Hirschsprung's Disease/Megacolon
Hirschsprung's is disease of gut caused due to congenital absence of ganglion cells (parasympathetic ganglion cells) in submucosal and myenteric plexus of intestine.This disorder affects the large intestine (colon).
Hirschsprung's disease is also known as Megacolon or Congenital Aganglionic Megacolon.
Pathophysiology
Due to absence of ganglionic cells
Results lack of peristalsis in affected portion
Functional obstruction of colon
Accumulation of gas and feces to the defect region
Enlargement of colon occurs, known as Megacolon
Clinical Manifestations
1. Failure to pass meconium
2. Constipation
3. Bloating
4. Abdominal pain or tenderness
5. Hard fecal masses called fecalomas
6. Abdominal distension
7. Bile stained vomiting
8. Weight loss and dehydration because of failure to take fluids and vomiting
9. Episodes of diarhoea
10. Enterocolitis (inflammation in small intestine and colon) due to faecal stagnation
11. Enterocolitis lead to dehydration and sepsis
12. Shock may develop
13. In order children; constipation with abdominal distension due to masses of faeces and gas.
14. Ribbon like form of stool passed
15. Malnourished and anemic due to malabsorption of nutrients.
Diagnostic Evaluation
1. Baby who not pass meconium within 48 hours of birth.
2. On rectal examination
Explosive leakage of gas and accumulated faeces.
3. On palpating abdomen, faecal mass is felt in left lower position of abdomen.
4. Anorectal manometry (evaluate the function of the rectal and anal muscles; motality study).
5. Barium Enema
6. Rectal biopsy; show absence of ganglion in submucosa.
Management
1. Medical Management
Administration of isotonic enema
Administration of stool softeners
Low residue diet
2. Surgical management
1. Remove Aganglionic bowel followed by anastomosis of remaining portion.
2. Surgery involve two steps;
a. Temporary colostomy done above zone of ganglionic and Aganglionic bowel in sigmoid or transverse colon.
b. Excision of Aganglionic segment with a "pull through" procedure enabling anastomosis to be done between ganglionic colon and anus.
c. After a year corrective surgery Colostomy is closed
a. Swenson procedure
Aganglionic colon resected just above anus.
Ganglionic colon brought outside and anus anastomosis is done outside.
After anastomosis, anastomosed anus and colon pushed inside.
b. Duhamel procedure
c. Soave's procedure
Rectum mucosa is removed.
Aganglionic colon is excised.
Ganglionic colon is pulled through of rectum and anastomosed with anus.
Nursing Management
Pre-Operative Nursing Care
1. Complete history of Newborn taken;
History shows failure to pass meconium and vomiting.
Physical assessment; show presence of abdominal distension
Enquire bowel habits of child
2. Administered isotonic enema, suppositories and stool softener.
3. Low residue diet given to child such as potato, rice, milk, soup, strained fruit juice.
4. Monitor vital sign and blood pressure to obtain baseline data of child.
5. Observe abdominal girth to detect abdominal distension, observe for absence of stool, vomiting, abdominal pain, and absence of bowel sounds on auscultation.
6. Given semi-fowler's position facilitate lung expansion and breathing.
7. Keep the patient NPO and nasogastric aspiration done on night before surgery.
8. Prepare bowel for surgery; in older children repeated saline enema and bowel wash with antibiotic solution like neomycin is done.
9. Observe dehydration; indicated by reduced urine output, increase thirst, sunken eyes, por skin turgor.
10. Prepare the child for colostomy and related procedure.
Post Operative Nursing Care
1. Monitor vital sign of child.
2. Observe the any discharge or leakage from abdominal dressing or ostomy bag for bleeding.
3. Placed the child in comfortable position according to physician's order
4. After surgery chid is NPO, administration IV fluids as ordered
5. Monitor for abdominal distension
86. Maintain fluid and electrolyte balance
7. Monitor for return of bowel sounds, then start oral feeding
8. Colostomy care is to be done;
a. Observe stoma and its colour (normally pink in colour, purplish colour indicate infection)
b. Observe stoma for any discharge, purulent discharge or edema
c. Apply zinc oxide ointment on skin around stoma to prevent skin picking cause scratched in skin.
d. Provide blant diet to child; non irritating to stoma.
e. Keep colostomy clean and dry
f. Frequently empty collecting bag applied on stoma
g. Educate the parents about colostomy care
h. Pull through surgery;
1. Incision site prevented from infection
2. IV fluids administerd untill peristalsis resumes, healing of anastomosis taken place
3. Bowel sounds returns, start oral feeds with glucose water and then milk
4. Observe for post operative complications;
Such as; hemorrhage, shock, wound infection, abdominal distension, enterocolitis, leakage at site of anastomosis leading to pelvic abscess observed by sudden abdominal distension, rise in body temperature.
5. Advice the caregivers after discharge from hospital provide adequate fluids, dietary fibre, stool softeners in order to achieve normal bowel activity.
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