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Complication: of:

Respiratory distress during the 1st 48 hours because

Swelling of tongue, mouth and nostrils put downward pressure on the chin to increase air passage Increased respiratory secretions Difficulty in adjusting to a smaller airway

Nursing Care: Incidence: Cleft lip with or without palate is more frequent among males; but cleft palate alone is more frequent among females. Etiology: primarily genetic therefore there is a greater than average possibility of having other children with same anomaly (i.e. a mother who has had a child with a cleft lip/palate is more likely to have another child with the same condition). Types: 1. Unilateral 2. Bilateral 3. Midline rare Minimize reasons for crying because it adds to an already irritable and fussy child Put inside mist tent to liquefy respiratory secretions Can lie only his back Elbow restraints should be used at all times to prevent him from putting his hands, or other objects into his mouth Logans bow is taped after surgery to protect the suture line from blows, pressure and sucking (never let the child suck on the apparatus) o Moisten OS with sterile NSS o OS is removed before feeding and cleansing o After feeding, the suture line is cleansed with cotton tipped applicator which has been dipped in one half strength hydrogen peroxide in order to prevent crusts which cause uneven healing and infection, leaving ugly scars. Feedings formula resumed around 3-4 weeks post-op. A rubber tipped medicine dropper which is placed to the side of the mouth away from the suture line is used to avoid tension on the suture line.

Associated Problems: 1. 2. Feeding the child is not able to maintain closed suction on the nipple Upper Respiratory Tract Infection (URTI) because they breathe through the mouth Ear Infection because the pharyngeal opening of the Eustachian tube is an abnormal position Speech defects Dental malformation Body image SURGERY

Postoperative Palatoplasty: Complication: Nursing Care: Position on abdomen to facilitate drainage of blood and mucus (Remember: suction is never done) Use of mist tent is recommended Elbow restraints are applied Sucking, blowing, talking, laughing or putting objects into the mouth is not allowed Feedings o Paper cups are used. Never spoon, fork, knife, straw or glass o Sterile water rinses are given after feeding o Just like post-op chelloplasty, feeding are resumed 3-4 weeks after repair, when healing has already taken place. Give small frequent feedings initially. Hemorrhage

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Definitive tx:



Chelloplasty surgery to close cleft lip; done as early as possible. Using the Rule of 10 At least 10 wks old Weighing at least 10lbs. Having at least 10 g hemoglobin Palatoplasty cleft palate surgery; is not done very early (not earlier than 10-12 months) because it can harm the tooth buds) nor too late (because the palate can become too rigid and child might develop undesirable speech patterns) Velopharyngeal flap operation at 8-9 years of age; to revise previous repair, correct nose deformities and reconstruct the nasopharynx for speech improvement

TRACHEOESOPHAGEAL FISTULA Definitions: Esophageal Atresia failure of the esophagus to form a continuous passage from the pharynx to the stomach Tracheoesophageal fistula abnormal sinus connection b/w the esophagus and the trachea Types:


Preoperative Nursing Care: 1. 2. Provide emotional support to parents parents have difficulty loving the infant and responding warmly to him; encourage verbalization of fears, guilt, anger, etc. Feeding the most immediate and obvious problem because the infant cannot maintain closed suction around the nipple nor use mouth movement adequately to pull on the nipple. Use soft, regular, cross-cut nipple Burp more frequently because he swallows more air than usual Do not feed lying down should be in sitting or semi-sitting position to aid successful swallowing and prevent choking Be patient he takes longer to feed Do not confine lying on back for long periods of time to prevent URTI and ear infections. Give small amounts of water after feeding in order to rinse the mouth. Prevent dry, cracked lips especially since the baby breathes through the mouth.

Type I/A Upper and lower segments of the esophagus are blind; no connection to the trachea

Type II/B Upper end of the esophagus opens into the trachea; blind lower segment

Postoperative Chelloplasty:

Type III/C Upper end is blind; lower end connects into the trachea. Most common type (85%)

Type IV/D Both upper and lower ends of the esophagus open into the trachea by a fistula

Type V/E H type; no esophageal atresia but with fistula; rare type Clinical Manifestations: 1.

Type VI/F Stenosis occurs 2/3 of the way down the esophagus. Obstruction may be partial of complete

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 Continue use of incubator 3. Maintain adequate nutrition oral feedings started 6-14 days post-op. Low residue diet to keep stools soft Feed slowly in upright position to allow time for swallowing 4. Oral hygiene to prevent bacterial growth 5. Allow infant to suck on pacifier to meet psychological and physiological needs 6. Encourage parental participation to promote strong parental-infant bonding HYPERTROPHIC PYLORIC STENOSIS

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Excessive amount of secretions outstanding symptom which occurs soon after birth Constant drooling Large amounts of secretions from the nose Intermittent cyanosis due to aspiration because of overflow from the blind pouch Abdominal distention air from the trachea passes through the fistula into the stomach When fed, infant responds violently after first or second swallow Coughs and chokes Fluid returns through the nose and mouth Infant struggles 5. Inability to pass catheter through the nose or mouth into the stomach Diagnostic Evaluation:

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


Preoperative Nursing Care: 1. Position newborn with the head and chest elevated 20-30 to prevent reflux of gastric juices into the tracheobronchial tree 2. Regular suctioning 3. Put in incubator with high humidity to aid in liquefying secretions and thick mucus 4. Administer oxygen PRN 5. Assist in bougie treatment (elongation of proximal pouch using a mercury-weighed dilator or firm catheter briefly each day) 6. Give antibiotics as ordered to prevent or treat pneumonitis Observe closely for: VS; respiratory behavior Amount of secretions Skin color

Congenital hypertrophy of the muscle of the pylorus in the stomach; the muscle becomes progressively thickened and elongated with narrowing of the lumen

Signs and Symptoms: Projectile vomiting after 1 or 2 feedings during the 2nd-4th week of life Important points to consider: o Vomiting is the initial symptom of upper GIT obstruction (abdominal distention is the major symptom in lower GIT obstruction) o Vomitus in UGIT problems is blood tinged not bile-streaked (vomiting in LGIT is bile-streaked or vomiting of fecal material) o Vomiting is usually projectile if there is an obstruction (non-projectile if w/o obstruction) Effects of vomiting: o Baby is hungry afterwards o Scanty, infrequent stools o Metabolic alkalosis and marked deficits of sodium and potassium o Marked weight loss and dehydration 1-2 cm olive-shaped mass along with the midepigastric plane to the right of the rectus muscle Gastric peristaltic waves are seen running across the abdomen from left to right after a feeding On barium enema, the string sign can be seen


Surgery: 1. 2. Primary Repair esophageal anastomosis and division of fistula Gastrostomy and cervical esophagostomy done temporarily until infant gains weight staging (repeated operations separated by periods of time, waiting for growth) is the accepted philosophy of treatment.

Postoperative Care:

Preoperative Nursing Care:

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Monitor IC Accurate recording of intake and output Feed by gavage Thickened feedings at frequent intervals. Thickened feedings delay the emptying time of the stomach, thus increasing satiety and making vomiting less likely. Feed slowly on semi-upright position Burp PRN because of poor peristalsis Observe rules regarding NGT feeding


Invagination or telescoping of a portion of the small intestine into a more distal segment of the intestine More frequent in infants and very young children than in adults because children have hyperactive lower intestinal tract


Signs and Symptoms: 1. 2. 3. 4. 5. 6. 7. Sudden onset of severe, spasmodic and explosive pain causing the child to pull up his legs on to his abdomen and give out a loud, shrill cry. As pain subsides, child lies limp, pale and sweaty The pain occurs successively more intense and at shorter intervals Vomiting is an associated symptom After an hour, the typical brown, bloody, mucoid stools (currant jelly) can be observed On palpation, sausage-shaped mass in the ascending or transverse colon can be felt On barium X-ray, the staircase or coiled spring pattern can be seen

Treatment of Choice: Fradet-Ramstedt separation of the hypertrophied muscles without incision of the mucosa Postoperative Management: (recovery is rapid) 1. NGT is inserted for gastric decompression drainage of fluid and gases prevents pressure on the suture line thru preventing paralytic ileus (the most common complication after abdominal surgery) Immediate postop, position child on his side with his back supported Resumption of feeding:

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Consist of gradually increasing amounts of clear fluids that contain glucose and electrolytes. If well tolerated, diluted formula is given on the 2nd day with increasing concentration at each feeding until full formula can be given On the first few occasions that a formula is given, give by medicine dropper to ensure that the child obtains only a small amount, thereby reducing stress at the pylorus Feed on semi-upright position for 45-60 minutes and position slightly on the right side to increase gastric emptying time. ACHALASIA Pathophysiology: On the 3rd to the 10th day of life, the cardiac sphincter muscles fail to function causing it to be relaxed and constantly patent. Unknown cause; self-limiting (disappears spontaneously within 3 months)

Complication: Gangrene of the bowel if not corrected immediately Management: 1. 2. Barium enema hopefully reduce invagination. Otherwise, surgery is done to remove gangrenous intestine and end-to-end anastomoses is done Postoperative care NGT for gastric decompression Proper positioning Limit childs activity after discharge Recurrence is highly unlikely (i.e. it is very rare)


HIRSCHSPRUNGS DISEASE / CONGENITAL AGANGLIONIC MEGACOLON Definition: Congenital absence of parasympathetic ganglion nerve cells (w/c control defecation) usually in the rectosigmoid area (therefore, it is a LGIT obstruction)


Signs and Symptoms: NO NERVE CELLS Delay in meconium passage Obstinate constipation Intermittent progressive abdominal distention

Signs and Symptoms: 1. 2. 3. 4. Prolonged, repeated non-projectile vomiting which is more pronounced when baby is lying flat on his back Often hungry after each vomiting episode Aspiration may occur Pressure on the abdomen causes reflux of stomach contents into the esophagu

Management: 1. 2. Thicken feedings (fomula + cereals) because they are less easily vomited Put on upright position for 30 minutes after everyfeeding

Large fecal mass accumulate

Foul odor of breath and stools

Thin abdominal wall; prominent superficial veins Abdominal Impaired absorption of water and nutrients Nausea

INTUSSUSCEPTION Malnutrition, lethargy and anemia

Respiratory distress

Infrequent, spontaneous ribbon-like stools

Preoperative Management: 1. Good hygiene is important

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Frequent, small feedings of low-residue diet if poorly nourished, apathetic and uncomfortable with distention and nausea Upright position for respiratory distress, supported with pillows and sandbags Administration of antibiotics and stool softeners Frequent daily enemas to remove accumulating fecal matter Oil retention o Use funnel or syringe o 75-150 ml mineral or olive oil at 100 degrees (37.7C) o Apply pressure over anus so as not to expel the solution

Management: 1. 2. 3. Temporary Colostomy if poor surgical risk (very young baby; malnourished; high agenetic or atretic type) Surgery anoplasty or abdominoperineal pull through Postoperative Care: Expose perineum to air by putting infant on supine position with legs suspended straight up or on prone position Check bowel sounds frequently NGT for gastric decompression Change position from side to side to decrease tension on suture line Oral feedings are resumed 1-2 days postop when peristalsis has resumed

Cleansing enema of isotonic solution (tap water is never used when there is impaired water absorption because water intoxication, as evidenced by muscular tremors and twitching can result and can even lead to convulsions, coma or death) o On supine, with pillows under the head and back, buttocks are placed on a diaper-lined bedpan o Dissolve 1 tsp. salt in 1quart of water (isotonic solution) o Temperature of solution is 105F or 40.5C o Enema should not be more than 18 inches above hip level o 10-12 French catheter is inserted 2-4 inches into the rectum o No more than 300ml should be given at a time



Treatment of Choice: Surgery: Swenson pull-through if the infant is of poor surgical risk, a temporary colostomy in the distal portion where normal ganglia are found is done IMPERFORATE ANUS Unknown etiology arrest in embryonic development at 8 weeks of intrauterine life Types:

Diaphragmatic hernia failure of development of the posterolateral portion of the diaphragm resulting in persistence of the pleuroperitoneal canal (Foramen of Bockladek). Abdominal organs (usually the stomach and the intestines) protrude into the chest cavity, usually on the left side, displacing the heart to the right of the chest and the lung collapses Signs and Symptoms: o Presenting sign cyanosis and severe respiratory distress immediately after birth o Abdomen is scaphoid (not domeshaped) o Breath sounds are diminished or absent; bowel sounds are heard in the chest cavity o Intracostal and subcostal retractions are observed Management: o Elevate head to improve breathing o NGT to prevent distention o Correction of respiratory acidosis with buffering agents (e.g. sodium bicarbonate, THAM) o Immediate surgical repair Hiatal hernia protrusion of the stomach through the esophageal hiatus Incidence: more frequent and severe in males; established family pattern Signs and symptoms: forceful vomiting between 1wk and 1mo of age, eventually containing old blood resulting in anemia, wt. loss, dehydration and malnutrition Complication: aspiration pneumonia Diagnostic procedure: barium x-ray Management of choice: surgery: GASTROPREXY hiatus is reduced and stomach is tacked in Preoperative care o Give thickened formula o Feed in upright position and let child stay upright for hour after feeding Postoperative care o Underwater chest drainage and basic respiratory therapy for several days Prognosis: recurrence is common Inguinal hernia protrusion of hernial sac through the abdominal wall, the inguinal opening or into the scrotum Umbilical hernia protrusion of the omentum or small intestine through a congenital weakness or opening of the umbilical ring


Type I Stenosis

Type II Membranous

High Low (distance less than 1.5 (distance greater than 1.5 cm) cm) Type III Agenesis (High and Low)

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Type IV Atretic