Khyber Medical University Post Graduate College of Nursing Peshawar 10-11-2009


Birth defect is a widely-used term for a congenital malformation, i.e. a congenital, physical anomaly which is recognizable at birth, and which is significant enough to be considered a problem. According to the (Centers for Disease Control and Prevention, CDC) most birth defects are believed to be caused by a complex mix of factors including genetics, environment, and behaviors, though many birth defects have no known cause.

AIM: To share the knowledge among the
participants about the Cogenital disorders of G.I system.
OBJECTIVES: At the end of presentation the participants will be able to:

Describe the normal characteristics of pediatric G.I system. Define congenital disorder. Enlist the congenital disorders of G.I system. Explain different congenital abnormalities of G.I system with causes, pathophysiology and signs & symptoms. Describe the nursing care plan for each disorder.

  


Characteristics of the pediatric GI System

Peristalsis occurs within 2 ½ to 3 hours in the neonate and extends to 3 to 6 hours in older infants and children. The stomach capacity of the neonate is 30 to 60 ml, which gradually increases to 200 to 350 ml by age 12 months and 1,500 ml in the adolescent.

Up until 4 to 8 weeks, the neonatal abdomen is larger than the chest and the musculature is poorly developed. The sucking and extrusion reflexes persist until 3 to 4 months. Saliva production begins at age 4 months and aids in the process of digestion.

Spit-ups are frequent in the neonate because of the immature muscle tone of the lower esophageal sphincter (LES) and the low volume capacity of the stomach.

Increased myelination of nerves to the anal sphincter allows for physiologic control of bowel function, usually at about age of 2 years.






glycogen storage capacity makes the infant prone to hypoglycemia.

From ages 1 to 3 years, the composition of intestinal flora becomes more adultlike and stomach acidity increases, reducing the number of GI infections.

What is congenital disorder?

Congenital disorder involves defects in or damage to a developing fetus. It may be the result of genetic abnormalities, the intrauterine (uterus) environment, errors of morphogenesis, or a chromosomal abnormality.

Any condition acquired during development in the uterus and not through heredity.

Describes a disease or condition with which someone is born.


Congenital G.I Disorders
         

Cleft lip & cleft palate. Esophageal atresia or transesophageal fistula. Gastroesophageal Reflux. Omphalocele. Hirschprung’s Disease. Intussusception. Pyloric Stenosis. Diaphragmatic Hernia. Celiac Disease. Imparforate anus.

Cleft Lip

Cleft lip and palate results when fusion involving the first brachial arch faills to to take place uring embryonic devellopment. failure of fusion of maxillary and median nasal process hereditary unilateral/bilateral males

Tx: surgery –

Cheiloplasty; usually 1-3 mos

Rule of 10’s10 wks, 10 lbs, Hgb 10 gm/dl

  


Cleft Palate
   

midline opening of palate usually with Cleft lip Female surgery 6-18 mos - allows anatomic changes; (ie, formation of palatine arch and tooth buds)


Pathophysiology of cleft lip & palate

Cleft lip
Varies from a notch in the lip to complete separation of the lip into the nose. Failure of maxillary process to fuse with nasal elevations on frontal prominence; normally occurs during 5th and 6th week of gestation. Merge of upper lip at midline complete b/w 7th and 8th week of gestation.

Cleft palate
Failure of mesodermal masses of lateral palatine process to meet and fuse; normally occurs b/w 7th and 12th week of gestatioon.

• s/s:



Nursing Dx:

Risk for imbalanced nutrition, less than body requirements R/T feeding problems

Risk for ineffective airway clearance R/T oral surgery Risk for infection during post op period



Nursing Care

Adequate nutrition; sips of fluid btw feeding in upright position; use rubber tipped syringe; Burp Prevent ear and upper respiratory tract infection Address body image and speech concerns Reassurance to parents


monitor respiratory distress d/t edema, hemorrhage Suction mucus and blood gently dropper feeding 1st 3 weeks; regular feeding after Anticipate needs and position to prevent tension on sutures
• Position side lying in cleft lip; prone in cleft palate


Esophageal Atresia with Tracheoesophageal Fistula

Esophgeal atresia is a failure of the esophagus to form a continuous passage from the pharynx to the stomach during embryonic development, Tracheo-esophageal fistula is the abnormal sinous connection b/w the trachea and esophagus. maternal hydramnios and prematurity

coughing, choking, cyanosis, dyspnea, excessive secretion, abdominal distention

Ba swallow


Nursing Dx:

Risk for imbalanced nutrition, less than body requirements R/T inability for oral intake

Risk for infection R/T aspiration or seepage of stomach contents into lungs

Risk for impaired skin integrity R/T gastrostomy tube insertion site

Nursing care
    

Suction regularly Elevate the head Gastrostomy feeding hydration O2

1. 2. 3. 4. 5.

Observe for respiratory distress Proper positioning –avoid hyperextension of neck Continue suction Prevent wound infection Provide pacifier

Post Gastrostomy


Hirschprung’s Disease

Aganglionic megacolon. Absence of parasympathetic ganglionic cells in a segment of the colon (usually at the distal end of the large intestine: rectosigmoid colon) Lack of innervation to a bowel segment causes a lack of, or alteration in, peristalsis in the affected part

Male predominance


Familial, congenital defect Commonly exists with other congenital anomalies, particularly Down’s syndrome (trisomy 21) and anomalies of the urinary tract

Absence of or reduce number of ganglion cells in the intetinal muscle wall, distal end of colon. No paristalsis in the affected portion. Affected portion is narrow > proximal end dialated. Abddominal ddistention & constpation results.

 

    

Constipation Ribbonlike stools Failure to pass meconium or stool abdominal distention Bile stained or fecal vomiting

Severe Constipation Enterocolitis – severe diarrhea, Hypovolemic shock, Death


Rectal biopsy provides definitive diagnosis by showing the absence of ganglion cells Ba enema – shows distention of the colon; spasm and a narrowed lumen in the affected bowel Abdominal X-rays show distention of the colon and air-fluid levels; No air is seen in the rectum

Tx: 1: primary resection of aganglionic segment.
2: colostomy, surgery: To decompress the colon, a temporary colostomy or ileostomy may be necessary



Nursing Dx:
 

Constipation R/T reduced bowel function Imbalanced nutrition, less than body requirements R/T reduced bowel function

Nursing Care Preop
1. Administer isotonic enemas: Normal saline solution or mineral oil to evacuate the bowel Daily enemas with 0.9% NaCl Don’t administer tap water. * Tap/hypotonic water will cause cardiac congestion or cerebral edema 2. Minimal residue diet with vitamin supplementation 3. Position semi fowlers to relieve dyspnea from distended abdomen 4. pacifier

After colostomy or Ileostomy

Monitor fluid intake and output (ileostomy will likely cause excessive electrolyte loss) Keep the area around the stoma clean and dry; use colostomy or ileostomy appliances to collect drainage Monitor for return of bowel sounds to begin diet

2. 3.

1. 2. 3. 4.

Observe for abdominal distention Small frequent feedings after NGT removal Colostomy care Assist parents to cope with children’s feeding problems


Is the invagination or telescoping of the portion of the intestine into an ajucent, more distal section of the intestine.410 months

S/S: Intense abdominal pain,
vomiting, blood in stool “currant jelly”, abdominal distention (sausage shaped mass)

 

Dx: sonogram “coiled spring” Tx: Ba enema (reduction by
hydrostatic pressure), surgery


Nursing Dx:

Pain R/T abnormal abdominal peristalsis Risk for deficient fluid volume R/T bowel obstruction


Nursing Care
1. 2. 3. 4. 5. 6. 7.

Provide comfort measures - pacifier for infants. NPO. Adequate hydration via IV therapy. Insertion of N.G tube to deflate stomach. Observe patient regularly for any complication. Promote parent-infant bonding. Post op nursing care.


Pyloric Stenosis

Hypertophy/hyperplasia of pyloric sphincter Males S/S: nonbillous vomiting, s/s of dehydration and wt loss, abdominal distention, “olive” sized mass RUQ, visible peristalsis

 

Dx: xray-”string sign”,
USG, endoscopy

Tx: surgery

Nursing Dx:

Risk for deficient fluid volume R/T inability to retain food

Risk for infection at site of surgical incision R/T danger of contamination from feces R/T proximity of incision to diaper area


Nursing care
  

Hydration Pacifier may give thickened feedings on upright position then NPO just before surgery Monitor I and O, weight, and vomiting


Dropper feeding 4-6 hrs after surgery 45 min- 1 hr duration; oral rehydration soln then half strength breastmilk/formula at 24 hr interval Side lying position Monitor weight and return of peristalsis Wound care Pacifier for oral needs

2. 3. 4. 5.

Hiatal or Diaphragmatic Hernia
 

Part of stomach protrudes through the esophageal hiatus of the diaphragm into thoracic cavity. OR Herniation of intestinal content into the thoracic cavity.

Predisposing factors include:
• • • • • Congenital weakness Increased intra-abdominal pressure Increased age Trauma Forced recumbent position

Diaphragmatic Hernia
Left side S/S: respiratory difficulty, cyanosis, retractions, (-) breath sounds affected side, scaphoid abdomen

 

Dx: CXR, Upper

Tx: Diaphragmatic
Hernia repair



Nursing Dx:
Risk for ineffective airway clearance R/T displaced bowel Risk for imbalanced nutrition, less than body requirements, R/T NPO status


Nursing Care
  

Mechanical ventilation Elevate head Low intermittent suction

1. 2. 3. 4. 5. 6. 7.

NICU care. Semi-fowlers Wound care Maintain warm, humidified environment– lung fluid drainage Suction prn Chest physio NPO – prevent pressure on diaphragm

Celiac Disease/Gluten sensitive enteropathy/Celiac sprue

Celiac disease also called gluten-induced enteropathy, is an inborn error of metabolism Characterized by poor food absorption and sensitivity or intolerance to gluten (a protein found in grains : wheat, rye, oats, barley) Usually becomes apparent between ages 6-18 months Causes: Gluten intolerance Immunoglobulin deficiency


A decrease in the amount and activity of enzymes in the intestinal mucosal cells causes the villi of the proximal small intestine to atrophy and decreases intestinal absorption. Malabsorption of fat-soluble vitamins (????), minirals & some protiens & carbohydrates. Complications

 

Lymphoma of the small intestine Impair growth, succeptibility


steatorrhea (????) because of inability to absorb ____? chronic diarrhea anorexia malnutrition; weight loss failure to thrive coagulation difficulty from the malabsorption of fat soluble vitamins irritability anemic (Fe deficiency) abdominal pain and distention

CELIAC CRISIS: constitutes acute medical emergency and threat to life acute vomiting and diarrhea,dehydration & acidosis Dx:clinical; IgA antigliadin Ab ↑↑, gluten free diet Mx: gluten free diet, vitamin & Fe supplementation NDx: Imbalance nutrition: Less than body requirements R/T impaired absorption

Nursing Intervention:
  

nutrition counseling Eliminate gluten from the diet: avoid cereals, bread, cake, cookies, spaghetti, pizza, instant soup, some chocolates Give the child: corn and rice products, soy and potato flour, breast milk or soy- based formula, and fresh fruits. Replace vitamins and calories; give small, frequent meals. Monitor for steatorrhea- its disappearance is a good indicator that the child’s ability to absorb nutrients is improving.

Imperforate Anus

Is the congenital presence of an intact anal membrane or internal blind pouch of the bowel.


  

An arrest in embryonic development of the anus, lower rectum and urogenital tract at the 8th week of embryonic life. Fistula is more likely to be formed. Others anomalies likely, especially TEF. Cause unknown.

Types ano-rectal malformations
   

Anal stenosis. Imperforate anal membrane. Anal agenesis. Rectal agenesis.

   

No anal opening. Absence of meconium stool. Green-tinged urine---if fistula is present. Progressive abdominal distention.


Treatment for

Low-type imperforate anus.
• Local dilatation of fistula. • Definitive repair. • Anoplasty.

High-type imperforate anus.
• Colostomy for decompression. • Definitive pull-through surgery, deferred untill 1 year of age.

Complications: before & after surgery

Nursing Assessment
   

Physical examination of newborn. No anal opening. Presence of perinial fistula. Presence of meconium-stained urine.

Pre-op nursing care:
     

With-hold feeding, note any vomiting (COCA). N.G intubation. Measure abdoominal girth. Keep the baby warm. Keep fistula area clean. Observe for any compliction.


Post-op nursing diagnosis

Risk for infection R/T surgical incision of anoplasty Risk for impaired skin integrity R/T ostomy Risk for fluid volume deficit R/T restricted intake


Poost-op nursing care
Depending on the location, type of imperforate anus, sex of child.  Prevent infection of suture line.  Expose perinium to air.  Proper positioning, prone or side to side.  Observe for redness, drainage, poor healing.  Preventing skin breakdown, (ostomy care).  Maintaining fluid & electrolyte balance.  Start oral feed as ordered (usually within hours after of anoplasty).  Moniter for return of paristalsis.  Parentral fluids untill oral feed not started.  Observe for abdominal distetion, bleedding from 51 perinium


Sandra Nettina: The Lippincott Manual of Nursing Practice, 6th edition, 1996 Lipponcott –Raven Publishers


Thank you!

Sign up to vote on this title
UsefulNot useful