2.1. Idiopathic constipation in children
Constipation is common in childhood. It is prevalent in around 5–30% of children, depending on the criteria used for diagnosis. Symptoms become chronic in more than one third of patients and constipation is a common reason for referral to secondary care.2,3,4,5 Morbidity may be under-reported as people may not seek advice because they are embarrassed.
The exact cause of constipation is not fully understood but factors that may contribute include: pain, fever, dehydration, dietary and fluid intake, psychological issues, toilet training, medicines and familial history of constipation. Constipation is referred to as ‘idiopathic’ if it cannot be explained by anatomical or physiological abnormalities.
Many people don't recognise the signs and symptoms of constipation and few relate the presence of soiling to constipation. The signs and symptoms of childhood idiopathic constipation include: infrequent bowel activity, foul smelling wind and stools, excessive flatulence, irregular stool texture, passing occasional enormous stools or frequent small pellets, withholding or straining to stop passage of stools, soiling or overflow, abdominal pain, distension or discomfort, poor appetite, lack of energy, unhappy, angry or irritable mood and general malaise.6
Painful defecation is an important factor in constipation but it is not always recognised; ‘withholding’ behaviours to prevent passage of painful stools are often confused with straining to pass stools. Families may delay seeking help for fear of a negative response from healthcare professionals. It has been suggested that some healthcare professionals underestimate the impact of constipation on the child or young person and their family.7 This may contribute to the poor clinical outcomes often seen in children and young people with constipation.
Soiling is debilitating but rarely life threatening, so it might be expected to have little impact on healthcare provision. But many children and young people experience social, psychological and educational consequences that require prolonged support.
Some children and young people with physical disabilities, such as cerebral palsy, are more prone to idiopathic constipation as a result of impaired mobility. Children and young people with Down's syndrome and autism are also more prone to the condition. It is important that assessment and ongoing management for these children and young people happen in the same way as is recommended for all children and young people.
Without early diagnosis and treatment, an acute episode of constipation can lead to anal fissure and become chronic. By the time the child or young person is seen they may be in a vicious cycle. Children and young people and their families are often given conflicting advice and practice is inconsistent, making treatment potentially less effective and frustrating for all concerned. Early identification of constipation and effective treatment can improve outcomes for children and young people.8,9,10 This guideline provides strategies based on the best available evidence to support early identification, positive diagnosis and timely, effective management. Implementation of this guideline will provide a consistent, coordinated approach and will improve outcomes for children and young people.
2.2. Aim and scope of the guideline
This guideline aims to provide guidance in the following areas:
diagnosis of idiopathic constipation, including:
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patient history
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clinical examination, including the role of digital rectal examination
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diagnostic criteria (for example, ROME III criteria)
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investigations to rule out alternative diagnoses such as Hirschsprung's disease or coeliac disease including:
- ◊
blood tests
- ◊
radiological investigations
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gastrointestinal endoscopy
- ◊
manometry
- ◊
rectal biopsy
management, including:
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dietary manipulation, such as the role of water and milk intake, fruits, vegetables (fibres and roughage), fruit juices and cereals
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exclusion of cows' milk protein
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physical activity
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pharmacological treatments, specifically bulk-forming laxatives, stimulant laxatives and osmotic laxatives
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psychological and behavioural management including:
- ◊
toilet training
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behavioural modification
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maintaining toilet diaries
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rewarding
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psychosocial counselling such as biofeedback therapy and intense psychotherapy
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complementary and alternative interventions, specifically abdominal massage, reflexology and hypnotherapy
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surgical management, including manual evacuation under general anaesthetic and antegrade colonic enema (ACE procedure)
indications for referral to specialist services
information and support needs for children and families.
The following areas are specifically excluded from the guideline:
diagnosis and treatment of another disease identified during the diagnosis of childhood idiopathic constipation
management and diagnosis of comorbidity
care received in specialist services after referral
additional management for children with an underlying, congenital, genetic, metabolic, endocrine or neurological disorder who also have constipation.
Further information about the areas that are covered by the guideline is available in the Scope of the guideline (reproduced in appendix A).
2.3. Abbreviations and glossary
Abbreviations
- A&E
accident and emergency department
- ACE
antegrade colonic enema
- AGA
antigliadin antibodies
- ALSPAC
Avon longitudinal study of parents and children
- AP
allergic proctitis
- ARM
anorectal manometry
- BET
balloon expulsion test
- BNFC
British National Formulary for Children
- BT
behavioural therapy
- CAM
complementary and alternative medicine
- CAMHS
child and adolescent mental health services
- CD
coeliac disease
- CFU
colony forming unit
- CI
confidence interval
- C-IBS
constipation-predominant irritable bowel syndrome
- CP
cerebral palsy
- CTT
colonic transit time
- DRE
digital rectal examination
- ED
emergency department
- EMA
endomysium antibodies
- EMG
electromyographic
- ESPGHAN
European Society of Paediatric Gastroenterology, Hepatology and Nutrition
- FAP
functional abdominal pain
- FNRFI
functional non-retentive faecal incontinence
- FC
functional constipation
- FFR
functional faecal retention
- FOS
fructo-oligosaccharide
- GC
geometric centre
- GDG
guideline development group
- GOS
galacto-oligosaccharide
- HD
Hirschsprung's disease
- IBS
irritable bowel syndrome
- IC
idiopathic constipation
- IcFOS
fructo-oligosaccharide (see FOS)
- ID
intestinal dysganglionoses
- IGA
immunoglobulin
- IND
intestinal neuronal dysplasia
- ITT
intention to treat
- LGG
lactobacillus rhamnosus GG
- LR
likelihood ratio
- LSQ
Leeds satisfaction questionnaire
- MACE
Malone antegrade colonic enema (see ACE)
- MOM
milk of magnesia
- MPOC
Measure of Processes of Care
- NCC-WCH
National Collaborating Centre for Women's and Children's Health
- NDTC
normal delayed transit constipation
- NHS
National Health Service
- NICE
National Institute for Health and Clinical Excellence
- NLC
nurse-led clinic
- PC
paediatric constipation
- PEDI
paediatric evaluation of disability inventory
- PEG
polyethylene glycol
- PGC
paediatric gastroenterology clinic
- PSTC
paediatric slow transit constipation
- QALY
quality adjusted life year
- RAIR
rectoanal inhibitory reflex
- RAP
recurrent abdominal pain
- RCT
randomised controlled trial
- ROC
receiver operator characteristic
- RSB
rectal suction biopsy
- RSTT
rectosigmoid transit time
- SSS
severity symptom score
- STC
slow transit constipation
- TGITT
total gastrointestinal transit time
- tTG
tissue transglutaminase
- US
ultrasound
- UTI
urinary tract infection
Glossary of terms
- Acute constipation
Self-limiting constipation
- Allergic proctitis
Proctitis is an inflammation of the rectum. Allergic proctitis is inflammation attributed to allergic causes. The causes of the allergies have been attributed mostly to dietary proteins
- Anal stenosis
A narrowing of the anus which results in a reduced lumen and particularly a loss of the capacity to dilate with passage of faeces. This, in turn, results in straining, passage of ribbon-like faeces and constipation.
- Anal wink
The reflex contraction of the external anal sphincter
- Antegrade colonic enema procedure
A surgical procedure in which a channel is created, most frequently into the caecum, in the large intestine. This allows a catheter to be inserted and the bowel to be washed out. Sometimes known as Malone antegrade colonic enema (MACE) procedure
- Anteriorly placed anus
A congenital malformation in which the anus is malpositioned
- Biofeedback
Treatment method involving teaching the individual how to relax the external anal sphincter during straining. Treatment modalities include manometric and electromyographic biofeedback
- Chronic constipation
Constipation lasting longer than 8 weeks
- Colony-forming unit
A measure of viable (living) bacterial or fungal cells numbers. Results are given as colony-forming units per millilitre (CFU/mL) for water and colony-forming units per gram (CFU/g) for soil or other porous material
- Constipation
A term to describe the subjective complaint of passage of abnormally delayed or infrequent passage of dry, hardened faeces often accompanied by straining and/or pain
- Diarrhoea
The frequent passage of loose or watery stools, usually accompanied by abdominal cramping and urgency
- Digital rectal examination
Examination of the lower rectum using a gloved, lubricated finger to check for abnormalities
- Disimpaction
The evacuation of impacted faeces
- Encopresis
Deliberate defecation in an inappropriate place. This is not to be confused with soiling
- Faecal impaction
Severe constipation with a large faecal mass in either the rectum or the abdomen, and/or overflow soiling
- Faecal incontinence
The involuntary leakage of faeces
- Functional constipation
See idiopathic constipation
- Hirschsprung's disease
A congenital abnormality in which the nerve cells in a section of the bowel are not present. As a result, faeces can become trapped in the bowel
- Idiopathic constipation
Constipation is termed idiopathic when it cannot (currently) be explained by any anatomical, physiological, radiological or histological abnormalities. The exact aetiology is not fully understood but it is generally accepted that a combination of factors may contribute to the condition
- Intractable constipation
Constipation which does not respond to sustained, optimum medical management
- Kerckring folds
Circular folds projecting into the lumen of the the small bowel composed of reduplications of the mucous membrane
- Macrogols
Osmotic laxatives. Macrogols with the mean molecular weight of 3350 and 4000 are used as laxatives
- Megacolon
An abnormally enlarged colon that can be congenital (as in Hirschsprung's disease) or acquired (as in chronic constipation)
- Megarectum
A large rectum as a result of chronic faecal loading
- Optimum management
Management as set out in this guideline
- Organic constipation
Constipation is termed organic when there is an identifiable physiological or anatomical cause
- Osmotic laxatives
Laxatives which increase the amount of water in the faeces thereby making them softer
- Patulous anus
Widely patent anal orifice
- Rectoanal inhibitory reflex
Relaxation of the internal anal sphincter in response to increased pressure of stool, gas or liquid entering the rectum. If voluntary muscle action occurs, the rectum empties through the anal canal. This reflex is absent in cases of congenital megacolon.
- Retentive posturing
Typical straight-legged, tiptoed, back-arching posture
- ROME (II and III) criteria
The Rome criteria is a system developed to classify functional gastrointestinal disorders (FGIDs); disorders of the digestive system in which symptoms cannot be explained by the presence of structural or tissue abnormality, based on clinical symptoms. Some examples of FGIDs include irritable bowel syndrome, functional dyspepsia, functional constipation and functional heartburn. The most recent revision of the criteria, the Rome III criteria, was published in 2006. Further details can be found on the Rome Foundation website [www.romecriteria.org].
- Side effects/adverse effects
An undesired effect resulting from treatment
- Smearing
The intentional spreading of faeces
- Soiling/overflow soiling
Involuntary passage of fluid or semi solid stool into clothing as a result of overflow from a faecally loaded bowel
- Specialist
Healthcare professional with either interest, experience and/or training in the diagnosis and treatment of constipation in children and young people, such as specialist continence nurse or community paediatrician with an interest
- Specialist services
Services for children and young people which include constipation management
- Stimulant laxatives
Laxatives which increase bowel motility
2.4. For whom is the guidance intended?
This guidance is of relevance to those who work in or use the National Health Service (NHS) in England and Wales, in particular:
GPs and primary care and child health teams
professional groups which are routinely involved in the care of children and families
professionals who may encounter children in the course of their professional duties, for example radiographers, mental health professionals and surgeons
those responsible for commissioning and planning healthcare services, including primary care trust commissioners, Health Commission Wales commissioners and public health and trust managers.
professionals working in social services and education/childcare settings, including school nurses.
2.5. Other relevant documents
This guideline is intended to complement other existing and proposed works of relevance, including the following guidance published by NICE.
‘Coeliac Disease’ (NICE clinical guideline 86
11)
‘Urinary tract infection in children’ (NICE clinical guideline 54
12)
‘Nocturnal enuresis’ (NICE clinical guideline 79, expected publication date October 2010)
‘Maternal and child nutrition’ (NICE public health guideline 11)
‘When to suspect child maltreatment’ (NICE clinical guideline 89)
‘Medicines adherence’ (NICE clinical guideline 76)
2.6. Who has developed the guidance?
The guidance was developed by the Guideline Development Group (GDG), a multi-professional and lay working group convened by the National Collaborating Centre for Women's and Children's Health (NCC-WCH). Membership included:
a specialist paediatric nurse (Chair)
two general practitioners
two paediatricians
a dietician
a psychologist
a paediatric surgeon
a gastrointestinal nurse
a community nurse
a health visitor
two patient/carer members.
Staff from the NCC-WCH provided methodological support for the guidance development process, undertook systematic searches, retrieved and appraised the evidence and wrote successive drafts of the guidance.
An external advisor was appointed by the GDG to advise on pharmacological interventions.
All GDG members' and external advisers' potential and actual conflicts of interest were recorded on declaration forms provided by NICE (summarised in appendix B). The group was asked to declare interests at every GDG meeting. At the end of development, it became apparent that one of the GDG members had a personal pecuniary interest which would have precluded them from discussions about clinical management had it been declared earlier. In light of this, the recommendations were considered in consultation with the Chair. It was agreed that the majority of the recommendations should stand, as they appropriately represented the evidence and the GDG's consensus in interpreting that evidence. Some amendments were made to the recommendations on clinical management to ensure that they reflected the strength of the underlying evidence.
2.7. Guideline development methodology
This guidance was commissioned by NICE and developed in accordance with the guideline development process outlined in the NICE Guidelines Manual (2009).13
summarises the key stages of the process and indicates which version of the Guidelines Manual was followed at each stage.
Stages in the NICE guideline development process and versions of ‘The Guidelines Manual’ followed at each stage.
In accordance with NICE's Equality Scheme, ethnic and cultural considerations and factors relating to disabilities have been considered by the GDG throughout the development process and specifically addressed in individual recommendations where relevant. Further information is available from the NICE website [www.nice.org.uk/aboutnice/howwework/NICEEqualityScheme.jsp].
Forming clinical questions and search strategies
The GDG formulated clinical questions based on the scope (see appendix D). These formed the starting point for subsequent evidence reviews. Relevant published evidence to answer the clinical questions was identified by applying systematic search strategies (see Appendix J) to the following databases: Medline (1950 onwards), Embase (1980 onwards), Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1982 onwards), and three Cochrane databases (Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews and the Database of Abstracts of Reviews of Effects). Searches to identify economic studies were undertaken using the above databases and the NHS Economic Evaluation Database (NHS EED). None of the searches was limited by date or language of publication (although publications in languages other than English were not reviewed). Generic and specially developed search filters were used to identify particular study designs, such as randomised controlled trials (RCTs). There was no systematic attempt to search grey literature (conferences, abstracts, theses and unpublished trials), and hand searching of journals not indexed on the databases was not undertaken.
Towards the end of the guideline development process, the searches were updated and re-executed, to include evidence published and indexed in the databases by 20 July 2009. See appendix D for full details of the systematic searches, including the sources searched and the search strategies for each review question.
Reviewing and grading the evidence
Evidence relating to clinical effectiveness was reviewed and graded using the hierarchical system presented in . This system reflects the susceptibility to bias inherent in particular study designs.
Levels of evidence for intervention studies.
The type of clinical question dictates the highest level of evidence that may be sought. In assessing the quality of the evidence, each study receives a quality rating coded as ‘++’, ‘+’ or ‘–’. For issues of therapy or treatment, the highest possible evidence level (EL) is a well-conducted systematic review or meta-analysis of RCTs (EL = 1++) or an individual RCT (EL = 1+). Studies of poor quality are rated as ‘–’. Usually, studies rated as ‘–’ should not be used as a basis for making a recommendation, but they can be used to inform recommendations.
For each clinical question, the highest available level of evidence was sought. Where appropriate, studies of a weaker design were not considered; for example, if a systematic review, meta-analysis or RCT was identified to answer a question. Where systematic reviews, meta-analyses and RCTs were not identified, other appropriate experimental or observational studies were sought. For diagnostic tests, test evaluation studies examining the performance of the test were used if the effectiveness (accuracy) of the test was required. However, where an evaluation of the effectiveness of the test in the clinical management of patients and the outcome of disease was required, evidence from RCTs or cohort studies was optimal. For studies evaluating the accuracy of a diagnostic test, sensitivity, specificity, positive predictive values (PPVs) and negative predictive values (NPVs) were calculated or quoted where possible (see ).
‘2 × 2’ table for calculation of diagnostic accuracy parameters.
This hierarchical system covers studies of treatment effectiveness. However, it is less appropriate for studies reporting accuracy of diagnostic tests. In the absence of a validated ranking system for this type of test, NICE has developed a hierarchy of evidence that takes into account various factors likely to affect the validity of such studies (see ).
Levels of evidence for studies of the accuracy of diagnostic tests.
Summary results and data are presented in the text. More detailed results and data are presented in the evidence tables provided in appendix J. Where possible, dichotomous outcomes are presented as relative risks (RRs) with 95% confidence intervals (CIs) and continuous outcomes are presented as mean differences with 95% CIs or standard deviations (SDs).
The body of evidence identified for each clinical question was synthesised qualitatively in clinical evidence statements. Studies were pooled and quantitative synthesis (meta-analysis) was undertaken for two clinical questions in this guideline as it was felt that there were a sufficient number of similar studies to merit such analysis: these questions were the effectiveness of polyethylene glycol 3350 compared to lactulose in the maintenance section and effectiveness of biofeedback in the section on psychological and behavioural interventions.
Health economics
The aims of the health economic input to the guideline were to inform the GDG of potential economic issues relating to the management of idiopathic constipation, and to ensure that recommendations represented a cost-effective use of healthcare resources. Health economic evaluations aim to integrate data on benefits (ideally in terms of quality adjusted life years [QALYs]), harms and cost of alternative options.
The GDG prioritised a number of clinical questions where it was thought that economic considerations would be particularly important in formulating recommendations. Systematic searches for published economic evidence were undertaken for these questions. For economic evaluations, no standard system of grading the quality of evidence exists and included papers were assessed using a quality assessment checklist based on good practice in economic evaluation.14 Reviews of the (very limited) relevant published economic literature are presented alongside the clinical effectiveness reviews or as part of appendices detailing original economic analyses (see below).
For this guideline an economic evaluation was conducted to support the area of cost effectiveness of methods of disimpaction and maintenance of idiopathic constipation in children.
Evidence to recommendations
For each clinical question, recommendations for clinical care were derived using, and linked explicitly to, the evidence that supported them. In the first instance, informal consensus methods were used by the GDG to agree clinical and, where appropriate, cost-effectiveness evidence statements. Statements summarising the GDG's interpretation of the evidence and any extrapolation from the evidence used to form recommendations were also prepared to ensure transparency in the decision-making process.
In areas where no substantial clinical research evidence was identified (key components of history-taking and physical examination), the GDG made consensus statements and used its collective experience to identify good practice. The health economics justification in areas of the guideline where the use of NHS resources (interventions) was considered was based on GDG consensus in relation to the likely cost-effectiveness implications of the recommendations. The GDG also identified areas where evidence to answer the group's clinical questions was lacking and used this information to formulate recommendations for future research.
Towards the end of the guideline development process, informal consensus methods (show of hands) were used to identify ten ‘key priorities for implementation’ (key recommendations) and five high priority research recommendations. The key priorities for implementation were those recommendations likely to have the biggest impact on patient care and patient outcomes in the NHS as a whole.
Stakeholder involvement in the guideline development process
Registered stakeholder organisations were invited to comment on the draft scope of the guideline and the draft guideline. Stakeholder organisations were also invited to undertake a pre-publication check of the final guideline to identify factual inaccuracies. The GDG carefully considered and responded to all comments received from stakeholder organisations. The comments and responses, which were reviewed independently for NICE by a Guidelines Review Panel, are published on the NICE website.
A full list of the stakeholders for this guideline can be found in appendix C.
In addition, children and young people were consulted on the content (scope) of the guideline using a questionnaire survey and on the guideline recommendations via a stakeholder meeting. See appendix F for further details of this work.
2.8. Schedule for updating the guidance
Clinical guidelines commissioned by NICE are published with a review date 3 years from date of publication. Reviewing may begin earlier than 3 years if significant evidence that affects guideline recommendations is identified sooner.
