Symptoms
Diagnosis
Umbilical pain
Organic problem the further, the pain originates from umbilicus
Early satiety, nausea, sour breath, belching
Peptic origin
Crampy pain and/or bloating and/or intestinal gas related to meals, as well as pain with dairy
Lactose intolerance, giardiasis
Cough, wheezing, laryngitis, pain supine
Gastroesophageal reflux
Irregular bowel movements, encopresis, mass in the left lower abdominal quadrant and abdominal distension
Constipation
Blood in stool
Inflammatory bowel disease
Bulimia behavior with or without weight loss
Gastroesophageal reflux from an eating disorder
Reference [2]
Box 11.2. ROME III [70]
ROME III classification of childhood functional abdominal pain disorders
H1.
Vomiting and aerophagia
H1a.
Adolescent rumination syndrome
H1b.
Cyclic vomiting syndrome
H1c.
Aerophagia
H2.
Abdominal pain-related FGIDs
H2a.
Functional dyspepsia
H2b.
Irritable bowel syndrome
H2c.
Abdominal migraine
H2d.
Childhood functional abdominal pain
H3.
Constipation and incontinence
H3a.
Functional constipation
H3b.
Nonretentive fecal incontinence
Keeping in mind not all abdominal pain is gastrointestinal in origin; establishing differential diagnosis of abdominal pain begins with good history and physical exam. The first step should identify any “red flags” that could suggest organic disease. Many clinicians, in the setting of chronic abdominal pain, will consider a baseline workup to include: CBC with differential, sedimentation rate, urine analysis, pancreatic enzymes, and fecal screening for ova and parasites.
The utility of imaging would depend on patients’ symptomatology. Abdominal ultrasound is a noninvasive test and does not expose the child to unnecessary radiation. The frequency of identification of abnormalities is as low as 1 % in children without “red flag” symptoms [5]. Still, many centers rely on ultrasound diagnosis to rule out possible organic cause of abdominal pain. Ultrasonographic examination of the abdomen is noninvasive and inexpensive test which is relatively painless for the pediatric patient. It can be utilized to detect irregularities of the kidneys, gallbladder, liver, pancreas, appendix, intestines, ovaries, and uterus. When symptoms and signs are present; such as jaundice, back pain, flank pain, vomiting, and/or abnormal physical exam findings, the probability of identifying organic abdominal abnormalities is increased to 11 %. In addition, imaging may have additional benefits as it can provide reassurance for both patients and families that some catastrophic event is not likely. In each case the potential for cost containment should be balanced against the risk that an incidental finding may cause more undue concern.
An example of a clinical entity where X-ray examination may be helpful is that of constipation. The contribution of constipation to chronic abdominal pain is often underappreciated. Abdominal pain caused by constipation is frequently left-sided, or suprapubic. A low-residue diet is the top cause of functional constipation, especially when it is greater than 3 months in duration. In some children whose habitus may prevent optimal abdominal evaluation, it may be reasonable to consider abdominal X-ray, if clinical history for constipation is highly suggestive.
Once the diagnosis of functional gastrointestinal disorder is made, it can be further categorized by the new Rome III criteria [9]. Into Functional Dyspepsia, Irritable Bowel Syndrome, Abdominal Migraine, Chronic Functional Abdominal Pain, and Chronic Functional Abdominal Pain Syndrome.
Functional Dyspepsia
To fulfill criteria for the functional dyspepsia symptoms listed below must occur at least once per week for at least 2 months. Those include:
1.
Persistent/recurrent pain or discomfort centered in upper abdomen (above the umbilicus).
2.
Pain not relieved by defecation, or associated with changes in stool frequency, or stool form.
3.
No evidence of inflammatory, anatomic, metabolic, or neoplastic process to explain such symptoms.
Upper endoscopy is no longer mandatory to establish diagnosis of functional dyspepsia. A recent publication describes the long-term outcomes in pediatric patients who underwent endoscopy and continue with dyspeptic symptoms and those with presence or absence of reflux esophagitis symptoms persisted for similar periods of time [10]. This prospective cohort study also demonstrated a strong association between pediatric dyspepsia and anxiety. About half of these patients studied had a lifetime history of one or more anxiety disorders [10].
Irritable Bowel Syndrome
Irritable Bowel Syndrome (IBS) is defined as an abdominal discomfort or pain associated with two or more of the following occurring at least 25 % of the time:
1.
Improvement with defecation
2.
Onset associated with change in frequency of stool
3.
Onset associated with change in form (appearance) of stool
Patients should not have any evidence of inflammatory, anatomic, metabolic, or neoplastic process to explain symptoms and such symptoms should be present once a week for at least 2 months.
Serious symptoms, like rectal bleeding, involuntary weight loss, growth retardation, and unexplained fevers necessitate further evaluation before consideration of functional gastrointestinal disease FGIDs such as IBS. Physical exam and history may assist in such determination. There can also be several other disease states that can mimic symptoms of IBS including lactose intolerance, sucrase-isomaltase deficiency, celiac disease, small bowel bacterial overgrowth, microscopic colitis, and bile acid malabsorption.
Post-infectious IBS is believed to be due to mild inflammation with subsequent visceral hypersensitivity that continues after infectious process has abated [11, 12]. It is therefore believed that childhood conditions associated with intestinal inflammation including cow’s milk allergy, and even recovery from pyloric stenosis may contribute to increased probability of developing childhood FGIDs including FAP and IBS [13, 14].
Abdominal Migraine
Abdominal migraine (AM) is defined as
1.
Pain severe enough to interfere with normal daily activities.
2.
Pain dull, or colicky in nature.
3.
Periumbilical, or poorly localized pain.
4.
Any two of anorexia, nausea, vomiting, headache, photophobia, or pallor.
5.
Attacks lasting for at least 1 h.
6.
Complete resolution of symptoms between attacks.
Children with migraine headaches are twice as likely to develop abdominal migraines, and children with abdominal migraines were twice as likely to have migraine headaches compared to general pediatric population [15].
In order to establish diagnosis of AM patients must have at least two or more of the following conditions over the preceding 12 months and are characterized by:
1.
Paroxysmal episodes of intense, acute periumbilical pain that lasts for 1 h or more.
2.
Intervening periods of usual health lasting weeks to months.
3.
Pain that interferes with normal activities.
4.
Pain associated with two of the following:
4.1
Anorexia
4.2
Nausea
4.3
Vomiting
4.4
Headache
4.5
Photophobia
4.6
Pallor
4.7
In addition, there should be no evidence of an inflammatory, anatomic, metabolic, or neoplastic process to explain above symptoms.
Abdominal migraines are present in 1–4 % of children, and more commonly in girls [16, 17]. Age of presentation is between 7 and 12 years of age. In one study investigators found that, in spite of the fact the patient may have met the diagnosis based on above criteria, in at least 4 % of patients presenting with chronic abdominal pain, none of the patients received the diagnosis of AM [18]. It is particularly important to recognize these cardinal features as well as the prevalence given for the potential abortive and preventative migraine-specific therapies [18].
Childhood Functional Abdominal Pain
Childhood functional abdominal pain is suspected if episodic or continuous pain is present once or twice a week for at least 2 months and there are insufficient criteria for other FGIDs, there should be no evidence of inflammatory, anatomic, metabolic, or neoplastic processes to explain such symptoms.
There is some degree of controversy concerning classification when children with FAP/IBS demonstrate low-grade inflammation. In 2008, Shulman published a prospective study of 65 children with FAP/IBS and compared to 39 age- and gender-matched controls. Their results suggested that proximal GI and colonic permeability were increased in affected patients. Furthermore, the same study proposed that the frequency low-grade inflammation in FAP/IBS patients was increased as measured rising fecal calprotectin levels [19]. This is in contrast to the previously published Norwegian study in which 90 % of pediatric patients with FGIDs had normal levels of fecal calprotectin.
Childhood Functional Abdominal Pain Syndrome
This new diagnostic category enables the inclusion of children with recurrent abdominal pain that does not impair the ability to maintain regular activities under the traditional diagnosis of functional abdominal pain. This syndrome includes both the impairment of some daily routine as well as extraintestinal symptoms.
Symptoms are present at least once a week for duration of at least 2 months and must satisfy the above listed criteria for childhood functional abdominal pain. In addition patients must have one or more of the following at least 25 % of the time:
1.
Some loss of daily functioning.
2.
Additional somatic symptoms including headache, limb pain, or difficulty sleeping.
Prospective studies with incorporation of Rome III criteria will make possible to gauge whether this distinction equates with significant changes in treatment strategies, and long-term prognosis [8].
Treatment
There is no clear, effective, evidence-based algorithm for treatment of abdominal pain. Currently, a multimodal, noninvasive treatment strategy is recommended. In a small percentage of cases, patients may benefit from an interventional or surgical intervention.
The goal of any therapy for functional abdominal pain is to reduce stress and alleviate tension for both the child and the parents. At the same time the therapy should promote normal patterns of physical activity, social interaction, and school attendance. The therapy must involve the patient, parents, pediatrician, gastroenterologist, psychologist, social workers, and teachers. It should be noted and reinforced that the pain the patient is experiencing is real pain; however, treatment goals should be aimed at minimizing disruption of daily activities due to abdominal complaints rather than treating a specific source of the pain. The overall focus should be on managing pain rather than eliminating pain as the target goal.
Families need to be cognizant that up to one-third of children with FGIDs may continue with symptoms in 5 years [20]. They need to feel comfortable validating the child’s abdominal complaints, without encouraging or reinforcing symptoms. For some children, discounting their symptoms leads to exacerbation of physical pain. An alliance with the child’s school nurse can impact ability to encourage school attendance. Specifically, familiarity with cognitive-behavioral therapy and guided imagery techniques, can allow for implementation of coping strategies for children with chronic abdominal pain.
In addition, arranging for a consultation with a child psychologist is a vital part to the treatment of functional abdominal pain. Psychological support is a valuable tool for the entire team in order to assist the child to cope with chronic pain. The goal of treatment is not the total elimination of symptoms, but rather the acquisition of strategies for coping with the pain and getting on with their life [21]. For children who miss school because of their symptoms, going back to school is a prime objective [22]. It is crucial to convey to the parents and the child that the pain is not due to organic causes. The family should be informed that stress may trigger symptoms. Taking time to explain to children (especially teens) about visceral hypersensitivity will help them to understand the physiology behind their pain. Patients should be given the details about the stretching of the bowel wall in relation to the child’s low-pain threshold. At the same time it should be reinforced that this does not mean that abdominal pain is not a real problem (or imply that the pain is “just in your head”) [21]. Often the explanation itself usually leads to a marked improvement of symptoms [23].
Cognitive-behavioral strategies have an established role in the treatment of children with anxiety and depression disorders. This seems appropriate given that cognitive-behavioral therapy is the treatment best supported by presently available evidence and is considered “probably efficacious” according to the widely established empirically supported treatment criteria [22, 24]. Due to the previously discussed observation of coexistence of anxiety and depressive disorders, such non-pharmacologic approaches are recommended as first-line therapy [8, 25, 26]. Two RCTs [27, 28] evaluated the efficacy of a cognitive-behavioral program and a cognitive-behavioral family intervention for the treatment of nonspecific abdominal pain. In the first study, results showed that both the experimental and the control groups had decreased levels of pain. However, the treated group improved more quickly, the effects generalized to the school setting, and a larger proportion of subjects were completely pain-free by 3 months of follow-up. In the second study, the children and mothers who were taught coping skills had a higher rate of complete elimination of pain, lower levels of relapse at 6 and 12 months’ follow-up, and lower levels of interference with their activities as a result of pain, and parents reported a higher level of satisfaction with the treatment. After controlling for pretreatment levels of pain, children’s active self-coping and mothers’ care giving strategies were significant independent predictors of pain behavior after treatment.
Multiple studies also indicate a supportive role for hypnotherapy [29–31]. Studies including hypnotherapy tend to show patients having decreased anxiety and improved activities of daily living. Dietary changes including increasing daily fiber are often recommended. Studies including adult experience are inconclusive. As it is inexpensive, it may be reasonable to consider. If added fiber therapy is chosen, the target goal for fiber (in grams per day) is the child’s age + 5—up to 30 g/day. Parents are advised that the introduction of fiber needs to be gradual, over several weeks.
If there are specific food triggers for pain that are identified, selective avoidance is indicated. Many families believe that food antigens are contributing to their child’s abdominal pain, as they perceive the pain is triggered by eating. Current evidence suggests that food allergies represent less than 5 % of children with FGIDs [32]. As such extensive allergy testing is unlikely to help with diagnosis or treatment of function chronic abdominal pain.
The use of probiotics has been shown to be beneficial in children with IBS [33–35]. Conversely studies have failed to find an association between pediatric functional abdominal pain and lactose intolerance [36]. Elimination of lactose from the diet is unlikely to improve chronic functional pain symptoms.
Pharmacological therapy for treatment of FGIDs has included the use of low-dose tricyclic antidepressants (TCAs); however, their utility is uncertain. A Cochrane review examined improvement of functional abdominal pain in children and adolescents and found that improvement did not differ substantially between those who did or did not receive medical therapy. The review was based on two small RCTs [37, 38]. There has since been a retrospective study of 98 patients in which there seemed to be an improvement in abdominal complaints with use of low-dose TCAs. On the other hand, Saps [38] published a study that included a placebo-controlled arm and did not demonstrate a difference between the treatment and control group.
Support for the possible utility of TCA’s stems from several investigations have reported higher levels of life stress in children with chronic abdominal pain compared with children without abdominal pain. Two studies compared pediatric abdominal pain patients with healthy school-age children and found significantly higher levels of life-event stress in patients with pain [39, 40] A separate diary study found that patients with recurrent episodes of abdominal pain reported significantly more daily stressors than school children without abdominal pain; moreover, the relation between daily stressors and somatic complaints was significantly stronger for patients with abdominal pain than for healthy school children [41].
Serotoninergic agents were studied via a small randomized-controlled trial of pizotifen in children with abdominal migraine. While the authors were able to demonstrate improvement in treatment versus placebo group, study size was limited to 14 patients, and this medication is not currently approved in the United States [42]. Another study evaluated the selective serotonin reuptake inhibitor Citalopram. Authors conducted a small (N = 25) 12-week open-label trial. The medication was well tolerated, but larger randomized controlled trials need to be considered [43].
In rare cases diagnostic laparoscopy may be indicated. Sringel et al. reported a case series of 13 children with chronic severe episodes of abdominal pain who were subjected to diagnostic laparoscopy. Laparoscopic findings identified the cause of abdominal pain in 12 of 13 patients. Laparoscopic appendectomy was performed in all patients. Abdominal pain resolved in ten patients. These authors concluded that diagnostic laparoscopy is a beneficial procedure in the management of some children with chronic recurrent abdominal pain resistant to other treatments [44].
When the child receives a diagnosis of a functional abdominal pain disorder, the rationale behind this diagnosis must be made evident. If a child continues to have abdominal pain, and organic causes have been excluded the physician should discuss cognitive-behavioral therapy with the patient and their family. Next, all treatment options must be discussed and appropriate time allowed for questions and answers. The family needs to understand that the symptoms support the diagnosis for the criteria pointing to the functional condition. All supporting data from the physical exam, laboratory results, and studies should be made readily available to reassure the patient and family. Any additional medical information that relates to the diagnosis should be provided to the family.