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Management of chronic constipation in children
SYMPOSIUM: GASTROENTEROLOGY
Management of chronic constipation in children*
Diagnosis of functional constipation is as per the ROME III criteria Infants up to 4 years should have at least two symptoms for 1 month prior to diagnosis and those over 4 years at least two of the following symptoms present for the previous 2 months*: 1. Two or fewer defaecations per week 2. At least one episode of faecal incontinence per week 3. Retentive posturing or stool retention 4. Painful or hard bowel movements 5. Presence of a large faecal mass in the rectum 6. Large diameter stools that may obstruct the toilet
Lucy J Howarth Peter B Sullivan
Abstract Children are commonly affected by constipation. Optimal management of chronic constipation requires a good understanding of the underlying pathophysiology. The presentation and management of constipation varies by age. This review aims to give the reader a clear guide to diagnosis, investigation, pharmacological and nonpharmacological management of chronic constipation in children. We describe the features typically evident in the clinical history and how the pathology can interrupt normal physiology. We outline the age dependent presentation and management of chronic, functional constipation based on the best available evidence and examine the NICE guideline for laxative use in children.
* Without objective evidence of a pathological condition and without fulfilling irritable bowel syndrome criteria.
Table 1
play a part. The association with behaviours is complex because constipation can be both caused by and cause changes in behaviour. Significantly higher rates of constipation have been reported amongst mothers of constipated children as opposed to fathers or siblings of a constipated child. A genetic component is likely to be part of the pathogenesis of functional constipation but no mutations in specific genes have been linked. Like many other functional gastrointestinal disorders the pathophysiology and prognosis are variably understood by medical practitioners. This results in a large variety of strongly held beliefs and management strategies. To understand constipation in childhood it is necessary to have a good knowledge of normal physiology, the wide range of normality and the role of diet and behaviour.
Keywords age dependent presentation; children; chronic constipation; clinical diagnosis; investigations; laxative treatment; non pharmacological treatment; red flag symptoms and signs
Introduction Constipation, derived from the Latin ‘constipare’, meaning ‘to cram together’, is the commonest gastrointestinal disorder comprising up to 25% of referrals to tertiary paediatric gastroenterology clinics. In primary, secondary and tertiary care, there are more consultations for constipation management than for other periodic, chronic conditions such as asthma or migraine. Chronic constipation is a heterogeneous group of disorders, and is often late-presenting. It is defined by infrequent and or difficult passage of stools, and is a clinical diagnosis that should be based on symptoms that fulfil the ROME III criteria (see Table 1).
Physiology of defaecation Enteric content enters the colon via the ileocaecal valve. Stools are formed by the progressive absorption of water, and are propelled along the colon to the rectum. Stool is stored until a socially acceptable time to defecate. The rectum stores and eliminates stool through a complex mechanism involving pelvic floor muscles, the autonomic and somatic nervous systems. The anorectal angle, formed by the anal sphincter complex and puborectalis muscle is crucial to successful storage and defaecation. This angle is 85e105 at rest. The rectum is usually empty but distension of the rectal wall with stool descending from the sigmoid colon causes a temporary reflex relaxation of the internal anal sphincter allowing stool to come into contact with sensitive receptors in the anal canal. The rectoanal inhibitory reflex results in a contraction of the internal sphincter, inhibiting defecation. The process, however, alerts the individual to the presence of stool, liquid or flatus in the rectum. An indication of the exquisite sensory innervation of the anorectum is the ability to distinguish between distension caused by solid, liquid or gas. Once a child has an opinion about the appropriate time to respond to this signal, a voluntary process of defaecation is either begun or overruled. If the sensation on passing stool is pain (for example when the stool is both large and hard) then even very young children can resist the urge to push. Such stool withholding e often misinterpreted as straining to evacuate stool e is frequent in toddlers. If the individual decides the time is right
Prevalence The reported prevalence of constipation varies from 0.8 to 28% and the condition has a wide geographic variability, with the highest reported prevalence in the USA and the lowest in Finland. Pathogenesis is multifactorial with research focussing on environmental factors, behavioural problems and genetic predisposition. Environmental factors such as activity level and diet but also low maternal education level or social circumstance *
This article is based on a previously published article of the same name, published in this journal issue 22(10): 401e8. Lucy J Howarth BM MRCPCH is a Consultant in Paediatric Gastroenterology in the University Department of Paediatrics, Children’s Hospital, John Radcliffe Hospital, Headington, Oxford, UK. Conflict of interest: none Peter B Sullivan MA MD FRCP FRCPCH is Reader in Paediatric Gastroenterology, Honorary Consultant Paediatrician, University Department of Paediatrics, Children’s Hospital, John Radcliffe Hospital, Headington, Oxford, UK. Conflict of interest: none
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SYMPOSIUM: GASTROENTEROLOGY
then increased intrarectal pressure comes from straining of intraabdominal muscles and pelvic floor muscles to push faeces towards the anal canal. The puborectalis muscle relaxes to allow the descent of the pelvic floor, straightening the anorectal angle and inhibiting the internal and external anal sphincters, allowing faeces to be expelled. In newborn babies and very young infants the voluntary element of control is not yet developed so defaecation occurs following initial distension of the rectal wall.
It is important that there is a general awareness of the wide range of rare pathologies that may present with constipation as effective management depends on an understanding of the underlying pathophysiology. Constipation should be regarded as a symptom and not a disease. Anorectal malformations and Hirschsprung’s disease are amongst the commoner pathologies underlying very early onset childhood constipation.
History Pathophysiology of constipation
Pitfalls Although the ROME III criteria (Table 1) appear self-explanatory, history-taking can be difficult. The key features in history are described in Box 1. Care must be taken. Often history depends upon reports by parents or other carers, and may be subject to over- or under-reporting bias. Functional constipation may often present late, or with abdominal pain or spurious diarrhoea. A large faecal mass in the rectum gives the sensation of incomplete evacuation and children may try to open their bowels several times a day. If only small amounts of soft/liquid stools are passed around the sides of the obstructive faecal mass, this is termed overflow, or overflow diarrhoea. Anal canal trauma from passage of hard or large stool can present with bright red rectal bleeding or severe anal pain. Pain may exacerbate the problem, as it will inhibit defecation.
Whilst the majority (more than 90%) of children with chronic constipation will be considered to have functional, idiopathic constipation, exclusion of organic causes is important (summarised in Table 2). Constipation is also an important side effect of several classes of medicines and is commonly encountered in children receiving opiates, antacids or iron. Coeliac disease is commonly thought of as causing diarrhoea, but constipation is seen, possibly due to anorexia or changes in ileal function or gut motility. Constipation is very common in children and adults with cystic fibrosis, where there is an association with low total fat absorption and a history of meconium ileus. Neuromuscular conditions can affect the gut. Smooth muscle cells and intestinal cells of Cajal play a major role in normal gut motility. These cells ensure regular contractions of the colonic wall and propulsion of content. Constipation is often seen in patients with cerebral palsy and Duchenne muscular dystrophy. In patients with spinal muscular atrophy (SMA) proximal muscle weakness is a cardinal feature. In most of these patients constipation is a problem because of reduced abdominal muscle tone as well as disturbed innovation of the myenteric plexus. Children with significant developmental delay are more prone to constipation for a wide variety of reasons depending on their underlying disorder. For example, children with cerebral palsy often have dysmotility problems. Disorders that affect the enteric nervous system such as hereditary sensory and autonomic neuropathy (previously known as RileyeDay syndrome) are associated with constipation. Normal gastrointestinal motility is disturbed by abnormal autonomic function. Constipation can be a feature of disorders that affect water/ electrolyte balance such as diabetes insipidus. This can lead to reduced water content i.e. harder stools or as a result of muscle weakness caused by electrolyte imbalance. Other endocrine diseases such as MEN3 and hypothyroidism can present with constipation.
Key features in history taking C C C C
C C
C C
C C
Delay in passage of meconium Age at onset Relation to toilet training Toileting history-stool frequency, consistency, pain, soiling, presence of blood Stool withholding behaviour Urinary symptoms (13% of those with constipation have urinary symptoms) Abdominal pain Diet-history of exacerbation e.g. with cow’s milk or a poor diet low in fruit and fibre General health and developmental milestones Family and social history
Box 1
Organic causes of childhood constipation Structural colorectal
Spinal cord
Systemic
Neuropathic lesions of intestine
Drugs
Other important causes
Anal stenosis Post NEC/IBD stricture
Spina bifida Sacral agenesis
Diabetes Hypothyroidism
Hirschsprung disease Intestinal neuronal dysplasia
Opioid analgesia Antacids
Coeliac disease Cystic fibrosis
Chronic intestinal pseudo- obstruction
Spinal cord tumours
Hypo/hypercalcaemia
Iron
Cows milk protein allergy
Neurofibromatosis Cerebral palsy
Cholestyramine
Table 2
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SYMPOSIUM: GASTROENTEROLOGY
Age dependent presentation
Age of toilet training
Newborn e 4 months 99% of term infants pass meconium within the first 24 hours following delivery. Very low birth weight or premature infants can have non-pathological delay in opening their bowels. 94% of children with Hirschsprung’s Disease and 25% of those with cystic fibrosis have initial delay in the passage of meconium. Newborn babies generally have a higher stool frequency of around 4/day but there is particularly high variability amongst breast-fed babies who can sometimes not open their bowels for days without being constipated. It is unusual for babies to become constipated in the first 4 months unless they have a congenital/anatomical abnormality, systemic upset or possibly cow’s milk protein allergy. A change from breast to formula feeding can result in constipation or the use of ‘hungrier baby’ milk with higher sodium content. It is unusual for young babies to develop chronic constipation Beware of red flags in the history and examination Late passage of meconium Systemically unwell Anatomical anomalies Developmental delay
This is a common time for children to become constipated. The age at which children start toilet training varies between and within families. There is evidence that early toilet training is associated with a higher incidence of constipation. The optimum time is when a child is able to communicate their desire to ‘do a poo’, has enough motor control to be able to overrule an urge if no toilet is available and can sit and actively strain on a toilet when the time is right. Many children and families find this stage of development a stressful, frustrating and unpleasant time. The transition can be very frightening to young children. The inconvenience of cleaning up accidents and the patience necessary to encourage young children to successfully toilet train should not be underestimated. Many families need help with this stage of normal development and may benefit from support groups or parenting classes. Long-standing constipation often starts with difficulties with early toilet training and adverse toileting behaviour. C
It is always important to ask about the ease of potty training as this is a common time for constipation to begin
Box 2
5 months to potty-training During this period infants start to wean from milk onto a variety of foods. The commonest cause of constipation in this age group is change in diet and consistency of stools. As baby rice and high fibre foods are added to an infant’s diet, stools become more bulky. It is common for parents to describe long periods of straining and sometimes pain associated with large diameter stools. Often early constipation can be treated effectively with change in weaning foods, such as an increase in fruits such as pear and less baby rice until the infant’s digestive system has adapted. In babies with reflux, no response to laxatives or a family history of atopy, a trial of a dairy-free diet (cow’s milk and soya) may be helpful. A clear association with cow’s milk protein allergy and constipation has been demonstrated. Many children start to take gluten in their diet at 9e12 months and if gastrointestinal symptoms including constipation start at this time then celiac disease should be considered. Toddlers who have had the sensation of pain following the passage of a hard stool are at high risk of stool withholding and rapidly worsening constipation. It is important to ask questions about posturing during a bowel movement as withholding behaviours can easily be misinterpreted as straining. In toddlers nearing three or at the time of potty training it is very important that a period of time has passed with soft stools to allow them to gain confidence and lose their fear of opening their bowels prior to any attempts to encourage them to use a potty or toilet. Often subtle manipulations in diet and fluid intake can be helpful. Some children are prone to constipation and some may drink insufficient fluid particularly in hot weather. A consultation with a paediatric dietician is invaluable, but may not be readily available (Box 2). Constipation is often related to dietary changes Do not forget the association with cow’s milk protein allergy and coeliac disease Stool withholding may result from painful defaecation and can be easily mistaken for straining
School age children Children generally learn to use their own toilet at home but need their parents’ ongoing support, praise and bottom wiping for some years afterwards. When children start full time school they need to be independent. Schools have variable amounts of support available for toileting needs of young reception age children. Toilets may be inaccessible from the playground or lunch hall
Key features in examination C
General health, plot on growth chart Look for evidence of systemic illness, faltering growth, anaemia
C
Abdominal palpation Evidence of faecal loading þ/ a faecal mass this is difficult in the obese child
C
Perianal region and digital rectal examination Examine perianal region for signs of fissures, soiling, infection (such as group A streptococcus) or anatomical anomalies A rectal examination should only be performed if it is likely to provide additional information: a sudden gush of stool may be found in a child with Hirschsprung’s disease post examination. Hard, impacted stool may be found confirming the need for a faecal disimpaction
C
Neurological exam, including inspection of the lumbar-sacral spine and examination of the lower limbs Spinal abnormalities or spinal cord tumours can present with constipation
Box 3
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Practical tips to improve toileting behaviour such as ensuring enough time for breakfast can be helpful Involve children in advice about lifestyle changes
and young children are often scared to go alone, unable to do up buttons or wipe their own bottom. This is a time where stool withholding and inevitable constipation is common. One approach that can be helpful is to try and emphasise the importance of adequate time before school for breakfast and going to the toilet. Sometimes there are improvements that can be made to privacy at school or the decorative state of the toilets to make them more appealing. There are often significant changes in diet at this time with children eating school dinners and having to make independent choices about what they do and don’t eat. Dietetic assessment at this age is very helpful and it is important to involve the child in education about healthy eating and a diet that has adequate fruit, fibre and fluid. Children are usually only allowed to drink water at school and many prefer juice prior to this and so drink much less than usual while running around more. This dehydration can exacerbate constipation.
Older children and early teens Children can become acutely constipated at any stage following an infection, change in diet, routine (e.g. moving house or school) or emotional upset. When reviewing older children early history remains important as symptoms are often long standing. Eating disorders need to be considered. Constipation can present with abdominal pain, nausea, anorexia or soiling. Rarely soiling may occur because of an involuntary leakage of soft stool around faeces impacted in the rectum. This will cause significant psychological morbidity. Older children who have been constipated for some time and who are soiling regularly are often bullied and become withdrawn and disengaged with education. Families find the symptom of soiling understandably
Investigation of children with chronic constipation Investigation
Indication
Change to management
Plain abdominal radiograph
Not usually indicated
Bowel transit studies Radio e opaque shapes swallowed daily for 5 days and then an abdominal radiograph performed to assess the transit time of the shapes
Not routinely indicated e interpret with caution as shapes can adhere to the colonic mucosa Useful in children with chronic diarrhoea or soiling for whom there is uncertainty about constipation as the underlying cause Not indicated in young children. Demonstrates whether there is normal relaxation of the internal anal sphincter in response to rectal dilation May be indicated if there is possibility of cow’s milk protein enteropathy To exclude Hirschsprungs disease if delay in early passage of meconium or only able to pass paste like stools with long standing constipation or abdominal distension All children with long standing constipation not responding to initial management
May be useful to exclude sacral agenesis or other spinal abnormalities Occasionally used to provide families with visual evidence of constipation If delayed transit shown it is more acceptable to families to comply with the advice for a child to undergo disimpaction of faeces
Anorectal manometry
Endoscopy Full thickness rectal biopsy
Coeliac screen tissue transglutaminase or endomysial antibodies þ immunoglobulin A levels
Electrolytes and glucose
Thyroid function
Fbc and iron studies
Concerning features of history or examination or not responding to treatment. Exclude hypo/ hypercalcaemia Urine dip-exclude diabetes Infants with early constipation and developmental delay. Family history of thyroid disease. Clinical suspicion Iron deficiency anaemia common because of poor diet. Iron therapy can cause constipation
Can demonstrate anatomical reasons underlying constipation. May need referral to a colorectal surgeon May demonstrate eosinophilic enteropathy Surgical excision of aganglionic bowel with temporary colostomy followed by a later pull through anastamoses If serology positive will need endoscopic duodenal biopsies and histological confirmation of the diagnosis while on full gluten containing diet Gluten free diet should lead to a resolution of symptoms Treat electrolyte abnormality
Thyroxine replacement
Treat iron deficiency anaemia
Table 3
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SYMPOSIUM: GASTROENTEROLOGY
difficult to manage and often feel anger and resentment towards the child who they often blame for the problem. Treatment must address underlying issues and offer support to the child and the family (Box 3). Constipation can present with a variety of symptoms and often families need a lot of reassurance that there is no serious underlying pathology Symptoms have often been longstanding
Investigations At all ages, if the history and examination are suggestive of functional constipation and there are no abnormal findings on examination, further investigations are not indicated. If a child has long standing constipation that has been resistant to treatment or there are worrying features then the following investigations may be considered [see Table 3].
Management The overall aim of management of childhood constipation should be rapid diagnosis and restoration of regular, pain free bowel movements at the appropriate time in the appropriate place with the minimum amount of laxatives possible. Effective management (summarised in Figure 1) relies on a good relationship with the family and interventions to treat the underlying cause and not just the symptom.
Figure 1 Non-pharmacological treatment of constipation.
to listen to music or read books while sitting and straining on the toilet. Breathing techniques can be useful to overcome fear. Some children find blowing bubbles or blowing up balloons help with their efforts to push a stool out. It can be helpful for children to have a foot-stool to rest their feet on while opening their bowels or a cushioned seat that can be smaller than the full adult sized seat. A non-punitive approach is necessary to improve toileting behaviour Aim to improve the whole toileting experience
Diet and lifestyle Education about normal physiology and healthy eating habits is a cornerstone of management. There is wide variability in the adequacy of children’s diets and multiple factors including socioeconomic status, health beliefs of families and eating habits of individual children. There is a clear correlation between inadequate fibre and fluid intake and also evidence that it is very difficult to change eating habits of families. There is insufficient evidence to suggest a high fibre or high fluid intake is beneficial and is difficult to comply with. A dietetic assessment is extremely useful to accurately assess an individual child’s diet and make recommendations that are likely to be acceptable/manageable for their family. The recommended fibre intake in children over 2 is their age in years plus 5 g per day. Two weetabix provide 3.8 g fibre. The importance of breakfast as a meal and starting the family day earlier to make time for breakfast, activity and then a relaxed attempt at opening bowels using the gastrocolic reflex and achieving a clear out prior to nursery/school is useful advice. Although there is a lack of trial evidence to support the effectiveness of increased physical activity in constipation management, it is a common observation that obesity and lack of physical activity are associated with constipation. A diet sufficient in fruit, fibre and adequate fluid will be essential to an effective cure Increased activity levels are helpful
Psychological assessment Many children and families who have been struggling with chronic constipation benefit enormously from psychological input. There are many issues which commonly arise such as toilet phobia, depression, distress from bullying, or abuse and clinical psychologist have many effective strategies that help children. Behaviour modification through reward Reward schemes are often successful; depend on a consistent approach and the child being old enough to respond. Star charts can be used, which can be as simple as a blank sheet of paper with stars for eating breakfast, sitting on the toilet, taking medication, doing a poo on the toilet or dealing with relevant issues. It is important that the rewards are awarded frequently enough to be seen as valuable by the child. Star charts should be used at appropriate ages
Toileting behaviour Difficulties with the transition from nappies to potty/toilet are common and it is always useful to help family’s access advice about toilet training. The basic principal of ignoring failure and praising success is important to convey. Improving the environment in the toileting area can be effective. Some children like
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Pharmacological treatment Success of pharmacological treatment (summarised in Figures 2 and 3) relies on addressing the underlying causes of constipation and the non-pharmacological advice should be
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SYMPOSIUM: GASTROENTEROLOGY
reviewed and reinforced each time a child comes to clinic. When medicines are required for treatment it is helpful to divide the treatment regimen into disimpaction and maintenance therapies [see below]. Constipation management is complicated and requires regular review and assessment particularly at the beginning of laxative treatment. In children with acute onset constipation or mild symptoms lifestyle manipulations may be enough. For the majority seeking medical help it will be necessary to start a laxative. If the underlying problem is infrequent passage of large, hard stools then it is logical that a faecal softener should be the first line treatment. For many children with overflow diarrhoea, soiling or long standing symptoms disimpaction of large volume, hard stool in the rectum is essential before any management strategies or lifestyle changes will have any effect. There is little trial data to guide stimulant laxative use but there is widespread
consensus amongst specialists that they are often required either to ensure adequate disimpaction or as a maintenance agent. A systematic review of the literature showed only 50% of all children started on laxative treatment followed for 6e12 months are shown to recover and be asymptomatic whilst no longer on laxatives. It is important that expectations are managed and the length of treatment with laxatives is not underestimated.
Disimpaction Disimpaction can be achieved at home but families will need support. Children are asked to drink large volumes of PEG 3350 which can be difficult to tolerate. A stimulant laxative is added if disimpaction has not been achieved by 2 weeks. In practice, in severe cases may require enemas. A recent RCT compared daily enemas with 1.5 g/day PEG 3350 and found no significant
Figure 2 Disimpaction: as recommended by NICE guideline.
Figure 3 Maintenance therapy for treatment of constipation: as recommended by NICE.
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SYMPOSIUM: GASTROENTEROLOGY
difference in efficacy or in behaviour scores, measuring fear/ distress, caused by the treatment. The NICE guideline recommends trying oral stool softeners and stimulants or sole agent stimulant laxative such as senna or sodium picosulpahte (elixir) or picolax (sachet) prior to rectal medication.
Maintenance treatment It is good practice to follow up the initial visit with a phone call and to review children regularly to ensure adequate disimpaction. Constipation is often most effectively managed in the community by a specialist nurse who forms a relationship with the family and
Osmotic laxatives Age
Dose
Mechanism of action
Side effects
Lactulose
Infants under 2
1e3 ml/kg
Relating to fermentation by products with excess gas production leading to flatulence, bloating and abdominal cramps
PEG 3350 (with electrolytes) Movicol
Only licensed for over 2 years but recommended by NICE as 1st line for all
0.3e0.8 g/kg/day maintenance 1e1.5 g/kg/day disimpaction
Non-absorbable disaccharide of sugars D-galactose and D-fructose. Fermented by colonic bacteria producing hyperosmolar byproducts Large molecular size. Not absorbed. Produces a direct osmotic effect. No impact on electrolyte balance
Side effects
Diarrhoea, abdominal distension, nausea
Table 4
Stimulant laxatives Age
Dose
Mechanism of action
Sodium picosulphate
Elixir 5 mg/5 ml Child 1 month to 4 years Child 4e18 years
2.5e10 ml od 2.5e20 ml od
Bisacodyl
Orally Child 4e18 years Rectally Child 2e18 years
5e20 mg od 5e10 mg od
Stimulating the mucosa of both the colon, causing peristalsis, and the rectum, causing increased motility and a feeling of rectal fullness By bacterial cleavage the active form is formed in the colon Action after 6e12 hours Diphenylmethane laxative, which, when activated, stimulates intestinal fluid secretion and colonic contraction
Senna
Elixir 7.5 mg/5 ml Child 1 month to 4 years Child 4e18 years
2.5e10 ml od 2.5e20 ml od
Docusate sodium
6 monthse2 years 2e12 years 12e18 years
12.5 mg tds 12.5e25 mg tds 500 mg/day in divided doses
Anthraquinone laxative increase fluid and electrolyte accumulation in the distal ileum and colon after metabolism. This occurs only when the prodrug comes into contact with intestinal micro-organisms. For this reason, sennosides can be given orally but not by suppository An anionic surfactant that reduces surface tension of water, allowing water to penetrate the intestine, softening the stool. Docusate also has a stimulatory affect by increasing cAMP concentrations in colonic mucosal cells which increases contractility
Withdrawn in the United States after rodent studies suggested carcinogenic links. Subsequent studies have not demonstrated an increased risk of cancer in humans taking phenolphthalein laxatives Changes in the colon produced by chronic anthraquinone use include melanosis coli, a benign and reversible condition. No evidence exists that anthraquinone laxatives given in clinically appropriate doses cause enteric damage
Table 5
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SYMPOSIUM: GASTROENTEROLOGY
provides regular support over the phone and in clinic. The addition of a stimulant laxative such as senna once stools are soft is often necessary to overcome stool withholding. Stimulant laxatives work by increasing intestinal motility. Intermittently stimulant laxatives receive adverse press and as the side effects can be more prominent. As the safety and long term efficacy in children is not proven, the use of stimulants should be regularly reviewed. There are a wide number of osmotic and stimulant laxatives available. There mechanisms of action and important side effects are summarised in Tables 4 and 5. Trial evidence commonly suffers from small sample sizes and short duration of follow-up. There have been recent randomised trials and a meta-analysis of efficacy for PEG 3550. Two systematic reviews compared PEG 3350 with placebo in the treatment of chronic constipation in children. PEG 3350 significantly improved pain during defaecation and increased the number of stools per week but did not significantly reduce the number of incontinent episodes.
Burnett CA, Juszczak E, Sullivan PB. Nurse management of intractable functional constipation: a randomised controlled trial. Arch Dis Child 2004; 89: 717e22. Caffarelli, et al. Characterization of irritable bowel syndrome and constipation in children with allergic diseases. Eur J Pediatr 2007; 166: 1245e52. Constipation in children and young people. Diagnosis and management of idiopathic childhood constipation in primary and secondary care. May 2010. National Institute for Health and Care Excellence. National Institute of Clinical Excellence Guideline, www.nice.org.uk/guideline/CG99. Kiefte-de Jong, et al. Infant nutritional factors and functional constipation in childhood: the generation R study. Am J Gastroenterol March 2010; 105: 940e5. Peeters B, Benninga MA, Hennekam RC. Childhood constipation; an overview of genetic studies and associated syndromes. Best Pract Res Clin Gastroenterol 2011; 25: 73e88. Pijpers MA, Bongers ME, Benninga MA, Berger MY. Functional constipation in children: a systematic review on prognosis and predictive factors. J Pediatr Gastroenterol Nutr 2010; 50: 256e68. Choung RS, Shah ND, Chitkara D, et al. Direct medical costs of constipation from childhood to early adulthood: a population-based birth cohort study. J Pediatr Gastroenterol Nutr 2011; 52: 47e54. Rajindrajith Shaman, Devanarayana Niranga Manjuri. Constipation in children: novel insight into epidemiology, pathophysiology and management. J Neurogastroenterol Motil 2011; 17. Sullivan PB, Alder N, Shrestha B, Turton L, Lambert B. Effectiveness of using a behavioural intervention to improve dietary fibre intakes in children with constipation. J Hum Nutr Diet 2011; 25: 33e42. Sullivan PB, Juszscak E, Burnett C. Parent satisfaction in a nurse-led clinic compared with a paediatric gastroenterology clinic for the management of intractable, functional constipation. Arch Dis Child 2006; 91: 499e501. Tabbers MM, Boluyt N, Berger M, Benninga MA. Diagnosis and treatment of functional constipation. Eur J Pediatr 2011; 170: 955e63.
Discussion Constipation is a distressing symptom for children and their families and is time consuming and difficult medical problem to manage. Management of constipation can be dispiriting as often symptoms persist and families often become very frustrated. It is important to provide targeted and appropriate lifestyle advice. Laxatives alone will rarely be sufficient to cure any patient of chronic constipation. While most children are unable to entirely change their lifestyle the importance of the non-pharmacological treatment of constipation is crucial to its successful management. With the development of chronic constipation, possibly exacerbated by stool withholding, the rectum changes from being highly sensitive to distension by stool to a large, distended sac unresponsive to distension. Moreover, the longer the condition persists the more likely it is that there will be histological changes in the lining (mucosa) of the rectum. Atrophy of the rectal musculature with degeneration (focal muscle fibre vacuolation) of muscle or even muscle fibre disappearance has been found in all chronically constipated children where this has been looked for. These changes are long-lasting and manometric (pressure) studies show that abnormal anorectal function is still present many years after the cessation of treatment and recovery from chronic constipation. Constipation is a distressing symptom for children and their families and is time consuming and difficult medical problem to manage. Management of constipation can be dispiriting as often symptoms persist and families often become very frustrated. It is important to provide targeted and appropriate life style advice. Laxatives alone will rarely be sufficient to cure any patient of chronic constipation. While most children are unable to entirely change their lifestyle the importance of the non-pharmacological treatment of constipation is crucial to its successful management. A
Practice points C
C
C
C
C
FURTHER READING Borrelli O, Barbara G, DiNardo G, et al. Neuroimmune interaction and anorectal motility in children with food allergy-related chronic constipation. Am J Gastroenterol 2009; 104: 454e63.
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C
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The diagnosis of functional constipation in childhood should be clinical and be based on fulfilling the ROME criteria It is important to exclude underling pathology that either directly or indirectly causes constipation as a symptom Investigations should be tailored to children who have concerning signs or symptoms and be based on the clinical history It is important to be aware of the differences in presentation at different ages, and underlying causes of constipation Treatment should be targeted on the underlying cause and be based on lifestyle modification and effective use of appropriate laxatives Management of constipation benefits from a multidisciplinary team approach and families need ongoing support to improve the success of treatment
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Management of chronic constipation in children*
Diagnosis of functional constipation is as per the ROME III criteria Infants up to 4 years should have at least two symptoms for 1 month prior to diagnosis and those over 4 years at least two of the following symptoms present for the previous 2 months*: 1. Two or fewer defaecations per week 2. At least one episode of faecal incontinence per week 3. Retentive posturing or stool retention 4. Painful or hard bowel movements 5. Presence of a large faecal mass in the rectum 6. Large diameter stools that may obstruct the toilet
Lucy J Howarth Peter B Sullivan
Abstract Children are commonly affected by constipation. Optimal management of chronic constipation requires a good understanding of the underlying pathophysiology. The presentation and management of constipation varies by age. This review aims to give the reader a clear guide to diagnosis, investigation, pharmacological and nonpharmacological management of chronic constipation in children. We describe the features typically evident in the clinical history and how the pathology can interrupt normal physiology. We outline the age dependent presentation and management of chronic, functional constipation based on the best available evidence and examine the NICE guideline for laxative use in children.
* Without objective evidence of a pathological condition and without fulfilling irritable bowel syndrome criteria.
Table 1
play a part. The association with behaviours is complex because constipation can be both caused by and cause changes in behaviour. Significantly higher rates of constipation have been reported amongst mothers of constipated children as opposed to fathers or siblings of a constipated child. A genetic component is likely to be part of the pathogenesis of functional constipation but no mutations in specific genes have been linked. Like many other functional gastrointestinal disorders the pathophysiology and prognosis are variably understood by medical practitioners. This results in a large variety of strongly held beliefs and management strategies. To understand constipation in childhood it is necessary to have a good knowledge of normal physiology, the wide range of normality and the role of diet and behaviour.
Keywords age dependent presentation; children; chronic constipation; clinical diagnosis; investigations; laxative treatment; non pharmacological treatment; red flag symptoms and signs
Introduction Constipation, derived from the Latin ‘constipare’, meaning ‘to cram together’, is the commonest gastrointestinal disorder comprising up to 25% of referrals to tertiary paediatric gastroenterology clinics. In primary, secondary and tertiary care, there are more consultations for constipation management than for other periodic, chronic conditions such as asthma or migraine. Chronic constipation is a heterogeneous group of disorders, and is often late-presenting. It is defined by infrequent and or difficult passage of stools, and is a clinical diagnosis that should be based on symptoms that fulfil the ROME III criteria (see Table 1).
Physiology of defaecation Enteric content enters the colon via the ileocaecal valve. Stools are formed by the progressive absorption of water, and are propelled along the colon to the rectum. Stool is stored until a socially acceptable time to defecate. The rectum stores and eliminates stool through a complex mechanism involving pelvic floor muscles, the autonomic and somatic nervous systems. The anorectal angle, formed by the anal sphincter complex and puborectalis muscle is crucial to successful storage and defaecation. This angle is 85e105 at rest. The rectum is usually empty but distension of the rectal wall with stool descending from the sigmoid colon causes a temporary reflex relaxation of the internal anal sphincter allowing stool to come into contact with sensitive receptors in the anal canal. The rectoanal inhibitory reflex results in a contraction of the internal sphincter, inhibiting defecation. The process, however, alerts the individual to the presence of stool, liquid or flatus in the rectum. An indication of the exquisite sensory innervation of the anorectum is the ability to distinguish between distension caused by solid, liquid or gas. Once a child has an opinion about the appropriate time to respond to this signal, a voluntary process of defaecation is either begun or overruled. If the sensation on passing stool is pain (for example when the stool is both large and hard) then even very young children can resist the urge to push. Such stool withholding e often misinterpreted as straining to evacuate stool e is frequent in toddlers. If the individual decides the time is right
Prevalence The reported prevalence of constipation varies from 0.8 to 28% and the condition has a wide geographic variability, with the highest reported prevalence in the USA and the lowest in Finland. Pathogenesis is multifactorial with research focussing on environmental factors, behavioural problems and genetic predisposition. Environmental factors such as activity level and diet but also low maternal education level or social circumstance *
This article is based on a previously published article of the same name, published in this journal issue 22(10): 401e8. Lucy J Howarth BM MRCPCH is a Consultant in Paediatric Gastroenterology in the University Department of Paediatrics, Children’s Hospital, John Radcliffe Hospital, Headington, Oxford, UK. Conflict of interest: none Peter B Sullivan MA MD FRCP FRCPCH is Reader in Paediatric Gastroenterology, Honorary Consultant Paediatrician, University Department of Paediatrics, Children’s Hospital, John Radcliffe Hospital, Headington, Oxford, UK. Conflict of interest: none
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SYMPOSIUM: GASTROENTEROLOGY
then increased intrarectal pressure comes from straining of intraabdominal muscles and pelvic floor muscles to push faeces towards the anal canal. The puborectalis muscle relaxes to allow the descent of the pelvic floor, straightening the anorectal angle and inhibiting the internal and external anal sphincters, allowing faeces to be expelled. In newborn babies and very young infants the voluntary element of control is not yet developed so defaecation occurs following initial distension of the rectal wall.
It is important that there is a general awareness of the wide range of rare pathologies that may present with constipation as effective management depends on an understanding of the underlying pathophysiology. Constipation should be regarded as a symptom and not a disease. Anorectal malformations and Hirschsprung’s disease are amongst the commoner pathologies underlying very early onset childhood constipation.
History Pathophysiology of constipation
Pitfalls Although the ROME III criteria (Table 1) appear self-explanatory, history-taking can be difficult. The key features in history are described in Box 1. Care must be taken. Often history depends upon reports by parents or other carers, and may be subject to over- or under-reporting bias. Functional constipation may often present late, or with abdominal pain or spurious diarrhoea. A large faecal mass in the rectum gives the sensation of incomplete evacuation and children may try to open their bowels several times a day. If only small amounts of soft/liquid stools are passed around the sides of the obstructive faecal mass, this is termed overflow, or overflow diarrhoea. Anal canal trauma from passage of hard or large stool can present with bright red rectal bleeding or severe anal pain. Pain may exacerbate the problem, as it will inhibit defecation.
Whilst the majority (more than 90%) of children with chronic constipation will be considered to have functional, idiopathic constipation, exclusion of organic causes is important (summarised in Table 2). Constipation is also an important side effect of several classes of medicines and is commonly encountered in children receiving opiates, antacids or iron. Coeliac disease is commonly thought of as causing diarrhoea, but constipation is seen, possibly due to anorexia or changes in ileal function or gut motility. Constipation is very common in children and adults with cystic fibrosis, where there is an association with low total fat absorption and a history of meconium ileus. Neuromuscular conditions can affect the gut. Smooth muscle cells and intestinal cells of Cajal play a major role in normal gut motility. These cells ensure regular contractions of the colonic wall and propulsion of content. Constipation is often seen in patients with cerebral palsy and Duchenne muscular dystrophy. In patients with spinal muscular atrophy (SMA) proximal muscle weakness is a cardinal feature. In most of these patients constipation is a problem because of reduced abdominal muscle tone as well as disturbed innovation of the myenteric plexus. Children with significant developmental delay are more prone to constipation for a wide variety of reasons depending on their underlying disorder. For example, children with cerebral palsy often have dysmotility problems. Disorders that affect the enteric nervous system such as hereditary sensory and autonomic neuropathy (previously known as RileyeDay syndrome) are associated with constipation. Normal gastrointestinal motility is disturbed by abnormal autonomic function. Constipation can be a feature of disorders that affect water/ electrolyte balance such as diabetes insipidus. This can lead to reduced water content i.e. harder stools or as a result of muscle weakness caused by electrolyte imbalance. Other endocrine diseases such as MEN3 and hypothyroidism can present with constipation.
Key features in history taking C C C C
C C
C C
C C
Delay in passage of meconium Age at onset Relation to toilet training Toileting history-stool frequency, consistency, pain, soiling, presence of blood Stool withholding behaviour Urinary symptoms (13% of those with constipation have urinary symptoms) Abdominal pain Diet-history of exacerbation e.g. with cow’s milk or a poor diet low in fruit and fibre General health and developmental milestones Family and social history
Box 1
Organic causes of childhood constipation Structural colorectal
Spinal cord
Systemic
Neuropathic lesions of intestine
Drugs
Other important causes
Anal stenosis Post NEC/IBD stricture
Spina bifida Sacral agenesis
Diabetes Hypothyroidism
Hirschsprung disease Intestinal neuronal dysplasia
Opioid analgesia Antacids
Coeliac disease Cystic fibrosis
Chronic intestinal pseudo- obstruction
Spinal cord tumours
Hypo/hypercalcaemia
Iron
Cows milk protein allergy
Neurofibromatosis Cerebral palsy
Cholestyramine
Table 2
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Age dependent presentation
Age of toilet training
Newborn e 4 months 99% of term infants pass meconium within the first 24 hours following delivery. Very low birth weight or premature infants can have non-pathological delay in opening their bowels. 94% of children with Hirschsprung’s Disease and 25% of those with cystic fibrosis have initial delay in the passage of meconium. Newborn babies generally have a higher stool frequency of around 4/day but there is particularly high variability amongst breast-fed babies who can sometimes not open their bowels for days without being constipated. It is unusual for babies to become constipated in the first 4 months unless they have a congenital/anatomical abnormality, systemic upset or possibly cow’s milk protein allergy. A change from breast to formula feeding can result in constipation or the use of ‘hungrier baby’ milk with higher sodium content. It is unusual for young babies to develop chronic constipation Beware of red flags in the history and examination Late passage of meconium Systemically unwell Anatomical anomalies Developmental delay
This is a common time for children to become constipated. The age at which children start toilet training varies between and within families. There is evidence that early toilet training is associated with a higher incidence of constipation. The optimum time is when a child is able to communicate their desire to ‘do a poo’, has enough motor control to be able to overrule an urge if no toilet is available and can sit and actively strain on a toilet when the time is right. Many children and families find this stage of development a stressful, frustrating and unpleasant time. The transition can be very frightening to young children. The inconvenience of cleaning up accidents and the patience necessary to encourage young children to successfully toilet train should not be underestimated. Many families need help with this stage of normal development and may benefit from support groups or parenting classes. Long-standing constipation often starts with difficulties with early toilet training and adverse toileting behaviour. C
It is always important to ask about the ease of potty training as this is a common time for constipation to begin
Box 2
5 months to potty-training During this period infants start to wean from milk onto a variety of foods. The commonest cause of constipation in this age group is change in diet and consistency of stools. As baby rice and high fibre foods are added to an infant’s diet, stools become more bulky. It is common for parents to describe long periods of straining and sometimes pain associated with large diameter stools. Often early constipation can be treated effectively with change in weaning foods, such as an increase in fruits such as pear and less baby rice until the infant’s digestive system has adapted. In babies with reflux, no response to laxatives or a family history of atopy, a trial of a dairy-free diet (cow’s milk and soya) may be helpful. A clear association with cow’s milk protein allergy and constipation has been demonstrated. Many children start to take gluten in their diet at 9e12 months and if gastrointestinal symptoms including constipation start at this time then celiac disease should be considered. Toddlers who have had the sensation of pain following the passage of a hard stool are at high risk of stool withholding and rapidly worsening constipation. It is important to ask questions about posturing during a bowel movement as withholding behaviours can easily be misinterpreted as straining. In toddlers nearing three or at the time of potty training it is very important that a period of time has passed with soft stools to allow them to gain confidence and lose their fear of opening their bowels prior to any attempts to encourage them to use a potty or toilet. Often subtle manipulations in diet and fluid intake can be helpful. Some children are prone to constipation and some may drink insufficient fluid particularly in hot weather. A consultation with a paediatric dietician is invaluable, but may not be readily available (Box 2). Constipation is often related to dietary changes Do not forget the association with cow’s milk protein allergy and coeliac disease Stool withholding may result from painful defaecation and can be easily mistaken for straining
School age children Children generally learn to use their own toilet at home but need their parents’ ongoing support, praise and bottom wiping for some years afterwards. When children start full time school they need to be independent. Schools have variable amounts of support available for toileting needs of young reception age children. Toilets may be inaccessible from the playground or lunch hall
Key features in examination C
General health, plot on growth chart Look for evidence of systemic illness, faltering growth, anaemia
C
Abdominal palpation Evidence of faecal loading þ/ a faecal mass this is difficult in the obese child
C
Perianal region and digital rectal examination Examine perianal region for signs of fissures, soiling, infection (such as group A streptococcus) or anatomical anomalies A rectal examination should only be performed if it is likely to provide additional information: a sudden gush of stool may be found in a child with Hirschsprung’s disease post examination. Hard, impacted stool may be found confirming the need for a faecal disimpaction
C
Neurological exam, including inspection of the lumbar-sacral spine and examination of the lower limbs Spinal abnormalities or spinal cord tumours can present with constipation
Box 3
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Practical tips to improve toileting behaviour such as ensuring enough time for breakfast can be helpful Involve children in advice about lifestyle changes
and young children are often scared to go alone, unable to do up buttons or wipe their own bottom. This is a time where stool withholding and inevitable constipation is common. One approach that can be helpful is to try and emphasise the importance of adequate time before school for breakfast and going to the toilet. Sometimes there are improvements that can be made to privacy at school or the decorative state of the toilets to make them more appealing. There are often significant changes in diet at this time with children eating school dinners and having to make independent choices about what they do and don’t eat. Dietetic assessment at this age is very helpful and it is important to involve the child in education about healthy eating and a diet that has adequate fruit, fibre and fluid. Children are usually only allowed to drink water at school and many prefer juice prior to this and so drink much less than usual while running around more. This dehydration can exacerbate constipation.
Older children and early teens Children can become acutely constipated at any stage following an infection, change in diet, routine (e.g. moving house or school) or emotional upset. When reviewing older children early history remains important as symptoms are often long standing. Eating disorders need to be considered. Constipation can present with abdominal pain, nausea, anorexia or soiling. Rarely soiling may occur because of an involuntary leakage of soft stool around faeces impacted in the rectum. This will cause significant psychological morbidity. Older children who have been constipated for some time and who are soiling regularly are often bullied and become withdrawn and disengaged with education. Families find the symptom of soiling understandably
Investigation of children with chronic constipation Investigation
Indication
Change to management
Plain abdominal radiograph
Not usually indicated
Bowel transit studies Radio e opaque shapes swallowed daily for 5 days and then an abdominal radiograph performed to assess the transit time of the shapes
Not routinely indicated e interpret with caution as shapes can adhere to the colonic mucosa Useful in children with chronic diarrhoea or soiling for whom there is uncertainty about constipation as the underlying cause Not indicated in young children. Demonstrates whether there is normal relaxation of the internal anal sphincter in response to rectal dilation May be indicated if there is possibility of cow’s milk protein enteropathy To exclude Hirschsprungs disease if delay in early passage of meconium or only able to pass paste like stools with long standing constipation or abdominal distension All children with long standing constipation not responding to initial management
May be useful to exclude sacral agenesis or other spinal abnormalities Occasionally used to provide families with visual evidence of constipation If delayed transit shown it is more acceptable to families to comply with the advice for a child to undergo disimpaction of faeces
Anorectal manometry
Endoscopy Full thickness rectal biopsy
Coeliac screen tissue transglutaminase or endomysial antibodies þ immunoglobulin A levels
Electrolytes and glucose
Thyroid function
Fbc and iron studies
Concerning features of history or examination or not responding to treatment. Exclude hypo/ hypercalcaemia Urine dip-exclude diabetes Infants with early constipation and developmental delay. Family history of thyroid disease. Clinical suspicion Iron deficiency anaemia common because of poor diet. Iron therapy can cause constipation
Can demonstrate anatomical reasons underlying constipation. May need referral to a colorectal surgeon May demonstrate eosinophilic enteropathy Surgical excision of aganglionic bowel with temporary colostomy followed by a later pull through anastamoses If serology positive will need endoscopic duodenal biopsies and histological confirmation of the diagnosis while on full gluten containing diet Gluten free diet should lead to a resolution of symptoms Treat electrolyte abnormality
Thyroxine replacement
Treat iron deficiency anaemia
Table 3
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SYMPOSIUM: GASTROENTEROLOGY
difficult to manage and often feel anger and resentment towards the child who they often blame for the problem. Treatment must address underlying issues and offer support to the child and the family (Box 3). Constipation can present with a variety of symptoms and often families need a lot of reassurance that there is no serious underlying pathology Symptoms have often been longstanding
Investigations At all ages, if the history and examination are suggestive of functional constipation and there are no abnormal findings on examination, further investigations are not indicated. If a child has long standing constipation that has been resistant to treatment or there are worrying features then the following investigations may be considered [see Table 3].
Management The overall aim of management of childhood constipation should be rapid diagnosis and restoration of regular, pain free bowel movements at the appropriate time in the appropriate place with the minimum amount of laxatives possible. Effective management (summarised in Figure 1) relies on a good relationship with the family and interventions to treat the underlying cause and not just the symptom.
Figure 1 Non-pharmacological treatment of constipation.
to listen to music or read books while sitting and straining on the toilet. Breathing techniques can be useful to overcome fear. Some children find blowing bubbles or blowing up balloons help with their efforts to push a stool out. It can be helpful for children to have a foot-stool to rest their feet on while opening their bowels or a cushioned seat that can be smaller than the full adult sized seat. A non-punitive approach is necessary to improve toileting behaviour Aim to improve the whole toileting experience
Diet and lifestyle Education about normal physiology and healthy eating habits is a cornerstone of management. There is wide variability in the adequacy of children’s diets and multiple factors including socioeconomic status, health beliefs of families and eating habits of individual children. There is a clear correlation between inadequate fibre and fluid intake and also evidence that it is very difficult to change eating habits of families. There is insufficient evidence to suggest a high fibre or high fluid intake is beneficial and is difficult to comply with. A dietetic assessment is extremely useful to accurately assess an individual child’s diet and make recommendations that are likely to be acceptable/manageable for their family. The recommended fibre intake in children over 2 is their age in years plus 5 g per day. Two weetabix provide 3.8 g fibre. The importance of breakfast as a meal and starting the family day earlier to make time for breakfast, activity and then a relaxed attempt at opening bowels using the gastrocolic reflex and achieving a clear out prior to nursery/school is useful advice. Although there is a lack of trial evidence to support the effectiveness of increased physical activity in constipation management, it is a common observation that obesity and lack of physical activity are associated with constipation. A diet sufficient in fruit, fibre and adequate fluid will be essential to an effective cure Increased activity levels are helpful
Psychological assessment Many children and families who have been struggling with chronic constipation benefit enormously from psychological input. There are many issues which commonly arise such as toilet phobia, depression, distress from bullying, or abuse and clinical psychologist have many effective strategies that help children. Behaviour modification through reward Reward schemes are often successful; depend on a consistent approach and the child being old enough to respond. Star charts can be used, which can be as simple as a blank sheet of paper with stars for eating breakfast, sitting on the toilet, taking medication, doing a poo on the toilet or dealing with relevant issues. It is important that the rewards are awarded frequently enough to be seen as valuable by the child. Star charts should be used at appropriate ages
Toileting behaviour Difficulties with the transition from nappies to potty/toilet are common and it is always useful to help family’s access advice about toilet training. The basic principal of ignoring failure and praising success is important to convey. Improving the environment in the toileting area can be effective. Some children like
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Pharmacological treatment Success of pharmacological treatment (summarised in Figures 2 and 3) relies on addressing the underlying causes of constipation and the non-pharmacological advice should be
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reviewed and reinforced each time a child comes to clinic. When medicines are required for treatment it is helpful to divide the treatment regimen into disimpaction and maintenance therapies [see below]. Constipation management is complicated and requires regular review and assessment particularly at the beginning of laxative treatment. In children with acute onset constipation or mild symptoms lifestyle manipulations may be enough. For the majority seeking medical help it will be necessary to start a laxative. If the underlying problem is infrequent passage of large, hard stools then it is logical that a faecal softener should be the first line treatment. For many children with overflow diarrhoea, soiling or long standing symptoms disimpaction of large volume, hard stool in the rectum is essential before any management strategies or lifestyle changes will have any effect. There is little trial data to guide stimulant laxative use but there is widespread
consensus amongst specialists that they are often required either to ensure adequate disimpaction or as a maintenance agent. A systematic review of the literature showed only 50% of all children started on laxative treatment followed for 6e12 months are shown to recover and be asymptomatic whilst no longer on laxatives. It is important that expectations are managed and the length of treatment with laxatives is not underestimated.
Disimpaction Disimpaction can be achieved at home but families will need support. Children are asked to drink large volumes of PEG 3350 which can be difficult to tolerate. A stimulant laxative is added if disimpaction has not been achieved by 2 weeks. In practice, in severe cases may require enemas. A recent RCT compared daily enemas with 1.5 g/day PEG 3350 and found no significant
Figure 2 Disimpaction: as recommended by NICE guideline.
Figure 3 Maintenance therapy for treatment of constipation: as recommended by NICE.
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difference in efficacy or in behaviour scores, measuring fear/ distress, caused by the treatment. The NICE guideline recommends trying oral stool softeners and stimulants or sole agent stimulant laxative such as senna or sodium picosulpahte (elixir) or picolax (sachet) prior to rectal medication.
Maintenance treatment It is good practice to follow up the initial visit with a phone call and to review children regularly to ensure adequate disimpaction. Constipation is often most effectively managed in the community by a specialist nurse who forms a relationship with the family and
Osmotic laxatives Age
Dose
Mechanism of action
Side effects
Lactulose
Infants under 2
1e3 ml/kg
Relating to fermentation by products with excess gas production leading to flatulence, bloating and abdominal cramps
PEG 3350 (with electrolytes) Movicol
Only licensed for over 2 years but recommended by NICE as 1st line for all
0.3e0.8 g/kg/day maintenance 1e1.5 g/kg/day disimpaction
Non-absorbable disaccharide of sugars D-galactose and D-fructose. Fermented by colonic bacteria producing hyperosmolar byproducts Large molecular size. Not absorbed. Produces a direct osmotic effect. No impact on electrolyte balance
Side effects
Diarrhoea, abdominal distension, nausea
Table 4
Stimulant laxatives Age
Dose
Mechanism of action
Sodium picosulphate
Elixir 5 mg/5 ml Child 1 month to 4 years Child 4e18 years
2.5e10 ml od 2.5e20 ml od
Bisacodyl
Orally Child 4e18 years Rectally Child 2e18 years
5e20 mg od 5e10 mg od
Stimulating the mucosa of both the colon, causing peristalsis, and the rectum, causing increased motility and a feeling of rectal fullness By bacterial cleavage the active form is formed in the colon Action after 6e12 hours Diphenylmethane laxative, which, when activated, stimulates intestinal fluid secretion and colonic contraction
Senna
Elixir 7.5 mg/5 ml Child 1 month to 4 years Child 4e18 years
2.5e10 ml od 2.5e20 ml od
Docusate sodium
6 monthse2 years 2e12 years 12e18 years
12.5 mg tds 12.5e25 mg tds 500 mg/day in divided doses
Anthraquinone laxative increase fluid and electrolyte accumulation in the distal ileum and colon after metabolism. This occurs only when the prodrug comes into contact with intestinal micro-organisms. For this reason, sennosides can be given orally but not by suppository An anionic surfactant that reduces surface tension of water, allowing water to penetrate the intestine, softening the stool. Docusate also has a stimulatory affect by increasing cAMP concentrations in colonic mucosal cells which increases contractility
Withdrawn in the United States after rodent studies suggested carcinogenic links. Subsequent studies have not demonstrated an increased risk of cancer in humans taking phenolphthalein laxatives Changes in the colon produced by chronic anthraquinone use include melanosis coli, a benign and reversible condition. No evidence exists that anthraquinone laxatives given in clinically appropriate doses cause enteric damage
Table 5
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SYMPOSIUM: GASTROENTEROLOGY
provides regular support over the phone and in clinic. The addition of a stimulant laxative such as senna once stools are soft is often necessary to overcome stool withholding. Stimulant laxatives work by increasing intestinal motility. Intermittently stimulant laxatives receive adverse press and as the side effects can be more prominent. As the safety and long term efficacy in children is not proven, the use of stimulants should be regularly reviewed. There are a wide number of osmotic and stimulant laxatives available. There mechanisms of action and important side effects are summarised in Tables 4 and 5. Trial evidence commonly suffers from small sample sizes and short duration of follow-up. There have been recent randomised trials and a meta-analysis of efficacy for PEG 3550. Two systematic reviews compared PEG 3350 with placebo in the treatment of chronic constipation in children. PEG 3350 significantly improved pain during defaecation and increased the number of stools per week but did not significantly reduce the number of incontinent episodes.
Burnett CA, Juszczak E, Sullivan PB. Nurse management of intractable functional constipation: a randomised controlled trial. Arch Dis Child 2004; 89: 717e22. Caffarelli, et al. Characterization of irritable bowel syndrome and constipation in children with allergic diseases. Eur J Pediatr 2007; 166: 1245e52. Constipation in children and young people. Diagnosis and management of idiopathic childhood constipation in primary and secondary care. May 2010. National Institute for Health and Care Excellence. National Institute of Clinical Excellence Guideline, www.nice.org.uk/guideline/CG99. Kiefte-de Jong, et al. Infant nutritional factors and functional constipation in childhood: the generation R study. Am J Gastroenterol March 2010; 105: 940e5. Peeters B, Benninga MA, Hennekam RC. Childhood constipation; an overview of genetic studies and associated syndromes. Best Pract Res Clin Gastroenterol 2011; 25: 73e88. Pijpers MA, Bongers ME, Benninga MA, Berger MY. Functional constipation in children: a systematic review on prognosis and predictive factors. J Pediatr Gastroenterol Nutr 2010; 50: 256e68. Choung RS, Shah ND, Chitkara D, et al. Direct medical costs of constipation from childhood to early adulthood: a population-based birth cohort study. J Pediatr Gastroenterol Nutr 2011; 52: 47e54. Rajindrajith Shaman, Devanarayana Niranga Manjuri. Constipation in children: novel insight into epidemiology, pathophysiology and management. J Neurogastroenterol Motil 2011; 17. Sullivan PB, Alder N, Shrestha B, Turton L, Lambert B. Effectiveness of using a behavioural intervention to improve dietary fibre intakes in children with constipation. J Hum Nutr Diet 2011; 25: 33e42. Sullivan PB, Juszscak E, Burnett C. Parent satisfaction in a nurse-led clinic compared with a paediatric gastroenterology clinic for the management of intractable, functional constipation. Arch Dis Child 2006; 91: 499e501. Tabbers MM, Boluyt N, Berger M, Benninga MA. Diagnosis and treatment of functional constipation. Eur J Pediatr 2011; 170: 955e63.
Discussion Constipation is a distressing symptom for children and their families and is time consuming and difficult medical problem to manage. Management of constipation can be dispiriting as often symptoms persist and families often become very frustrated. It is important to provide targeted and appropriate lifestyle advice. Laxatives alone will rarely be sufficient to cure any patient of chronic constipation. While most children are unable to entirely change their lifestyle the importance of the non-pharmacological treatment of constipation is crucial to its successful management. With the development of chronic constipation, possibly exacerbated by stool withholding, the rectum changes from being highly sensitive to distension by stool to a large, distended sac unresponsive to distension. Moreover, the longer the condition persists the more likely it is that there will be histological changes in the lining (mucosa) of the rectum. Atrophy of the rectal musculature with degeneration (focal muscle fibre vacuolation) of muscle or even muscle fibre disappearance has been found in all chronically constipated children where this has been looked for. These changes are long-lasting and manometric (pressure) studies show that abnormal anorectal function is still present many years after the cessation of treatment and recovery from chronic constipation. Constipation is a distressing symptom for children and their families and is time consuming and difficult medical problem to manage. Management of constipation can be dispiriting as often symptoms persist and families often become very frustrated. It is important to provide targeted and appropriate life style advice. Laxatives alone will rarely be sufficient to cure any patient of chronic constipation. While most children are unable to entirely change their lifestyle the importance of the non-pharmacological treatment of constipation is crucial to its successful management. A
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FURTHER READING Borrelli O, Barbara G, DiNardo G, et al. Neuroimmune interaction and anorectal motility in children with food allergy-related chronic constipation. Am J Gastroenterol 2009; 104: 454e63.
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The diagnosis of functional constipation in childhood should be clinical and be based on fulfilling the ROME criteria It is important to exclude underling pathology that either directly or indirectly causes constipation as a symptom Investigations should be tailored to children who have concerning signs or symptoms and be based on the clinical history It is important to be aware of the differences in presentation at different ages, and underlying causes of constipation Treatment should be targeted on the underlying cause and be based on lifestyle modification and effective use of appropriate laxatives Management of constipation benefits from a multidisciplinary team approach and families need ongoing support to improve the success of treatment
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Please cite this article in press as: Howarth LJ, Sullivan PB, Management of chronic constipation in children, Paediatrics and Child Health (2016), http://dx.doi.org/10.1016/j.paed.2016.06.007