speculated that this disorder occurs when neonates fail to coordinate increased intraab- TREATMENT dominal pressure with relaxation of the pelvic In infants, fruit juices which contain fructose floor. Thus, the complete history (including and sorbitol such as prune and pear increase Childhood functional gastrointestinal disorders II67

stool water. Barley, corn syrup, lactulose, and lactulose, and colonic lavage solutions assures a

sorbitol can also be used, but mineral oil is not prolonged period of painless defecation until Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from recommended.42 When solid food is introduced the disorder resolves. provision of adequate amounts of liquids and fibers is encouraged (age in years +5 = number G4d. Functional non-retentive fecal of grams of dietary fiber per day). soiling Functional non-retentive soiling may be a G4c. Functional fecal retention manifestation of an emotional disturbance in a Functional fecal retention is the most common school-aged child. Soiling episodes may have a cause of constipation and fecal soiling in relationship to the presence of a specific person children. It consists of repetitive attempts to (e.g., a parent) or time of day, and may repre- sent impulsive action triggered by unconscious avoid defecation because of fears associated 45 with defecation.43 Consequently, a fecal mass anger. accumulates in the rectum. DIAGNOSTIC CRITERIA Once a week or more for the preceding 12 DIAGNOSTIC CRITERIA From infancy to 16 years old, a history of at weeks, in a child older than four years, a his- least 12 weeks of: tory of: (1) Passage of large diameter stools at (1) Defecation into places and at times intervals < 2 times per week; and inappropriate to the social context; (2) Retentive posturing, avoiding defeca- (2) In the absence of structural or inflam- tion by purposefully contracting the matory disease; and pelvic floor. As pelvic floor muscles (3) In the absence of signs of fecal retention fatigue, the child uses the gluteal (listed in section G4c). muscles, squeezing the buttocks to- gether. CLINICAL AND DIAGNOSTIC RECOMMENDATIONS Most children with non-retentive fecal soiling Accompanying symptoms may include fecal have daily bowel movements and often have soiling, irritability, abdominal cramps, de- complete stool evacuation in their undergar- creased appetite, and/or early satiety. The ments. There are few complaints of associated accompanying symptoms disappear immedi- constipation and no fecal mass is found upon ately following passage of a large stool. physical or x ray examination.

CLINICAL AND DIAGNOSTIC RECOMMENDATIONS TREATMENT Physical examination allows assessment of rec- The goal is to help parents acknowledge the tal fecal mass, which is judged for height above absence of organic disease and accept a referral http://gut.bmj.com/ the pelvic brim and consistency with bimanual to a mental health professional for the treat- palpation on either side of the rectus sheath. In ment of emotional problems. these children, who fear painful defecation, the necessity of a rectal examination remains 1 Drossman DA, Thompson WG, Talley NJ, et al. Identifica- debatable. A neurological examination ex- tion of subgroups of functional bowel disorders. Gastroen- terol Int 1990;3:159–72. cludes occult spinal dysraphism as the cause of 2 Drossman DA, Creed FH, Fava GA, et al. The functional retentive or non-retentive fecal soiling. Anorec- gastrointestinal disorders: Diagnosis, pathophysiology and treat- ment. McLean, VA: Degnon Associates, 1994. tal manometry, anal sphincter ultrasound, or 3 Drossman DA. Do the Rome criteria stand up? In: Goebell on September 27, 2021 by guest. Protected copyright. radiography is unnecessary.44 The child’s be- H, Holfman G, Talley NS, eds. Functional dyspepsia and irritable bowel syndrome: concepts and controversies. Falk Sym- havior during the interview may assist in mak- posium 98. Dordrecht: Kluwer Academic Publishers, ing the diagnosis. These children appear as if 1998:11–18. 4 Boyce WT, Barr RG, Zeltzer LK. Temperament and the they are unaware and unconcerned, but in psychobiology of stress. Pediatrics 1992;90:483–6. reality they are ashamed and feel isolated. 5 Liv D, Dioro J, Tannenbaum B, et al. Maternal care hippoc- ampal glucocorticoid receptors and hypothalamic- pituitary-adrenal responses to stress. Science 1997;277: 1659–62. TREATMENT 6 Schor E, Starfield B, Stidley C, et al. Family health. Utiliza- Incontinence occurs when stool seeps around tion and eVects of family membership. Med Care 1987;25: 616–26. the fecal mass and leaks out. Therefore, the cli- 7 Hyman PE, Rasquin-Weber A, Fleisher DR, et al. Child- nician, child, and parent should agree on a plan hood functional gastrointestinal disorders. In: Drossman DA, ed. The functional gastrointestinal disorders. McLean, for evacuating the rectal fecal mass, often using VA: Degnon Associates (in press). daily oral mineral oil. Many toddlers with 8 Hyman PE (ed). Childhood functional gastrointestinal disor- ders. New York: Academy Professional Information Serv- functional fecal retention are frightened of any ices (in press). anal manipulation, and find enemas particu- 9 Fleisher DR. Functional vomiting disorders in infancy: innocent vomiting, nervous vomiting, and infant rumina- larly scary. Other experts view the fecal mass as tion syndrome. J Pediatr 1994;125:S84–94. an obstruction, and favor enemas and stimu- 10 Orenstein SR, Whittington PF. Positioning for preventing infant gastroesophageal reflux. Pediatrics 1982;69:768–72. lant laxatives to expedite its expulsion. They 11 Orenstein SR, Magill HL, Brooks P. Thickening of infant argue that early passage of the obstructing mass feedings for therapy of gastroesophageal reflux. J Pediatr will give the child immediate relief, and provide 1987;110:181–6. 12 Scott RB, Ferreira C, Smith L, et al. Cisapride in pediatric the confidence necessary for continuing toilet gastroesophageal reflux. J Pediatr Gastroenterol Nutr 1997; learning. In toddlers, toilet training will not 25:499–506. 13 Fleisher DR. Infant rumination syndrome. Am J Dis Child proceed smoothly until the child’s fear of pain- 1979;133:266–9. 14 Sauvage D, Leddet I, Hameury L, et al. Infantile ful defecation resolves. Long term maintenance rumination: diagnosis and follow-up study of twenty cases. using stool softeners, including mineral oil, J Am Acad Child Psychiatry 1985;24:197–203. II68 Rasquin-Weber, Hyman, Cucchiara, et al

15 Fleisher DR, Matar M. The cyclic vomiting syndrome: A 29 Pilowsky I, Barroro GG. A controlled study of psycho- report of 71 cases and a literature review. J Pediatr Gastro- therapy and amitriptyline used individually in the treat- enterol Nutr 1993;17:361–9. ment of chronic intractable psychogenic pain. Pain Gut: first published as 10.1136/gut.45.2008.ii60 on 1 September 1999. Downloaded from 16 Pfau BT, Li BUK. DiVerentiating cyclic from chronic vom- 1990;40:3–19. iting patterns in children—quantitative criteria and diag- 30 Symon DNK, Russell G. Abdominal migraine: A childhood nostic implications. Pediatrics 1996;97:367–8. syndrome defined. Cephalagia 1986;6:223–8. 17 Forbes D, Withers G. Prophylactic therapy in cyclic vomit- 31 Mortimer MJ, Kay J, Jaron A. Clinical epidemiology of ing syndrome. J Pediatr Gastroenterol Nutr 1995;21(suppl childhood abdominal migraine in an urban general 1):S57–9. practice. Devel Med Child Neurol 1993;35:243–8. 18 Anderson J, Sugerman K, Lockhart JR, et al.EVective pro- 32 Symon DNK, Russell G. Double blind placebo controlled phylactic therapy for cyclic vomiting syndrome in children trial of pizotifen syrup in the treatment of abdominal using amitriptyline or cyproheptadine. Pediatrics 1997;100: migraine. Arch Dis Child 1995;72:48–50. 977–81. 33 Gauderer MWL, Halpin TC, Izant RJ. Pathologic child- hood aerophagia: a recognizable clinical entity. 19 Fleisher DR. Management of cyclic vomiting syndrome. J J Pediatr 1995; (suppl 1):S52–6. Surg 1981;16:301–5. Pediatr Gastroenterol Nutr 21 34 Lloyd-Still JD. Chronic diarrhea of childhood and the mis- 20 Carey WB. “Colic” — Primary excessive crying as in infant- use of elimination diets. J Pediatr 1979;95:10–3. environment interaction. Pediatr Clin North Am 1984;31: 35 Fenton TR, Harries JT, Milla PJ. Disordered small intestinal 993–1005. motility: a rational basis for toddler’s diarrhea. Gut 21 Appley J, Naish N. Recurrent abdominal pains: A field sur- 1983;24:897–903. vey of 1,000 school children. Arch Dis Child 1958;33:165– 36 Weaver LT. Bowel habit from birth to old age. J Pediatr Gas- 70. troenterol Nutr 1988;7:637–40. 22 Hyams JS, Burke G, Davis PM, et al. Abdominal pain and 37 Levine MD. Children with : A descriptive irritable bowel syndrome in adolescents: a community- analysis. Pediatrics 1979;56:412–16. based study. J Pediatr 1996;129:220–6. 38 Loening-Baucke V. Chronic constipation in children. 23 Di Lorenzo C, Sigurdsson L, GriYths J, et al. Rectal and Gastroenterology 1993;105:1557–64. gastric hyperalgesia in children with recurrent abdominal 39 Molnar D, Taitz LS, Uurwin OM, et al. Anorectal manom- pain [abstract]. Gastroenterology 1998;114:A743. etry results in defecation disorders. Arch Dis Child 1983;58: 24 Talley NJ, Colin-Jones D, Koch KL, et al. Functional 257–61. dyspepsia: a classification with guidelines for diagnosis and 40 Swenson O, Sherman JO, Fisher JH. Diagnosis of congeni- management. Gastroenterol Int 1991;4:145–60. tal megacolon: An analysis of 501 patients. J Pediatr Surg 25 Graham DY, Malaty HM, Evans DG, et al. Epidemiology of 1973;8:587–94. Helicobacter pylori in an asymptomatic population in the 41 Hyams JS, Treem WR, Etienne NL, et al. EVect of infant United States. EVect of age, race, and socioeconomic formula on stool characteristics of young infants. Pediatrics status. Gastroenterology 1991;100:1495–501. 1995;95:50–4. 26 Sigurdsson L, Flores A, Putnam PE, et al. Postviral 42 Fan LL, Graham LM. Radiological cases of the month. gastroparesis: Presentation, treatment, and outcome. J Lipid pneumonia from mineral oil aspiration. Arch Pediatr Pediatr 1997;131:751–3. Adolesc Med 1994;148:205–6. 27 Walker LS, Garber J, Green JW. Psychosocial correlates of 43 Partin JC, Hamill SK, Fischel JE, et al. Painful defecation recurrent childhood pain: a comparison of pediatric and fecal soiling in children. Pediatrics 1992;89:1007–9. patients with recurrent abdominal pain, organic illness, and 44 Sutphen J, Borowitz S, Ling W, et al. Anorectal manometric psychiatric disorders. J Abnormal Psychol 1993;102:248– examination in encopretic-constipated children. Dis Colon 58. Rectum 1997;40:1051–2. 28 Greenbaum DS, Mayle JE, Vangeren LE, et al.EVects of 45 Benninga MA, Buller HA, Heymans HSA, et al. Is encopre- desipramine on irritable bowel syndrome compared with sis always the result of constipation? Am J Dis Child 1994; atropine and placebo. Dig Dis Sci 1987;32:257–66. 71:186–93. http://gut.bmj.com/

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