Childhood constipation Childhood constipation DR/Medhat Mohamed Ibrahim DR/Medhat Mohamed Ibrahim

EpidemiologyEpidemiology

Prevalence of functional constipation in children Prevalence of functional constipation in children ranges from 4-36%.ranges from 4-36%. Vnder wal MF, B. 2005 Vnder wal MF, B. 2005

34% of toddlers in the United Kingdom and 37% of 34% of toddlers in the United Kingdom and 37% of Brazilian children younger than 12 years of age Brazilian children younger than 12 years of age were considered by their parents to be constipated.were considered by their parents to be constipated.

Encopresis is three to six times more common Encopresis is three to six times more common among males than females and is acknowledged in among males than females and is acknowledged in 3% of 4-year-olds and 1.6% of 10-year-olds. 3% of 4-year-olds and 1.6% of 10-year-olds.

Cont.,Cont.,

A positive family history has been found in 28-50% A positive family history has been found in 28-50% of constipated children and a higher incidence of constipated children and a higher incidence reported in monozygotic than dizygotic twins .reported in monozygotic than dizygotic twins .

Constipation tends to be equal in both sexes below Constipation tends to be equal in both sexes below 5 years, commoner in girls above 13 years of age.5 years, commoner in girls above 13 years of age.

peak incidence is at the time of toilet training peak incidence is at the time of toilet training around 2-3 years of age. around 2-3 years of age.

Cont.,Cont.,

In the hospital setting, pediatric constipation In the hospital setting, pediatric constipation forms 3% of all referrals to pediatric clinic and forms 3% of all referrals to pediatric clinic and up to 25% to pediatric gastroenterologists up to 25% to pediatric gastroenterologists clinic.clinic.

American study suggests that there is a cost of American study suggests that there is a cost of health resources for children with constipation, health resources for children with constipation, estimated at $3.9 billion/year, 2011.estimated at $3.9 billion/year, 2011.

Objective Objective

This presentation aims to be a practical guide This presentation aims to be a practical guide for physicians, to outline the current :for physicians, to outline the current :

diagnostic criteria . diagnostic criteria . provide an evidence-base for the provide an evidence-base for the

medical management of constipation in medical management of constipation in children. children.

(In the light of recent ,National Institute of (In the light of recent ,National Institute of Clinical Excellence (NICE) guidelines on Clinical Excellence (NICE) guidelines on constipationconstipation.).)

Definition (Definition (According to ROME According to ROME шшfor for Evidence Based Medicine & Research Evidence Based Medicine & Research

practice .practice .Rasquin A,2006 Rasquin A,2006 ))

ConstipationConstipation generally is defined by the generally is defined by the HARD nature of the stoolHARD nature of the stool

PAIN associated with its passage . PAIN associated with its passage . 33%33% Painful or hard bowel movementPainful or hard bowel movement Failure to pass ONE stools / week. (Iowa criteria of Failure to pass ONE stools / week. (Iowa criteria of

constipation three/week) . constipation three/week) . 58%58% History of large diameter stool History of large diameter stool Presence of a large fecal mass in the rectum(palpable).Presence of a large fecal mass in the rectum(palpable).

History of History of retentive posturing retentive posturing or excessive or excessive volitional stool retentionvolitional stool retention

Definition ,Definition ,

would be preferable to define constipation as would be preferable to define constipation as the failure to evacuate the lower colon the failure to evacuate the lower colon completely with a normal bowel movement. completely with a normal bowel movement.

EncopresisEncopresis

EncopresisEncopresis or fecal soiling usually is the result of or fecal soiling usually is the result of looser stool leaking or overflowing from a rectum looser stool leaking or overflowing from a rectum

that has been distended by retained stool. that has been distended by retained stool. 90%90% IncontinenceIncontinence: non-retentive fecal soiling has been : non-retentive fecal soiling has been

described for children soiling without difficult described for children soiling without difficult infrequent defecation. PACCT (Paris consensus on infrequent defecation. PACCT (Paris consensus on childhood constipation terminology group) have childhood constipation terminology group) have defined this as passage of stools in an inappropriate defined this as passage of stools in an inappropriate place, occurring in children with a mental age of 4 place, occurring in children with a mental age of 4 years and older, with no evidence of constipation on years and older, with no evidence of constipation on history or examinationhistory or examination

What is normal ?What is normal ?

The The frequency of stoolsfrequency of stools in most children in most children decreases from a mean of four per day in the decreases from a mean of four per day in the first week of life to 1.7 per day by the age of 2 first week of life to 1.7 per day by the age of 2 years.years.

Over this interval, Over this interval, stool volume stool volume increases increases more than tenfold while maintaining a more than tenfold while maintaining a consistent water content of approximately 75%.consistent water content of approximately 75%.

Intestinal transit Intestinal transit time from mouth to rectum time from mouth to rectum increases from 8 hours in the first month of life increases from 8 hours in the first month of life to 16 hours by 2 years of age to 26 hours by the to 16 hours by 2 years of age to 26 hours by the age 10.age 10.

Breast feeding

Artificial formula

How does it worksHow does it works

Normal continence is maintained by the resting Normal continence is maintained by the resting tonicity of the internal anal sphincter tonicity of the internal anal sphincter

It can be enhanced by contraction of the puborectalis It can be enhanced by contraction of the puborectalis muscle, which creates a 90-degree angle of rectum muscle, which creates a 90-degree angle of rectum to the anal canal.to the anal canal.

When more than 15 cc of stool enters the normal When more than 15 cc of stool enters the normal rectum, stretch receptors and nerves in the rectum, stretch receptors and nerves in the intramural plexus are activated.intramural plexus are activated.

Inhibitory interneurons decrease the resting tone in Inhibitory interneurons decrease the resting tone in the involuntary smooth muscle of the internal anal the involuntary smooth muscle of the internal anal sphincter.sphincter.

How does it works How does it works (cont.)(cont.)

Relaxation of the sphincter allows the Relaxation of the sphincter allows the stool to reach the external anal stool to reach the external anal sphincter and the urge to defecate is sphincter and the urge to defecate is signaled. signaled.

If the child relaxes the external anal If the child relaxes the external anal sphincter, squats to straighten the sphincter, squats to straighten the anorectal canal, and increases intra-anorectal canal, and increases intra-abdominal pressure the rectum is abdominal pressure the rectum is evacuated of stool.evacuated of stool.

Why it may not workWhy it may not work

If, however, the child tightens the external anal If, however, the child tightens the external anal sphincter and the gluteal muscles, the fecal mass is sphincter and the gluteal muscles, the fecal mass is pushed back into the rectal vault and the urge to pushed back into the rectal vault and the urge to defecate subsides.defecate subsides.

Repetitive denial of evacuation leads to stretching of Repetitive denial of evacuation leads to stretching of the rectum and eventually of the lower colon, the rectum and eventually of the lower colon, producing a reduction in muscle tone and retention of producing a reduction in muscle tone and retention of stool. stool.

The longer the stool remains in the rectum, the more The longer the stool remains in the rectum, the more water is removed, and the harder the stool becomes water is removed, and the harder the stool becomes to the point of impaction.to the point of impaction.

EncopresisEncopresis

Presents predominantly between 3 and 7 years Presents predominantly between 3 and 7 years

The child is not aware of the soiling until it is nearly The child is not aware of the soiling until it is nearly complete.complete.

Manometry shows decreased sensitivity to Manometry shows decreased sensitivity to distension. distension.

In one study 40% experienced delays in toilet In one study 40% experienced delays in toilet training, and 60% reported painful stools before the training, and 60% reported painful stools before the age of 3 years.age of 3 years.

More than 90% of chronic encopresis occurs in the More than 90% of chronic encopresis occurs in the context of functional constipation.context of functional constipation.

It may not be functional…..It may not be functional…..

Organic incontinence can occur in: Organic incontinence can occur in:

Children who have damaged corticospinal pathways Children who have damaged corticospinal pathways such as lumbosacral myelomeningocele.such as lumbosacral myelomeningocele.

Anorectal dysfunction after operative pullthrough Anorectal dysfunction after operative pullthrough surgery for high imperforate anus or colectomy.surgery for high imperforate anus or colectomy.

Prolonged diarrhea (pelvic floor muscles fatigue)Prolonged diarrhea (pelvic floor muscles fatigue)

Psychological counseling may be equally valuable in Psychological counseling may be equally valuable in all forms of encopresis because the stress of soiling is all forms of encopresis because the stress of soiling is independent of etiology.independent of etiology.

Functional constipation backgroundFunctional constipation background

Straining with the passage of soft stool is normal in Straining with the passage of soft stool is normal in neonates and infants. (first few days of live)neonates and infants. (first few days of live)

It is related to their inability to coordinate pelvic floor It is related to their inability to coordinate pelvic floor relaxation with the Valsalva maneuver and to straighten relaxation with the Valsalva maneuver and to straighten the anorectal canal when lying down. the anorectal canal when lying down.

Once a week may be still normal in infantsOnce a week may be still normal in infants

Insertion of a glycerin suppository or thermometer will Insertion of a glycerin suppository or thermometer will induce reflex anal sphincter relaxation and the desired induce reflex anal sphincter relaxation and the desired evacuation, but unplanned rectal manipulation is evacuation, but unplanned rectal manipulation is discouraged. discouraged.

If the stools become firm balls or rectal fissures evolve, If the stools become firm balls or rectal fissures evolve, softening of the stool is indicated.softening of the stool is indicated.

Functional constipation Functional constipation backgroundbackground

Functional fecal retention is the most Functional fecal retention is the most common explanation for childhood common explanation for childhood constipation. constipation.

Generally, the passage of a large soft stool at Generally, the passage of a large soft stool at intervals of less than 72 hours in infants is not intervals of less than 72 hours in infants is not associated with rectal fecal retention.associated with rectal fecal retention.

Why they may do itWhy they may do it

““control” issue with unsuccessful toilet trainingcontrol” issue with unsuccessful toilet training

the logical response to painful stools (anal the logical response to painful stools (anal inflammation from fissures, perianal streptococcal inflammation from fissures, perianal streptococcal infection, perianal abscess)infection, perianal abscess)

threatening event such as a television show, birth of threatening event such as a television show, birth of a siblinga sibling

desire to avoid defecation in a strange toilet when desire to avoid defecation in a strange toilet when away from home.away from home.

Some toddlers and older children are too distracted Some toddlers and older children are too distracted to evacuate .to evacuate .

And the resultAnd the result

Enormous stools to the point of being “toilet-plugging-Enormous stools to the point of being “toilet-plugging-specials”specials”

Significant pain and a prepassage ritual of gluteal tightening Significant pain and a prepassage ritual of gluteal tightening and posturingand posturing

Early satiety, small meals all day, irritability, and Early satiety, small meals all day, irritability, and unpredictable spasms of abdominal pain usually located in unpredictable spasms of abdominal pain usually located in the lower abdomen. the lower abdomen.

Encopresis becomes increasingly frequent. Encopresis becomes increasingly frequent.

Painless rectal bleeding after defecation. Painless rectal bleeding after defecation.

After the passage, symptoms generally resolve for a few After the passage, symptoms generally resolve for a few days, then recur.days, then recur.

Hirschsprung diseaseHirschsprung disease

Hirschsprung disease or aganglionosis occurs in 1 in Hirschsprung disease or aganglionosis occurs in 1 in 5,000 births. 5,000 births.

Male-to-female ratio is 4 to 1, and the incidence Male-to-female ratio is 4 to 1, and the incidence increases with longer segments of disease. increases with longer segments of disease.

The diagnostic lack of ganglion cells in the myenteric The diagnostic lack of ganglion cells in the myenteric and submucosal plexus of the bowel wall extends and submucosal plexus of the bowel wall extends proximally from the internal anal sphincter. proximally from the internal anal sphincter.

In among 80% of the involved children, the In among 80% of the involved children, the aganglionic segment does not extend above the aganglionic segment does not extend above the sigmoid. sigmoid.

Hirschsprung diseaseHirschsprung disease

Hirschsprung disease is a heterogenous genetic Hirschsprung disease is a heterogenous genetic disorder with risk rates for siblings ranging from 3% disorder with risk rates for siblings ranging from 3% with short segment disease to 25% with a female with short segment disease to 25% with a female who has long segment disease.who has long segment disease.

An autosomal dominant form occurs with mutation in An autosomal dominant form occurs with mutation in the RET gene. the RET gene.

Syndromes associated with Hirschsprung disease Syndromes associated with Hirschsprung disease include trisomy 21, deletion of chromosome 13q, include trisomy 21, deletion of chromosome 13q, Smith-Lemli-Opitz, Waardenberg, Laurence-Moon-Smith-Lemli-Opitz, Waardenberg, Laurence-Moon-Biedl-Bardet, congenital deafness, and congenital Biedl-Bardet, congenital deafness, and congenital central hypoventilation.central hypoventilation.

Hirschsprung diseaseHirschsprung disease

Difficulty with evacuation is present from birth;Difficulty with evacuation is present from birth;

Meconium is not passed in the first 48 hours of life Meconium is not passed in the first 48 hours of life in 40% of involved infants.in 40% of involved infants.

Recurrent abdominal distension, emesis, failure to Recurrent abdominal distension, emesis, failure to thrive, and acute enterocolitis allow diagnosis of thrive, and acute enterocolitis allow diagnosis of 60% of patients by 3 months of age. 60% of patients by 3 months of age.

The presence of early obstructive features, onset The presence of early obstructive features, onset in infancy, and nearly complete absence of in infancy, and nearly complete absence of encopresis distinguish Hirschsprung disease from encopresis distinguish Hirschsprung disease from functional fecal retention. functional fecal retention.

Hirschsprung diseaseHirschsprung disease

On rectal examination, the aganglionic bowel On rectal examination, the aganglionic bowel is tight around the finger and the rectal is tight around the finger and the rectal ampulla is not dilated ampulla is not dilated Finger glove signFinger glove sign. .

A barium enema usually allows visualization A barium enema usually allows visualization of the transition zone between the tonically of the transition zone between the tonically contracted aganglionic segment and the contracted aganglionic segment and the dilated proximal bowel. dilated proximal bowel.

The enema should be performed without The enema should be performed without preparation, which distorts the distal preparation, which distorts the distal anatomy. anatomy.

Hirschsprung diseaseHirschsprung disease

The entire colon and some small bowel may The entire colon and some small bowel may be involved in 3%.be involved in 3%.

The aganglionic segment of bowel fails to The aganglionic segment of bowel fails to relax because of the absence of inhibitory relax because of the absence of inhibitory neurons neurons containing nitric oxide and containing nitric oxide and vasoactive intestinal peptide.vasoactive intestinal peptide.

Hirschsprung diseaseHirschsprung disease

Manometric studies of the rectum Manometric studies of the rectum demonstrate the failure of the internal anal demonstrate the failure of the internal anal sphincter to relax when distended.sphincter to relax when distended.

Confirmation of the diagnosis requires rectal Confirmation of the diagnosis requires rectal biopsy.biopsy.

The aganglionic bowel has nerve fibers The aganglionic bowel has nerve fibers staining for acetylcholinesterasestaining for acetylcholinesterase

Neuronal dysplasiaNeuronal dysplasia

Neuronal dysplasia, in contrast to aganglionic Neuronal dysplasia, in contrast to aganglionic disease, is associated with increased disease, is associated with increased numbers of ganglion cells (hyperganglionosis) numbers of ganglion cells (hyperganglionosis) in the lower colon. in the lower colon.

It may present throughout childhood with It may present throughout childhood with variable constipation or features of pseudo-variable constipation or features of pseudo-obstruction.obstruction.

It is more frequent among children who have It is more frequent among children who have neurofibromatosis and has been associated neurofibromatosis and has been associated with MEN type IIb due to glioneuromas of the with MEN type IIb due to glioneuromas of the intestinal tract. intestinal tract.

Neuronal dysplasia Neuronal dysplasia (cont.)(cont.)

Surgical intervention is individualized and Surgical intervention is individualized and based on the severity of symptoms and based on the severity of symptoms and manometric demonstration of severely manometric demonstration of severely impaired rectal relaxation.impaired rectal relaxation.

Reduced numbers of ganglion cells Reduced numbers of ganglion cells (hypoganglionosis) usually is an acquired (hypoganglionosis) usually is an acquired disease of ganglion cell destruction seen in disease of ganglion cell destruction seen in Chagas disease or paraneoplastic syndrome. Chagas disease or paraneoplastic syndrome.

ObstructiveObstructive

Congenital Congenital Anterior ectopic anus is Anterior ectopic anus is

defined by a measurable defined by a measurable displacement of the anal displacement of the anal opening based on the opening based on the ratio of the anus-to-ratio of the anus-to-fourchette to the coccyx-fourchette to the coccyx-to-fourchette being less to-fourchette being less than 0.34 in females and than 0.34 in females and less than 0.46 in males.less than 0.46 in males.

Anal opening

Anal sphincter

urethra

No vaginal opening

CONT.,CONT.,• the shift creates a the shift creates a

broad posterior rectal broad posterior rectal shelf that increases shelf that increases with distension of the with distension of the ampulla.ampulla.

• Surgical correction Surgical correction occasionally is occasionally is required, but most required, but most patients do well if patients do well if distension of the distension of the ampulla is minimized.ampulla is minimized.

Obstructive (cont.)Obstructive (cont.)

Congenital (cont.)Congenital (cont.) Anal ring stenosis presents with painful Anal ring stenosis presents with painful

constipation in infancy. constipation in infancy. A narrow ring or band is noted on rectal A narrow ring or band is noted on rectal

examination with a dilated ampulla above it. examination with a dilated ampulla above it. The stenosis usually responds to gradual The stenosis usually responds to gradual

dilatationdilatation

Post-operative Post-operative

One week post-operative45 days post operative

Endocrine/metabolicEndocrine/metabolic

The term “pseudo-obstruction syndrome” has been The term “pseudo-obstruction syndrome” has been applied to all non anatomic disorders of abnormal applied to all non anatomic disorders of abnormal peristalsis. peristalsis.

Motility abnormalities may manifest with delayed Motility abnormalities may manifest with delayed gastric emptying, small bowel stasis, and/or gastric emptying, small bowel stasis, and/or constipation. constipation.

The primary form is familial, presenting in infancy The primary form is familial, presenting in infancy with FTT, distension, and progressive dysmotility with with FTT, distension, and progressive dysmotility with delayed gastric emptying and constipation.delayed gastric emptying and constipation.

Endocrine/metabolic Endocrine/metabolic (cont.)(cont.)

The secondary or acquired forms of pseudo-The secondary or acquired forms of pseudo-obstruction encompass a wide variety of obstruction encompass a wide variety of neurologic, muscular, pharmacologic, and neurologic, muscular, pharmacologic, and metabolic disorders.metabolic disorders.

Complications of Complications of constipationconstipation

Abdominal or rectal pain and encopresisAbdominal or rectal pain and encopresis

Enuresis is acknowledged in more than 40% of Enuresis is acknowledged in more than 40% of children who have encopresischildren who have encopresis

In some, the enuresis resolves when the pelvic mass In some, the enuresis resolves when the pelvic mass of retained rectal stools evacuated. of retained rectal stools evacuated.

Increased frequency of urinary tract infection and Increased frequency of urinary tract infection and potential obstruction of the left ureter.potential obstruction of the left ureter.

The dilated lower colon may lose enough tone to The dilated lower colon may lose enough tone to allow internal prolapse or intussusception.allow internal prolapse or intussusception.

Complications of Complications of constipationconstipation

Chronic low-grade internal prolapse creates an Chronic low-grade internal prolapse creates an ischemic ulcer of the rectal wall (solitary rectal ulcer ischemic ulcer of the rectal wall (solitary rectal ulcer syndrome).syndrome).

The diffuse irritation of the colon caused by firm stool The diffuse irritation of the colon caused by firm stool even may lead to protein-losing enteropathy.even may lead to protein-losing enteropathy.

The social stigma associated with encopresis can be The social stigma associated with encopresis can be very debilitating to any child. very debilitating to any child.

Diagnostic approachDiagnostic approach HistoryHistory

birth history of gestational complications, birth history of gestational complications, birthweight, timing of passage of meconium, and birthweight, timing of passage of meconium, and tolerance of early feedings.tolerance of early feedings.

introduction of cow milk is the most constipating introduction of cow milk is the most constipating component of the young child’s diet.component of the young child’s diet.

Transitions to child care, all-day school, diaper to Transitions to child care, all-day school, diaper to toilet trainingtoilet training

Family history is reviewed for evidence of genetic Family history is reviewed for evidence of genetic factors, as aganglionosis, cystic fibrosis, factors, as aganglionosis, cystic fibrosis, hypothyroidism, neurofibromatosis, or myopathieshypothyroidism, neurofibromatosis, or myopathies

History (cont.)History (cont.)

The character of the stools is reviewed from The character of the stools is reviewed from birth, especially for the first 24 hours, for birth, especially for the first 24 hours, for consistency, caliber, volume, and frequency.consistency, caliber, volume, and frequency.

The age and circumstances at onset of The age and circumstances at onset of encopresis should be documented.encopresis should be documented.

Encopresis in the absence of constipation Encopresis in the absence of constipation suggests an organic or behavioral origin. suggests an organic or behavioral origin.

A history of possible sexual or rectal abuse A history of possible sexual or rectal abuse should be elicitedshould be elicited

History (cont.)History (cont.)

Of prior surgeryOf prior surgery

neonatal complications (NEC)neonatal complications (NEC)

courses of medications that may contribute to courses of medications that may contribute to constipationconstipation

Physical examinationPhysical examination

Documentation of growth and weight gainDocumentation of growth and weight gain

Signs of systemic diseases include a thorough Signs of systemic diseases include a thorough neurologic evaluation.neurologic evaluation.

The abdomen is examined for degree of The abdomen is examined for degree of distension Bowel sounds are documented, distension Bowel sounds are documented,

perineum is inspected for evidence of perineum is inspected for evidence of encopresis, streptococcal or monilial encopresis, streptococcal or monilial infection, fissures, and trauma (abuse) infection, fissures, and trauma (abuse)

The anal opening is observed, watch for The anal opening is observed, watch for perirectal manifestations of Crohn‘s diseaseperirectal manifestations of Crohn‘s disease

Physical examination Physical examination (cont.)(cont.)

A dilated ampulla filled with retained firm A dilated ampulla filled with retained firm stool is a feature of functional retention.stool is a feature of functional retention.

The abdominal examination may The abdominal examination may demonstrate palpable dilated loops of demonstrate palpable dilated loops of sigmoid and distal colon. sigmoid and distal colon.

The back should be examined for sacral skin The back should be examined for sacral skin clues to lower spine deformity.clues to lower spine deformity.

Tendon reflexes should also be assessed to Tendon reflexes should also be assessed to rule out neurological problem. rule out neurological problem.

Laboratory Laboratory investigationsinvestigations

Thyroid functionsThyroid functions

Serum calcium, electrolyte levels, magnesium Serum calcium, electrolyte levels, magnesium and urea nitrogen. and urea nitrogen.

Urinalysis and urine culture Urinalysis and urine culture

The plain abdominal radiograph may be of The plain abdominal radiograph may be of value in the child in whom an abdominal value in the child in whom an abdominal examination is difficult or to monitor examination is difficult or to monitor compliance.compliance.

Lumbosacral spine radiographs or magnetic Lumbosacral spine radiographs or magnetic resonance imaging if indicated. resonance imaging if indicated.

Unprepared barium enema for the transition zone Unprepared barium enema for the transition zone or strictures from necrotizing enterocolitis.or strictures from necrotizing enterocolitis.

The contrast enema defecogram has a definite The contrast enema defecogram has a definite role in assessing pelvic muscle function following role in assessing pelvic muscle function following surgery or in the context of central nervous surgery or in the context of central nervous system disease.system disease.

Anorectal manometry is available to evaluate Anorectal manometry is available to evaluate internal anal sphincter relaxation and determine internal anal sphincter relaxation and determine the level of pressure awareness in older children.the level of pressure awareness in older children.

It also will identify the 25% of chronically It also will identify the 25% of chronically constipated children who exhibit a paradoxic constipated children who exhibit a paradoxic increase in external anal sphincter pressure. increase in external anal sphincter pressure.

Red FlagsRed Flags

Laboratory Laboratory investigations (cont.)investigations (cont.)

A few centers now offer total colonic motility, a A few centers now offer total colonic motility, a valuable tool in the evaluation of neuropathic valuable tool in the evaluation of neuropathic or muscular dysmotility in chronic intestinal or muscular dysmotility in chronic intestinal pseudo-obstruction.pseudo-obstruction.

The value of the suction rectal biopsy has The value of the suction rectal biopsy has increased with the ability to stain the tissue for increased with the ability to stain the tissue for both ganglion cells and acetylcholinesterase.both ganglion cells and acetylcholinesterase.

The rectal biopsy also can be of diagnostic The rectal biopsy also can be of diagnostic value in the child who has amyloidosis, graft value in the child who has amyloidosis, graft versus host disease, lipid storage disease, or versus host disease, lipid storage disease, or Crohn disease.Crohn disease.

InvestigationInvestigation Plain X Ray.Plain X Ray.

the Leech system ,divides the Leech system ,divides the abdominal X-Ray into the abdominal X-Ray into three sections, three sections, ascending colon and ascending colon and

proximal transverse colon; proximal transverse colon; distal transverse colon; distal transverse colon;

descending colon descending colon rectosigmoid area.rectosigmoid area.

Each segment is then Each segment is then assessed for the presence assessed for the presence of stools (score 0-5where of stools (score 0-5where 0 indicates no stool; 5 0 indicates no stool; 5 means gross fecal loading means gross fecal loading with bowel dilation).with bowel dilation).

Presence of firm, packed hard stool in the rectum cor-relates closely with radiological evidence of faecal reten-tion, with sensitivity and positive predictive valuesexceeding 90%

Management of chronic Management of chronic constipation and encopresis constipation and encopresis

The three phases of management:The three phases of management:

complete evacuation or disimpactioncomplete evacuation or disimpaction sustained evacuation to restore normal sustained evacuation to restore normal

colorectal tonecolorectal tone weaning from intervention. weaning from intervention.

The success of each depends on the The success of each depends on the cooperation and understanding of the parent cooperation and understanding of the parent and, when possible, the child.and, when possible, the child.

Management of chronic constipation and Management of chronic constipation and encopresis encopresis

The older child is encouraged to be involved The older child is encouraged to be involved by keeping a chart or calendar to document by keeping a chart or calendar to document efforts, successes, and failures. efforts, successes, and failures.

They are encouraged to establish a “habit” of They are encouraged to establish a “habit” of toilet use independent of the rest of toilet use independent of the rest of treatment. treatment.

Use a foot stool for the child to maximize Use a foot stool for the child to maximize abdominal pressure during the Valsalva abdominal pressure during the Valsalva maneuver.maneuver.

Diet in the Diet in the managementmanagement

The family is counseled to reduce The family is counseled to reduce constipating foods .constipating foods .

Additional fiber has no value when colorectal Additional fiber has no value when colorectal tone is diminished in the child who has active tone is diminished in the child who has active functional fecal retention. functional fecal retention.

In the second phase, when tone is being In the second phase, when tone is being restored, additional fiber is of great value to restored, additional fiber is of great value to improve the “efficiency” of evacuationimprove the “efficiency” of evacuation

Once remission is achieved, dietary fiber is Once remission is achieved, dietary fiber is maximized.maximized.

Phase 1: completePhase 1: completeevacuation or disimpactionevacuation or disimpaction

No management plan will succeed if complete No management plan will succeed if complete evacuation is not achieved initially. evacuation is not achieved initially.

Occasionally manual disimpaction is required, but the Occasionally manual disimpaction is required, but the general rule is to minimize rectal manipulation.general rule is to minimize rectal manipulation.

If the rectal examination reveals a firm, wide If the rectal examination reveals a firm, wide impaction of stool, enemas will be required for up to impaction of stool, enemas will be required for up to 2 to 5 days2 to 5 days

Use beyond 3 to 5 days is discouraged because of the Use beyond 3 to 5 days is discouraged because of the potential for electrolyte disturbance. potential for electrolyte disturbance.

Tap water and herbal enemas should be avoidedTap water and herbal enemas should be avoided

Phase 1 (cont.)Phase 1 (cont.)

A preferred management is the use of A preferred management is the use of Bisacodyl tablets given in 3 consequent Bisacodyl tablets given in 3 consequent evenings.evenings.

If the child fails to evacuate with the use of If the child fails to evacuate with the use of mineral oil and phosphate enemas, a hyper-mineral oil and phosphate enemas, a hyper-osmolar enema using a water-soluble osmolar enema using a water-soluble contrast media can be given.contrast media can be given.

Disimpaction occasionally is achieved with Disimpaction occasionally is achieved with oral high-dose mineral oil for 3 days.oral high-dose mineral oil for 3 days.

And if it still does not And if it still does not workwork

If disimpaction is not achieved with enemas or If disimpaction is not achieved with enemas or irrigation, the child must be admitted to the irrigation, the child must be admitted to the hospital for oral lavage with hospital for oral lavage with polyethyleneglycol-electrolyte solution. polyethyleneglycol-electrolyte solution.

Because volumes of 30 to 40 mL/kg per hour Because volumes of 30 to 40 mL/kg per hour are used, most children will require a are used, most children will require a nasogastric tube.nasogastric tube.

If it is not work colostom for 6 months.If it is not work colostom for 6 months.

Irrigation of the distal loop with normal saline . Irrigation of the distal loop with normal saline .

Phase 2:sustain complete Phase 2:sustain complete evacuation evacuation

Habitual toilet useHabitual toilet use

Stool softeners, to achieve daily complete evacuation.Stool softeners, to achieve daily complete evacuation.

This phase can last from 2 to 6 months or longer. This phase can last from 2 to 6 months or longer.

The aim is to allow the distended colon to return to normal The aim is to allow the distended colon to return to normal calibre and tone. calibre and tone.

The best approach is a combination of medical therapy, The best approach is a combination of medical therapy, behavioral modification, and counseling.behavioral modification, and counseling.

Older children who have incontinence, paradoxic failure of Older children who have incontinence, paradoxic failure of anal relaxation, or postoperative low imperforate anus are anal relaxation, or postoperative low imperforate anus are candidates for biofeedback therapy with an experienced candidates for biofeedback therapy with an experienced therapist.therapist.

The “hard” groupThe “hard” group

Toddlers are the most resistant to early Toddlers are the most resistant to early treatment. treatment. They are hard-core fecal withholdersThey are hard-core fecal withholders poorly compliant with medicationpoorly compliant with medication resistant to behavior modification. resistant to behavior modification. The parents also tend to undermedicate unless The parents also tend to undermedicate unless

they are convinced of the need for and safety of they are convinced of the need for and safety of such agents. such agents.

Toilet training should not be attempted until both Toilet training should not be attempted until both the physical and psychological components of the physical and psychological components of the constipation are under control. the constipation are under control.

Lactulose Lactulose is begun at 5 to 10 mL BID, increasing as required is begun at 5 to 10 mL BID, increasing as required up to 45 mL BID. up to 45 mL BID.

Increased gas and looser-than-desired stools occur initially. Increased gas and looser-than-desired stools occur initially.

Emulsified mineral oilEmulsified mineral oil is an alternative for toddlers and older is an alternative for toddlers and older children. It is begun at 2 mL/kg per dose BID and increased children. It is begun at 2 mL/kg per dose BID and increased as needed up to 6 to 8 oz per day. as needed up to 6 to 8 oz per day.

Transient oil leakage may occur until the right dose is Transient oil leakage may occur until the right dose is achieved.achieved.

Concerns about mineral oil interfering with absorption of fat-Concerns about mineral oil interfering with absorption of fat-soluble vitamins have not been substantiated in short-term soluble vitamins have not been substantiated in short-term studies.studies.

Phase 3: weaning from Phase 3: weaning from medicationsmedications

Once regular evacuation has been sustained, the use of Once regular evacuation has been sustained, the use of laxatives is reduced gradually. laxatives is reduced gradually.

Softening agents are continued daily. Softening agents are continued daily.

Ingestion of insoluble or soluble fiber should be increased. Ingestion of insoluble or soluble fiber should be increased.

Most fiber supplements are soluble, fermented in part by Most fiber supplements are soluble, fermented in part by fecal bacteria, which contributes to increased fecal mass. fecal bacteria, which contributes to increased fecal mass.

Doses are increased slowly to minimize gas production Doses are increased slowly to minimize gas production and distension. and distension.

Rates of recurrence approach 50%. Rates of recurrence approach 50%.