Box 26.3 Symptoms and signs suggestive of underlying pathology in children with constipatio n \231 Administration of oral pancreatic enzymes may reduce pain via negative feedback inhi bition to the pancreas, with some benefi t shown in adults (Layer et al., 1990 ), but data in children is lacking. \227\206 Constipation reported from birth or fi rst few weeks of life. \227\206 Failure to pass meconium/delay (more than 48 h) in a term baby. \231 Antioxidant therapy has been suggested for chronic pancreatitis in adults (Bhardwaj et al., 2004 ), but there is confl icting data about effi cacy, and further research is nee ded (Gachago et al., 2008 ). \227\206 Infrequent/very large (ribbon) stools. \227\206 Faltering growth. \231 Antibiotics may prevent secondary bacterial infections, limit complications, and imp rove outcome; however, they are best reserved for severe cases, especially where pancreatic necrosis is present (Sainio et al., 1995 ). \227\206 Previously known or undiagnosed weakness in legs/locomotor delay. \227\206 Abdominal distension with vomiting. \231 Surgical intervention is rarely required in management of pan- creatitis except in c ases of congenital malformations or manage- ment of complications such as drainage of pseud ocysts. \227\206 Perianal inspection \200\224 abnormal appearance/patency/position of anus, anter iorly placed anus or absent anal wink, fi ssures, fi stulae, bruising, tight or patulous an us. \227\206 Gross abdominal distension, palpable fecal mass (in at least Constipation Constipation is a very common (5 \200\22330%) cause of abdominal pain in child ren (van den berg et al., 2006 ). Most children do not have an underlying cause and the ROM E III criteria for diagnosis of func- tional constipation include presence of two or more o f the follow- ing for more than 1 month (Rasquin et al., 2006 ): half of these patients). \227\206 Spine and gluteal examination \200\224 asymmetry of gluteal muscles, scoliosis, evidence of sacral agenesis, overlying skin over sacral region with discoloration, naevi, s inus, hairy patch, lipoma or central pit. \227\206 Deformity in lower limbs such as talipes. \231 Two or fewer defecations per week. \227\206 Abnormal neuromuscular signs unexplained by any existing \231 At least one episode/week of incontinence aft er the acquisition of condition, such as cerebral palsy. toileting skills. \227\206 Abnormal tendon refl exes. \231 History of excessive stool retention. \231 History of painful or hard bowel movements. for compliance (sitting on the toilet) at fi rst and then for success (opening bowel in the toilet). \231 Presence of a large fecal mass in the rectum. \231 History of large-diameter stools which may obstruct the toilet. \231 Drug treatment: laxatives are used in the management of acute as well as chronic con stipation. As abdominal pain in these children is principally due to fecal impaction it is poorly responsive to analgesics. History and clinical examination aim to assess the severity of symp- toms and resulting fun ctional impairment. Symptoms and signs that are suggestive of underlying pathology ( Box 26 .3 ) should be sought to ensure diagnosis or exclusion of organic conditions. \231 Disimpaction of stools can reduce pain (Poenaru et al., 1997 ). Senna or polyethylen e glycol can be used as sole agents in high doses. In severe cases, sodium picosulphate (li quid or sachets) may be required. Rectal medications (suppositories, enemas) are used if al l oral medications have failed. Manual evacuation of the bowel under anesthesia is rarely c onsidered, and only if oral and rectal treatments fail. Management Th e UK National Institute for Health and Clinical Excellence (NICE) produced a clinical guideline \200\230 CG99 Constipation in children and young people: Diagnosis and management of idiopathic childhood constipation in primary and secondary care \200\231 in M ay 2010 (< http:// www.nice.org.uk/cg99 >). Th e management of chronic functional constipat ion oft en requires a multidisciplinary approach, based on the following principles: \231 Laxatives are oft en needed for prolonged maintenance therapy, but need to be closel y monitored, weaned only aft er a sustained period of normal bowel actions with no soiling, and used in conjunction with other supportive strategies. Polyethylene gly- col (Movicol ) is widely used. Senokot given in the evening, at reasonable doses, is eff ective. Th e choice of agent depends on local preference as well as individual patient circumstances ( Table 26.2 ). Young children should remain on laxatives until toi- let training is well e stablished. \231 Explanation of normal bowel function aids the family \200\231s understand- ing and i mproves compliance. Parents and children should be reas- sured that constipation is common and responds well to treatment. \231 High-fi bre diet is recommended along with adequate fl uid intake because it adds bulk to the stool. \231 Regular exercise promotes peristalsis and helps bowel transit. Neuromuscular motility disorders Motility disorders present with features of gastrointestin al obstruc- tion in the absence of a mechanical cause. Th e spectrum of motil- ity disorder s ranges from relatively benign conditions such as \231 Regular toileting is a crucial part of the management to encour- age regular emptying of the rectum. \231 Behavioral advice and reward schemes (star charts) are helpful to gain child \200 \231s trust in the management. Rewards can be given CHAPTER 26 pain and gastroenterological diseases 265 gastro-oesophageal refl ux and functional constipation, to life-threat- ening illnesses suc h as chronic intestinal pseudo-obstruction. It can result from any process that interferes with coordinated patterns of propulsive movement of intestinal musculature. Chronic disorde rs of motility account for 15% of patients with intestinal failure necessitat- ing the use of parenteral nutrition (Connor et al., 2006 ) and irrevers- ible cases may require intesti nal transplant (Gupte et al., 2007 ) cases, symptoms are present from birth or early infancy. Pain is usually a prominent featur e and is a result of uncoordinated con- traction of bowel musculature (Takahashi, 2005 ). Management Management requires a multidisciplinary team that includes a gastroenterologist, surgeon, dietician, and psychologist. Chronic abdominal pain or the fear of pain is a comm on problem in children with chronic intestinal pseudo-obstruction. Luminal dilatation, repe ated invasive procedures, and mucosal infl ammation may all contribute to the development o f visceral hyperalgesia with sensiti- zation of peripheral and central pain pathways (Anand et al., 2004 ; Christianson et al., 2009 ; Feng et al., 2012 ). Th e stress of chronic dis ease can also infl uence pain report and altered family dynamics (see also Birnie et al., C hapter 12; Fogeron and King, Chapter 13, this volume). Intestinal pseudo-obstruction can be divided into acute or chronic. Chronic pseudo obstruct ion can be primary or second- ary to systemic illness. Primary pseudo-obstruction is a resu lt of disorders of intestinal smooth muscle (myopathic) or myentric plexus (neuropathic), a nd can involve either the whole of or a localized segment of the bowel ( Box 26.4 ). Neurop athic disorders are more common and may be primary or secondary (e.g. in utero insults such as fetal alcohol syndrome or postnatal injuries such as ischaemic events or viral infectio ns; Rudolph et al., 1997 ). Antro- duodenal manometry measures the intraluminal pressure of the antrum and duodenum and is useful to distinguish myopathy (con- traction amplitude is reduced but spatial and temporal organization is preserved), from neuropathy (contractions have normal ampli- tude but are uncoordinated and lack normal physiologic patterns) (Hyman et al., 1993 ). Analgesics may have limited effi cacy, and use of morphine can worsen symptoms due to furth er slowing of gut (Brunton et al., 2011 ). Controlling bowel dilatation and infl ammation c an reduce pain. Prokinetics used to improve gastric emptying and gut motility include eryth romycin (Ng et al., 2012 ), domperidone, and neostigmine in severe cases (Gmora et al., 200 2 ). Octreotide is being increasingly studied as a prokinetic to increase small intestinal motility, inhibit gastric emptying, and gallbladder con- tractility, but benefi t has only been reported in adults (Soudah et al., 1991 ). Mitochondrial disorders such as mitochondrial neurogastroin- testinal encephalopathy (MNGIE ; an autosomal recessive multisys- tem disorder caused by thymidine phosphorylase defi cien cy) may also be complicated by pseudo-obstruction, with gastrointestinal symptoms (diarrhea , dysphagia, and failure to thrive) oft en pre- ceding the neurological dysfunction (leucoe ncephalopathy, exter- nal ophthalmoparesis and demyelinating peripheral neuropathy) (Shoff ner et al., 2010 ). Recently, abnormalities of the gastroin- testinal pacemaker cells, the interstitial cells of Cajal, have been described in patients with motility disorders. In mo st pediatric Stimulant laxatives have shown to be benefi cial in relieving constipation as well as incre asing motility (Connor et al., 2006 ). Reduced motility is also associated with bacterial o vergrowth which causes further mucosal infl ammation and damage, and worsens the gastrointe stinal motility. Oral antibiotics help reduce infl ammation and reduce distension by limiti ng gas-forming bacteria. Probiotics along with antibiotics have also been used in cases of overgrowth though their superiority above a placebo is yet to be proven (Shanahan, 2003 ). A dilated non-motile segment of the gut can be surgically removed or gastrostomy, jejunosto my, or loop enterostomy may be required to shorten the gut and facili- tate transit of intr aluminal contents, and can improve enteral feed tolerance in some cases (Shibata et al., 20 03 ). Small intestinal transplantation is indicated in total parenteral nutrition (TPN)- de pendent pseudo-obstruction patients with life-threatening complications of PN or with dwind ling venous access (Mittal et al., 2003 ). Box 26.4 Neuromuscular motility disorders associated with primary chronic pseudo-obstructio n \227\206 Aganglionosis: \227\206 Hirschsprung \200\231s disease. \227\206 Primary visceral myopathies: \227\206 Familial myopathy with megaduodenum \227\206 Megacystis microcolon \227\206 Hollow visceral myopathy Conclusion Pain is an important and common symptom of gastrointestinal dis- ease. Pain may be the presenting feature, or an indicator of acute complications, that requires symptomati c treatment until the under- lying gastrointestinal disorder is controlled by specifi c tre atments. 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Howard Introduction Postoperative pain management begins prior to surgery and includes not only th e prevention and pharmacological treatment of pain, but also a holistic and well-coordinate d strategy that allays fears and anxieties, and allows children and their carers to partici pate in the selection and implementation of safe and suitable analgesia. Admission to hospi tal for surgery is a signifi cant and potentially traumatic event. Coping with a strange an d unknown environment, fear of separation, anticipation of painful procedures, and postop- erative pain or adverse eff ects such as nausea are all prominent causes of anxiety and str ess that can increase the perception of pain and impact on the quality of perioperative car e. Th erefore, a suc- cessful postoperative pain management program will include: ongoing t raining of hospital staff , adequate preparation of children and families that provides tim ely verbal and written information, and the development and implementation of audited insti tutional analgesic protocols that ensure the safety and effi cacy of pain man- agement stra tegies in a child-friendly and secure environment. response, as treatment must be fl exible enough to adapt to inter- individual variation and unexpected circumstances or events. Side eff ects, such as PONV (postoperative nausea and vomiting), over- sedation, or depression of respiration should also be anticipated, monitor ed, and managed. Pain aft er surgery can vary in intensity and duration, but is virtu- ally always greatest in the early perioperative period. Pain inten- sity decreases in the succeeding days or wee ks depending not only on the type and extent of surgery but also on less predictable inter- individual factors, but can normally be expected to resolve spontaneously over time. In pra ctical terms this implies that in the fi rst postoperative hours and days analgesia should be given regularly, by the clock, until requirements start to fall. Analgesia can then be g iven \200\230when required \200\231 at times when pain is reported or measured, or pain is anticipated, e.g. prior to mobilization. A number of professional organizations have produ ced detailed and helpful guidelines on the analgesic management of postoperative pain in ch ildren. \200\230Good Practice in Postoperative and Procedural Pain \200\231 from the Assoc iation of Pediatric Anesthetists of Great Britain and Ireland are comprehensive, systematic ally developed, evidence-based guidelines on procedure-specifi c analgesic strat- egies for postoperative pain in neonates and children (Howard et al., 2012 ). \200\230Acute Pain Ma nagement: Scientifi c Evidence \200\231 from the Australian and New Zealand College of Anes thetists (Macintyre et al., 2010 ), and \200\230Practice Guidelines for Acute Pain Managem ent in the Perioperative Setting \200\231 from the American Society of Anesthesiologists al so include advice on the management of post- operative pain in children (American Society o f Anesthesiologists, 2012 ). Th e pediatric anaesthesiologist is responsible for the select ion and initiation of an appropriate perioperative analgesic regimen. In many institutions ongoing care is devolved to a multi- disciplinary postoperative pain management service, bu t all pro- viders of postoperative pain treatment should be familiar with the methods, indi cations, doses, and side eff ects of the most commonly used analgesics and analgesic techni ques. Aims \227\206 Understand basic principles of postoperative analgesia. \227\206 Ability to select an age-appropriate postoperative pain assess- ment tool. \227\206 Understand the pharmacology, advantages and disadvantages of the principal analgesics, and analgesic techniques. \227\206 Ability to describe suitable analgesic regimens for frequently encountered surgical procedures and in special situations. General principles Postoperative pain is predictable, but pain intensity and the response t o analgesia are subject to many diff erent patient-deter- mined variables that are not full y predictable. Th erefore, postopera- tive pain should be anticipated and its intensity ass essed frequently during the postoperative period so that appropriate analgesia can be admin istered promptly, and the response to treatment assessed. Analgesia should be planned accor ding to the expected intensity and duration of pain, but must always be titrated against cl inical Negative e \200 ects of postoperative pain Aside from its unpleasant and distressing quali ties, poorly managed pain has negative physiological, psychological, and behavioral consequ ences. Changes in cardiorespiratory, gastrointestinal, endocrine, and immune function that can be prevented by eff ective SECTION 4 pain in specific populations and diseases 270 analgesia are known to occur (see Acute Pain Management: Scientifi c Evidence (3rd edition) for an up-to-date account; Macintyre et al., 2010 ). Postoperative pain can delay re-mobil ization and recovery from surgery and can lead to undesirable long-term eff ects. re-assessment; thereby establishing a cycle of evaluation and treat- ment that should conti nue throughout the postoperative period (Howard, 1997 ). Th is requires training of health care profession- als in the principles of acute pain assessment and the selection and use o f structured pain measurement tools that are suitable for the developmental age of the chil d and the context in which they are to be used. As postoperative pain management must oft e n continue at home following discharge from hospital, parents and carers may also require g uidance and instruction on the assessment of pain. Pain and injury during infancy and childhood can adversely infl uence pain processing mecha nisms in later life, including the response to subsequent pain and injury many years later (see Walker, Chapter 3, this volume). In addition, a small proportion of children may exper ience persistent postsurgical pain for months or years. Predisposing factors include poorly controlled postoperative pain, extensive or repeated surgery involving nerve damage, and p sy- chological vulnerability (Kehlet et al., 2006 ; Macintyre et al., 2010 ; Macrae, 2008 ) . Poorly managed postoperative pain also contributes to adverse and problematic changes in children \200\231s behavior and sleep pattern, which may be observed for prolonged periods aft er surgery, and cause considerable distress and disruption to family life (Karling et a l., 2007 ; Kotiniemi et al., 1997a ; Power et al., 2012 ). Basic principles of measurement For pain to be measured as accurately as possible, the prin ciples underpinning assessment at diff erent developmental ages and in diff erent settings must be understood. Standardized pain measure- ment tools use various diff erent methods of self-report or obser- vations to quantify pain intensity. Older children are able to use s elf-report scales such as the visual analog scale (VAS) or numer- ical rating scale (NRS) b ut these methods are not accurate in chil- dren who are too young to understand such concep ts and cannot be used in infants and pre-verbal or non-verbal children. In order to overcom e these obstacles a variety of scales have been devised using three basic methods of measur ement: Postoperative pain management services Anaesthesiology-led acute pain management services w ere devel- oped to improve the quality and consistency of care following surgery. Th eir re sponsibilities include: provision of education pro- grammes for staff , provision and maint enance of specialized equip- ment (such as patient-controlled analgesia (PCA) pumps), and o verall supervision of pain management and recognition/manage- ment of side eff ects or comp lications ( Box 27.1 ). 1. Self-report (e.g. using diagrams or pictures). 2. Observations of behavior. 3. Observations of physiological parameters. Multidisciplinary institutional Pain Management Services have been established in many hosp itals to fulfi l this role, several mod- els have been described; they are an essential res ource to ensure the quality and consistency of care (Lloyd-Th omas and Howard, 1994 ; Shapi ro et al., 1991 ; Stomberg et al., 2003 ). Such services have facilitated the introduction of complex and innovative infu- sion techniques for pain management in children including P CA, nurse-controlled analgesia (NCA), and continuous regional local anesthesia (LA), e.g. e pidural and paravertebral blocks.