Paediatric constipation

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EARN 3 FREE CPD POINTS constipation Leader in digital CPD for Southern African healthcare professionals Best practice Paediatric constipation Diagnosis and treatment Dr Tim De Maayer Paediatric

1 EARN 3 FREE CPD POINTS constipation Leader in digital CPD for Southern African healthcare professionals Best practice Paediatric constipation Diagnosis and treatment Dr Tim De Maayer Paediatric Gastroenterologist Rahima Moosa Mother and Child Hospital Johannesburg Introduction The European Society for Paediatric Gastroenterology, Hepatology and Nutrition/ North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN/NASPGHAN) and National Institutes of Clinical Excellence (NICE) guidelines are currently the most widely used for the diagnosis and treatment of paediatric constipation. These international guidelines form the basis of an in-depth presentation by Dr Tim De Maayer on the subject, hosted in Johannesburg. KEY MESSAGES Paediatric constipation has a high prevalence It is usually functional in nature, but it is important to exclude non-functional causes Be alert for any red flags that point to the latter Education and empowerment are key to successful treatment Early treatment confers a better prognosis. This report was made possible by an unrestricted educational grant from Cipla. The content of the report is independent of the sponsor. october 2018 I 1

With a prevalence as high as 30% (the range is 5-30%, given varying definitions), paediatric constipation is very common. It is under-reported, poorly understood and poorly treated.

2 With a prevalence as high as 30% (the range is 5-30%, given varying definitions), paediatric constipation is very common. It is under-reported, poorly understood and poorly treated. Most paediatric constipation is idiopathic/functional. The longer treatment is delayed, the less likelihood of a successful outcome, said Dr De Maayer. It is a multifactorial condition and diet, fluid intake, genetics, being too busy playing, medications, inadequate ablution facilities and toilet training can all play a role in functional constipation. The key aetiological factor, however, is stoolwithholding behaviour, which manifests as retentive posturing, e.g. bending, crossing of legs, holding on to furniture. A child with constipation associates defecation with pain, and avoids stooling by keeping the stool in the rectum, where it gets progressively larger and harder (Figure 1). When they finally do pass it, the experience will be even more painful. The result is a vicious circle whereby the association with pain is reinforced on an ongoing basis, leading to chronic constipation. Retaining stool leads to faecal impaction, and may result in overflow diarrhoea/incontinence as liquid stool can form higher up in the rectum. One consequence of this may be misdiagnosis and hence delayed treatment. Holding stools The longer treatment is delayed, the less likelihood of a successful outcome. Painful bowel movement Larger and harder stools Figure 1. Stool-retentive behaviour Earn free CPD Points Join our CPD community at and start to earn today! Diagnosis The ROME IV criteria 1 for functional paediatric constipation are two or more of the following: two or fewer movements per week; faecal incontinence lasting a week or longer; retentive posturing/voluntary stool retention; painful or hard movements; large faecal mass in the rectum and large diameter stools (Table 1). Symptoms should be present at least once a month for a minimum of one month and there should be insufficient criteria to diagnose irritable bowel syndrome. Table 1. ROME IV criteria Two or more of the following: Two or fewer defecations per week Faecal incontinence 1 per week Retentive posturing or volitional stool retention Painful or hard bowel movements Large faecal mass in the rectum Large diameter stools that may obstruct the toilet Symptoms occurring at least once per month for a minimum of one month Insufficient criteria to diagnose irritable bowel syndrome 2 I october 2018

If any of these red flags are found, the patient should be referred to the appropriate subspecialist.

They include coeliac disease, electrolyte abnormalities, anatomical malformations, hypothyroidism, cystic fibrosis, neuropathic disorders and spinal cord abnormalities.

There is no need to investigate in the absence of red flags, however, as paediatric constipation is a mainly clinical diagnosis (Figure 2).

red flags in paediatric constipation Blood in stool Systemic symptoms (such as faltering growth and weight loss) Urinary symptoms Abnormal lower limb neurology Extra-intestinal symptoms

3 If any of these red flags are found, the patient should be referred to the appropriate subspecialist. While 90% of paediatric constipation is functional, non-functional causes, though rare, need to be excluded. They include coeliac disease, electrolyte abnormalities, anatomical malformations, hypothyroidism, cystic fibrosis, neuropathic disorders and spinal cord abnormalities. 2 Dr De Maayer recommends looking for red flags (Table 2). If any of these are found, the patient should be referred to the appropriate subspecialist. There is no need to investigate in the absence of red flags, however, as paediatric constipation is a mainly clinical diagnosis (Figure 2). Many children are nonetheless subjected to unnecessary investigations. Table 2. red flags in paediatric constipation Blood in stool Systemic symptoms (such as faltering growth and weight loss) Urinary symptoms Abnormal lower limb neurology Extra-intestinal symptoms that suggest inflammatory bowel disease Perianal disease. Figure 2. No need for further investigations in the absence of red flags Cow s milk allergy may be a cause if there is no response to treatment, but Dr De Maayer noted that it is not a common one. Clues to consider include: onset of constipation after dietary changes, the presence of eczema/rhinitis/asthma and straining despite soft stools. If you consider it might indeed be the cause, try a two-week to one-month dairy-free diet with calcium supplementation, reintroducing dairy after 6-12 months. October 2018 I 3

4 Treatment options Education and empowerment are the cornerstones of treatment and key to its success, as the patient and their parents need to understand the feedback loop that underpins the vicious circle referred to previously (Figure 3). Among other things, they need to understand the pathophysiology and be made aware that this is a chronic condition requiring long-term treatment. Toilet training needs to be child-led and positioning needs to be taken into account, as the child needs to feel comfortable when sitting on the toilet. The ideal time to go to the toilet is immediately after breakfast or dinner, and a reward system should be considered to positively reinforce attempted defecation. Keeping a diary may also be helpful. Education and empowerment are the cornerstones of treatment and key to its success. Stool Rectum Sphincter muscle Anus More stool forms and backs into colon Soft stool Large stool gets stuck (impacted) Enlarged dialated rectum Anus Normal Chronic Constipation Figure 3. Constipation is a vicious circle Earn free CPD Points Join our CPD community at and start to earn today! Many parents have reservations about their child taking chronic medications so reassurance is important. Oral medications include the osmotic laxatives, lactulose (1-3ml/kg in divided doses) and polyethylene glycol (PEG-3350) for disimpaction (1-1.5g/kg/day) and maintenance ( g/kg/day). Dr De Maayer advises against the use of stimulant laxatives, e.g. senna, sodium picosulphate, bisacodyl, for longer than a few days as they can induce dependence, but osmotic laxatives carry no risk of dependence, something to underscore to concerned parents. Lactulose and PEG-3350 have a similar efficacy, but the latter has fewer side effects and is the preferred choice worldwide. It is therefore the treatment of choice in the private sector. (Only lactulose is available to public sector patients in South Africa.) PEG-3350 is associated with better compliance and acceptability than all other osmotic laxatives. However, the effective dose is unpredictable and varies from patient to patient, so titration may be necessary. Side effects, which include diarrhoea, bloating/flatulence and abdominal pain, are few and dose-dependent, and can be reduced by lowering the dose. 3-5 Chronic use of PEG-3350 is not associated with any electrolyte disturbances. However, concerns have been raised about the neurotoxicity of ethylene glycol, one of PEG-3350 s metabolites. Dr De Maayer pointed out that while PEG administration is associated with a transient increase in ethylene glycol levels, these remain well below toxic levels and are only 1/15 th of those permissible in drinking water (Table 3). 6 4 I october 2018

5 Lactulose and PEG-3350 have a similar efficacy, but the latter has fewer side effects and is the preferred choice worldwide. Table 3. PEG-3350 With/without electrolytes Better acceptability and higher compliance rates Unpredictable effective dose titrate! Side effects: Diarrhoea 10% Bloating/flatulence 6% Abdominal pain 2% No significant electrolyte disturbances in chronic use Neurotoxicity of ethylene glycol Metabolite of PEG Transient increase in ethylene glycol after PEG administration Remains well below toxic levels Total dose of ethylene glycol ingested <1/15 th of allowed drinking water levels Older alternatives to lactulose and PEG are mineral oil ( liquid paraffin ) and magnesium hydroxide ( milk of magnesia ) (Table 4). The former was once very popular but has a number of drawbacks, notably potential vitamin deficiencies with long-term use, anal leakage, lack of palatability and the risk of aspiration pneumonia. The latter is also a possibility with PEG Magnesium hydroxide is similarly unpalatable and its effectiveness has been questioned. When it comes to non-oral therapy, phosphate enemas (2.5ml/kg held in the rectum for as long as possible) work well, but patient resistance is common and they should be reserved only for patients who do not get results with oral treatment. Rarely, children may even require general anaesthesia for disimpaction if especially resistant. Phosphate enemas should not be used in children under one year of age, however. Some newer therapies that act on A role for surgery? There is a role for surgery in severe constipation, particularly if underlying disease is present, but very uncommonly. The antegrade continence enema, also known as the Malone procedure, is fairly simple and entails the following: The appendix is brought to the surface of the skin and a stoma is created around the bikini line A small plastic trap door, which opens Table 4. Other therapies Mineral oil Potential vitamin deficiencies with long-term use Anal leakage Not palatable Risk of aspiration pneumonia Magnesium hydroxide Lower palatability and?efficiency Lactulose Well tolerated Similar efficacy to PEG but more side effects chloride channels have shown promising results in adult patients. These include lubiprostone, linaclotide and prucalopride, but they are not yet available locally and probably won t be for the foreseeable future. However, there are no paediatric trials to date. There is also no adequate evidence yet to support a role for transcutaneous nerve stimulation. Other interventions are not supported and tend not to work. Where diet is concerned, specifically with regard to increased fibre intake, the evidence is weak and conflicting. Fibre is thought to be important in the weaning off medication phase. Evidence is similarly weak with regard to the role of exercise and probiotic supplementation. When it comes to duration of treatment, there is no adequate evidence base. Expert opinion holds that treatment should continue for a minimum of two months and only be withdrawn gradually after one month with no constipation. If a child is in the developmental stage of potty training, treatment should only be stopped once potty training has been achieved. and closes, is inserted into the stoma to allow access to the bowel via the appendix A catheter into the bowel is placed in the stoma A solution is injected into the bowel via the catheter The fluid irrigates the bowel and, after about 20 minutes, flushes out faeces through the anus. October 2018 I 5

6 Earn free CPD Points Are you a member of Southern Africa s leading digital Continuing Professional Development website earning FREE CPD points with access to best practice content? Only a few clicks and you can register to start earning today Visit For all Southern African healthcare professionals Prognosis The prognosis of children referred to paediatric gastroenterologists is that after six months 50% will be asymptomatic and off treatment, 10% asymptomatic but still on treatment and 40% both symptomatic and on-treatment (Table 5). Early treatment confers a better prognosis and we need greater doctor/nurse awareness to enable earlier identification and thus treatment, concluded Dr De Maayer. Eighty percent versus 32% of patients become asymptomatic if adequate treatment is started within three months of symptom onset. Recovery rates are good over time 50% after five years and 80% after 10 years. Relapses are frequent, however, and when they occur it is important References 1. ROME-IV. Functional Gastrointestinal Disorders. theromefoundation.org/the-new-rome-iv-diagnosticcriteria-for-functional-gi-disorders-debuting-atddw-2016/ 2. Tabbers MM, DiLorenzo C, Berger MY, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr 2014; 58: Pashankar DS, Loening-Baucke V, Bishop WP. Safety of polyethylene glycol 3350 for the treatment of chronic constipation in children. Arch Pediatr Adolesc Med to reintroduce treatment immediately to address them. Table 5. Prognosis Of children referred to paediatric gastroenterologists, after 6/12: 50% will be asymptomatic and off treatment 10% will be asymptomatic and on treatment 40% will remain symptomatic on treatment Early treatment confers a better prognosis: 80% vs 32% were asymptomatic if adequate treatment was started within 3/12 of symptom onset 50% and 80% recovery rates after five and 10 years Relapses are frequent 2003; 157: Alper A, Pashankar DS. Polyethylene glycol: a gamechanger laxative for children. J Pediatr Gastroenterol Nutr 3013; 57: Loening-Baucke V, Krishna R, Pashankar DS. Polyethylene glycol 3350 without electrolytes for the treatment of functional constipation in infants and toddlers. J Pediatr Gastroenterol Nutr 2004; 39: Williams KC, Rogers LK, Hill I, Barnard J, DiLorenzo C. PEG 3350 administration is not associated with sustained elevation of glycol levels. J Pediatr 2018; 195: Find us at DeNovo Disclaimer The views and opinions expressed in the article are those of the presenters and do not necessarily reflect those of the publisher or its sponsor. In all clinical instances, medical practitioners are referred to the product insert documentation as approved by relevant control authorities. Published by denovo Medica Reg: 2012/216456/07 70 Arlington Street, Everglen, Cape Town, 7550 Tel: (021) I info@denovomedica.com 6 I october 2018

Prescribing Guidance for the Treatment of Constipation in Children

Prescribing Guidance for the Treatment of Constipation in Children Effective Date: July 2007 Reviewed: September 2009 & December 2011 Review Date: December 2013 Gateshead Medicines Management Approved