SILLIMAN UNIVERSITY College of NursingDumaguete City

Resource Uniton

Hirschsprung’s Disease

SUBMITTED TO: Ms. Charmaine Joy V. Quilnet, RN

SUMITTED BY:

Karen Michelle Boniel

NCM 105 – A1Pediatric Rotation

College of NursingSilliman University

Dumaguete City

Placement: NCM 105, 1st Semester, 2nd Rotation (SY 2010-2011)

Time Allotment: 1 hour

Description: This ward class deals on the topic Hirschsprung’s Disease. Discussion will include overview of the disease, etiology and pathophysiology, clinical manifestations, management and/or treatment, as well as nursing responsibilities.

Central Objective: By the end of the 1-hour presentation, the learners shall be able to gain appropriate and adequate comprehension on Hirschsprung’s Disease as a disease, acquire sufficient knowledge and skills on the management of this condition, and develop affirmative approach or mind-set in the use of these concepts in rendering efficient nursing care to clients.

Specific Objectives Content Time Allotment

Evaluation

At the end of the 1-hour discussion, the learners will be able to:

Concisely define

I. Overview of the Condition

A. Description

10 mins. Question and Answer through Roll

Hirschsprung’s Disease in one’s own words.

Briefly state the etiology of the condition.

Hirschsprung's disease occurs when some of the nerve cells that are normally present in the intestine do not form properly while a baby is developing during pregnancy.

As food is digested, muscles move food forward through the intestines in a movement called peristalsis. When we eat, nerve cells that are present in the wall of the intestines receive signals from the brain telling the intestinal muscles to move food forward.

In children with Hirschsprung's disease, a lack of nerve cells in part of the intestine interrupts the signal from the brain and prevents peristalsis in that segment of the intestine. Because stool cannot move forward normally, the intestine can become partially or completely obstructed (blocked), and begins to expand to a larger than normal size.

The problems a child will experience with Hirschsprung's disease depend on how much of the intestine has normal nerve cells present. Seventy percent of babies with Hirschsprung's disease are missing nerve cells in only a portion of the last one to two feet of the large intestine.

This condition is also known as congenital aganglionic megacolon. In most instances, the lower portion of the sigmoid colon just above the anus is affected.

B. Etiology

Between the 5th and the 12th weeks of pregnancy, while the fetus is growing and developing, nerve cells form in the digestive tract, beginning in the mouth and finishing in the anus. For unknown reasons, the nerve cells do not grow past a certain point in the intestine in babies with Hirschsprung's disease.

Scientists are not sure why the intestinal nerve cells do not form completely. Nothing has been shown to cause this problem, including medications a mother takes while pregnant or what a mother eats during pregnancy.

the Dice and Fishbowl Game at 75% level of competency.

*The reporter will draw a name from the bowl. The name picked out will be the person who will roll the dice. After he/she rolls the dice, he/she will answer the question that corresponds to the number the dice yields.

C. Risk Factors

Hirschsprung's disease occurs five times more frequently in males than in females. Children with Down syndrome have a higher risk of having Hirschsprung's disease.

There is possibly a genetic, or inherited, cause for Hirschsprung's disease. If a family has a child with Hirschsprung's disease, there is a 3 to 12 percent chance that another baby they have will also have the disease. Also, there is an increased chance that a couple will have a child with Hirschsprung's disease if one of the parents has the disease. The chances are higher if the mother is the parent with Hirschsprung's disease.

D. Frequency

Hirschsprung's disease occurs in 1 out of every 5,000 live births.

E. Mortality/Morbidity

Approximately 20% of infants will have one or more associated abnormality involving the neurological, cardiovascular, urological, or gastrointestinal system.

Untreated aganglionic megacolon in infancy may result in a mortality rate of as much as 80%. Operative mortality rates for any of the interventional procedures are very low. Even in cases of treated Hirschsprung disease, the mortality rate may be as high as 30% as a result of enterocolitis.

F. Race

Untreated aganglionic megacolon in infancy may result in a mortality rate of as much as 80%. Operative mortality rates for any of the interventional procedures are very low. Even in cases of treated Hirschsprung disease, the mortality rate may be as high as 30% as a result of

Differentiate the four types of Hirschsprung’s Disease.

enterocolitis.

G. Sex

Hirschsprung disease occurs more often in males than in females, with a male-to-female ratio of approximately 4:1. However, with long-segment disease, the incidence increases in females.

H. Age

Hirschsprung disease is uncommon in premature infants.

The age at which Hirschsprung disease is diagnosed has progressively decreased over the past century. In the early 1900s, the median age at diagnosis was 2-3 years; from the 1950s to 1970s, the median age was 2-6 months.

Currently, approximately 90% of patients with Hirschsprung disease are diagnosed in the newborn period.

II. Types of Hirschsprung’s Disease

A. Short-Segment Disease

It is also called Type I HD. In this type, the parts affected are from the internal rectal sphincter up to the sigmoid or proximal descending large intestine. It is the more common type accounting for about 60-85% of cases.

B. Long-Segment Disease

This one is also called Type II HD. In this type, the transverse or even portions of the ascending colon can be affected. It is the less common type accounting for 15-25% of cases.

C. Total Hirschsprung’s Disease

5 mins.

Satisfactorily trace the pathophysiology of the condition.

Enumerate at least 3 clinical manifestations characteristic of the condition.

In this rare type, the affected person has no ganglion cells at all in the colon.

D. Small Intestine Hirschsprung’s Disease

As rare as Total Hirschsprung’s, Small Intestine HD starts in the small intestine.

III. Pathophysiology (Concept Map Presentation)

An arrest in embryologic development affecting the migration of parasympathetic nerve innervation of the intestine.

o Normally, the nerve cells migrate to the upper end of the alimentary tract and then proceed in the caudal direction, with migration to the distal colon complete by 12 weeks.

o Migration occurs first in the intermuscular layer, called Auerbach's plexus, and then moves into the submucosal plexus, moving along the GI tract in a descending manner.

The process of aganglionosis is almost always continuous within the affected segment, ending in proximal segment with ganglion cells. Intermittent ganglion cells in the colon have been reported, but this is extremely unusual.

o Most commonly affected site is the rectosigmoid colon (referred to as short-segment disease) (80%)

o Long-segment disease extends to the upper descending colon and transverse colon (10% to 15%) and occasionally throughout the entire colon, involving the small bowel (5%).

o Total angiogliosis of the bowel, involving the entire small and large bowel, is rare. No peristalsis occurs in the affected portion of intestine (ie, spastic and contracted). This

section is usually narrow; therefore, no fecal material passes through it. Proximal to the narrow affected section, the colon is dilated (see Figure 48-5).

o Filled with fecal material and gas.

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State at least 2 complications of the disease.

o Hypertrophy of muscular coating.o Ulceration of mucosa may be seen in neonate.

The internal rectal sphincter fails to relax, and evacuation of fecal material and gas is prevented. Abdominal distension and constipation result.

V. Complications

Intestinal distension and ischemia may result as a result of obstruction. As the bowel dilates, intraluminal pressure increases, resulting in decreased blood flow and deterioration of the mucosal barrier. Stasis allows proliferation of bacteria (Clostridium difficile, Staphylococcus aureus, anaerobes, coliforms), which may lead to the development of enterocolitis (inflammation of the small bowel and colon) with associated sepsis and signs of bowels obstruction. Enterocolitis is the leading cause of death in children with Hirschsprung’s disease. (DiLorenzo, 2001 as quoted in Wong’s Essentials of Pediatric Nursing)

Perforation of the intestine and Short bowel syndrome, a condition that can lead to malnourishment and dehydration, can also result.

Preoperativeo Enterocolitis is a major cause of deatho Hydroureter or hydronephrosis- Analogously, hydroureter is defined as a dilation of the

ureter. The presence of hydronephrosis or hydroureter should be considered a physiologic response to the interruption of the flow of urine. Although this is often due to an obstructive process, in some cases, such as megauretersecondary to prenatal reflux, the collecting system may be dilated in the absence of obstruction. In addition, obstruction can sometimes occur in the absence of a dilated urinary tract. Thus, the terms hydronephrosis and obstruction should not be used interchangeably.

o Water intoxication from tap water enemaso Cecal perforation

5 mins.

Briefly describe at least 4 ways on how Hirschsprung’s disease is diagnosed.

Postoperativeo Enterocolitis: remains the major cause of morbidity and mortality (mortality 6% to

30%).o Diarrhea (69%)o Vomiting (51%)o Fever (34%)o Lethargy (27%)o Leaking of anastomosis and pelvic abscesso Stenosis, sudden inability to evacuate colono Long term: intestinal obstruction from adhesions, volvulus, intussusception

VI. Diagnostic Procedures

A. Rectal ExaminationThis is diagnostic test which may show a lack of stool in the large intestine or near the anus and dilated segments of the large and small intestine. This demonstrates absence of fecal material in the long segment disease; in short segment disease, rectal impaction may be present and, when the finger is removed, it may cause a rush of stool as the obstruction is temporary relieved.

B. X-RaysAn x-ray of the abdomen shows strictures (narrowed areas), obstructions (blockages), and dilated intestine above the obstruction. It shows severe gaseous distention of the bowel, with absence of gas in the rectum.

C. Radiopaque MarkersWhen ingested, measure intestinal transit time. Children with short segment disease retain the markers in the rectum for long periods.

5 mins.

Differentiate the four types of surgical interventions.

D. Barium EnemaThis is a procedure performed to examine the large intestine for abnormalities. A fluid called barium (a metallic, chemical, chalky, liquid used to coat the inside of organs so that they will show up on an x-ray) is given into the rectum as an enema. This shows narrowed intestine proximal to anus and dilated intestine proximal to narrow segment.

E. Rectal BiopsyThis is a test that takes a sample of the cells in the rectum or large intestine and then looks for nerve cells under a microscope. This may be done to demonstrate absent or reduced number of ganglion nerve cells, and confirm diagnosis.

F. Anorectal ManometryThis is a test that measures nerve reflexes which are missing in Hirschsprung's disease. It may be done to record the reflex response to anal sphincter, requires cooperation of the child. a balloon is inflated in the rectum to measure pressure in the area.

G. UltrasonogramThis may be done to demonstrate dilated colon.

VII. Surgical Intervention

The Pull-Through Procedure used to be composed of 2 steps. First, was the conventional colostomy which is used to cleanse the bowel prior to the main part of the operation. In the second step, which is the main operation, and was typically done when the child’s age, weight, and condition is right, a resection of the affected portion is done, and after the resection, reanastomisis is performed. The original procedure was devised by Orvar Swenson but the pull-through procedure has already been modified for about 5 times but still with equal therapeutic results. Today, a pull-through procedure can already be done without a colostomy due to the

5 mins.

Give at least two nursing management approaches for pre-operative care and

advancement in technology available provided that it would be diagnosed on time.

A. Boley’s ProcedureThis endorectal pull-through procedure described by Boley involves stripping the

mucosa from the aganglionic rectum and bringing down normally innervated colon through the residual muscular cuff, thus bypassing the abnormal bowel from within.

B. Duhamel’s Procedure

Duhamel describe a procedure to create a neorectum, bringing down normally innervated bowel behind the aganglionic rectum. The neorectum created in this procedure has an anterior aganglionic half with normal sensation and a posterior ganglionic half with normal propulsion.

C. Soave’s Procedure

This approach utilizes an anorectal mucosa-existing technique and self-anastomosis by the bpwel. Intraabdominally, the seromuscular layer of the entire length of affected bowel is separated from the (inner) mucosal layer beginning at the proximal normal bowel downward while the same is done to the anus upward till the entire outer layer forms a “tube” detached from the inner bowel. The “tube” is brought out through the previously excised “circle” of anal tissue (internal and external sphincters) to the point of normal bowel but not sutured. The protruding bowel consists of aganglionic bowel and the distal distended portion. A rectal tube is inserted and left for 15-20 days till auto-anastomosis takes place. Protruding bowel is cauterized, under anesthesia. To avoid abdominal infection, no intraabdominal excision is used.

D. Swenson’s Procedure

The aganglionic segment is excise and anastomose the normal proximal bowel to the rectum 1-2 cm above the dentate line. This procedure is particularly difficult to

5 mins.

post-operative care, respectively.

Provide at least 3 nursing diagnoses applicable to the disease condition.

perform which led to the development of two other procedures.

After resection of the distended segment, the aganglionic portion and the rectum are everted through the anus. Then the normal bowel is brought down and sutured (a) as the aganglionic segment is excised and, finally, the normal bowel is sutured (b) to the anus and trimmed. The anastomosis features two layers (muscle and mucosa) and pelvic dissection.

VIII. Nursing Management

A. Assessment

1. Health History and Medical Diagnostic Evaluation Elicit a description of the present illness and chief complaint. Newborn stool patterns are akey element in recognizing this diagnosis. Assess whether the newborn passed a meconium stool: most children with Hirschsprung’s do not pass out stool within the first 24-28 hours of life.

Supportive findings on history and physical examination for Hirschsprung's disease. Digital rectal examination reveals an anal canal and a rectum that are narrow and empty of

stool (in long-segment disease). In short-segment disease, rectal impaction may be present; removal of finger may be associated with a rush of stool as the obstruction is relieved.

Plain films show severe gaseous distension of the bowel, with absence of air in the rectum. Radiopaque markers, ingested, measure intestinal transit time. Children with short-

segment disease retain the markers in the rectum for long periods. Barium enema is used to demonstrate a transition zone between the proximal dilated,

normal innervated colon, and the distal, narrow, aganglionic colon.o May be nondiagnostic in young infants who have not had sufficient time to develop

a transition zone.

o Delayed passage of barium is suggestive, but not definitive for Hirschsprung's disease.

Anorectal manometer demonstrates failure of the intestinal sphincter to relax in response to transient rectal distention. Requires cooperation of the child.

Full thickness or suction rectal biopsy absence or reduced number of ganglion nerve cells; definitive diagnosis.

2. Clinical Manifestation.Inspect and palpate the abdomen. The abdomen is typically distended, and often

stool masses can be palpated in the abdomen. Perform rectal examination to assess for rectal tone and presence of stool in the rectum. If a gloved finger is inserted in the rectum of a child with true constipation, the examining finger will touch a hard, caked stool. But with Hirschsprung’s, the rectum is empty because fecal material cannot pass into the rectum through the obstructed portion.

Neonate’s symptoms appearing at birth or within first weeks of life.o No meconium passed in the first 48 hours of life.o Vomiting a bile-stained or fecalo Abdominal distentiono Constipation—occurs in 100% of patientso Overflow-type diarrheao Dehydration; failure to thriveo Temporary relief of symptoms with enema

Older child’s symptoms not prominent at birth. Short-segment disease commonly presents later.

o History of constipation at birtho Progressive abdominal distensiono Peristaltic activity observable over abdomeno Absence of retentive posturing (ability to contract the internal and external

sphincter to purposefully avoid defecation)o Constipation is unresponsive to conventional remedieso Absence of encopresis (common feature of functional constipation)o Ribbonlike, fluidlike, or pellet stoolso Failure to grow or loss of subcutaneous fat; appears malnourished, stunted growtho Presentation insidious or catastrophic as with enterocolitis

Enterocolitis consists of severe toxemia and a proliferation of bacteria in the colonic lumen.

o Abdominal distensiono Explosive diarrhealo Vomitingo Fevero Lethargyo Rectal bleedingo Shock

B. Nursing Diagnoses (Nursing Dx with Management; Please refer to the last pages)

1. Preoperative Ineffective Breathing Pattern related to abdominal distension Acute Pain related to intestinal obstruction Imbalanced Nutrition: Less Than Body Requirements related to poor intake Constipation due to pathophysiologic process

2. Postoperative Risk for Injury related to postoperative course Risk for Infection of Surgical Incision Risk for Injury related to decreased peristalsis postoperatively

Ineffective Family Coping related to care of child with colostomy

C. Pre-Operative Care

1. Preventing ConstipationDecreased bowel motility may lead to constipation, which in turn may lead to injury.

Enemas may be given to achieve bowel elimination and also before diagnostic and surgical procedures are performed. Administer colonic irrigations with saline solutions. Neomycin or other antibiotic solutions re used to cleanse the bowel and prepare the GI tract. Never administer soapsuds or tap water enemas, because the lack of peristaltic action causes the enemas to be retained and absorbed into the tissues causing water intoxication. This could cause syncope, shock, or even death after only one or two irrigations.

2. Maintaining Adequate NutritionParenteral nutrition may be needed to improve nutritional status because constipation

and distended abdomen cause loss of appetite. The infant or child does not want to eat and they have a poor nutritional status. In older children a low residue diet is given.

3. Reducing FearChildren who are preschool-age and older are more aware of the approaching surgery

and have a number of fears reflective of the developmental stage. Preschoolers are still in the magical stage of thinking. They may overhear a word or two that they misinterpret and exaggerate; this can lead to imagined pain and danger. Careful explanations must be provided for the preschooler to reduce any fears about mutilation. Talk about the surgery, reassure the child that the “insides won’t come out,” and answer questions seriously and sincerely. Encourage family caregivers to stay with the young child if possible to increase the child’s feeling of security.

The older child may have a realistic view of what’s going to happen but may still fear

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the impending surgery. Peer contact may help comfort the school-age child.

4. Promoting Family CopingFamily caregivers are apprehensive about the preliminary procedur as well as the

impending surgery. Explain all aspects of the preoperative care including examinations, colonic irrigations, and IV fluid therapy. As with the other surgical procedures, inform the caregivers about the waiting area, PACU, the approximate length of time of the procedure, and answer any questions. Building good rapport preoperatively is an essential aspect of good nursing care.

D. Post-Operative Care

1. Promoting Skin Integrity Skin integrity of the surgical site, especially around the colostomy stoma, is very

important. When the routine colostomy care is performed, give careful attention to the area around the colostomy. Report any redness, irritation, and rashy appearances of the skin around the stoma. Prepare the skin with skin-toughening preparations that strengthen it and provide better adhesion of the appliance.

Preventing Wound Infection Change wound dressing using sterile technique. If done laparoscopically, wound is

minimal. Prevent contamination from diaper.

o Apply diaper below dressing.o Change diaper frequently.

Be aware 7 to 10 stools per day can be passed postoperatively. When cleared by surgery, prevent perianal and anal excoriation by thorough cleansing sitz baths and application of zinc oxide paste and karaya after soiling.

If skin is denuded and moist, apply a skin barrier and provide specific instruction to the family to prevent further damage to skin with aggressive removal.

Use careful hand-washing technique. Report wound redness, swelling or drainage, evisceration, or dehiscence immediately. Suction secretions frequently to prevent infection of the tracheobronchial tree and lungs. Encourage frequent coughing and deep breathing to maintain respiratory status. Allow the infant to cry for short periods to prevent atelectasis. Change the infant's position frequently to increase circulation and allow for aeration of all

lung areas.

2. Relieving PainThe child may have abdominal pain postoperatively. Observe for signs of pain such as

crying, pulse and respiratory rate increase, restlessness, guarding of the abdomen, or drawing up of the legs. Administer analgesics promptly as ordered. Additional nursing measures that can be used are changing the child’s position, holding the child when possible, stroking, cuddling, and engaging in age-appropriate activities. Observe for abdominal distention, which must be documented and reported promptly.

3. Preventing Abdominal Distension Maintain patency of NG tube immediately postoperatively.

o NG suction for 24 to 48 hours or until adequate bowels sounds, gas from rectum or gas from ostomy.

o Watch for increasing abdominal distention; measure abdominal girth.o Measure fluid loss because amount will affect fluid replacement.

Maintain NPO status until bowel sounds return and the bowel is ready for feedings as determined by provider.

Administer fluids to maintain hydration and replace lost electrolytes. Maintain Foley catheter for 24 to 48 hours. Provide frequent oral hygiene while NPO. Begin oral feedings as ordered.

o Avoid overfeeding.

o Turn head to side or elevate after feeding to prevent aspiration

4. Maintaining Fluid IntakeThe NG tube is left in place after the surgery and IV fluids are given until bowel

function is established. Accurate intake and output determinations and reporting the character, amount, and consistency of stools help determine when the child may have oral feedings. To monitor fluid loss, record and report the drainage from the NG tube every 8 hours. Immediately report any unusual drainage such as bright-red bleeding.

5. Providing Oral and Nasal Care Perform good mouth care at least every 4 hours. At the same time, gently clean the

nares to relieve any irritation from the NG tube. If the infant is young, sucking needs to be satisfied with a pacifier.

6. Providing Family EducationShow the family caregiver how to care for the colostomy at home. If available, an

ostomy nurse may be consulted to help teach the family caregivers. Discuss topics such as devices and their use, daily irrigation, and skin care. The caregivers should demonstrate their understanding by caring for the colostomy under the supervision of the nursing personnel several days before discharge. Family caregivers also need referrals to support personnel.

Supporting the Parents Acknowledge that even a temporary colostomy can be a difficult procedure to accept and

learn to manage.o Initiate ostomy referral.o Support the parents when teaching them to care for the colostomy.o Include the parents soon after surgery in dressing changes and any other

appropriate activities.o Assist and encourage the parents to treat the infant or child as normally as

possible.o Reassure parents that colostomy will not cause delay in the child's normal

development. Encourage the parents to talk about their fears and anxieties. Anticipating future surgery

for resection may be confusing and frightening. Initiate community-nurse referral to help the parents care for the child at home away from

the comforting situation of the hospital, and obtain necessary equipment. Initiate a genetic counseling referral, especially if the parents plan to have more children.

IX. Related Reading (Please refer to the last pages of the Resource Unit)

X. Open Forum and Evaluation

Evaluation Questions:

1. Concisely define Hirschsprung’s Disease in one’s own words.2. Briefly state the etiology of the condition.3. Differentiate the four types of Hirschsprung’s Disease.4. Enumerate at least 3 clinical manifestations characteristic of the condition.5. State at least 2 complications of the disease.6. Briefly describe at least 4 ways on how Hirschsprung’s disease is diagnosed.7. Differentiate the four types of surgical interventions.8. Give at least two nursing management approaches for pre-operative care and post-operative care, respectively.9. Provide at least 3 nursing diagnoses applicable to the disease condition.10. What is another name for Hirschsprung’s Disease?

PRE-OPERATIVE NURSING DIAGNOSES

Ineffective Breathing Pattern related to abdominal distention

Monitor for respiratory embarrassment that may result from abdominal distention; watch for rapid, shallow respirations; cyanosis; sternal retractions. Elevate infant's head and chest by tilting the mattress. Administer oxygen, as ordered, to support respiratory status

Acute Pain related to intestinal obstructiono Note degree of abdominal tenderness.

o Infant's legs drawn upo Chest breathingo Note color of abdomen and presence of gastric waves; take sequential measurements of abdominal girth for evidence of changes.o Assist in emptying the bowel by giving repeated colonic irrigations.

o Procedure for irrigation in an infant is similar to that in an older child, except that less fluid and pressure are used.o Physiologic saline solution (warmed) should be used for irrigations. Tap water may result in large quantities of water being absorbed and in water

intoxication.o Administer medications (antibiotics) to reduce the bacterial flora of the bowel.o Note any change in degree of distention before and after irrigation. Record if location of distention changes (ie, upper or lower abdomen).o Record all intake and output of irrigant and drainage. Report marked discrepancies in retention or loss of fluid.

Insert rectal tube for escape of accumulated fluid and gas as ordered.o If abdominal distention is not relieved by irrigation and decompression and discomfort is significant, insert an NG tube as ordered.

o Note drainage from NG tube, and chart characteristics.o Check for patency; saline irrigations may be requested. Carefully record intake and output.o Perform frequent mouth care.

o Alternate nares when changing NG tube every 24 hours, and use minimal amount of tape to prevent skin irritation.o Offer pacifier for nonnutritive sucking if on parenteral fluids.

Encourage parents to hold and rock infant.o Maintain position of comfort with head elevated. Offer soothing stimulation (eg, music, touch, play therapy).

Imbalanced Nutrition: Less Than Body Requirements related to poor intake

Obtain a dietary history regarding food and eating habits. (for children)o This will contribute to planning dietary alterations.o Explain to parents that eating problems are common with Hirschsprung's disease.

Monitor I.V. fluids appropriately. Measure all output. Offer small, frequent feedings. (Low-residue diet will aid in keeping stools soft.)

o Feed child slowly.o Provide as comfortable a position as possible for child during feedings.

Inform parents that defect can be corrected, but it may take some time for the child's physical status and feeding habits to improve.o Feeding may cause additional discomfort because of distention and nausea.o Parenteral nutrition may be necessary.

Constipation due to pathophysiologic process

Be aware that older children who present with milder forms of the disorder may be treated medically (rare). Note and record frequency and characteristics of stools (constipation is likely to occur). Provide demonstration and written and verbal instructions to family for saline enema administration and use of stool softeners. Obtain dietary consultation for teaching of low-residue diet.

POST-OPERATIVE NURSING DIAGNOSES

Risk for Injury related to postoperative course

Monitor vital signs and respiratory status closely. Monitor for proper functioning of colostomy, if present.

o Note drainage from colostomy: characteristics, frequency, fecal material, or liquid drainage.o Record stoma color, size, and return of function.o Note abdominal distention.o Measure fluid loss from colostomy because the amount will affect fluid replacement.

Report signs of obstruction from peritonitis, paralytic ileus, handling bowel, or swelling.o No output from colostomyo Increased tendernesso Irritabilityo Vomitingo Increased temperature

Place child in a lateral position on a flat or only slightly elevated bed. When head of bed is elevated, the residual carbon dioxide in the child's abdominal cavity causes referred pain in neck and shoulder.

Differentiate type and cause of pain to determine appropriate pain management interventions. Proper positioning, patent catheters and tubes, and timely administration of analgesics are key to ensuring patient comfort.

NURSING ALERT !!

Prevent injury to rectal mucosa by taking axillary or external ear temperature. Any rectal examination or procedure has the potential to cause serious harm to the patient and surgical site. (Not practiced today)

Risk for Infection of Surgical Incision

Change wound dressing using sterile technique. If done laparoscopically, wound is minimal. Prevent contamination from diaper.

o Apply diaper below dressing.o Change diaper frequently.

Be aware 7 to 10 stools per day can be passed postoperatively. When cleared by surgery, prevent perianal and anal excoriation by thorough cleansing sitz baths and application of zinc oxide paste and karaya after soiling.

If skin is denuded and moist, apply a skin barrier and provide specific instruction to the family to prevent further damage to skin with aggressive removal. Use careful hand-washing technique. Report wound redness, swelling or drainage, evisceration, or dehiscence immediately. Suction secretions frequently to prevent infection of the tracheobronchial tree and lungs. Encourage frequent coughing and deep breathing to maintain respiratory status.

Allow the infant to cry for short periods to prevent atelectasis. Change the infant's position frequently to increase circulation and allow for aeration of all lung areas.

Risk for Injury related to decreased peristalsis postoperatively

Maintain patency of NG tube immediately postoperatively.o NG suction for 24 to 48 hours or until adequate bowels sounds, gas from rectum or gas from ostomy.o Watch for increasing abdominal distention; measure abdominal girth.o Measure fluid loss because amount will affect fluid replacement.

Maintain NPO status until bowel sounds return and the bowel is ready for feedings as determined by provider. Administer fluids to maintain hydration and replace lost electrolytes. Maintain Foley catheter for 24 to 48 hours. Provide frequent oral hygiene while NPO. Begin oral feedings as ordered.

o Avoid overfeeding.

o Bubble frequently during feeding.o Turn head to side or elevate after feeding to prevent aspiration

Ineffective Family Coping related to care of child with colostomy

Acknowledge that even a temporary colostomy can be a difficult procedure to accept and learn to manage.o Initiate ostomy referral.o Support the parents when teaching them to care for the colostomy.o Include the parents soon after surgery in dressing changes and any other appropriate activities.o Assist and encourage the parents to treat the infant or child as normally as possible.o Reassure parents that colostomy will not cause delay in the child's normal development.

Encourage the parents to talk about their fears and anxieties. Anticipating future surgery for resection may be confusing and frightening. Initiate community-nurse referral to help the parents care for the child at home away from the comforting situation of the hospital, and obtain necessary

equipment. Initiate a genetic counseling referral, especially if the parents plan to have more children.

BIBLIOGRAPHY

BOOKS:

Dunphy, L. & Winland-Brown, J. (2001). Primary care: The art and science of advanced practice nursing. Philadelphia, PA : F.A. Davis. Pp. 456.

Hatfield, Nancy T. (2003). Broadribb’s Introductory Pediatric Nursing. 6th edition. Philadelphia: Lippincott Williams and Wilkins. Pp. 207-210.

Hockenberry, Marilyn J. (2005). Wong’s Essentials of Pediatric Nursing. 7th edition. Philippines: Elsevier PTE LTD. Pp. 853-855.

Kyle, Terri. (2008). Essentials of Pediatric Nursing. Philadelphia: Lippincott Williams and Wilkins. pp. 698-699.

Lewis, S., Heitkemper, M., Dirksen, S. (2004). Medical-surgical nursing: Assessment and management of clinical problems. 6th ed. Mosby, Inc.: St. Louis Missouri, USA. pp.1134-1136.

Meeker, M. & Rathrock, J. (1999). Alexander’s Care of the Patient in Surgery, 10th ed. St. Louis, Missouri: Mosby, pp. 366-382.

Pilliteri, Adele (2007). Maternal and Child Health Nursing: Care of the Childbearing and Childrearing Family. 5th edition. Philadelphia: Lippincott Williams and Wilkins. pp. 1441-1443.

Polaski, A. & Tatro, S. (1996). Luckmann’s core principles and practice of medical-surgical nursing . Philadelphia, PA : W.B. Saunders. P. 686.

Urdan, L. et. al. (2002). Thelan’s critical care nursing: Diagnosis and management. Philadelphia, PA : W.B. Saunders. Pp. 412-413.

JOURNAL:

Kanto, W.P. (April 2002). Quality of life after surgery for Hirschsprung’s disease. Journal Watch Pediatrics, 34(4). 639. Springhouse, PA: Lippincott Williams & Wilkins.