Exam 2: GI Flashcards
Hirshprung’s Disease
- also known as congenital aganglionic megacolon
- Mechanical obstruction caused by inadequate motility of part of the intestine
- Lack the ganglion that are necessary for peristalsis
- Due to this aganglionic portion, you end up having an accumulation of stool proximal to the portion of the large intestine that is affected by the Hirshprung’s
Areas affected by Hirshprung’s Disease
Usually includes rectum and proximal portion of the large intestine
- 15-20% of children can have it spread to the rest of the colon
- ~10% of children can have it spread to the entire intestines
Incidence of Hirshprung’s Disease (6)
- ¼ of all neonatal obstruction but may not be diagnosed until later in infancy or childhood
- Is usually diagnosed at/around birth
- More common in children with Downs Syndrome
- 4x more common in males
- 1 in 5,000 births
- Familial component/genetic predisposition → if you have a child with Hirshprung’s then your next child has a slightly increased likelihood of getting it too
EXACT ETIOLOGY IS UNKNOWN
Pathophysiology of Hirshprung’s Disease (4)
1st: Absence of ganglionic cells in one or more segments of the colon
2nd: Results in absence of propulsive movements (peristalsis) leading to accumulation of intestinal contents and distention of bowel proximal to defect (megacolon)
3rd: Internal anal sphincter fails to relax
4th: Intestinal distention and ischemia of bowel wall which leads to enterocolitis (inflammation of small bowel and colon) which is leading cause of death in children with Hirshprungs (worry about this if patient can’t have surgery)
Number one complication of Hirshprung’s
Entercolitis then Peritonitis
Entercolitis: inflammation of intestine and colon
Peritonitis: perforation of bowel wall that will occur second to entercolitis
Symptoms of Peritonitis (6)
- severe abdominal pain,
- very rigid and tender abdomen,
- distended abdomen,
- fever,
- dyspnea,
- no bowel sounds upon auscultation
Newborn Signs and Symptoms of Hirshprung’s (4)
- Failure to pass meconium (first stool) within 24 to 48 hrs. after birth
- -Meconium is black and tarry and difficult to clean
- -Failure to pass means you need further evaluation to see if the patient has Hirshprung’s - Reluctance to ingest fluids
- Bile-stained vomitus
- Abdominal distention
Infancy Signs and Symptoms of Hirshprung’s (7)
- Failure to thrive
- Constipation due to lack of peristalsis
- Abdominal distention
- Episodes of diarrhea and vomiting
- Explosive watery diarrhea
- Fever
- Severe exhaustion
Childhood Signs and Symptoms of Hirshprung’s (6)
- Constipation
- Ribbon-like, foul-smelling stool that leaks around where the mega-colon is
- Abdominal distention
- Visible peristalsis in abdomen
- Fecal masses easily palpable
- Poorly nourished child and anemic
Very rare!
Diagnosing Hirshprung’s (4)
- Rectal exam –> tight internal sphincter and absence of stool
- Barium enema
- Anorectal manometry
- Definitive diagnosis is rectal biopsy; there will be a lack of nerve fibers with Hirshprung’s disease
Therapeutic Managment of Hirshprung’s
- At birth, total surgery correction where they remove the diseased portion of the bowel and reinastimose the healthy parts (
- At 9kg pull through procedure and colostomy closed
* Depending on age of child, may have to do temporary ostomy after removal of aganglionic portion and once mega-colon area gets back to normal shape they reinastimose the healthy parts - Outcome of surgery is very good; 65-85% end up achieving normal bowel function
Enterocolitis
Inflammation of intestine and colon; the biggest concern of pre-op patient’s with Hirshprung’s
*Will need to assess for signs and symptoms of perforated bowel (peritonitis)
Assessing for Signs of Perforated Bowel (7)
- Vital signs – looking for shock; tachycardia, increased RR, high fever, may have hypotension
- Absent bowel sounds
- Abdominal distention and tenderness
- Vomiting
- Irritable
- Fever – DO NOT DO RECTAL TEMPS
- Dyspnea and cyanosis
Post-Op Care of Hirshprung’s
- Colostomy care
- Prevent contamination of wound with urine
- Impaired skin integrity due to incontinence
- NPO until bowel sounds return or flatus passed – IV fluids
- Pain control
- Strict I&O
Gastroesophogeal Reflux
Very common, could be due to/in conjunction with something else such as a treatment
- Dysfunction of lower esophageal sphincter (LES)
- Delayed gastric emptying
- Poor clearance of esophageal acid
- Susceptibility of esophageal mucosa to acid injury
Etiology of Gastroesophogeal Reflux (9 with most common cause)
- Prematurity
- Tracheal-esphogeal atresia
- Neurological disorders; delay in the message from the brain to the sphincter to close after eating
- Scoliosis
- Asthma
- Cystic Fibrosis
- Partial or incomplete swallowing dysfunctions
- Theophylline and caffeine
- Increased abdominal pressure
- Infants with short LES; Immature sphincter
- **This is the most common cause!
Tracheal-Esophogeal Atresia
When esophagus ends with a blind pouch; no connection to stomach
- Will have a fistula that is connected to a trachea
- When you try feeding them, they will present with cough, congestion, respiratory distress because the feed is going into the fistula then into trachea
- Surgical repair of this can put a child at risk for developing reflux
Incidence of Gastroesophogeal reflux (3)
- 3% of all newborns, but rare to see it in the newborn period
- Peaks between 1-4 months of age, usually resolves by 6-12 months of age
* **This is because regurgitation/irritability incidence decreases since they begin to sit up and are beginning to eat solids - Boys affected 3x more than girls
Signs and Symptoms of Gastroesophageal Reflux (10)
- Vomiting/may be constantly regurigtaing
- Weight loss
- Failure to thrive (may not be gaining weight)
- Very Irritable because the mucosa is getting excoriated; every time they reflux it’s very painful (May increase after eating)
- Respiratory illness
- Coughing, choking, apnea, bradycardia (May be aspirating)
- Hiccups
- Recurrent weight loss
- Heme (+) stools; blood in stool from bleeding in esophagus
- Sandifer’s syndrome
Apnea with Gastroesophogeal Reflux
Aspiration and regurgitation stimulates mucous production in the throat which can lead to apnea or bradycardia
*Near life threatening event: apneic episode at home and they need resuscitation