Summary of diagnostic criteria, pathophysiology, clinical evaluation and management plan for FGID in children based on Rome IV guidelines

1. Functional Gastrointestinal Disorders in
Children
Lokesh Tiwari
Associate Professor and Head of Pediatrics
All India Institute of Medical Sciences Patna
2nd June 2019

2. Objectives
• Updated classification of FGID
• Diagnostic Criteria for each disorder
• Pathophysiology
• Evaluation
• Treatment
• Role of probiotics

3. Rome criteria for FGID
• In Rome IV, the phrase “no evidence of an inflammatory, anatomic,
metabolic, or neoplastic process that explain the subject’s symptoms”
has been removed from diagnostic criteria.
• Instead “after appropriate medical evaluation, the symptoms cannot
be attributed to another medical condition.” is included
• It permits selective or no testing to support a positive diagnosis of an
FGID.
• FGIDs can coexist with other medical conditions.

4. Functional Gastrointestinal Disorders
H1. Functional nausea and vomiting disorders
• H1a. Cyclic vomiting syndrome
• H1b. Functional nausea and functional vomiting
• H1c. Rumination syndrome
• H1d. Aerophagia
H2. Functional abdominal pain disorders
• H2a. Functional dyspepsia
• H2b. Irritable bowel syndrome
• H2c. Abdominal migraine
• H2d. Functional abdominal pain not otherwise specified
H3. Functional defecation disorders
• H3a. Functional constipation
• H3b. Non-retentive fecal incontinence

5. H1. Functional nausea and vomiting disorders
• H1a. Cyclic vomiting syndrome
• H1b. Functional nausea and functional vomiting
• H1c. Rumination syndrome
• H1d. Aerophagia

6. H1a. Cyclic Vomiting Syndrome
Diagnostic Criteria: Must include all of the following:
1. The occurrence of 2 or more periods of intense, unremitting nausea and
paroxysmal vomiting, lasting hours to days within a 6-month period.
2. Episodes are stereotypical in each patient
3. Episodes are separated by weeks to months with return to baseline
health between episodes.
4. After appropriate medical evaluation, the symptoms cannot be
attributed to another condition.
*If the predominant feature is abdominal pain, consider abdominal migraine

7. Clinical evaluation
Underlying neurometabolic diseases:
• Higher likelihood in children with early onset of symptoms
• If considering metabolic testing, carry out during the vomiting
episode and before administration of IV fluids
Cannabinoid hyperemesis syndrome:
• Chronic use of cannabis in adolescents may leads to repeated
episodes of severe vomiting, nausea, and abdominal pain

8. Treatment
• Cyproheptadine in children <5 years of age
• Amitriptyline in children >5 years.
• Second-line treatment includes prophylaxis with propranolol
• Combinations of drugs or complementary treatments, such as
acupuncture and/or cognitive behavioral therapy
• Mitochondrial cofactors co-enzyme Q10 and L-carnitine as adjunctive
therapy
North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

9. H1b. Functional Nausea and Functional
Vomiting
Functional Nausea must include all of the following fulfilled for the last
2 months:
1. Bothersome nausea as the predominant symptom, at least twice
per week, and generally not related to meals
2. Not consistently associated with vomiting
3. After appropriate evaluation, the nausea cannot be fully explained
by another medical condition

10. H1b. Functional Nausea and Functional
Vomiting
Functional Vomiting must include all of the following:
1. On average, 1 or more episodes of vomiting per week
2. Absence of self-induced vomiting or criteria for an eating disorder
or rumination
3. After appropriate evaluation, the vomiting cannot be fully explained
by another medical condition

11. Clinical evaluation
• The presence of severe vomiting in addition to nausea: central
nervous system disease, GI anatomic abnormalities (eg, malrotation),
gastroparesis, and intestinal pseudo-obstruction should be excluded.
• Biochemical testing: serum electrolytes, calcium, cortisol, and thyroid
hormone levels.
• Consider and exclude Intestinal obstruction and motility disorders (eg,
gastroparesis, intestinal pseudo-obstruction) if recurrent vomiting.
• Normal upper GI endoscopy is not necessary for a diagnosis
• Psychological evaluation

12. Treatment
• Mental health intervention in patients with obvious psychological
comorbidities.
• Cognitive behavioral therapy and hypnotherapy have been used
• Cyproheptadine in children with functional dyspepsia with nausea
• Gastric electrical stimulation to treat intractable dyspepsia and may
be effective even in the absence of gastroparesis

13. H1c. Rumination Syndrome
Must include all of the following:
1. Repeated regurgitation and rechewing or expulsion of food that:
a. Begins soon after ingestion of a meal
b. Does not occur during sleep
2. Not preceded by retching
3. After appropriate evaluation, the symptoms cannot be fully
explained by another medical condition.
4. An eating disorder must be ruled out

14. Rumination Syndrome
Pathophysiologic features
• Increased intragastric pressure due to the contraction of the
abdominal muscles
• Displacement of gastroesophageal junction is into the thorax, rather
than relaxation of the lower esophageal sphincter (mechanism of
voluntary regurgitation)
• Triggering event: An intercurrent infectious process, a traumatic
psychosocial event, depression, anxiety disorder, obsessive
compulsive disorder, post-traumatic stress disorder, adjustment
disorder, developmental delays, and attention deficit-hyperactivity
disorder

15. Treatment
• Strategies for management of habit disorders.
• Inpatient interdisciplinary approach involving pediatric psychology,
pediatric gastroenterology, clinical nutrition, child life, therapeutic
recreation, and massage therapy has been reported in adolescents
with this condition

16. H1d. Aerophagia
Diagnostic Criteria: Must include all of the following
1. Excessive air swallowing
2. Abdominal distention due to intraluminal air which increases during
the day
3. Repetitive belching and/or increased flatus
4. After appropriate evaluation, the symptoms cannot be fully
explained by another medical condition.

17. H1d Aerophagia
Pathophysiologic features.
• When air swallowing is excessive, gas fills the GI lumen, resulting in
excessive belching, abdominal distention, flatus, and pain,
presumably as a consequence of luminal distention.
• In children who are unable to belch, symptoms of distention and pain
may be more severe.
• Underlying stressful events and anxiety can be a cause for excessive
air swallowing.

18. Clinical evaluation
• Motility disorders: gastroparesis, chronic intestinal pseudo-
obstruction.
• Bacterial overgrowth and malabsorption
• Celiac disease
• Disaccharidase deficiency
• In older children, chewing gum or drinking very quickly.

19. Treatment
• No controlled studies in children to guide therapy
• Supportive and behavioral therapy, psychotherapy, and
• Benzodiazepines

20. H2. Functional Abdominal Pain Disorders
• H2a. Functional dyspepsia
• H2b. Irritable bowel syndrome
• H2c. Abdominal migraine
• H2d. Functional abdominal pain not otherwise specified

21. H2a. Functional Dyspepsia
Diagnostic Criteria: Must include 1 or more of the following
bothersome symptoms at least 4 days per month:
1. Postprandial fullness
2. Early satiation
3. Epigastric pain or burning not associated with defecation
4. After appropriate evaluation, the symptoms cannot be fully
explained by another medical condition.

22. Pathophysiology
• Abnormalities of gastric motor function, visceral hypersensitivity due
to central or peripheral sensitization, low-grade inflammation, and
genetic predisposition.
• Impaired gastric accommodation, as determined by a decreased
ability of the stomach to relax in response to a meal,
• Sequela of an acute bacterial gastroenteritis
• No evidence in children that Helicobacter pylori gastritis causes
dyspeptic symptoms in the absence of duodenal ulcer.

23. Clinical evaluation: Potential Alarm Features
• Family history of inflammatory
bowel disease, celiac disease, or
peptic ulcer disease
• Persistent right upper or right
lower quadrant pain
• Dysphagia
• Odynophagia
• Persistent vomiting
• Gastrointestinal blood loss
• Nocturnal diarrhea
• Arthritis
• Perirectal disease
• Involuntary weight loss
• Deceleration of linear growth
• Delayed puberty
• Unexplained fever

24. Treatment
• Avoid NSAIDS and spicy, fatty or caffeine containing foods
• Address psychological factors
• H2 blockers and PPIs: If pain is predominant symptoms
• Omeprazole is superior to ranitidine, famotidine, and cimetidine
• Low-dose TCAs (amitriptyline and imipramine) in difficult cases.
• Prokinetics if nausea, bloating, and early satiety
• Cyproheptadine is safe and effective
• Gastric electrical stimulation is a promising option

25. H2b. Irritable Bowel Syndrome
• Diagnostic Criteria: Must include all of the following:
1. Abdominal pain at least 4 days per month associated with one or
more of the following:
a. Related to defecation
b. A change in frequency of stool
c. A change in form (appearance) of stool
2. In children with constipation, the pain does not resolve with
resolution of the constipation (children in whom the pain resolves
have functional constipation, not irritable bowel syndrome)

26. Pathophysiology of functional abdominal pain
disorders

27. Clinical evaluation
• Functional constipation: A careful history and physical examination
• In case of possible IBS with diarrhea: infection, celiac disease,
carbohydrate malabsorption, and, less commonly, inflammatory
bowel disease.
• Celiac disease can rarely present with constipation as well
• Likelihood of an organic disease if alarm symptoms
• Fecal calprotectin is good screen for intestinal mucosal inflammation
and superior to standard testing such as C-reactive protein.

28. Treatment
• Data supporting the utility of
• Probiotics
• Peppermint oil in reducing pain severity.
• Elimination diet reducing intake of fermentable oligosaccharides,
disaccharides, monosaccharides, and polyols.
• Behavioral treatments for symptom coping skills.

29. Abdominal Migraine
Diagnostic Criteria must include all of
the following occurring at least twice:
1. Paroxysmal episodes of intense,
acute periumbilical, midline or
diffuse abdominal pain lasting 1
hour or more (should be the most
severe and distressing symptom)
2. Episodes are separated by weeks to
months.
3. The pain is incapacitating and
interferes with normal activities
4. Stereotypical pattern and symptoms
in the individual patient
The pain is associated with 2 or more of
the following:
a. Anorexia
b. Nausea
c. Vomiting
d. Headache
e. Photophobia
f. Pallor

30. Clinical evaluation
• Supporting the diagnosis: prodromal symptoms, such as behavior or
mood changes, photophobia and vasomotor symptoms similar to
migraine headaches, and a history of symptom relief with
antimigraine therapy
• Exclude intermittent small bowel or urologic obstruction, recurrent
pancreatitis, biliary tract disease, familial Mediterranean fever,
metabolic disorders such as porphyria, and psychiatric disorders

31. Treatment
• Immediate symptom relief
• Oral pizotifen (anti-serotonin and antihistamine effects)
• Prophylaxis
• Amitriptyline,
• Propranolol and
• Cyproheptadine

32. H2d. Functional Abdominal Pain Not Otherwise
Specified
• Diagnostic Criteria must be fulfilled at least 4 times per month and
include all of the following:
1. Episodic or continuous abdominal pain that does not occur solely
during physiologic events (eg, eating, menses)
2. Insufficient criteria for irritable bowel syndrome, functional
dyspepsia, or abdominal migraine

33. Treatment
• Antispasmodics not significantly better than placebo in children.
• Amitriptyline
• Citalopram found a trend toward effectiveness compared to placebo
(black box warning for an increased risk of suicidal ideation in
adolescents)
• Cognitive behavioral therapy and hypnotherapy

34. H3. Functional defecation disorders
• H3a. Functional constipation
• H3b. Non-retentive fecal incontinence

35. H3a. Functional Constipation
Must include 2 or more of the following occurring at least once per week for
a minimum of 1 month with insufficient criteria for a diagnosis of irritable
bowel syndrome:
1. 2 or fewer defecations in the toilet per week in a child of a
developmental age of at least 4 years
2. At least 1 episode of fecal incontinence per week
3. History of retentive posturing or excessive volitional stool retention
4. History of painful or hard bowel movements
5. Presence of a large fecal mass in the rectum
6. History of large diameter stools that can obstruct the toilet

36. Pathophysiology
• Triggering event is universal instinct to avoid defecation because of
pain or social reasons (eg, school, travel).
• Colonic mucosa absorbs water from the feces and the retained stools
become progressively more difficult to evacuate.
• Viciouss cycle of stool retention
• Rectum is increasingly distended, resulting in overflow fecal
incontinence, loss of rectal sensation, and ultimately, loss of the
normal urge to defecate.
• Decreased motility in the foregut, leading to anorexia, abdominal
distention and pain

37. Alarm signs for underlying disease
• Passage of meconium >48 h in a
term newborn
• Constipation starting in the first
month of life
• Family history of Hirschsprung’s
disease
• Ribbon stools
• Blood in the stools in the absence
of anal fissures
• Failure to thrive
• Bilious vomiting
• Severe abdominal distension
• Abnormal thyroid gland
• Abnormal position of the anus
• Absent anal or cremasteric reflex
• Decreased lower extremity
strength/tone/reflex
• Sacral dimple
• Tuft of hair on spine
• Gluteal cleft deviation
• Anal scars

38. Some of the recommendations in FC
• Digital examination to confirm the diagnosis and exclude underlying medical
conditions
• No role for the routine use of an abdominal x-ray
• A plain abdominal radiograph may be used in a child if fecal impaction is
suspected but in whom physical examination is unreliable/not possible
• Routine allergy testing for cow’s milk allergy is not recommended in children with
constipation in the absence of alarm symptoms
• Laboratory testing to screen for hypothyroidism, celiac disease, and
hypercalcemia is not recommended in children with constipation in the absence
of alarm symptoms
• Rectal biopsy is the gold standard for diagnosing Hirschsprung’s disease
• A barium enema should not be used as an initial diagnostic tool

39. Treatment
2 steps:
1. Dis-impaction for children who present with fecal impaction
2. Maintenance therapy to prevent re-accumulation of feces using a
variety of agents.
• Polyethylene glycol is first-line therapy for constipated children
(superior to lactulose in 3 Cochrane Reviews)
• Only 50% of children were taken off laxatives successfully

40. Treatment
• Education: counseling families to recognize with-holding behaviors
and to use behavioral interventions,
• Regular toileting
• Use of diaries to track stooling,
• Reward systems for successful evacuations
• Normal fiber and fluid intake
• Addition of prebiotics and probiotics currently not supported by
adequate evidence.

41. H3b. Nonretentive Fecal Incontinence
• Diagnostic Criteria: At least a 1-month history of the following
symptoms in a child with a developmental age older than 4 years:
1. Defecation into places inappropriate to the sociocultural context
2. No evidence of fecal retention
3. After appropriate medical evaluation, the fecal incontinence cannot
be explained by another medical condition

42. Pathophysiology
• Patients with non-retentive fecal incontinence (NFI) have normal
defecation frequencies and colonic and anorectal motility parameters,
differentiating this condition from FC.
• NFI might be a manifestation of an emotional disturbance in a school-
aged child and represent impulsive action triggered by unconscious
anger.
• NFI has been described as a result of sexual abuse in childhood

43. Treatment
• Address psychological disturbances, learning difficulties, and behavioral
problems.
• Victims of sexual abuse must be identified and referred for appropriate
counseling.
• Behavioral therapy.
• Regular toilet training use with rewards and diminishing toilet phobia
contribute to lower distress, restore normal bowel habits, and re-establish
self-respect.
• Only 29% of the children were completely free of fecal incontinence.
• At 18 years of age, 15% of adolescents with NFI still had the disorder.
• No prognostic factors for success were identified

44. Probiotics

46. Abdominal pain frequency

47. Abdominal pain intensity

48. Treatment success

51. Defecationfrequency

52. TreatmentSuccess

54. Life Beyond Numbers
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