NURSING CARE OF THE HIGH RISK NEWBORN TO MATURITY

BY: Criselda N. Olaguer, R.N,M.N

PROBLEMS RELATED TO NUTRITION

MOUTH
CLEFT LIP AND PALATE

ESOPHAGUS
ESOPHAGEAL ATRESIA TRACHEOESOPHAGIAL FISTULA

STOMACH
CHALASIA HYPERTROPIC PYLORIC STENOSIS

INTESTINES
INTUSSUSCEPTION HIRSPRUNGS DISEASE

ANUS
IMPERFORATE ANUS

CLEFT LIP AND PALATE CLEFT LIP

CLEFT PALATE

CLEFT LIP AND PALATE

1.Nonunion of tissue and bone of upper lip and hard/soft palate during embryonic development 2. Familial disorder associated with congenital abnormalities 3. Cleft Palate : failure of the bone and tissues to fuse results in a communication between mouth and nose.

4. Assessment Findings a. Facial abnormality visible at birth, cleft lip or palate or both b. Difficulty sucking, inability to form airtight seal around the nipple c. Nasal speech d. lack of normal dental function and appearance e. Formula escapes through the nose in infants with cleft palate f. Predisposition to infection because of free communication between mouth and nose g. Possible difficulty swallowing h. Abdominal distention due to swallowed air

There are generally three different kinds of clefts:

1. Cleft lip without a cleft palate 2. Cleft palate without a cleft lip 3. Cleft lip and cleft palate together In addition, clefts can occur on one side of the mouth (unilateral clefting) or on both sides of the mouth (bilateral clefting). More boys than girls have a cleft lip, while more girls have cleft palate without a cleft lip. Because clefting causes specific visible symptoms, its easy to diagnose. It can be detected through a prenatal ultrasound. If the clefting has not been detected prior to the babys birth, its identified immediately afterward.

NURSING DIAGNOSIS AND NURSING INTERVENTIONS FOR PREOPERATIVE CLEFT LIP REPAIR
5. Nursing Diagnosis a. Altered Nutrition : less than body requirements related to eating difficulties. b. Risk for infection related to open wound created by malformation. c. Impaired Adjustment to infant related to malformation and special care needs. d. Risk for Aspiration related to tongue and palate deformities e. Knowledge deficit related to home management of infant with cleft malformation.

MEDICAL MANAGEMENT
1.Speech therapist 2.Dentist and orthodontist 3.Audiologist,otolaryngologist,pediatrician (these children are prone to otitis media and possible hearing loss) 4.Surgical correction a. Timing varies with severity of defect; early correction helps to avoid speech defects. b. Cheiloplasty: correction of cleft lip 1. Goal is to unite edges to allow lips to be both functional and cosmetically attractive. 2. Usually performed approximately age 2 months (to prepare gums for eruption of teeth) when child is free from respiratory infection. 3. Steri-Strips or Logan bar usually used to take tension off suture line. c. Cleft palate repair is usually not done until age 18 months in anticipation of speech development. 1. Between lip and palate repair child is maintained on normal nutritional and respiratory status; also maintains normal immunization schedule. 2. Child should be weaned and able to take liquids from a cup before palate repair.

NURSING INTERVENTIONS:PREOP CLEFT LIP REPAIR

1.

2.
3. 4. 5. 6. 7. 8.

Feed in upright position to decrease chance of aspiration and decrease amount of air swallowed. Burp frequently; increased swallowed air causes abdominal distension and discomfort. Use large-holed nipple; press cleft lip together with fingers to encourage sucking and to strengthen muscles needed later for speech. If infant unable to suck, use a rubber-stripped syringe and drip formula into side of mouth. Administer gavage feeding as ordered if necessary. Finish feeding with water to wash away formula in palate area. Provide small, frequent feedings. Provide emotional support for parents/family. a. Demonstrate benefits of surgery by showing before and after pictures. b. Reinforce that disorder is not their fault and that it will not affect childs life span or mental ability.

NURSING CARE: POST-OP CLEFT REPAIR

1. Maintain patent airway (child may appear to have respiratory distress because of closure of previously open space; adaptation occurs quickly). 2. Asses color; monitor amount of swallowing to detect hemorrhage. 3. Do not place in prone position or with pressure on cheeks; avoid any pressure or tension on suture line. 4. Avoid straining on suture line by anticipating childs needs. a. Prevent crying b. Keep child comfortable and content. 5. Use elbow restraints or pin sleeves of shirt to diaper to keep childs hands away from suture line. 6. Resume feeding as ordered. 7. Keep suture line clean; clean after each feeding with saline, peroxide, or water to remove crusts and prevent scarring. 8. Provide pain control/relief.

NURSING INTERVENTIONS: PRE- OP CLEFT PALATE REPAIR

1. 2.

Prepare parents to care for child after surgery. Instruct concerning feeding methods and positioning.

NURSING INTERVENTIONS: POST-OP CLEFT PALATE REPAIR 1. 2. 3. Position on side for drainage of blood/ mucus. Have suction available but only use in emergency. Prevent injury or trauma to suture line. Use cups only for liquids; no bottles. Avoid straws, utensils, popsicle sticks, chewing gum. Provide soft toys. Use elbow restraints. Provide liquid diet initially, then progress to soft before returning to normal. Give water after each feeding to clean suture line. Hold and cuddle these babies to help distract them.

ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA

CONGENITAL ESOPHAGEAL ATRESIA absence of usual body opening represents a failure of the esophagus to develop as a continuous passage, instead, it ends as a blind pouch. It is characterized by incomplete formation of the esophagus. TRACHEOESOPHAGEAL FISTULA passage between organs represents an abnormal opening between the trachea and esophagus. EA and TEF can occur separately or together.

ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA 1. Open connection between trachea and esophagus This deformity is found more often in low birth weight or premature infant and is associated with polyhydramnios in the mother and multiple congenital anomalies. Medical Management: a. Drug therapy: antibiotics b. Surgery: Palliative Gastrostomy for feeding Corrective End to end anastomosis Diagnostics: Flouroscopy with contrast

2.

3.

A blind pouch of the esophagus with a fistula from the lower esophageal segment connecting with the trachea; this is the most common type and accounts for 86 percent of cases.

An absence of a portion of the esophagus with no fistula to the trachea; each end is a blind pouch. This is more rare, accounting for 8 percent of cases, and is a true esophageal atresia with no fistula.

Here, the esophagus is complete (but may have a narrowed portion) with a fistula to the trachea. This is an Htype case and accounts for 4 percent

A blind pouch with a fistula to the trachea from the upper portion of the esophagus accounts for 1 percent of cases.

Two fistula connections to the trachea from the upper and lower portions of the esophagus accounts for 1 percent.

Normal development of the esophagus and trachea.

ASSESSMENT
ESOPHAGEAL ATRESIA a. History of polyhydramnios in mother (from infants inability to swallow and excrete amniotic fluid) b. Inability to pass an NG tube. c. Increased drooling and salivation. d. Immediate regurgitation of undigested formula/milk when fed. e. Intermittent cyanosis from choking on aspirated secretions. TRACHEOESOPHAGEAL FISTULA a. Normal swallowing but some food/mucus crosses fistula, causing and intermittent cyanosis. b. Distended abdomen from inhaled air crossing fistula into stomach. c. Aspiration pneumonia from reflux of gastric secretions into the trachea. ESOPHAGEAL ATRESIA WITH TEF a. All findings for esophageal atreasia b. Abdominal distention and aspiration pneumonia from gas and reflux of gastric acids into the trachea.

DIAGNOSTIC EVALUATION
1. Maternal history of polyhydramnios helpful clue 2. Flat plate x-ray of abdomen and chest reveals presence of gas in the stomach and chest. 3. X-ray with radiopaque catheter; radiopaque contrast medium never used because of aspiration

PREOPERATIVE NURSING CARE

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2. 3.
4. 5. 6. 7. 8.

Position newborn with the head and chest elevated to prevent reflux of gastric juices into the tracheobronchial tree. Regular suctioning. Put in incubator with high humidity to aid in liquefying secretions and thick mucus. Administer oxygen PRN. Assist in bougie treatment (elongation of proximal pouch using a mercury weighted dilator or firm catheters inserted briefly each day). Give antibiotics as ordered to prevent or treat associated pneumonitis. Monitor IV or hyperalimentation. Observe closely for; Vital signs, respiratory behavior Amount of secretions Abdominal distention Skin color

SURGERY
1. Primary repair esophageal anastomosis and division of fistula 2. Gastrostomy and cervical esophagostomy temporarily until infant gains weight. Staging repeated operations separated by periods of time, waiting for growth is the accepted philosophy of treatment.

POSTOPERATIVE CARE
1. Observe for signs of stricture at the anastomosis site; refusal to feed; pronounced coughing; dysphagia; atelectasis, pneumonia. 2. Maintain patent airway Suction PRN mark catheter to determine how far it can be inserted without disturbing anastomosis site Change position frequently and stimulate baby to cry but avoid hyperextension of the neck to prevent tension on the suture line. 3. Maintain adequate nutrition oral feeding started 6 to 14 days post-op. Low residue diet to keep stool soft Feed slowly in upright position to allow time for swallowing. 4. Oral hygiene to prevent bacterial growth. 5. Allow infant to suck on a pacifier to meet psychological and physiological needs. 6. Encourage parental participation to promote strong parentalinfant bonding.

CHALASIA
ALTERNATIVE NAMES GASTROESOPHAGEAL REFLUX IN INFANTS, GASTROESOPHAGEAL REFLUX DISEASE (GERD, ACID REFLUX), CARDIO ESOPHAGEAL RELAXATION

CHALASIA
Is the movement of stomach contents up to the esophagus towards the mouth, rather than down through the digestive system (peristalsis). The liquid can inflame and damage the lining of the esophagus although this occurs in a minority of patients. The regurgitated liquid usually contains acid and pepsin that are produced by the stomach. (PEPSIN is an enzyme that begins the digestion of proteins in the stomach). The reflux liquid may also contain bile that has backed-up into the stomach from the duodenum. (The duodenum is the first part of the small intestine that attaches to the stomach.) Acid is believed to be the most injurious component of the refluxed liquid. Pepsin and bile may also injure the esophagus, but their role in the production of esophageal inflammation and damage (esophagitis) is not as clear as the role of acid.

PATHOPHYSIOLIGY
Gastroesophageal reflux is both a normal physiologic phenomenon that occurs in the general population and a pathophysiologic phenomenon that can result in mild to severe symptoms. Gastroesophageal reflux disease can be described as any symptomatic clinical condition or change in tissue structure that results from the reflux of stomach or duodenal contents into the esophagus. On the 3rd and 10th day of life, the cardiac sphincter muscle fail to function, causing it to be relaxed and constantly patent. It is also caused by overdistension of stomach by gas or overfeeding. It results in local irritation of the lining of the esophagus from backflow of acidic gastric contents.

ETIOLOGY
The etiology of GERD can be attributed to such factors as transient lower esophageal sphincter (LES) relaxations, decreased LES resting tone, delayed stomach emptying, ineffective esophageal clearance, and diminished salivation. Other contributing factors include the potency of the refluxed material, and the inability of the esophageal tissue to resist injury and repair itself. Factors that may exacerbate the symptoms of GERD in some patients include smoking, caffeine, chocolate, fatty foods, overeating with gastric distension, tight clothing, the presence of a hiatal hernia, and certain medications. A careful history will often show what factors are important for individual patients. While avoidance of exacerbating factors may be helpful, there is relatively little data supporting the efficacy of lifestyle modification alone for the treatment of GERD.

CLINICAL MANIFESTATIONS
1. 2. 3. 4. 5. 6. 7. 8. 9. 1. 2. 3. 4. Excessive vomiting during the first few weeks of life. Extremely forceful vomiting Chronic cough Wheezing Apnea or breath holding spells Slow growth Excessive crying as if in pain Weight loss Irritability

DIAGNOSTIC EVALUATION
Muscle tone of cardiac sphincter reduced Esophageal pH: contents acidic Fluoroscopy: presence of refluxed contrast material not quickly cleared or repeated reflux. There may be history of episodes of aspiration pneumonia.

NURSING INTERVENTIONS
1. Position with head elevated 30 45 degrees. 2. Give small, frequent feedings with adequate burping. 3. Thickened feeding (formula + cereals) because they are less easily vomited. 4. Provide client teaching and discharge planning: teach parents how to position and feed infants.

HYPERTROPHIC PYLORIC STENOSIS

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2. 3. 4.
5.

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Hypertrophy (thickening) of the pyloric sphincter causing stenosis and obstruction. Etiology: unknown Incidence: Caucasian, firstborn, full term boys Medical MGT: Non-invasive: thickened feeding Surgery:Pyloromyotomy Assessment: - olive size bulge (right rib cage) - vomiting that becomes projectile as the obstruction increases - peristaltic waves during and after feeding - FTT - dehydration Diagnostic test: - Upper GI series (narrowed diameter of the pylorus) - Na, K Cl decreased - metabolic alkalosis

Diagram of normal stomach and pylorus. Note the cross-section showing normal pyloric opening.

Diagram of stomach with pyloric stenosis. Note the cross-section showing how the pyloric opening is very narrowed.

PATHOPHYSIOLOGY
Full-thickness biopsies demonstrate both hypertrophy and hyperplasia of the circular muscle layer of the pylorus. No clear pathophysiologic sequence or etiology has been described. Proposed theories include, among others, abnormal circular muscle innervation, immature ganglion cells, decreased nitric-oxide stimulation of muscle fibers, immature ganglion cells, and abnormal levels of gastrin. The gastric outlet obstruction due to the hypertrophic pylorus impairs emptying of gastric contents into the duodenum. As a consequence all ingested food and gastric secretions can only exit via vomiting which can be of a projectile nature. The vomited material does not contain bile because the pyloric onstruction prevents entry of duodenal contents (containing bile) into the stomach. This results in loss of gastric acid (hydrochloric acid). The chloride loss results in hypochloremia which impairs the kidney's ability to excrete bicarbonate. This is the significant factor that prevents correction of the alkalosis. A secondary hyperaldosteronism develops due to the hypovolaemia. The high aldosterone levels causes the kidneys to: avidly retain Na+ (to correct the intravascular volume depletion) excrete increased amounts of K+ into the urine (resulting in hypokalaemia). The body's compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2.

CLINICAL MANIFESTATIONS
1. Olive-size bulged under right rib cage. 2. Vomiting as obstruction increases, vomiting becomes more forceful and projectile. - Vomiting does not contain bile (bile duct is distal to the pylorus). 3. Peristaltic waves during and after feeding (look like rolling balls under abdominal cavity). 4. Failure to thrive, even though infant appears hungry after vomiting. 5. Dehydration: sunken fontanels, poor skin turgor, decreased urinary output

DIAGNOSTIC TEST
1. Upper GI series (narrowed diameter of the pylorus) 2. Na, K, Cl decreased 3. Hematocrit increase 4. metabolic alkalosis

MEDICAL MANAGEMENT
1. 2. Noninvasive thickened feedings. Surgery pyloromyotomy (Fredet-Ranstedt procedure) dividing the muscle of the pylorus to open up the gastric outlet). This is a relatively straightforward surgery that can possibly be done through a single incision (usually 3-4 cm long) or laparoscopically (through several tiny incisions), depending on the surgeon's experience and preference.

Diagram of stomach after repair of pyloric stenosis. Note (in the cross-section) how an incision has been made in the muscle, enlarging the pylorus and relieving the obstruction.

NURSING INTERVENTIONS
PREOPERATIVE
1. Administer replacement fluids and electrolytes as ordered. 2. Prevent vomiting.
Give thickened feedings. Keep in high fowlers position. Place on right side after feedings. Minimize handling. Record strict I and O, daily weights, and urine specific gravity.

3. Observe for sumptoms of aspiration of vomitous.

NURSING INTERVENTIONS
1. 2. 3. 4. 5. POSTOPERATIVE Advance diet as tolerated. Place on right side after feeding. Monitor strict I and O, daily weights. Observe incision for signs of infection. Provide client teaching and discharge planning concerning feeding and positioning of infant.

INTUSSUSCEPTION
Intussusception is a condition where part of the intestine (bowel) folds into itself like a telescope. The telescoping may block the bowel and its blood supply, which can result in damage to the bowel. Intussusception often involves both small and large bowels. It is the most common cause of bowel obstruction (blockage) in children. The part of the intestine that folds inward may lose some or all of its blood supply. This section of the intestine becomes swollen and painful. If intussusception is not treated, the intestine may become blocked. Intussusception usually happens in young children. It is rare in adults.

ETIOLOGY
In infants, the causes of intussusception are unknown, although there are some theories about why it occurs. Because intussusception is seen most often in spring and fall, this seems to suggest a possible connection to the kinds of viruses that children catch during these seasons, including upper respiratory infections. In some cases, intussusception may follow a recent bout of gastroenteritis. Gastrointestinal infections may cause swelling of the infection-fighting lymph tissue that lines the intestine, which may pull one part of the intestine into the other. Intussusception is most common around the age that infants are being introduced to solid foods. It has been suggested that the introduction of new foods may also cause some swelling of the lymph tissue in the intestine, increasing the chance of developing intussusception. It is also associated with cystic fibrosis and celiac disease. Usually, when an adult or a child older than 3 develops an intussusception, its often the result of enlarged lymph nodes, a tumor, or a polyp in the intestine.

CLINICAL MANIFESTATIONS
Children with intussusception have intense abdominal pain, which often begins so suddenly that it causes loud, anguished crying and causes the child to draw the knees up to the chest. The pain is usually intermittent, but recurs and become stronger. As the pain subsides, a child with an intussusception may stop crying and seem fine. Other common symptoms include Abdominal swelling or distention Passing stools mixed with blood and mucus, known as CURRANT JELLY STOOL( 60% of infants with an intussusception will pass currant jelly stool) Vomiting Vomiting up bile, a bitter tasting fluid secreted by the liver thats often golden brown to greenish in color Lethargy (drowsiness or sluggishness) Shallow breathing sausage-shaped lump in the upper right side of the belly. Grunting As the illness progresses, a child will become progressively weaker and may develop a fever and appear to go into shock. Symptoms of shock include lethargy, rapid heartbeat, weak pulse, low blood pressure, and rapid breathing.

DIAGNOSTIC EVALUATION
1. 2. Abdominal X-ray a diagnostic test which may show intestinal obstructoin. Barium enema 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. An xray of the abdomen shows strictures (narrowed areas), obstructions (blockages), and other problems. On some occasions, the pressure exerted on the intestine while inserting barium will help the intestine to unfold, correcting the intussusception.

MEDICAL MANAGEMENT
1. 2. BARIUM ENEMA to reduce telescoping by hydrostatic pressure. SURGERY An operation is necessary for intussusception that does not resolve with barium enema, or for those who are too ill to have the diagnostic procedure. Under anesthesia, the surgeon will make an incision in the abdomen, locate the intussusception, and pushed the telescoped sections back into place. The intestine will be examined for damage, and, if any sections are not working correctly, they will be removed. If there is damage to the intestine and the section removed is small, the two sections of healthy intestine will be sewn back together. If the injured section of intestine is large, a significant amount of intestine may be removed. In these case, the parts of the intestine that remain after the damaged section is removed cannot be attached to each other surgically.

NURSING INTERVENTIONS
1. Provide routine pre and post operative care for abdominal surgery. 2. Monitor for fluid and electrolyte imbalance and intervene as needed. 3. Monitor for peritonitis and intervene as needed.

HIRSCHSPRUNGS DISEASE
1. Absence of autonomic parasympathetic ganglion cells in a portion of the large colon.

2. Results in decrease motility and signs of function obstruction.

3. Familial disease : More common in boys than in girls. Often associated with Downs Syndrome. 4. When stool enters the affected part of the colon, lack of peristalsis causes it to remain there until additional stool pushes through, colon dilates as stool is impacted. 5. Medical Management : Stool Softeners Isotonic Enemas Low Residue Diets Surgery : Loop or Double Barrel Colostomy

6. Assessment Findings a. Failure or delay in passing meconium b. Abdominal distention; Failure to pass stool c. Temporary relief following digital rectal exam. d. Loose stools, only liquid can get around impaction ( ribbon like stools ) e. Nausea, anorexia, lethargy f. Possible bile stained or fecal vomiting g. Loss of weight and failure to grow h. Volvulus I. Diagnostic Test : Rectal Biopsy

1.Administer Enemas as ordered a. Use mineral oil or isotonic saline. b. Do not use tap water or soap suds enemas in infants. c. Use volume appropriate to weight of infant. a. Infants : 150-200 ml. b. Children : 25-500 ml. d. Do not treat the loose stools, the child is actually constipated. e. Administer TPN as ordered. f. Provide low reside diet. g. Provide patient teaching on low residue diet and colostomy care.

FAILURE TO THRIVE
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3.

A condition in which a child fails to gain weight and is persistently less than 5th percentile on growth charts. When related to non organic cause: disrupted maternal-child relationship. It is important to rule out other pathology first ( absorption or hormonal problems) before a disorder can be diagnosed as FTT. Assessment Finding: - sleep disturbances - rumination - history of parental isolation and social crisis with inadequate support system -PE: delayed growth (decreased vocalization, decreased interest in environment), characteristic postures (floppy or stiff, resist cuddling) -disturbed maternal-infant interaction (feeding techniques, amount of stimulation, ability to respond to infants cues.

FAILURE TO THRIVE
4. Nursing Intervention: 1. Provide consistent care. 2. Teach parents positive feeding techniques: - provide a quiet environment - follow childs rhythm of feeding - maintain face to face posture with child - talk to child encouragingly during feeding 3. Involve parents in care 4. Refer to appropriate community agencies.

IMPERFORATE ANUS
Congenital malformation caused by abnormal fetal development. Associated with fistula formation to rectum or vagina and other congenital anomalies. Surgical correction performed in stages with completion at about 1 yr. May need temporary colostomy.

IMPERFORATE ANUS
Medical Management a. Manual Dilatation b. Surgery: anoplasty (reconstruction of the anus). c. Prophylactic antibiotics Assessment findings a. No stool passage within 24 hours of birth. b. Meconium stool from inappropriate orifice. c.Inability to insert thermometer. Nursing Diagnosis a. Risk for injuries of infant before surgery. b. Risk for infection related to surgical incision of anoplasty. c. Risk for impaired skin integrity related to ostomy. d. Risk for fluid volume deficit related to restricted intake. e. Family coping: Potential for growth related to increased needs of infant. c. Risk for injury after definitive repair surgery.

IMPERFORATE ANUS
Types of Imperforate Anus: 1. Anal Stenosis 2. Imperforate anal membrane 3.Anal agenesis 4.Rectal agenesis 5. Recto vaginal fistula Nursing Interventions: a. If suspected do not take anal temperature. c. After surgery, prevent infection. Keep anal incisional area as clean as possible. d. after surgery, use side lying position or have the child lie in prone with hips elevated.

IMPERFORATE ANUS

IMPERFORATE ANUS

IMPERFORATE ANUS

IMPERFORATE ANUS