Constipation in Infants and Children Jason Dranove, MD Levine Children’s Hospital Carolinas Medical Center Division of Pediatric Gastroenterology, Hepatology, and Nutrition 2011

Newborns First meconium stool usually within the first 36 hours of birth in normal newborns 90% pass stool within 24 hours This may happen later in preterm infants without underlying structural defects First week of life normal newborn has 4 stools per day, with some variability Breastfed infants can stool with each feeding or only once every 7 to 10 days Formula fed infants tend to stool more regularly than breastfed infants Soy formulas known to cause harder stools Protein Hydrolysate and Elemental formulas associated with looser stools

Delayed passage of meconium Intestinal Obstruction / Anatomical Malformation Hirschsprung’s Disease Meconium Ileus Functional Ileus Small left colon Maternal Drugs Hypothyroidism

Normal Frequency of Bowel Movements The gradual decrease in bowel movement frequency with advancing age correlates with shorter transit time and varying patterns of colonic motility. The mean total gastrointestinal transit time is 8.5 hours at 1 to 3 months of age, 16 hours at 4 to 24 months, 26 hours at 3 to 13 years of age, and 30 to 48 hours after puberty [4]. Evaluation and Treatment of Constipation in Infants and Children: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology & Nutrition. 43(3):e1-e13, September 2006.

Constipation in Infants and Toddlers At least two of the following present for at least one month Two or fewer defecations per week At least one episode of incontinence after the acquisition of toileting skills History of excessive stool retention History of painful or hard bowel movements Presence of a large fecal mass in the rectum History of large-diameter stools that may obstruct the toilet Constipation is generally defined as a delay or difficulty in passage of stool This is very subjective, so over the years investigators have tried to make a more objective criteria so that constipation is not over or under diagnosed.

Infantile Dyschezia In children less than 6 months old At least 10 minutes of straining and crying Successful passage of a soft stool Otherwise healthy and thriving Resolves spontaneously No interventioni required Reassurance is key Laxatives and osmotic agents can lead to more gas

Constipation in Children 4-18 YO Children with developmental age of at least 4 to 18 years Two of the following present for at least two months Two or fewer defecations per week At least one episode of fecal incontinence per week History of retentive posturing or excessive volitional stool retention History of painful or hard bowel movements Presence of a large fecal mass in the rectum History of large-diameter stools that may obstruct the toilet

Nonretentive Fecal Incontinence Must include all of the following in a child with a developmental age at least 4 years Defecation into places inappropriate to the social context at least once per month No evidence of an inflammatory, anatomic, metabolic, or neoplastic process that explains the subject’s symptoms No evidence of fecal retention

Functional vs. Organic -- Functional Over 95% of Constipated children has functional constipation Functional: persistent, difficult, infrequent, or seemingly incomplete defecation without evidence of underlying structural or metabolic defect Most commonly due to with-holding after a painful bowel movement Presents most commonly at three age periods At introduction of cereals and solid foods At toilet training At the start of school

Functional Constipation Classic History Child has a painful bowel movement When urge to have a bowel movement happens, the child consciously withholds stool by contracting their external anal sphincter and gluteal muscles The child might rise on their toes, rock back and forth, stiffen their buttocks and legs, assume unusual postures, and often will hide in a corner Eventually, the rectum habituates to the stimulus of the enlarging fecal mass, the urge to defecate subsides, and the retentive behavior becomes almost second nature or subconscious Can develop soiling (encopresis)

Functional vs. Organic -- Organic Accounts for less than 5% of all constipation Anatomic malformations Metabolic causes Neuropathic conditions Intestinal nerve and muscle disorders Drugs Hypotonia Miscellaneous

Distinguishing Functional vs. Organic -- History Presentation in neonatal period more likely to be organic as compared to older children Clues from history (red flags) Delayed growth Delayed passage of meconium Urinary incontinence or bladder disease Passage of blood (unless attributable to an anal fissure) Constipation from birth or very early infancy Acute onset of constipation Vomiting Signs of systemic illness, multisystem involvement Recurrent respiratory infections History of sexual, physical, or emotional abuse

Functional vs. Organic – Physical exam clues Abdominal distension Findings of spinal dysraphism Patulous anus Absent cremasteric reflex (boys) Absent anal wink Pigmentation, dimples, or tufts of hair over lumbosacral region Anorectal malformation Anteriorly displaced anus Sensory or motor defects of the lower extremities Inability to insert a pinky in the anal canal Gush of stool after a rectal exam upon which no stool is felt in the rectal vault

Clues to Hirschsprung’s disease Aganglionic bowel extending for variable lengths from the internal anal sphincter 75-80% confined to rectosigmoid Incidence about 1:5000 Male to female 4:1 Almost exclusively a disease of full term infants 80-90% diagnosed within first 3 years Mean age of diagnosis is 2.6 months

Barium enema for Hirschsprung’s Transition zone

Other Metabolic Causes of Constipation Celiac disease Hypothyroidism Spinal dysraphism Cystic Fibrosis Botulism Hypokalemia / Hypercalcemia Lead poisoning

Evaluation of Constipation Evaluation and Treatment of Constipation in Infants and Children: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology & Nutrition. 43(3):e1-e13, September 2006.

Overall approach to management Determine whether fecal impaction present Treat impaction if present Initiate treatment with oral medications Provide parental education Close follow up Adjust medications as necessary

Management and Treatment of Constipation -- > 1 YO Red Flags Present Yes – Evaluate further No – Presume and treat functional constipation Is a fecal impaction present (mass of stool felt on abdominal exam or large rectal fecal mass on DRE) If not comfortable performing rectal exam – KUB can help Yes – Disimpact with oral or rectal medication, then move to maintenance medication If fail disimpaction, refer to Pediatric GI No – Start maintenance treatment with with education, diet, medication Continued …..

Management and treatment of Constipation > 1 YO Maintenance therapy Effective – Attempt to wean after several months of success Ineffective – Reassess, re-educate, increase or change medications If effective on second try – go into maintenance phase and attempt to wean again in future If ineffective on second trial of treatment T4, TSH, Calcium, Celiac panel (total IgA and anti ttG IgA antibody), lead level If abnormal, treat as indicated If tests normal, refer to pediatric GI

Pediatric GI After referral is made Determine appropriate workup Vast majority of referral patients can be managed without much diagnostics When refractory to treatment, consider further workup Anorectal manometry Barium enema Spine MRI Radio-opaque marker studies Full thickness rectal biopsy Colonic manometry

Management and treatment of Constipation -- < 1 YO Similar to that of Children > 1 YO Differences If delayed passage of meconium Refer to Pediatric GI or Surgery for rectal biopsy If rectal biopsy normal – sweat test 1st line treatment can be diet alone Prune or apple juice, 2-4 ounces a day

Education Family friendly explanation of constipation Reassure that this is not a willful or defiant behavior Maintain consistent, positive, supportive attitude Avoid punishment Establish a reward system

Toilet Hygiene Twice a day for 10-15 minutes after breakfast and dinner Gastrocolic reflex Sit up straight Thighs parallel to ground Good foot support Valsalva maneuver to increase abd pressure Blow up balloon No distractions Reasonable reward system

Disimpaction Impaction Disimpaction a hard mass in the lower abdomen identified during physical examination, or A dilated rectum filled with a large amount of stool on rectal exam, or Excessive stool in the colon identified by radiography Disimpaction Oral Rectal Oral and Rectal Best determined after discussion with family Manual

Disimpaction LCH approach High dose Polyethylene Glycol (Miralax) Age 1-2 2 teaspoons of Miralax with 4 oz of clear liquid and drink repeat every hour until stool is clear Age 3-5 4 capfuls of Miralax in 24 ounces of Gatorade given 4 oz q 30 – 60 minutes until gone Age 6-11 6 capfuls of Miralax in 32 oz of Gatorade given 4 oz q 30-60 minutes until gone Age 12 and older 8 capfuls of Miralax in 32 oz of Gatorade given 4 oz q 30-60 minutes until gone Stimulant laxative Age 3-11 Bisacodyl 5 mg at beginning and end of cleanout Age 12 and up Bisacodyl 10 mg at beginning and end of cleanout

Enema Fleets Phosphosoda enema Retention of enema < 2 YO not recommended 2 – 4 YO = 33.75 ml (1/2 of a Pediatric Fleets enema <Pedia Lax>) 5-11 YO = 67.5 ml (full Pediatric Fleets enema <Pedia-Lax>) 12 YO and up – 118 ml (adult Fleet enema) Retention of enema Hyperphosphatemia Hypocalcemia Never give more than one enema per day If enema not evacuated, do not give a second enema

Goals of treatment 1 to 2 soft (mashed potato or soft ice cream) consistency stools per day Resolution of soiling Return of rectal sensation Empowerment of child Make defecation a positive experience

Non absorbed sugars Juices OK to use after roughly 2 mo of age Prune Apple White grape juice Increase osmotic load and draw water into bowel OK to use after roughly 2 mo of age

Lactulose Second line in infants < 6 mo not responding to juice Limited role in those over 6 mo secondary to success of PEG 3350 Comes 10 g / 15 ml Dose = 1-3 ml/kg/day in single or divided doses Usually start ½ to 1 teaspoon a day and increase as needed Side effects Cramps, flatulence, “colicky” behavior

PEG 3350 Safe for use down to 6 months of age Comes 17 grams in a cap Roughly 4 teaspoons is in one cap (1 teaspoon = roughly 4 to 5 grams of PEG 3350 Easier to dose by teaspoon in infants Typical dose for maintenance is roughly 0.7 g/kg/day In older children typically start at max of 17 grams twice a day but can increase if needed Technically no max dose If not responding to 34 grams a day in older child or roughly 1 g/kg/day in younger child, consider adding a stimulant laxative, re-education, or referral

Sample Treatment regimen for older child (non infant) Start Miralax at discussed doses Increase or decrease dose by small amounts until desired effect is reached Follow up within 1 month Aggressive Approach After 8 weeks of soft daily bowel movements, begin to taper by small amounts every couple of weeks (1/4 of dose at a time is a good guide) until BM achieved without laxative If stools become hard again during taper, increase to the last effective dose and maintain for another 8 weeks Conservative approach Continue laxatives for 6 months of soft daily bowel movements, then wean slowly

Stimulants Senna Bisacodyl Comes 8.8 mg/5ml or 8.8 mg tabs 2-6 YO – 2.5 to 7.5 ml a day 6-12 YO – 5-15 ml a day Try to limit to periodic dosing With regular use drug can lose effectiveness Anecdotal evidence Bisacodyl 0.2 mg/kg/dose, max 10 mg per dose Comes in 5 and 10 mg tabs Use intermittently or for short periods Has very high side effect profile Cramping, diarrhea, abdominal pain, nausea

Others Mineral oil Magnesium citrate Magnesium hydroxide Karo Syrup Do not use for < 1 YO due to aspiration risk Magnesium citrate Magnesium hydroxide Karo Syrup Suppositories

Diet Controversial whether dietary changes can treat constipation Mild constipation Increase fluid intake Increase fiber intake Goal is age + 5 in grams per day Mild to severe constipation Diet alone unlikely to treat constipation Role of excessive cow’s milk intake controversial

Inpatient bowel cleanout Polyethylene Glycol Electrolyte solution 100 ml / kg up to max of 4 liters Likely will need Nasogastric administration Can run at rates up to 1 L per hour Personal experience Run in over 8 hours If stool not clear may repeat If severe impaction not responding Consult Pediatric Gastroenterology Manual Disimpaction under anesthesia

Treatments Not recommended Milk and Molasses enema Soap suds enema Tap water enema Oral Phosphosoda Nephropathy FDA warning

Non retentive encopresis Soiling in the absence of fecal impaction or constipation Cause unknown High correlation with attention deficit and psychological comorbidities Up to 40% were never fully toilet trained Treatment Unfortunately limited Psychologist Regimented toileting schedule

Colonic Manometry

Colonic Manometery

Anorectal Manometry

Anorectal Manometry

Case 1 3 YO male with infrequent, hard bowel movements. Stools can clog the toilet. He has a normal physical and is thriving? --What is the diagnosis --Is any workup indicated --What is the treatment?

Case 2 18 month old female Constipation, abdominal distension, poor growth, frequent wheezing and chronic cough Mom can not remember if she passed meconium within 1st day of life What is the differential diagnosis? Would you do any workup?

Case 3 3 month old male, full term infant Abdominal distension, poor growth, has developed vomiting Rectal exam – can not get pinky into anal canal Differential diagnosis? Workup?

Case 4 3 YO female. Was doing well until about 9 months of age, then started to fall off growth curve. Has distended abdomen, extremity wasting, no history of respiratory infection. Differential diagnosis Workup?

Case 5 2 YO female with constipation since birth Did pass meconium on day of life 1 No abdominal distension, normal growth Physical exam reveals a pit over the lumbosacral area with hair covering it? Differential diagnosis Workup

Question 1 What is the appropriate screening test for celiac disease A. Anti gliadin antibody B. HLA DQ2-DQ8 genotype C. Ttg IgG and serum IgA D. Ttg IgA and serum IgA E. TgG Iga alone

Question 2 Infantile dyschezia is straining with passage of soft bowel movements in babies up to _____ months old. A. 3 B. 6 C. 9 D. 12 E. 15

Question 3 The presence of meconium ileus is almost pathognomonic for A. Hirschsprung’s disease B. Infant of diabetic mother C. Ileal atresia D. Cystic Fibrosis E. Hypothyroidism

Question 4 Which of the following home remedies is strongly discouraged for fecal disimpaction A. Milk and Molasses enema B. Soap Suds enema C. Tap Water enema D. All of the above E. None of the above

Question 5 The following tests should be routinely performed in all constipated children A. TSH B. Sweat test C. Basic metabolic panel D. All of the above E. None of the above