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  1. HYPERTROPHIC PYLORIC STENOSIS
    1. Introduction: Hypertrophic pyloric stenosis (HPS) causes obstruction of the upper GI tract, but the infant frequently does not have symptoms until 2 to 4 weeks of age. HPS results in thickening, or hypertrophy, of the pyloric sphincter located at the distal end of the stomach; this causes a mechanical intestinal obstruction that becomes increasingly evident as the infant begins to consume larger amounts of formula during the early weeks of life. Pyloric stenosis is five times more common in boys than girls and is most often found in full-term Caucasian infants. The exact etiology remains unknown; however, there does seem to be a genetic predisposition.
    2. Assessment:
      1. Classic symptom is vomiting:
        1. Begins as nonprojectile at age 2 to 4 weeks.
        2. Advances to projectile at age 4 to 6 weeks.
        3. Vomitus is non–bile stained (stomach contents only).
        4. Most often occurs shortly after a feeding.
        5. Major problem is the mechanical obstruction of the flow of stomach contents to the small intestine due to the anatomical defect of stenosis of the pyloric sphincter.
        6. No apparent nausea or pain, as evidenced by the fact that infant eagerly accepts a second feeding after episode of vomiting.
        7. Metabolic alkalosis develops due to loss of hydrochloric acid.
      2. Inspection of abdomen reveals:
        1. Palpable olive-shaped mass in right upper quadrant.
        2. Visible peristaltic waves, moving from left to right across upper abdomen.
      3. Weight: fails to gain or loses.
      4. Stools: constipated, diminished in number and size—due to loss of fluids with vomiting.
      5. Signs of dehydration may become evident (Table 5.16. Signs and Symptoms of Dehydration in Infants and Young Children).
      6. Upper GI series and ultrasonography reveal:
        1. Delayed gastric emptying.
        2. Elongated and narrowed pyloric canal.
    3. Analysis/nursing diagnosis:
      1. Fluid volume deficit related to vomiting.
      2. Altered nutrition, less than body requirements, related to vomiting.
      3. Risk for injury/infection related to altered nutritional state.
      4. Impaired skin integrity related to dehydration and altered nutritional state.
      5. Knowledge deficit related to cause of disease, treatment and surgery, prognosis, and follow-up care.
    4. Nursing care plan/implementation:
      1. Goal (preoperative): restore fluid and electrolyte balance.
        1. pillImage Generally NPO, with IVs preoperatively to provide fluids and electrolytes.
        2. Observe and record I&O, including vomiting and stool.
        3. Weight: check every 8 hours or daily.
        4. Monitor laboratory data.
      2. Goal (postoperative): provide adequate nutrition.
        1. pillImage Maintain NPO with IVs for 4 to 6 hours postoperatively, as ordered ( can offer pacifier).
        2. foodImage Follow specific feeding regimen ordered by doctor—generally start with clear fluids in small amounts hourly, increasing slowly as tolerated. Full feeding schedule reinstated within 48 hours. Offer pacifier between feedings.
        3. Fed only by RN for 24 to 48 hours, because vomiting tends to continue in immediate postoperative period.
        4. Burp well—before, during, and after feeding.
        5. infoImage  Position after feeding: high Fowler's turned to right side; minimal handling after feeding to prevent vomiting.
      3. Goal (preoperative and postoperative): institute preventive measures to avoid infection or skin breakdown.
        1. Use good hand-washing technique.
        2. Administer good skin care, especially in diaper area (urine is highly concentrated); give special care to any reddened areas.
        3. Give mouth care when NPO or after vomiting.
        4. Tuck diaper down below suture line to prevent contamination with urine (postoperatively).
        5. Note condition of suture line—report any redness or discharge immediately.
        6. Screen staff and visitors for any sign of infection.
      4. Goal: do discharge teaching to prepare parents to care for infant at home.
        1. Teach parents that defect is anatomical and unrelated to their parenting behavior/skill.
        2. Demonstrate feeding techniques, and remind parents that vomiting may still occur.
        3. Stress that repair is complete; this condition will never recur.
        4. Instruct parents in care of the suture line: no baths for 10 days, tuck diaper down, report any signs of infection promptly.
        5. Offer follow-up referrals as indicated.
    5. Evaluation/outcome criteria:
      1. Infant survives surgical repair without untoward difficulties (including infection/skin breakdown).
      2. Adequate nutrition is maintained, and infant begins to grow and gain weight.
      3. Parents verbalize confidence in their ability to care for their infant on discharge.
  2. HIRSCHSPRUNG'S DISEASE (congenital aganglionic megacolon)
    1. Introduction: Hirschsprung's disease is a congenital anomaly of the lower GI tract, but the diagnosis often is not established until the infant is 6 to 12 months old. The major problem is a functional obstruction of the colon caused by the congenital anatomical defect of lack of nerve cells in the walls of the colon, resulting in the absence of peristalsis. Hirschsprung's disease is four times more common in boys than girls and is frequently noted in children with Down syndrome.
    2. Assessment:
      1. In the newborn, failure to pass meconium (in addition to other signs and symptoms of intestinal obstruction).
      2. Obstinate constipation—history of inability to pass stool without stool softeners, laxatives, or enemas; persists despite all attempts to treat medically.
      3. Stools are infrequent and tend to be thin and ribbonlike.
      4. Vomiting: bile stained, flecked with bits of stool (breath has fecal odor), due to GI obstruction and eventual backing up of stools.
      5. Abdominal distention can be severe enough to impinge on respirations, due to GI obstruction and retention of stools.
      6. Anorexia, nausea, irritability due to severe constipation.
      7. Malabsorption results in anemia, hypoproteinemia, and loss of subcutaneous fat.
      8. Visible peristalsis and palpable fecal masses may also be detected.
    3. Analysis/nursing diagnosis:
      1. Constipation related to impaired bowel functioning.
      2. Altered nutrition, less than body requirements, related to poor absorption of nutrients.
      3. Risk for injury/infection related to malnutrition.
      4. Pain related to surgery and treatments.
      5. Knowledge deficit regarding care of the child with a colostomy and follow-up care.
    4. Nursing care plan/implementation:
      1. Goal (preoperative): promote optimum nutritional status, fluid and electrolyte balance.
        1. Monitor for signs and symptoms of progressive intestinal obstruction: measure abdominal girth daily.
        2. pillImage Administer IV fluids, as ordered—may include total parenteral nutrition (TPN) or intravenous lipids.
        3. Daily weights, I&O, urine specific gravity.
        4. foodImage Monitor for possible dehydration.
        5. Diet: low residue.
      2. Goal (preoperative): assist in preparing bowel for surgery.
        1. Teach parents what will be done and why—enlist their cooperation as much as possible.
        2. Insert NG tube, connect to low suction to achieve and maintain gastric decompression.
        3. infoImage  Position: semi-Fowler's.
        4. Bowel is cleansed with a series of isotonic saline (0.9%) enemas.
        5. pillImage Administer oral antibiotics and colonic irrigations to decrease bacteria.
        6. Take axillary temperatures only.
        7. If child can understand, prepare for probable colostomy using pictures, dolls (usual age at surgery is 10 to 16 months).
      3. Postoperative goals: same as for adult having major abdominal surgery or a colostomy (see Chapter 6. Physiological Integrity).
      4. Goal (postoperative): discharge teaching to prepare parents to care at home for infant with a colostomy.
        1. Home care of colostomy of infant is essentially same as for adult (see Chapter 6. Physiological Integrity).
        2. Teach parents to keep written records of stools: number, frequency, consistency.
        3. Teach parents to tape diaper below colostomy to prevent irritation.
        4. Because colostomy is usually temporary, discuss:
          1. Second-stage repair (closure and pullthrough) done when the child weighs approximately 20 lb.
          2. Possible difficulties in toilet training.
        5. Stress need for long-term follow-up care.
        6. Make referral to home care if indicated.
    5. Evaluation/outcome criteria:
      1. Infant is prepared for surgery and tolerates procedure well.
      2. Postoperative recovery is uneventful.
      3. Parents verbalize confidence in ability to care at home for infant with a colostomy and verbalize their understanding that second surgery will be needed to close the colostomy.
  3. INTUSSUSCEPTION
    1. Introduction: Intussusception is the apparently spontaneous telescoping of one portion of the intestine into another, resulting in a mechanical obstruction of the lower GI tract. There is no known cause, and intussusception is three times more common in boys than girls; the child with intussusception is usually between 3 and 36 months of age.
    2. Assessment:
      1. Typically presents with sudden onset in child who is healthy, thriving.
      2. Pain: paroxysmal, colicky, abdominal, with intervals when the child appears normal and comfortable.
      3. Stools: "currant-jelly," bloody, mixed with mucus.
      4. Vomiting due to intestinal obstruction.
      5. Abdomen: distended, tender, with palpable, sausage-shaped mass in right upper quadrant (RUQ).
      6. Late signs: fever, shock, signs of peritonitis as the compressed bowel wall becomes necrotic and perforates.
    3. Analysis/nursing diagnosis:
      1. Fluid volume deficit related to diarrhea and vomiting.
      2. Pain related to bowel-wall ischemia, necrosis, and death.
      3. Risk for injury/infection related to bowel-wall perforation and peritonitis.
      4. Knowledge deficit regarding the disease, medical or surgical treatment, and prognosis.
    4. Nursing care plan/implementation:
      1. Goal: assist with attempts at medical treatment.
        1. Explain to parents that a barium enema will be given to the child in an attempt to reduce the telescoping through hydrostatic pressure (succeeds in 75% of cases).
        2. Stress that if this treatment is not successful, or if perforation of the bowel wall has already occurred, surgery will be necessary.
        3. If medical treatment is apparently successful, monitor child for 24 to 36 hours for recurrence before discharge.
      2. Preoperative and postoperative goals: same as for adult with major abdominal surgery (see Chapter 6. Physiological Integrity).
      3. Goal: discharge teaching to prepare parents for care of the child at home.
        1. Stress that recurrence is rare (10%) and most often occurs within the first 24 to 36 hours after reduction.
        2. Other teaching: same as for adult going home after bowel surgery (see Chapter 6. Physiological Integrity).
    5. Evaluation/outcome criteria:
      1. Infant tolerates medical-surgical treatment and completely recovers.
      2. Parents verbalize confidence in ability to care for infant after discharge.