What Is Known
* Transanal irrigation is an increasingly accepted treatment in children with bowel dysfunction who do not respond to conservative treatments.
* In recent years, data have been published on the efficacy of transanal irrigation in pediatric patient populations such as anorectal malformations and functional constipation.
* Although the use of transanal irrigation in adults has been standardized, the practice in children still remains largely empirical.
What Is New
* The first best-practice article based on published evidence and professional experience, aimed at healthcare professionals who manage pediatric bowel dysfunction and who currently use transanal irrigation or would like to initiate in its use.
See “Transanal Irrigations: A Few Considerations” by Ambartsumyan on page 341.
In children, constipation and fecal incontinence (FI) may be the result of either organic or functional disorders (1–7). Organic causes are rare and typically congenital, and predominately have a neurological or anatomical origin. This is the case in patients with neurogenic bowel dysfunction (NBD), which in children is mainly related to open or closed spina bifida, and in patients with anorectal malformations or Hirschsprung disease.
In >95% of the children, after appropriate medical evaluation, the symptoms cannot be attributed to another medical condition and are therefore called functional (1,2). Indeed, functional constipation (FC), complicated by FI, affects up to 29.6% of children and can negatively impact their quality of life (QoL) (3,4). In >90% of affected patients, FI is a result of fecal retention, whereas the remaining cases fulfill the Rome IV criteria for functional nonretentive fecal incontinence (FNRFI) (1).
Today, transanal irrigation (TAI) is an accepted treatment in children and adults with bowel dysfunction (BD) who do not respond to conservative and medical treatments. TAI use in adults is well-defined (8) in a stepwise pyramid of care, that can be applied when conservative and medical treatment of BD (such as dietary and lifestyle advice, regular use of laxatives, suppositories, enemas, or manual evacuation) have failed.
TAI involves a large-volume water irrigation of the rectum and colon performed by introducing a catheter (often with a balloon) or a cone through the anus. TAI was introduced into current clinical practice by Shandling and Gilmour (9) in 1987 to treat constipation and improve fecal continence in children with NBD. On the basis of high success rates, reaching 100% in some studies, TAI was further applied to adults and children in whom other medical treatments had failed (10–13). Other empirical treatments and procedures have been proposed to treat nonresponsive BD including biofeedback and neuromodulation with inconsistent results in children (14–16). Therefore more invasive surgical interventions are sometimes offered, for example, the malone antegrade colonic enema (MACE) (17). Importantly, recent studies using TAI in children have reported high rates of success, both in clinical bowel outcomes and in improvement of QoL (13,18–21). Therefore, some authors recommend that TAI should be considered before any surgical treatment in children with BD (20,21).
Because there is still some uncertainty about the correct use of TAI in pediatric populations, the aim of this work is to provide a best-practice consensus review based on experience and a literature review to facilitate its use in clinical practice.
MATERIALS AND METHODS
A consensus group of specialists from France, Germany, Italy, the Netherlands, United Kingdom, and USA, and from various pediatric disciplines including gastroenterology, colorectal surgery, pediatric surgery, and neurourology, all with a long-term experience of NBD and TAI, produced this consensus review on the basis of existing published literature and their own clinical experience.
For the literature review, PubMed, CINAHL, and The Cochrane Library were searched from inception to June 2016. The inclusion criteria were articles published in the English language from January 1, 1980 to July 1, 2016 resulting from using the following search terms: (“transanal irrigation,” OR “anal irrigation,” OR “colonic irrigation,” OR “bowel enema”) AND (“neurogenic bowel,” OR “constipation,” OR “fecal incontinence,” OR “faecal incontinence”) AND (“children,” OR “pediatric,” OR “paediatric,” OR “pediatr*,” OR “child*”). The results of the search were then reviewed by at least 2 of the authors, as a minimum to title and abstract level (when available). Articles were rejected on the initial screen if they failed to meet our inclusion/exclusion criteria, whereas potentially relevant studies, and studies in which the title and abstract provided insufficient information were retrieved as full-text articles. Exclusion criteria were acute use of TAI (eg, for disimpaction or diagnostic use), studies in which adult patients (>18 years of age) were included and in which results from children were not reported separately, children receiving enemas (understood as instillation per rectum of a low-volume medicated substance), children with an antegrade continence enema (ACE/MACE) only or not reported separately, and educational materials and opinion papers. To support, complement, or contrast the results of the literature search, the authors made use of their clinical and practical expertise, experience, and opinions. Individual group members prepared a write-up each on a single section, and consensus was reached by several round-table discussions and common writing and review of the overall article.
A total of 404 potentially relevant articles and abstracts were identified; 369 through the search in PubMed and The Cochrane Library and 35 articles in CINAHL (all of which were previously identified in the search in PubMed or deemed not relevant). After applying the exclusion criteria to the results, 27 articles were included and 377 articles were excluded (15 of the latter were excluded only after being reviewed as full-text papers) (Fig. 1). Reasons for exclusion were “abstract not available” (n = 1), “editorial comment” (n = 1), “duplicates” (n = 39), “acute use only, disimpaction or preparation for colonoscopy” (n = 6), “educational and/or review papers” (n = 21), “out of scope MACE/ACE” (n = 120), “out of scope other reason” (n = 185), and “other including animal studies” (n = 4).
Four of the 27 included studies were cohort studies (1 prospective and 3 retrospective) and had an Oxford Centre for Evidence-Based Medicine rating of 3. The remaining 23 studies were case series (14 prospective and 9 retrospective) and had an Oxford Centre for Evidence-Based Medicine rating of 4. Data from the 27 included studies regarding underlying condition of the patient population are depicted in Table 1.
In total, 1040 patients were included in the 27 studies. Most patients (686/1040) had NBD as underlying condition, mainly spina bifida. Three hundred seventy-two were boys and 388 were girls, although some studies did not report sex, leaving 280 patients (27%) as undefined. The average age of the patients was 8.5 years (range 1–25 years).
Based on the literature review, the average success rates of TAI in children are estimated to be 78% (77.7%, range 53%–97%) for FI, 78% (range 53%–97%) for constipation, and 84% (range 60%–100%) when reported as overall improvement of symptoms (Supplemental Digital Content, Table, http://links.lww.com/MPG/A862). Success rates in terms of satisfaction with TAI and QoL were scarce in the selected articles, but Cazemier et al 2007 (23) reported satisfaction rates of approximately 90%, Corbett et al, 2014 (20) reported QoL improvement in 95% of children, and Koppen et al 2016 (13), reported a parent satisfaction of 86%. Discontinuation or failure of treatment with TAI ranged from 5% to 36%. Not all studies reported the reason for failure or discontinuation of the treatment and in some cases there may be more than 1 reason. In the studies that reported such reasons, the most common were lack of efficacy (responsible for approximately 36% of discontinuations), dislike or embarrassment of treatment (approximately 17%), and later remission of symptoms (responsible for approximately 15% of discontinuations). Other less frequent reasons reported for failure or discontinuation included noncompliance, pain on insertion, and catheter expulsion.
The median follow-up time was 23 (range 1–144) months of treatment. Most selected articles concerned patients with spina bifida, and it was therefore not possible to infer associations between TAI performance and etiology. The literature did not allow comparison of results according to which TAI device was used.
Indications for Transanal Irrigation and Management of Neurogenic and Functional Bowel Dysfunction
Management of neurogenic/anatomical and functional BD consists of nonpharmacological and pharmacological treatment modalities. As described in both the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)/North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and NICE guidelines (42) education and demystification are the first steps before starting the treatment of children with functional BD. It is important to provide information on prevalence, symptoms, treatment options, and prognosis. A nonaccusatory approach is of major importance, because these entities may be accompanied by feelings of guilt, shame, and anger in both children and their parents (42). The most important step in the nonpharmacological management is instituting a toilet program. Although inadequate fiber intake is associated with FC, there is insufficient evidence to support the use of supplementary fiber in addition to the daily recommended intake in children with FC. The ESPGHAN/NASPGHAN guidelines recommend a normal fiber and fluid intake and normal physical activity in children with constipation. The ESPGHAN/NASPGHAN guidelines recommend a normal fiber and fluid intake and normal physical activity in children with constipation.
Pharmacological treatment of constipation-associated FI consists of disimpaction by either oral or rectal route followed by maintenance treatment and follow-up (42). For the minority of patients with FNRFI, constipating agents rather than laxatives may be more appropriate (43). It is well known that retrograde enemas are therapeutically effective in treating constipation in children with a neurological disorder, including spina bifida, but little knowledge exists about the role of retrograde enemas in the maintenance treatment of children with FC (44). A randomized clinical trial showed that application of enemas on a regular basis is well-tolerated in children with chronic constipation, but also that they had no additional benefit over conventional treatment with oral laxatives in the maintenance phase of treatment (45). Other conservative methods include biofeedback (14) and noninvasive forms of nerve stimulation (46). In patients for whom conservative and medical methods for managing either organic or FC and/or FI are not successful, or where there is a different patient preference, TAI is proposed (13,18). If TAI is ineffective, the next step to consider would be more invasive therapies such as sacral nerve modulation (16) or MACE (17). Furthermore, new oral drugs such as lubiprostone and linaclotide have been shown effective in adult patients (47,48). Few data are, however, currently available in the pediatric literature to suggest the best next step if TAI fails. In patients not responding to any of the previous treatments, or again when patient preference dictates, the last step in the proposed pyramid would be surgical procedures including bowel resection and/or stoma formation (17). A bowel management pyramid for children (Fig. 2) is proposed based on an adaptation of the one suggested for the adult population by Emmanuel et al, 2013 (8). Briefly, the pyramid is divided into 4 levels based on the invasive nature of the method. Conservative management and TAI constitute non- or minimally invasive methods, whereas MACE, sacral neuromodulation, bowel resection, and stoma formation are more invasive.
Even though it may be less invasive than the MACE procedure, sacral nerve modulation (SNM) lacks the amount of evidence and its conclusiveness that several years of positive experience with MACE has. Furthermore, SNM is not licensed in some regions for use in children.
Patient Selection for Transanal Irrigation
Progress and eventual outcome of TAI are highly dependent on an individual child and family, and their interaction together. A full assessment in advance is often needed of the child's and family's motivation and cognitive ability. Moreover, physical factors such as anorectal anatomy, paraplegia, obesity, manual dexterity (especially in those with spinal conditions, or with upper-limb abnormalities), and balance may dictate the child's capacity, amongst other things, to sit on the toilet and successfully perform TAI.
It is important to differentiate between a functional and organic defecation disorder, and more specifically differentiate between FC and FNRFI. A normal colonic transit time, using radio-opaque markers, in combination with the absence of either abdominal or rectal fecal impaction confirms the diagnosis FNRFI. In a minority of cases magnetic resonance imaging of the spine and anorectal manometry are helpful to find organic causes for defecation problems (5,42).
A list of possible indications for TAI in children is provided in Table 2. Although rare congenital anorectal conditions such as Hirschsprung disease and anal stenosis typically present at the neonatal age, occasionally milder forms may persist unrecognized in older children with constipation and/or FI. Therefore, a proper history should be taken in any child under consideration for TAI, to exclude a delay in the first passage of meconium and to ensure that the caliber of solid stool is normal. The anus should, at the very least, be externally inspected to ensure normal anatomy and physiology. Children who have previously been operated on for any congenital anorectal condition may have constipation and/or FI because of a secondary postoperative stricture or anal stenosis. This possibility mandates assessment by a specialist pediatric surgeon, ideally the original operator, so that TAI is not administered inappropriately to a child who instead needs revisionary or alternative surgery.
Constipation and/or FI in children can be a presenting feature of nonaccidental injury, whether psychological or physical, including sexual abuse. Therefore, it is mandatory to consider this possibility as an underlying cause before pursuing any treatment. If there are any suspicions, referral is necessary to an expert in child protection for further evaluation. Furthermore, nonaccidental injury should be reconsidered if any child is unduly upset by passage of the rectal catheter/cone. Similarly, fabricated illness may manifest with symptoms of BD, necessitating the involvement of an appropriate pediatric specialist and/or pediatric psychiatrist, which may obviate the need for intrusive physical treatments such as TAI.
Probably the most important prerequisite for pediatric TAI to be effective in the early stages is to ensure that the patient is not affected before instituting TAI. Instead, starting with a relatively empty colon will allow TAI to begin with low volumes and minimal use of stimulant laxatives, thereby reducing the risk of off-putting cramps. Fecal impaction may sometimes be verified by simple abdominal examination alone. If necessary, rectal fecal impaction can be confirmed by rectal examination (which is appropriate if the child has absent anorectal sensation), abdominopelvic ultrasound, or abdominal x-ray. The cumulative radiation burden, however, makes x-ray inadvisable for repeated assessments (49). Fecal impaction before starting TAI can be treated with high-dose oral laxatives, enemas, or, as a last resort, manual disimpaction under general anesthesia (50).
The contraindications for the use of TAI in children are similar to those established for the adult patient population (8), except for the minimum age of the patient, which is dictated by the regulatory approvals in each region. As a guide, this is usually 3 years of age in Europe and 2 years of age in the USA. Some contraindications that are especially relevant in the pediatric patient population are listed in Table 2. Healthcare professionals should always study in detail the latest approved and valid Instructions for Use of the device(s) available in their territory, and be aware of both the absolute and relative contraindications that may be listed there.
Special Pediatric Considerations Before Starting Transanal Irrigation
There are many parallels to the approach in adults advocated by Emmanuel et al (8). There are, however, several distinct differences, which must be fully understood before embarking on treatment of children, particularly if the healthcare professional involved is more experienced with an adult patient population. Essential preparation requires an appropriately experienced healthcare professional to provide the child and parents with detailed explanations and discussions, which may take several meetings. Regarding the assessor/trainer, their competencies are more important that their actual title (eg, nurse, urotherapist, continence advisor, stoma nurse or therapist, doctor, etc). They have to be properly trained and experienced not only in TAI, but also in the management of constipation/FI, recognizing the need for safeguarding vulnerable children and families. A supervising pediatric clinician is recommended for the benefit of both the child/family and the other professionals involved. Long-term success also mandates the availability of regular and on-going support from these same healthcare professionals, to encourage patience and perseverance in the early stages, because it may take several weeks and sometimes longer to achieve reliable success. When age, mental and emotional maturity, and physical condition allow it, we encourage the patient's self-management of the TAI procedure. Rates of independent use in children vary in the literature, but seem to relate to the underlying pathology and to age alone. In one study, 79% of children with anorectal malformations ages 4 to 18 (mean 11) years were performing TAI themselves, including one 7-year-old (32), compared with only 16% of younger children in another study with mostly neurogenic conditions (20). Advice can also later be offered on how to adapt the acquired routines to permit and encourage social events, family holidays, residential trips, sleepovers, and other normal childhood activities. Children may not fully understand the rationale for their treatment, particularly if they are especially young or have associated learning difficulties. Therefore, the approach needs to be tailored toward the cognitive, educational, and psychological status, maturity, and motivation of the individual child and family, on whom the ultimate success of TAI is so dependent. Explanation of the technique is required in a positive child-friendly way, for example, with picture books, films, Web sites, or toy models. Children are often understandably embarrassed by, or wary of, the rectal approach, and are particularly likely to be put off if they experience pain, a burst balloon, or premature expulsion of the catheter in the early stages of starting TAI (37). In certain cases, the input of a clinical psychologist familiar with the pediatric population may prove helpful in unraveling the basis of any fears that a child may have, and may optimize adherence with the proposed treatment. When the child is still too anxious, it may be best to deliver training in small steps or consider reintroduction of TAI at a later stage as the child matures.
Training Before Starting Transanal Irrigation
Because TAI carries a small risk of serious complications (see section “Complications of Transanal Irrigation” and Table 3), it is absolutely essential that patients, families, or caregivers are properly trained before starting treatment. This also implies a need for formalized training of those healthcare professionals who are guiding the families in the use of TAI. In addition to familiarization with the equipment, and how to perform the technique safely, training must include explaining to the caregivers the symptoms of colonic perforation, and how to proceed in these emergency circumstances (52). The first session of TAI must be performed under the supervision of an experienced healthcare professional and preferably in a medical facility (52). Because TAI will generally be performed in the child's home, there is an obvious benefit to a third party such as a nurse specialist supervising TAI there during follow-up.
Proposed Treatment Regimen in Transanal Irrigation
Tonicity of Irrigant
Most clinicians in Europe and North America recommend simple tap water as the irrigant.
Any condition that features severe colonic dilatation or dysmotility may, however, theoretically predispose to prolonged retention of the irrigant, which, if hypotonic, could in theory be absorbed and cause iatrogenic hyponatremia. Some authors recommend “periodic evaluations” of serum electrolytes (53). To avoid this theoretical risk altogether, some units instead use normal (0.9%) saline as the irrigant. On the contrary, there are now enough published reports of successful and safe colonic irrigation including TAI with tap water to suggest that these concerns are unlikely (11,12,32,35). Therefore, the authors of this consensus paper do not routinely measure electrolytes, but if symptoms or signs of electrolyte imbalance present themselves, laboratory tests should be performed.
Sterility of Irrigant
Any source of drinkable water should suffice. Where the cleanliness of the tap water is doubtful, either cooled boiled or bottled water is recommended to avoid transmission of organisms such as amoeba or cryptosporidium.
Temperature of Irrigant
It is recommended that the irrigating solution should be close to body temperature (36°C–38°C), to reduce discomfort and nausea/vomiting from reflex bowel spasm, which can also lead to premature expulsion of the solution before it has had a chance to act on the stool.
Volume of Irrigant
The lowest volume of irrigant should be used that achieves the desired effect. Most specialist groups known to the authors use a volume of irrigant of 10 to 20 mL/kg (13,18,38), with a maximum total volume of 1 L (8). This calculation should be based on ideal body weight for height rather than the actual weight of an obese child. In selected or nonresponding patients, a more individualized approach can be considered, which may include information from imaging studies (6,31).
Use of Laxatives
Although some patients may be able to discontinue the use of laxatives after initiating TAI, others will require continued use, either at the same or reduced doses. This should be assessed individually in each patient. For a patient who has been requiring oral laxatives for a long time, there may be some merit in continuing these until he/she has become successfully established on TAI, and then gradually weaning off the laxatives as tolerated. Other clinicians add a stimulant or lubricant (such as bisacodyl, glycerin, polyethylene glycol, or Castile soap) to the colonic irrigation fluid (13). By achieving a more thorough colonic evacuation (54), this may permit a longer interval between TAI sessions, which may be an important option for those with busy social lives. Very little evidence or experience, however, exists regarding the addition of substances to the irrigation water, and to the knowledge of this authors this constitutes and off-label use. Finally, hyperphosphatemia, hypocalcemia, and hypokalemia have been associated with the use of phosphate enemas (55) and chemical colitis with use of castile or glycerin soap (56).
Frequency of Transanal Irrigation
Ideally TAI should be carried out at roughly the same time of the day, to recruit the beneficial effect of the “body clock” on gastrointestinal motility. Most units begin TAI on a daily basis until a successful routine is established. After that, some reduce 1 day of TAI per week every few weeks, progressing to 6 days per week, then to 5 days per week, and so on. Others reduce more rapidly by simply moving straight to TAI on alternate days. The emphasis should be on reducing the total time the child spends in the bathroom each week, as this has been shown to be a major benefit of TAI (11,20,32). Clearly, if the original constipation/incontinence relapses after reducing the frequency of TAI, the patient should return to the previously successful frequency.
It is almost inevitable that some difficulties will arise during the initiation of TAI. Therefore, it is recommended that the trainer and/or the clinician arrange a review visit after several weeks, and again several months later, to fine-tune the technique as needed. Even long after a successful schedule has been established, the long-term need for the readily available support (ideally via initial telephone or email contact) of a healthcare professional who is familiar with the child and family, and trained and experienced in the use of TAI, cannot be overemphasized. Some manufacturers of TAI devices offer a follow-up and support program to TAI users, which can be a helpful complement. Some suggestions for the more common problems that may occur, adapted from the adult patient population (8) are provided in Table 4.
Success Rates of Transanal Irrigation
Success rates of TAI obviously vary between the different underlying conditions of the patients, indications, and circumstances of use. Also, definitions of success differed among studies and among patient populations. The use of TAI in children with spina bifida and neurogenic BD resulted in significant improvement of FI in one of the studies, as 72% of 35 patients gained continence with the treatment modality suggested (11). Notably, the rate of patients with partial to total independence in toileting increased from 28% to 48% in the same study. A questionnaire study (41) compared 2 different methods of bowel management (TAI vs MACE) in pediatric patients with NBD due to myelomeningocele. The authors found no difference between the groups in the rates of fecal leakage or children's satisfaction, but a significantly higher satisfaction in parents of patients using MACE. In a review article of pediatric TAI (19), children ages 3 to 16 years with spina bifida, anorectal malformation, and sacral agenesis were studied. Most parents reported an improvement of the child's FI and a positive effect on children and family life was noted. A database study (37) reported on a heterogeneous group of pediatric patients using TAI in which most patients (62%) experienced idiopathic constipation; one quarter of the patients were classified as “nonadopters,” meaning that they discontinued the use of TAI within 1 month. Among the so-called “adopters,” a successful outcome was seen in 84%. Younger age was predictive for nonadopters.
An Italian multicenter study was conducted in children with anorectal malformation and spinal cord lesions with previous unsatisfactory bowel management. Patients were initiated on TAI (18) and evaluated at baseline and after 3 months of using TAI by completing a questionnaire on bowel function and QoL, and the Bristol Stool Chart. The authors concluded that all patients had improvement in these scores. Furthermore, all patients initiated on TAI continued its use at 3 months’ follow-up. Some studies on children with myelomeningocele (12,30) have also reported a significant reduction in urinary tract infections after starting TAI.
Complications of Transanal Irrigation
The most severe complication of TAI is bowel perforation (Table 3). A recent review article by Christensen et al (51), estimated the overall risk of perforation in the most recent years available to be in the order of 2 per 1 million procedures (all patient groups and ages). The same article provided extensive data on bowel perforation in 49 cases during 2005 to 2013, which pointed to increased risk during initiation of treatment and after pelvic surgery. Bowel perforation appears to be rare in children, with only 1 of those 49 cases being a child, corresponding to a rate in the order of 1 in 1 million procedures. Historically, perforations were not rare in diagnostic procedures such as contrast enema. By comparison, perforation risk during other procedures may be as high as 1 in 1000 during colonoscopy (57), or 1 in 40,000 during flexible sigmoidoscopy (58). In addition to perforations, some minor complications and complaints have been recorded. Some of these are frequent, and they represent a concern as they can lead to noncompliance and discontinuation of the TAI procedure.
This article reports data from the review of the existing literature and our personal shared experiences, with the intention to define indications and provide practical advice on using TAI. Different surgical therapeutic approaches have been used in children with BD over time including colostomy and MACE (17). Over the last 10 years, some centers, however, report having largely replaced surgical procedures for bowel continence in children with the introduction of TAI (21,28).
Based on the literature and our personal experience, TAI represents an effective and safe therapeutic approach for treating BD in children. Although TAI practice has been standardized in adults (8), its introduction in children has so far been without a standardized approach; hence, this consensus report on best practice.
Different commercial devices have been designed to facilitate the TAI practice and are available using a balloon catheter or cone tip, with water instillation regulated either by gravity or by manual or electronic pumps (Peristeen Coloplast, t, Qufora MBH, Irypump BBraun, Navina Wellspect). It shall be noted that at the time of writing this article, some of these devices had not been tested and/or approved for use in pediatric patients. Most of the reported experiences and recent published evidence are related to 1 device (Peristeen), but the possible advantages or disadvantages of the different devices remain unclear and are beyond the scope of this article. Just as for the choice of catheter for clean intermittent urethral catheterization, the specific patient's needs should guide the product choice, which may also be influenced by availability, reimbursement, and clinical support in different global settings. Whatever device is to be used to perform TAI, it is important that it will be available to the family as soon as training has been completed. Conversely, the device should only be used by the patient once the training has been completed. Parameters must be individualized and attention must be paid to adherence and follow-up. In some of the published studies with adult patients, a large number of patients discontinued TAI (59), and critical points determining compliance included education, training, and ongoing support over time (eg, home visit, phone call, outpatient clinic evaluation, patient support programs). Adherence to treatment seems to be higher in the studies with a pediatric population, but many of those are for the moment based on short- or mid-term, with only a few studies reporting data beyond 3 years of use (21,23,39). It still remains to be confirmed if this higher adherence remains in longer-term follow-up and for all indications.
Because one of the major concerns remains the limited evidence base, future research should include randomized controlled clinical trials around the world in patients with different pathologies, comparing safety, efficacy, and defining outcome measures including patient and parental satisfaction. Moreover, industry should be encouraged to produce specific devices for the pediatric age group that are different from adapted devices designed for adults. Finally, the putative benefits and disadvantages of different irrigation fluids (saline, added laxatives) and concomitant use of probiotics and prebiotics should be examined.
Pediatric patients with either functional or organic BD represent a complex group in whom management is often difficult. Many patients undergo multiple noninvasive and invasive treatments without benefit. Because of the frequent failure of these standard treatments, TAI has become a valuable therapeutic alternative that may prevent surgical intervention. Patient selection, dedicated healthcare professionals, thorough training, and careful follow-up are the key to TAI success. Healthcare professionals should always use a tailored approach to the individual patient considering the different underlying bowel pathologies, presenting symptoms, and personal and family dynamics, to increase efficacy and adherence.
The authors would like to thank Dr Steve James Hodges, Pediatric Urologist affiliated to Wake Forest University Health Service, Winston-Salem, (NC, USA) for his contribution to and review of the manuscript. Editorial support was provided by Dr Jacob Thyssen, Denmark.
Because most of the training and practical trouble shooting is performed by nurses, continence nurse specialists, urotherapists, or representatives of device manufacturers, the Troubleshooting table has been written with input from delegates attending the annual congress of the Nurses Group of the European Society for Pediatric Urology (ESPU-N) held in Prague in October 2015 and additional significant input from UK nurses Jo Searles (Sheffield Childrens Hospital), Martina Thomas (Colchester Hospital), Lenus Buzgoi (Chelsea and Westminster Hospital, London), Yvette Perston (Queen Elizabeth Hospital, Birmingham), and Brigitte Collins (St.Mark's Hospital, London), for which the authors are grateful.
1. Rasquin A, Di Lorenzo C, Forbes D, et al. Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology
2. Benninga MA, Faure C, Hyman PE, et al. Childhood functional gastrointestinal disorders: neonate/toddler. Gastroenterology
3. Mugie SM, Benninga MA, Di Lorenzo C. Epidemiology of constipation in children and adults: a systematic review. Best Pract Res Clin Gastroenterol
4. Wald A, Sigurdsson L. Quality of life in children and adults with constipation. Best Pract Res Clin Gastroenterol
5. Rajindrajith S, Devanarayana NM, Benninga MA. Review article: fecal incontinence in children: epidemiology, pathophysiology, clinical evaluation and management. Aliment Pharmacol Ther
6. Bischoff A, Levitt MA, Peña A. Bowel management for the treatment of pediatric fecal incontinence. Pediatr Surg Int
7. Keshtgar AS, Ward HC, Clayden GS, et al. Investigations for incontinence and constipation after surgery for Hirschsprung's disease. Ped Surg Int
8. Emmanuel AV, Krogh K, Bazzocchi G, et al. Consensus review of best practice of transanal irrigation in adults. Spinal Cord
9. Shandling B, Gilmour RF. The enema continence catheter in spina bifida: successful bowel management. J Ped Surg
10. Del Popolo G, Mosiello G, Pilati C, et al. Treatment of neurogenic bowel dysfunction using TAI: a multi center Italian study. Spinal Cord
11. Lopez Pereira P, Salvador OP, Arcas J, et al. TAI for the treatment of neuropathic bowel dysfunction. J Pediatr Urol
12. Ausili E, Focarelli B, Tabacco F, et al. Transanal irrigation in myelomeningocele children: an alternative safe and valid approach for neurogenic constipation. Spinal Cord
13. Koppen IJN, Kuizenga-Wessel S, Voogt HW, et al. Transanal irrigation in the treatment of children with intractable functional constipation. J Pediatr Gastroenterol Nutr
14. Loening-Baucke V. Efficacy of biofeedback training in improving faecal incontinence and anorectal physiologic function. Gut
15. Marshall DF, Boston VE. Altered bladder and bowel function following cutaneous electrical field stimulation in children with spina bifida—interim results of a randomized double-blind placebo-controlled trial. Eur J Pediatr Surg
1997; 7 (suppl 1):41–43.
16. Sulkowski JP, Nacion KM, Deans KJ, et al. Sacral nerve stimulation: a promising therapy for fecal and urinary incontinence and constipation in children. J Pediatr Surg
17. Siminas S, Losty PD. Current surgical management of pediatric idiopathic constipation: a systematic review of published studies. Ann Surg
18. Midrio P, Mosiello G, Ausili C, et al. Peristeen transanal irrigation in pediatric patients with anorectal malformation and spinal cord lesions: a multicentric Italian study. Colorectal Dis
19. Bray L, Sanders C. An evidence-based review of the use of transanal irrigation in children and young people with neurogenic bowel. Spinal Cord
20. Corbett P, Denny A, Dick K, et al. Peristeen integrated transanal irrigation system successfully treats fecal incontinence in children. J Ped Urol
21. Alenezi H, Alhazmi H, Trbay M, et al. Peristeen anal irrigation as a substitute for the MACE procedure in children who are in need of reconstructive bladder surgery. Can Urol Assoc J
22. Blair GK, Djonlic K, Fraser GC, et al. The bowel management tube: an effective means for controlling fecal incontinence. J Pediatr Surg
23. Cazemier M, Felt-Bersma RJ, Mulder CJ. Anal plugs and retrograde colonic irrigation are helpful in fecal incontinence or constipation. World J Gastroenterol
24. Choi EK, Han SW, Shin SH, et al. Long-term outcome of transanal irrigation for children with spina bifida. Spinal Cord 2014; 1–5.
25. Choi EK, Shin HS, Han SW, et al. The effects of transanal irrigation as a stepwise bowel management program on the quality of life of children with spina bifida and their caregivers. Spinal Cord
26. Chrzan R, Klijn AJ, Vijverberg MA, et al. Colonic washout enemas for persistent constipation in children with recurrent urinary tract infections based on dysfunctional voiding. Urology
27. Fernandez Eire P, Varela Cives R, Castro Gago M. Fecal incontinence in children with spina bifida: the best conservative treatment. Spinal Cord
28. Kelly MS, Dorgalli C, McLorie G, et al. Prospective evaluation of peristeen transanal irrigation system with the validated neurogenic bowel dysfunction score sheet in the pediatric population. Neurourol Urodyn
2016; [Epub ahead of print].
29. Liptak GS, Rewell GM. Management of bowel dysfunction in children with spinal cord disease or injury by means of the enema continence catheter. J Pediatr
30. Marte A, Borrelli M. Transanal irrigation and intestinal transit time in children with myelomeningocele. Minerva Pediatr
31. Märzheuser S, Schmidt D, David S, et al. Hydrocolonic sonography: a helpful diagnostic tool to implement effective bowel management. Pediatr Surg Int
32. Märzheuser S, Karsten K, Rothe K. Improvements in incontinence with self-management in patients with anorectal malformations. Eur J Pediatr Surg
33. Matsuno D, Yamazaki Y, Shiroyanagi Y, et al. The role of the retrograde colonic enema in children with spina bifida: is it inferior to the antegrade continence enema? Pediatr Surg Int
34. Mattsson S, Gladh G. Tap-water enema for children with myelomeningocele and neurogenic bowel dysfunction. Acta Paediatr
35. Nasher O, Hill RE, Peeraully R, et al. Peristeen transanal irrigation system for pediatric fecal incontinence: a single centre experience. Int J Pediatr
36. Neel KF. Total endoscopic and anal irrigation management approach to noncompliant neuropathic bladder in children: a good alternative. J Urol
37. Ng J, Ford K, Dalton S, et al. Transanal irrigation for intractable faecal incontinence and constipation: outcomes, quality of life and predicting non-adopters. Pediatr Surg Int
38. Pacilli M, Pallot D, Andrews A, et al. Use of Peristeen transanal colonic irrigation for bowel management in children: a single-center experience. J Pediatr Surg
39. Vande Velde S, Van Biervliet S, Van Laecke, et al. Colon enemas for fecal incontinence in patients with spina bifida. J Urol
40. Walker J, Webster P. Successful management of faecal incontinence using the enema continence catheter. Z Kinderchir
1989; 44 (suppl 1):44–45.
41. Wide P, Mattsson GG, Drott P, et al. Independence does not come with the method—treatment of neurogenic bowel dysfunction in children with myelomeningocele. Acta Paediatr
42. Tabbers MM, DiLorenzo C, Berger MY, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESGHAN and NASPHGAN. J Pediatr Gastroenterol Nutr
43. Voskuijl WP, Van Ginkel R, Taminiau JA, et al. Loperamide suppositories in an adolescent with childhood-onset functional non-retentive fecal soiling. J Pediatr Gastroenterol Nutr
44. Velde SV, Biervliet SV, Bruyne RD, et al. A systematic review on bowel management and the success rate of the various treatment modalities in spina bifida patients. Spinal Cord
45. Bongers ME, Van den Berg MM, Reitsma JB, et al. A randomized controlled trial of enemas in combination with oral laxative therapy for children with chronic constipation. Clin Gastroenterol Hepatol
46. Clarke MC, Chase JW, Gibb S, et al. Decreased colonic transit time after transcutaneous interferential electrical stimulation in children with slow transit constipation. J Pediatr Surg
47. Johanson JF, Morton D, Geenen J, et al. Multicenter, 4-week, double-blind, randomized, placebo-controlled trial of lubiprostone, a locally-acting type-2 chloride channel activator, in patients with chronic constipation. Am J Gastroenterol
48. Lembo AJ, Schneier HA, Shiff SJ, et al. Two randomized trials of linaclotide for chronic constipation. N Engl J Med
49. Benninga MA, Tabbers MM, Van Rijn RR. How to use a plain abdominal radiograph in children with functional defecation disorders. Arch Dis Child Educ Pract Ed
50. Bekkali NL, Van den Berg MM, Dijkgraaf MG, et al. Rectal fecal impaction treatment in childhood constipation: enemas versus high doses oral PEG. Pediatrics
51. Christensen P, Krogh K, Perrouin-Verbe B, et al. Global audit on bowel perforations related to transanal irrigation. Tech Coloproctol
52. Norton C. Guidelines for the use of Rectal Irrigation for Healthcare Professionals. St Mark's Hospital and Burdett Institute of Gastrointestinal Nursing editor, 2009, pp. 1–24.
53. Yerkes EB, Rink RC, King S, et al. Tap water and the Malone antegrade continence enema: a safe combination? J Urol
54. Bani-Hani AH, Cain MP, King S, et al. Tap water irrigation and additives to optimize success with the Malone antegrade continence enema: the Indiana University algorithm. Urol
55. Craig JC, Hodson EM, Martin HC. Phosphate enema poisoning in children. Med J Aust
56. Sheibani S, Gerson LB. Chemical colitis. J Clin Gastroenterol
57. Loffeld RJ, Engel A, Dekkers PE. Incidence and causes of colonoscopic perforations: a single-center case series. Endoscopy
58. Atkin WS, Cook CF, Cuzick J, et al. Single flexible sigmoidoscopy screening to prevent colorectal cancer: baseline findings of a UK multicentre randomised trial. Lancet
59. Christensen P, Krogh K, Buntzen S, et al. Long-term outcome and safety of transanal irrigation for constipation and fecal incontinence. Dis Colon Rectum