quizlet 3

• True or false: long segment disease, involving the rectum, sigmoid colon and variable length of colon, of Hirschsprung disease is the most common disease.

  False.
• Discuss the Rome IV criteria with regards to constipation in infants and toddlers up to 4 years of age. [TH3P]

  At least two of the following present for at least one month:

  two or less defecations per week.

  history of excessive fecal retention.

  history of large diameter stool.

  history of painful or hard bowel movements.

  presence of large fecal mass in the rectum.

  in toilet trained children, these additional criteria may be used:

  at least one episode of incontinence per week.

  history of large diameter stools that may obstruct the toilet.
• What is the normal frequency of stools in breastfed babies?

  7 stools in one day when mom has lots of milk or 1 stool in 7 days when mom has just enough milk.

  this should not be mistaken for diarrhea or constipation.
• What are the functional causes of constipation? [DLLLSUP]

  diet low in fibre.

  lack of exercise.

  low fluid intake.

  lack of colon discipline.

  side effects of meds.

  unavailability of toilets.

  psychological factors.
• What are the anatomical causes of constipation? [HAAVM]

  Hirschsprung disease.

  anal stenosis.

  anorectal malformation.

  voluntary inhibition of defecation due to painful disorders such as fissures and dermatitis.

  megarectum.
• What are the neuromuscular causes of constipation? [SPACH]

  spina bifida/meningocele.

  paraplegia.

  absence of abdominal muscle (prune belly).

  cerebral palsy.

  hypotonia.
• How is constipation treated? [DEAFGR]

  disimpaction.

  education of family/parents.

  adjust diet.

  fecal softeners when necessary.

  good bowel habits.

  regular follow up and support.
• What are the anatomical causes of fecal incontinence?

  surgery.

  spinal cord dysfunction.

  congenital Ano-rectal malformation.
• What is retentive functional incontinence?

  it is characterized by constipation and impaction thus resulting in overflow incontinence.

  this is the majority of paediatric patients.
• What causes non-retentive functional incontinence?

  emotional problems.
• What is the management of incontinence? [PREW]

  psychological evaluation and emotional support.

  regular follow up.

  education of family.

  well organized precise plan involving education, disimpaction, high fibre diet and water, and daily routine to keep bowel empty.
• What are diet changes that are needed in the management of constipation and incontinence?

  exclude excessive intake of cane sugar.

  add plain fibre in diet.

  encourage foods high in fibre such as brown bread, popcorn, fruits and vegetables, and lentils.

  replace cold drinks with water.
• What does the maintenance therapy of constipation include? [PHAB]


  physiotherapy (correct toilet sitting).

  high fibre diet.

  adequate water.

  bowel routine.
• What is anorectal malformation?

  spectrum of anal/rectum abnormalities.
• Anorectal malformations are commonly associated with what other malformations?

  VACTERL association.

  V- vertebral defects.

  A- anorectal malformation.

  C- cardiac anomalies.

  TE- tracheal-esophageal fistulas

  L- limb defects.
• List the anatomical classifications of anorectal malformations (Krickenbeck) [PRRRNC].

  perineal fistula.

  rectovestibular fistula.

  rectovesical fistula.

  rectourethral fistula.

  no fistula.

  cloaca.
• What fistula is mostly present in boys with anorectal malformation and what does this entail?

  What do you expect to see on a perineal inspection?

  recto-urinary fistula.

  they pass meconium through urethral orifice.

  no fistula will be seen on the perineal inspection.
• What fistula is mostly present in girls with anorectal malformation?

  What do you expect to see on a perineal inspection?

  recto-vestibular fistula.

  a fistula to the perineum or vestibulum will be seen on perineal inspection.
• What are the clinical signs of anorectal malformations in girls?

  they do not develop bowel obstruction.

  sufficient stool passes through recto-vestibular fistula.
• What are the clinical signs of anorectal malformations in boys?

  they develop bowel obstruction because they can't pass sufficient stool through the recto-urinary fistula.

  they develop abdominal distention and vomiting, which is a late sign.
• How is anorectal malformation treated?

  it is treated as a bowel obstruction.

  hydration, NPO, NG tube on free drainage.

  keep warm and send to paediatric surgeon.
• What is the workup for anorectal malformation wherein the baby has no anus?

  clinical evaluation of perineum/buttocks.

  babygram.

  screening sonar.

  an invertogram to confirm the level of lesion.
• What is the surgical management of anorectal malformation?

  if there is no visible fistula, colostomy.

  posterior sagittal ano-rectoplasty.

  four weeks after colostomy, check to see if anal dilation is needed.

  colostomy closure.

  if there is a visible fistula, mostly no colostomy is necessary.

  an anoplasty or ano-rectoplasty can be done.
• What is the result of the pathophysiology of Hirschsprung disease?

  increased muscle tone and thus failure to pass stool or flatus.
• What are the anomalies associated with Hirschsprung disease?

  cardiac.

  urogenital.

  gastro-intestinal.

  down syndrome.
• What are clinical signs of Hirschsprung disease?


  history of no meconium/constipation.

  clinical signs include distended abdomen and explosive stools on presentation.
• What do you expect to find on an abdominal X-ray of a baby with Hirschsprung disease?

  dilated bowel loops filled with air and no fluid-air levels.
• What would you expect to find on a contrast study of a baby with Hirschsprung disease?

  narrow distal segment.

  dilated proximal bowel.

  retention of contrast for more than 24 hours.
• What is Hirschsprung's disease definitively diagnosed by?


  full thickness rectal biopsy.

  histological findings viz. no ganglion cells visible in plexus, myenteric (Auerbach) and submucosal (Meissner).
• What is the clinical presentation of infants with Hirschsprung's disease? [PPREEN]

  perforation in 3%.

  poor feeding.

  resonant abdominal distention and vomiting.

  enterocolitis.

  explosive stools on rectal exam.

  no meconium passed in 24 hours.
• How is Hirschsprung's disease managed?

  Resuscitation.

  diverting colostomy to relieve obstruction.

  once the infant has recovered from acute disease, abnormal bowel is resected and an anastomosis to the anus is made.
• What is the emergency treatment of Hirschsprung disease?


  treat as bowel obstruction.

  nasogastric tube on free drainage and NPO.

  IV fluid according to weight and age.

  refer to paediatric surgeon.
• What is the emergency treatment of Hirschsprung disease with enterocolitis?

  IV fluid with Bolus ringers then maintenance fluids.

  nasogastric tube suctioning and NPO.

  saline enemas to remove debris from colon until it runs clear.

  antibiotics.
• What is the clinical presentation of Hirschsprung disease in older children? [RREMMS


  rule out myopathy.

  rectum may be empty.

  enterocolitis which is rare.

  malnutrition.

  massive distention.

  severe constipation.
• What is degenerative leiomyopathy?


  it is a progressive disease.

  infants are normal at birth, have constipation as toddlers.

  the muscles of the urinary tract may be affected as well.

  they unfortunately die as teenagers as there is no cure.
• What is the clinical picture of degenerative leiomyopathy?

  rectum may be empty.

  enterocolitis.

  malnutrition.

  massive distention.

  severe constipation.
• What is characteristic of a rectal biopsy of a child with degenerative leiomyopathy?

  normal ganglion cells are present.
• True or false: long segment disease, involving the rectum, sigmoid colon and variable length of colon, of Hirschsprung disease is the most common disease.

  False.
• Discuss the Rome IV criteria with regards to constipation in infants and toddlers up to 4 years of age. [TH3P]

  At least two of the following present for at least one month:

  two or less defecations per week.

  history of excessive fecal retention.

  history of large diameter stool.

  history of painful or hard bowel movements.

  presence of large fecal mass in the rectum.

  in toilet trained children, these additional criteria may be used:

  at least one episode of incontinence per week.

  history of large diameter stools that may obstruct the toilet.
• What is the normal frequency of stools in breastfed babies?

  7 stools in one day when mom has lots of milk or 1 stool in 7 days when mom has just enough milk.

  this should not be mistaken for diarrhea or constipation.