Chapter 24: Hirschsprung's Disease (Children) Flashcards
Hirschsprung’s Disease
as known as congenital aganglionic megacolon
- caused by a congenital absence of Meissner’s and Auerbach’s autonomic plexus in the bowel wall; this absence of ganglion cells results in a lack of motility in the affected portion of the bowel
- usually limited to distal colon
What happens when there is the absence of ganglion cells in the affected portion of the bowel?
lack of nervous system stimulation to that portion of the colon
- leads to abnormal or absence of peristalsis in the involved segment and an inability of the internal sphincter to relax
- a complete or partial bowel obstruction may occur as a result of this inability of the smooth muscles to relax; leads to a accumulation of bowel contents in the involved segment of bowel
Signs and Symptoms
- failure to pass meconium within the first 48 hours of life
- failure to thrive
- poor feeding
- chronic constipation
- down syndrome
> Physical findings:
- vomiting
- abdominal obstruction
- failure to pass stools
- diarrhea
- flatus
- explosive bowel movements
> In older children, the initial symptoms is chronic constipation. Child’s stools may be described as ribbon or pellet shaped and foul smelling
What is the most doomy presentation of Hirschsprung’s Disease?
Enterocolitis
- may present with an abrupt onset of foul-smelling diarrhea, abdominal distention, and fever
- rapid progression may indicate perforation of the bowel and sepsis
Diagnosis
- in the newborn is suspected based on clinical presentation or intestinal obstruction and failure to pass meconium
- radiographic studies show evidence of a dilated loop of bowel
- barium enema often demonstrates the transition between the dilated proximal colon and the aganglion distal segment, though this may not be evident until age 2 months or later
- absence of ganglion cells is determined by a biopsy; Rectal manometry and rectal suction biopsy
- In anorectal manometry a balloon is distended in the rectum and measures the pressure of the internal and anal sphincter; in normal patients, rectal distention initiates a reflex decline in internal sphincter pressure; in patients with this disease, the pressure fails to drop or there is a rise in pressure with rectal distention
Prevention
b/c this condition is familial and associated with chromosomal disorders = comprehensive prenatal care and counseling in families with a family history of Hirschsprung’s disease
Nursing Care
preoperative assessment of the child’s fluid and electrolyte status
- child place NPO
- nasogastric (NG) tube inserted
- IV fluids and electrolytes administered to prevent and/or correct imbalances
- accurate intake and output maintained to include colostomy and nasogastric tube drainage
Surgical Care
surgical resection of the affected bowel with or without a colostomy
-generally include a temporary colostomy, with a subsequent ostomy takedown and re-anastomosis (a connection made surgically between adjacent blood vessels, parts of the intestine, or other channels of the body, or the operation in which this is constructed) at age 6 to 12 months
> other options:
-excising the aganglionic segment and anastomosing the normal proximal bowel to the rectum 1 to 2 cm above the denate line
Postoperative Care
- physical assessment
- NPO
- pain management
- wound care
- fluid maintenance
- patency of NG tube
- monitor for abdominal distention
- assess return of bowel sounds
Education/ Discharge
- education on how to care for the colostomy; care of skin, appliance application, and referral to community resources
- symptoms of complications such as enterocolitis, leaks, and strictures at the site of anastomosis
- Signs of leaks= abdominal distention and irritability
- Signs of enterocolitis= abdominal distention, pain, fever, diarrhea, or shock-like symptoms
