Children s Hospital of Pittsburgh Continuity Clinic Curriculum Week of April 10, 2017 Nader Shaikh, MD, MPH Topic: Toileting: Constipation and Fecal Incontinence Learning Objectives: At the end of this learning experience, viewers will be able to: 1. Define constipation. 2. Identify signs and symptoms that differentiate functional from organic causes of constipation. 3. Describe the appropriate management strategies for functional constipation and fecal incontinence. (Original module prepared by Jennifer Chianese, MD, December 2004; revised by Nader Shaikh, MD, March 2008 and Aimee Biller, MD, January 2011 and September 2013) Content copyright 2011, 2017 University of Pittsburgh of the Commonwealth System of Higher Education. Created at the Division of General Academic Pediatrics, Children s Hospital of Pittsburgh of UPMC All permissions to reprint materials from other sources are received or pending. For educational purposes only. Do not copy and distribute Disclaimer: The University of Pittsburgh School of Medicine, The Children s Hospital of Pittsburgh of UPMC, authors, editors, producers and sponsors of this educational program do not guarantee the accuracy of the information contained herein and assume no liability for decisions made and actions taken based on this information. This information is provided for general medical education purposes only and is not meant to substitute for the independent medical judgment of a physician or other health professional relative to diagnostic and treatment options of a specific patient s medical condition. The information provided is not intended to be all-inclusive. The viewer should supplement this information with additional readings, other educational materials and discussion. No actual or implied endorsement or promotion of any specific reference(s) or product(s) is made or intended by the University of Pittsburgh School of Medicine, The Children s Hospital of Pittsburgh of UPMC, authors, editors, producers and sponsors of this material. By proceeding with this course, you are acknowledging this disclaimer. 1
Case 1: The parents of Nina, a 1 month-old female, are concerned that she may be constipated. For the past few weeks, her face turns red and she cries and appears to be straining many times a day. Some of these episodes are not associated with a bowel movement, but she does pass a soft bowel movement about three times a day after these straining episodes. Question 1: Is Nina constipated? 2
Question 1: Is Nina constipated? Children <4 years of age with two or more of the following symptoms at least once per week for at least 1 month are defined as having functional constipation: 2 defecations per week Painful or hard bowel movements >1 episode of fecal incontinence (after acquisition of toileting skills) per week Voluntary stool retention Presence of a large fecal mass in the rectum on examination Large-diameter stools that may obstruct the toilet Note the wide range of normal stool frequencies in healthy children in the table below: Age Normal Stool Frequencies in Healthy Children Bowel movements per day (mean) Bowel movements per week (+/- 2 SD) 0-3 months Breast-fed 2.9 (5-40) Formula-fed 2.0 (5-28) 6-12 months 1.8 (5-28) 1-3 yrs 1.4 (4-21) > 3 yrs 1.0 (3-14) Adapted from Fontana M. Bianch C, Cataldo F, et al. Bowel frequency in healthy children. Acta Paediatr Scand 1987; 78:682-4. (Appeared as Table 1, Normal Frequency of Bowel Movements, in Evaluation and treatment of constipation in infants and children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr Sep 2006;43(3):e2.) The soft consistency and daily frequency of Nina s stools are not suggestive of constipation. However, her parents report significant distress associated with bowel movements. Straining with passage of soft stool is normal in neonates and infants and is called infantile dyschezia. It is thought to be related to their inability to coordinate the timing of pelvic floor relaxation with the Valsava maneuver. The criteria for a diagnosis of infantile dyschezia are: Age less than 6 months At least 10 minutes of straining and crying Successful passage of soft stools in an otherwise healthy infant. Question 2: What would you tell Nina s parents? 3
Question 2: What would you tell Nina s parents? Infants generally outgrow infantile dyschezia in early infancy. Parents should be reassured that it is benign and no intervention is needed. Case 2: Christopher is a 3 year-old male whose mother is concerned that he only passes a bowel movement twice a week. On further questioning, she reports that his stools are hard as rocks and sometimes very large ( clog the toilets ) and he complains of pain during defecation. Mom has tried to increase the amount of fiber in his diet, but he is picky about eating fruits and vegetables. Question 3: Define nonorganic constipation and organic constipation and give examples of causes of each. 4
Question 3: Define nonorganic constipation and organic constipation and give examples of causes of each. Nonorganic constipation is constipation without objective evidence of a pathologic condition. Also called functional constipation, this is the cause of constipation in 90 to 95% of children with constipation. Organic constipation is constipation with an underlying medical, structural, toxic or pharmacologic cause. Differential diagnosis of constipation Nonorganic: Developmental: Cognitive handicaps Attention-deficit disorders Situational: Coercive toilet training Toilet phobia School bathroom avoidance Excessive parental interventions Sexual abuse Organic: Anatomic malformations Imperforate anus Anal stenosis Anterior displaced anus Pelvic mass (e.g., sacral teratoma) Metabolic and gastrointestinal Hypothyroidism Hypercalcemia Hypokalemia Cystic fibrosis Diabetes mellitus Multiple endocrine neoplasia type 2B Gluten enteropathy Neuropathic conditions Spinal cord abnormalities Spinal cord trauma Neurofibromatosis Static encephalopathy Tethered cord Intestinal nerve or muscle disorders Hirschsprung disease Intestinal neuronal dysplasia Visceral myopathies Visceral neuropathies Other: Depression Constitutional Colonic inertia Genetic predisposition Reduced stool volume & dryness Low fiber in diet Dehydration Underfeeding or malnutrition Abnormal abdominal musculature Prune belly Gastroschisis Down syndrome Connective Tissue Disorders Scleroderma Systemic lupus erythematosus Ehlers Danlos syndrome Drugs Opiates Phenobarbital Sucralfate Antacids Antihypertensives Anticholinergics Antidepressants Sympathomimetics Other Heavy metal ingestion (e.g., lead) Vitamin D intoxication Botulism Cow s milk protein intolerance Celiac disease Adapted from Evaluation and treatment of constipation in infants and children: recommendations of the North American Society for Pediatric Gastorenterology, Hepatology and Nutrition, Table 4, Differential Diagnosis of Constipation J Pediatr Gastroenterol Nutr, Vol. 43, September 2006 e6. 5
Question 4: What are the important points to cover in eliciting a medical history from Christopher s mother? 6
Question 4: What are the important points to cover in eliciting a medical history from Christopher s mother? According to the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) Clinical Practice Guideline 2014: Time after birth of the first bowel movement What the family means when using the term constipation Length of time the condition has been present Frequency of bowel movements Consistency and size of the stools Whether defecation is painful Whether blood has been present on the stool or on the toilet paper Presence or absence of abdominal pain Whether there is a history of stool withholding Medication history Changes in diet Treatments used to date and their effectiveness Changes in the child s environment (major change in family, new school, etc) If the child is in school it is important to learn whether the child uses the school restrooms and if not, why. Red flags for organic disorders include: Passage of meconium >48 hours of life Onset of symptoms in first month of life Family history of Hirshsprung disease Abdominal distension Anorexia Nausea Vomiting Weight loss or poor weight gain Bloody diarrhea in an infant with a history of constipation could indicate enterocolitis complicating Hirschsprung disease 7
Case 2 (Continued): On further questioning, you learn that Christopher was a healthy full-term male who passed meconium in the first 24 hours. He has no significant past medical history. In the past, he has had periods of normal stooling patterns interrupted by bouts of hard stools which improved with the addition of prune juice to his diet. His current bowel problems have been persisting for the past few months. Question 5: What would you look for on Christopher s growth charts and physical examination to differentiate nonorganic from organic constipation? 8
Question 5: What would you look for on Christopher s growth charts and physical examination to differentiate nonorganic from organic constipation? Failure to thrive Abdominal distension Sacral abnormalities (dimple, tuft of hair, gluteal cleft deviation) Flat buttocks (sign of malnutrition, i.e. celiac disease) Extreme fear during anal inspection Anteriorly displaced or patulous (spreading) anus Absent anal wink or cremasteric reflex Decreased lower extremity tone and/or strength Absence or delay in relaxation phase of lower extremity deep-tendon reflexes Most cases of organic constipation can be ruled out by doing a thorough history and physical examination. Note: The perineum and perianal area should always be visually examined. Evidence does not support use of digital rectal exam to diagnose functional constipation. Summary of features in Hirschsprung disease and functional constipation Feature Hirschsprung disease Functional constipation Onset in infancy Common Rare Delayed passage of meconium Common Rare Painful defecation Rare Common Soiling Rare Common Stool withholding Rare Common Stool in the rectal vault Rare Common Failure to thrive Common Rare Case 2 (continued): You review Christopher s growth charts and complete his physical exam. You find that Christopher has been growing along the 50 th percentile for height and weight (current weight = 17 kg). His abdominal exam is notable for palpable stool in the left lower quadrant. His sacrum and anus appears WNL. The exam is otherwise normal. Question 6: What criteria have to be met for the diagnosis of functional constipation? 9
Question 6: What criteria have to be met for the diagnosis of functional constipation? Children >4 years of age with two or more of the following for at least one month are defined as having functional constipation: 2defecations in the toilet per week >1 episode of fecal incontinence per week Painful or hard bowel movements Voluntary stool retention with or without posturing Presence of a large fecal mass in the rectum on examination Large-diameter stools that may obstruct the toilet Routine use of abdominal radiography is NOT recommended for the diagnosis of functional constipation. Question 7: How would you treat the patient? 10
Question 7: How would you treat the patient? Components of Treatment for Functional Constipation: 1. Education Goal: Demystify constipation and remove negative attributions Educate parents that treatment requires time and patience Educate parents that relapses are common Encourage parents to be consistent, positive, and supportive, not punitive 2. Disimpaction Goal: Evacuate stool without pain (Oral or Rectal disimpaction) Disimpaction via the oral or rectal route (i.e., by using enemas) are equally efficacious; the oral route is preferred because it is non-invasive. Oral disimpaction For children with mild constipation start by using twice the usual dose of Polyethylene glycol (Miralax) (1 to 1.5 g/kg daily) over the weekend. For Christopher, this would come out to 1 cap twice a day (normal dose would be one cap daily). Although other laxatives (mineral oil, magnesium hydroxide, magnesium citrate, lactulose, senna, and bisacodyl) have been used for oral disimpaction, there are no controlled trials documenting their efficacy. Rectal disimpaction Phosphate soda enema Mineral oil enema followed by a phosphate enema Saline enema Glycerin suppository (in infants) Counsel parents against regular use of these in infants The success of disimpaction should be evaluated after ~1 week. If treatment was not effective, need to determine reasons for lack of efficacy (e.g., lack of adherence) and consider other options (higher dose, different medication, referral, re-education). 11
3. Maintenance Therapy Goal: Prevent the reaccumulation of stool Dietary modifications: The NASPGHAN guideline recommends a balanced diet that includes whole grains, fruits and vegetables. Sorbitol, which naturally occurs in some fruit juices (prune, pear) can increase the frequency and water content of stools. Current evidence does not support the use of fiber supplements in children. Medications: PEG starting at 0.4 g/kg daily and titrated to achieve one soft stool per day (~1/2 cap for Christopher). Maintenance should continue for at least 2 months. It is prudent to wait at least 1 month after all symptoms have resolved before stopping maintenance. If the child is toilet training, wait until training is completed. Mineral oil, magnesium hydroxide, and stimulant laxatives (senna, bisacodyl) are considered second line or additional treatment. Behavior modifications: Unhurried time on the toilet after meals (may need a note for school to allow this following lunch) Daily schedules of using the toilet regardless of whether there is an urge to defecate Diaries of stool frequency combined with a reward system Case 3: Brianna is a 10 year-old girl whose grandmother reports that she soils her underpants a few times a week. Her grandmother does not know many details of Brianna s history because she has only been the child s guardian since her mother died two years ago. Brianna has been on daily PEG and painfully passes at most one formed bowel movement a week. Her grandmother has tried punishment, but the situation has not improved. She believes Brianna soils herself because she forgets to go the bathroom or is too lazy to go. Her grandmother is interested in instituting therapy at this time; however, she cannot bring Brianna in for additional visits due to transportation concerns. You review Brianna s chart and find a consult note dated four years ago from pediatric gastroenterology. After a thorough work-up, the diagnosis was constipation-associated 12
functional fecal incontinence. The plan was an initial disimpaction phase but Brianna did not return for follow-up. Brianna s physical examination was normal. You notice soft stool on her underpants. Question 8: What are the key points to convey to Brianna and her grandmother when explaining encopresis and its treatment? 13
Question 8: What are the key points to convey to Brianna and her grandmother when explaining encopresis and its treatment? Key points: Soiling is not her fault. She is unaware of the passage of stool. The rectum has become distended due to the long-standing presence of large, hard stools. This results in the loss of the sensory stimulus to defecate and the tone to propel a stool into the anus. Functional fecal incontinence is a problem that builds over many years and takes a long time to resolve. At least 6 months of keeping the rectum empty are required for normal rectal sensation and tone to return. Family tensions, emotional stressors, and lack of consistency in the home routine may have contributed to the problem. Advocate non-punitive approaches to soiling accidents and the use of positive reinforcement for successes. Reassure the child and caregiver that, with appropriate treatment and family support, functional fecal incontinence can be cured. Question 9: What is your treatment plan? 14
Question 9: What is your treatment plan? 1. Disimpaction: Plan to occur over the weekend or when not at school Phone follow-up during disimpaction for encouragement, instructions, questions Follow-up office visit to check that disimpaction is complete and to discuss initiation of maintenance therapy 2. Maintenance Medication: o Take 15 capfuls of Polyethylene Glycol and add it to 64 oz of a non-red Gatorade or other sports drink. Mix. Have child drink this on a Saturday over 4 hours. o Take a chewable laxative (e.g., senna or ex-lax) right before and right after the 4 hour period. Take maintenance dose of PEG. The dose should be adjusted every 3 days, until she is having one or two soft stools a day, with a pudding-like consistency. Goal is one soft BM per day. Establishment of a daily bathroom schedule and a reward system for following that schedule (although there are no trial data to support this). Establishment of a schedule for follow-up office visits or phone follow-ups to discuss relapses, problems. 3. Assist with transportation: (Public health in action!) This grandmother s report of difficulty with transportation as a major barrier to medical treatment reminds us of the importance of addressing the social determinants of health, a key public health concept. Look for transportation resources in your area. In Allegheny County, Pennsylvania, you can refer Brianna s grandmother to the Medical Assistance Transportation Program (MATP), if eligible. MATP in Pennsylvania provides transportation to and from MA-billable, non-emergency medical services. MATP Eligibility Must have a valid Pennsylvania Medical Assistance (MA) Card and be a resident of Allegheny County. Medical Assistance eligibility must be verified according to Pennsylvania Department of Human Services guidelines. MATP paperwork must be signed and returned before transportation services can begin. For forms and information, go to: http://www.alleghenycounty.us/dhs/matp.aspx 15
Take Home Points: 1) Most cases of organic constipation can be ruled out by doing a thorough history and physical examination. 2) Components of Treatment for Functional Constipation are: a. Education b. Disimpaction c. Maintenance Therapy 16
REFERENCES 1. Baker S, Liptak G, Colletti R, Croffie J, DiLorenzo C, Ector W, et al. Constipation in Infants and Children: Evaluation and Treatment. J Pediatr Gastroenterol Nutr 1999;29(5):612-626. 2. Boccia G, Manguso F, Coccorullo P, Masi P, Pensabene L, Staiano A. Functional defecation disorders in children: PACCT criteria versus Rome II criteria. J Pediatr 2007;151(4):394-398, 398 e391. 3. Evaluation and treatment of constipation in infants and children: Evidence Based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr Sep 2014;58:258-274. 4. Padhye Phatak U and Pashankar DS. Role of Polyethylene Glycol in Childhood Constipation. Clinical Pediatrics 2014. 5. Pall H, Zacur GM, Kramer RE, et al. Bowel Preparation for Pediatric Colonoscopy: Report of the NASPGHAN Endoscopy and Procedures Committee. J Pediatr Gastroenterol Nutr 2014. 6. Pashankar DS, Loening-Baucke V and Bishop WP. Safety of Polyethylene Glycol 3350 for the Treatment of Chronic Constipation in Children. Arch Pediatr Adolesc Med 2003. 7. Pasbankar DS, Bishop WP. Efficacy and optimal dose of daily polyethylene glycol 3350 for treatment of constipation and encopresis in children. J Pediatr 2001; 139(3):428-432. 8. Torres M, McGregor T, Wilder L. What is the most effective way for relieving constipation in children aged > 1 year? J Fam Prac 2004;53(9):744-746. 9. Youssef N, DiLorenzo C. Childhood Constipation: Evaluation and Treatment. J Clin Gasteroenterol 2001;33(3):199-205. 17