Cleft Lip / Cleft Palate

Predisposing Factors 1. 2. 3. 4. Genes or Heredity Polyhydramnios Multiple Births Teratogenic Drugs Anti-convulsant (anti-seizure) Anti neoplastic (anti-cancer) Anti viral 3rd generation Antibiotics Vitamin A Preparations (Retionoids, Retinoic Acid, Tretinoin ex. Accutane, Raccutane) Radiation Therapy Proscar drug of choice for Prostate Gland Cancer

Cleft Lip Failure of union of Embryonic Structures (Maxillary and Premaxillary Process) that occurs between 5 8 weeks of Fetal life Common in Males (with or without cleft palate) Types: Unilateral, Bilateral, Midline (rare)

Cleft Palate Failure of union of Palatal Structures that occurs between 9 12 weeks of Fetal Life Common in Females

Associated Problems 1. 2. 3. Feeding Upper Respiratory Tract Infection (URTI) Ear Infection (ex. Otitis Media) 4. 5. 6. Speech Problem Dental Malformation Body Image

Treatment: Surgery 1. Cleft Lip: Cheiloplasty or Closed Cleft Lip Reconstruction of the lip done asap using the RULE OF 10 a. @ least 10 lbs b. @ least 10 weeks c. @ least has 10 gms of Hemoglobin Cleft Palate: Palatoplasty or Uraroplasty Not done < 10 12 months because it can harm the Tooth Buds Not done > 12 months because palate can be rigid (stiff) and may develop speech problem. Veloplasty or Velopharyngeal Flap operation done during 8 9 years old (needs small space)

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PREOPERATIVE NURSING CARE 1. 2. Emotional Support for the parents and encourage verbalization of feelings Proper Feeding a. Use soft, round, large criss cross cut nipple; may use medicine dropper b. Burp frequently every after 1 oz of milk c. Dont feed lying on the back (supine) d. Dont confine lying on the back for long time to prevent URTI or Ear infections e. Rinse mouth with water after feeding f. Prevent dry or cracked lips

POSTOP INTERVENTIONS FOR CHEILOPLASTY 1. Complication: Respiratory Distress during 48 hours due to the ff a. Swelling of ttongue, mouth, and nostrils (Put downward pressure on the chin to air passage) b. Increased Respiratory Secretions c. Difficulty adjusting to a smaller airway Nursing Interventions a. Position: Supine or Sidelying (to prevent aspiration) b. Avoid reasons for crying to avoid stress on the sutures c. Put Mist tent to liquefy secretions d. Elbow Restraint to prevent touching the surgical site e. Logans Bow taped to maintain an intact suture line after surgery f. Maintain OS with sterile NSS; OS is removed before feeding and cleaning g. Feeding: Upright Position Use rubber tip asepto syringe with large hole and administer at the side of the mouth to prevent trauma to the sutures Clean sutures after feeding with half strength hydrogen peroxide or saline solution to prevent crusts and scarring. E nlarged criss cross cut nipple S ucking after healing of Cheiloplasty S wallowing R est OP INTERVENTIONS FOR POST PALATOPLASTY 1. 2. Complication: Hemmorhage Nursing Interventions a. Position: Prone (to prevent aspiration of blood) Change head position every 2hours b. Mist Tent c. Elbow Restraint d. No sucking, blowing, laughing, or putting objects on mouth e. Oral packing at palate during first 2 3 days f. Feeding: Use Paper cups or Plastic Cups (no nipple, no pacifier) No spoon, fork, straw, and knife Resumed 3 4 weeks after repair; Small frequent feedings initially Avoid Harsh Foods No tooth brushing (older children)

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OTHER INTERENTIONS ON CLEFT LIP AND CLEFT PALATE 1. 2. 3. 4. Monitor I & O Monitor Dehydration (check scanty urine) Monitor Defecation Patient always vomit if theres regurgitation

4 Persons
1. 2. 3. 4.

Speech Therapist Speech problem Audiologist URTI / Ear Infection /Otitis Media Dentist Dental Malformation Geneticist Hereditary

Tracheoesophageal Fistula (TEF)

1. 2.

Esophageal Atresia Failure of the Esophagus to form a continuous passage between Pharynx and Stomach Tracheoesophageal Fistula Abnormal sinus connection between Esophagus and Trachea

Clinical Manifestations 1. 2. 3. 4. 5. Excessive amount of secretions from nose and mouth even after birth Constant Drooling Intermittent Cyanosis Abdominal Distention When fed, infant responds violently after 1st or 2nd swallow a. 4 Cs: Cough, Choke, Cyanosis, Constant Drooling b. Fluid returns to nose and mouth c. Infant struggles Cannot pass catheter thru the nose and mouth into the stomach Cannot aspirate stomach contents

6. 7.

Diagnostic Evaluation 1. 2. 3. Materal History of Polyhydramnios Flat Plate Xray of Abdomen and Chest reveals gas in stomach Xray with Radiopaque Catheter / Radiopaque Contrast Medium / Fluoroscopy not done to prevent aspiration

Preoperative Nursing Management 1. 2. 3. 4. 5. 6. 7. 8. Elevate Head (20 30 degree; Fowler) Regular Suctioning Place in incubator with High Humidity Oxygen PRN Antibiotics as ordered Monitor IV or IV Hyperalimentation (thru central venous catheter into SVC or Jugular Vein) Assist in Bougie Tx (elongation of proximal pouch using mercury weighed dilator or firm catheter inserted each day) Observe: V/S, respirations, amount of secretions, skin color, abdominal distention

Postoperative Nursing Management 1. 2. Observe signs of Stricture at Anastomosis Site Maintain Patent Airway a. Suction PRN mark the catheter to determine how long it will be inserted w/o disturbing anastomosed site b. Change position frequently, stimulate to cry, dont hyperextend head c. Continued use of Incubator Maintain Adequate Nutrition a. Position: Upright (to allow time for swallowing), Feed Slowly b. Clear Liquids 4 5 days after surgery c. Low Residue Diet (Avoid high fiber that cannot be digested) Oral Hygiene Allow to suck on Pacifier Monitor I & O Encourage Parental Participation

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4. 5. 6. 7.

Gastroesophageal Reflux Disorder (GERD)

Acid Indigestion; Heartburn or Pyrosis; Similar to Chalasia Transfer of Gastric contents into the Esophagus GER becomes a disease, failure to thrive bleeding, Dysphagia develops Occurs when upper portion of stomach and lower esophageal contents move above the diaphragm

Clinical Manifestations 1. Frequent Spitting and Vomiting 2. Coughing 3. Laryngitis Pathophysiology 1. 2. 3. 4. 5. 4. Repeated Rugurgitation, Abdominal Pain Nausea, and

Vagus Nerve (CN X) responsible for the relaxation of LES With Afferent Stimulation (gastric distention, meals, associated with weak LES) Failure of LES to control food from the stomach Backward Movement of Gastric Contents Irritation of Esophagus (Esophagitis)

Avoid these Foods 1. 2. 3. Citrus Foods (ex. Orange, Lemon) Spicy Foods Fatty and Fried Foods 4. 5. 6. Tomato based foods Alcohol Chocolate

Diagnosis 1. 2. 3. GI Barium Swallow Radiograph to check areas high in acid contents; if sphincter is open or relaxed Endoscopy to assess presence of Esophagitis Esophageal Ph Monitoring 2 to 36 hours

Management 1. Children with Severe Complication Observe Recurrent: Aspiration Pneumonia, Severe Esophagitis, Failure to Thrive Treatment Surgery: NISSEN FUNDOPLICATION (wrapping a portion of gastric fundus) Children w/o complication a. Small frequent feedings and position after each feeding Medications a. H2 Antagonist Cimetidine (Tagament) for Reflux b. Proton Pump Inhibitor Omeprazole (Phisolec / Losec) to decrease release of gastric juice; block the final stage of acid production; inhibit H +K + Atpase (H+ is an irritant that appears on the last stage of acid secretion; increases alkalinity of stomach)

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Nursing Considerations 1. 2. 3. 4. 5. Identify Children with symptoms Educate parents about home care, feeding, positioning, and medications Care after surgery Weight Management Lifestyle Change

INTUSSUSCEPTION
Invagination OR Telescoping a portion of Small Intestine into a more distal segment of the intestine (almost near in Ileocecal Junction) Incidence: Frequent in Infants and Very Young Children than adults because they have Hyperactive Lower Intestinal Tract Signs and Symptoms: 1. 2. 3. 4. 5. 6. Sudden onset of severe spasmodic and explosive pain causing to pull the legs toward abdomen and gives a shrill cry; As pain subsides, child lies limp, pale, and sweaty Pain occurs intense at short intervals Vomiting is an associated symptom Brown, mucoid stools (CURRANT JELLY) maybe a combination of mucus and blood Sausage Shaped Mass in Ascending and Transverse Colon is felt (Right Quadrant) Barium Xray STAIRCASE or COILED SPRING pattern

Complication: Gangrene of Bowel (if not corrected asap; d/t blood circulation in intestine) Management: 1. 2. Barium Enema to decrease Invagination; to stretch out intestine Surgery: a. Anastomosis b. Pull Through Surgery if gangrenous

Post Op Care 1. 2. 3. NGT for gastric decompression Proper Positioning: Left Sidelying Limit childs activity for a while after discharge

Prognosis: Recurrence is highly unlikely, very rare

Hypertrophic Pyloric Stenosis

Congenital Hypertrophy of the muscles of Pylorus in the stomach; muscles becomes thickened and elongated, ;with narrowing of the lumen Hypertrophy of the Pyloric Sphicnter

Clinical Manifestations 1. PROJETILE VOMITING after 1 2 feedings during 2 4 weeks of life Remember: a. Vomiting is an initial symptom of UGIT Obstruction; Vomitus is Blood Tinged. b. Abdominal Distention is the major symptom of LGIT Problems; Vomitus is Bile Streaked or Fecal Material Effects of Vomiting a. b. c. d. 2. 3. 4. Baby is hungry afterwards Scanty infrequent stool Metabolic Acidosis (High Ph) and Deficit of Sodium and Potassium (electrolytes) Dehydration and Weight Loss

1 2 OLIVE SHAPED MASS along Mid Epigastric Right of Rectus Muscles PERISTALTIC WAVES across abdomen from Left Right Barium Enema: STRING SIGN revealed

PREOPERATIVE MANAGEMENT 1. 2. 3. Monitor IV Accurate recording of I & O Feeding: a. Semi Upright Position b. Feed by GAVAGE: thickened formula at frequent intervals to delay the emptying of stomach to increase satiety and make vomit less; feed slowly c. Burp PRN because of Poor Peristalsis d. Observe rules of NGT Feeding

TREATMENT: FREDET RAMSTEDT (separation of hypertrophied muscles without incision of muscles) POSTOPERATIVE MANAGEMENT (Fast Recovery) 1. 2. 3. NGT for Gastric Decompression (drainage of fluids and gas prevents pressure on suture line to prevent PARALYTIC ILEUS, which is the most common complication after abdominal surgery) Position: SIDELYING with back support Feeding: a. Resume Feeding in gradually amts of Clear Liquids that has glucose & electrolytes. If tolerated, DILUTED FORMULA on 2nd day, w/ increasing concentration each feeding until FULL FORMULA can be given. b. Use medicine dropper so child can only obtain a small amount reducing stress at Pylorus c. Semi Upright position for 40 60 minutes d. Right Sidelying to increase gastric emptying (after meals)

CELIAC DISEASE
(Malabsorption Syndrome / Gluten Induced Enteropathy / Gluten Sensitive Enteropathy) Disease of the proximal small intestine characterized by abnormal mucosa and permanent intolerance to Gluten.

Clinical Manifestations 1. 2. 3. 4. 5. 6. Retarded Growth; Thin Steatorrhea (passage of abnormally fatty and oily stools d/t malabsorption of ADEK) Symptoms of ADEK deficiency Chronic Diarrhea Grossly Distended Abdomen Abdominal Pain, Irritability and Vomiting

Diagnostic Evaluation 1. 2. Gastroscopy to visualize GIT Biopsy of Small Intestine to check if sensitive to gluten

Management 1. Permanent Gluten Free Diet. Lifetime avoidance of: B arley R ye O ats W heat Supplements of ADEK, IRON, and CALORIES

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Nursing Considerations 1. 2. Monitor Gluten Free Diet and Provide ADEK Client Teaching and Discharge Planning a. Gluten free diet and Importance of Adherence to it. b. Avoidance of Infection

Prognosis: Unlikely if gluten is eliminated

Malrotation and Volvulus

Abnormal rotation of intestine around Mesenteric Artery during Embryologic development Complete volvolus of intestine; compromise blood supply resulting to Intestinal Necrosis, Perforation, and Peritonitis

Clinical Manifestations 1. 2. 3. 4. 5. Intermittent Vomiting (bile streaked or fecal material) Foul Feculent Breath Recurrent Abdominal Pain Abdominal Distention Lower GIT Bleeding

Diagnosis 1. 2. GI Series Infant with BILIOUS VOMITING should be evaluated

Management 1. 2. Abdominal Surgery to remove gangrenous area Anastomosis

Nursing Considerations 1. 2. Educate parents the need of Hospitalization Post Op Care: V/S, Bowel Sounds, Care of Incision Site, No repositioning

CHALASIA
Abnormal relaxation of Cardiac Sphincter resulting to NON PROJECTILE, NON BILE, and SELF LIMITING VOMITING Etiology: Unknown; Self Limiting Disappears spontaneously within 3 months

Clinical Manifestations 1. 2. 3. 4. Prolonged, repeated NON PROJECTILE vomiting more often in Supine Position; Regurgitation of gastric contents Pressure on abdomen most especially when legs are flexed causes reflux of gastric contents into esophagus Aspiration may occur Often hungry after vomiting

Management: 1. 2. Thickened Feeding (Formula + Cereal) Position After Feeding: Upright for 30 mins

HIRSCHSPRUNG DISEASE / AGANGLIONOSIS / CONGENITAL AGANGLIONIC MEGACOLON

Congenital absence of Parasympathetic Ganglion Nerve Cells (controls defecation) usually in Rectosigmoid area (LGIT Obstruction) PNS responsible for peristalsis and defecation

Clinical Manifestations 1. 2. 3. 4. 5. 6. 7. 8. 9. Delayed passage of Meconium Obstinate Constipation (cannot defecate; older children) Accumulated Large Fecal Masses Infrequent spontaneous RIBBON LIKE STOOLS Foul Breath (Feculent, Fetid) and Stools Intermittent Abdominal Distention Abdominal Pain, Fever Nausea and Vomiting Malnutrition, Lethargy, Anemia, and Respiratory Distress

Preoperative Management 1. 2. 3. 4. 5. 6. Good Hygiene Small Frequent Feedings of LOW RESIDUE DIET Upright Position supported with pillows or sandbags for Respiratory Distress Administer Antibiotics and Stool Softeners Frequent Daily Enemas to remove accumulated feces Oil Retention Enema a. Position: Left or Right Full Sidelying b. Use funnel or syringe c. 75 150 ml Mineral or Olive Oil @ 37.7 C d. Apply pressure on the anus so as not to expel the solution Cleansing Enema of Isotonic Solution a. Not for Hypertensive Patients use Surfactant instead b. Tap water is never used when theres impaired water absorption because of water intoxication, as evidenced by tremors and twitching which can result to convulsions, coma or death. c. Position: Supine with pillows under head and back. Buttocks are placed on a diaper linen bed pan d. Dissolve 1 tsp salt in quart water (Isotonic Solution) with a temperature of 105F or 40.5 C e. Enema should not be above 18 inches above hip level f. 10 12 French Catheter inserted 2 4 inches into rectum (2inches for infant and 4 inches for older children) g. No > 300ml should be given @ a time. (for older child)

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Treatment: Surgery SWENSON PULL THROUGH (If patient is a poor surgical risk, a temporary COLOSTOMY in the distal portion where normal ganglia are found and done) Disadvantages of Temporary Colostomy 1. 2. Infection Needs frequent cleaning and monitoring *Always watch if bag is detached and always replace bag

POISONING
Definition: Ingestion of Toxic Substances Incidence: More common in BOYS because they are more active and adventurous Signs and Symptoms 1. 2. 3. GIT Disturbances abdominal pain, vomiting, diarrhea, anorexia Respiratory Circulatory Disturbances shock, collapse, unexplained cyanosis CNS Sudden Loss of Consciousness, convulsions

Management 1. 2. Identify the Poison Induce Vomiting by giving 15ml of Ipecac Syrup then repeated once within 20 mins, except: a. Patient is unconscious b. Poor Gag Reflex c. Not given >15 ml in children and >30 ml in adults 3. Give Antidote opposite of the poison (ex. Acid Alkali Vice Versa) 4. If definite poison is unknown, give Universal antidote a. Pulverized Charcoal (Burned Toast) absorbs phenol and strychnine; 2 parts b. Magnesium Oxide or MOM neutralizes acids; 1 part c. Tannic Acid (Strong Tea) precipitates acids, glycosides, and metals; 1 part 5. Gastric Lavage a. depends on the length of time which has elapsed and how much substance was ingested b. Not often done in children because of danger of fluid and electrolyte imbalance ASPIRIN POISONING common in Toddlers and Preschoolers because its appealing to the eye. Signs and Symptoms 1. Hyperpnea abnormal increase of rate and depth of respiration; earliest sign due to acidosis brought by aspirin which is an ASA (NSAIDS) 2. Hyperpyrexia, anorexia, vomiting, sweating 3. Purpuric Skin because salicylates inhibits Prothrombin formation 4. Potassium Deficiency seen as arrhythmias (irregular heart rate; tachy or brady) 5. Prone in bleeding Management 1. 2. 3. 4. Lock all medicines in a cabinet and not reachable by children Usual management for poisoning Give IV and VITAMIN K via IM as ordered Observe signs of bleeding

HERNIA
1. Diaphragmatic Hernia Failure of the posteriorlateral portion of diaphragm to develop resulting to persistence in Pleuroperitoneal Canal (Foramen Bochdalek) Signs and Symptoms 1. 2. 3. 4. 5. Presenting Sign cyanosis and severe RDS Schaphoid Abdomen (boat shape, nabicular shape, concave) Breath Sounds are diminished or absent Bowel Sounds heard in chest cavity Intercostal and Subcostal Retractions (Seesaw Retractions)

Management 1. 2. 3. 4. Elevate head to improve breathing NGT for gastric decompression prevent gastric distention Correction of Respiratory Acidosis w/ Buffering Agents (ex. Sodium Bicarbonate - Systemic Antacid; Slow IVP 5 10 mins; another 5 10 max of 20) Immediate Surgical Repair

------------------------------------------------------------------------------------------------------------------------ ------------2. Hiatal Hernia Protrusion of the Stomach thru to Esophageal Hiatus Incidence: Frequent and severe in Males with established familial pattern S/S: 1. 2. Forceful Vomiting between 1 week to 1 month of age Contains Old Blood resulting in Anemia, Weight Loss, Dehydration, and Malnutrition

Complication: Aspiration Pneumonia Diagnostic Evaluation: Barium Xray 2 Forms: 1. 2. Sliding stomach goes upward the diaphragm to the esophagus Rolling Parasesophageal enlargement of stomach laterally near or below the diaphragm.

Management of Choice SURGERY: GASTROPEXY - Hiatus is reduced and stomach is tucked in downwards to its normal position. Preoperative Care: 1. 2. Give Thickened Formula Feed in Upright Position; Let child stay in Upright Position for hr after feeding

Postoperative Care 1. 2. 3. Underwater Chest Drainage and Basic Respiratory Therapy for several days; UCD (tube is directed downwards to remove excess fluids) NGT for gastric decompression Monitor IV and Record I & 0

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Oral feedings resumed in few days; Small Frequent Feedings with PATIENCE because child eats slowly, inconsistent, tires easily, and may refuse food. a. Use soft, PREEMIE NIPPLE (small lumen) for Infants b. Finger Foods for Older Children to encourage Independence.

Progonosis: Recurrence is common if child is active 3. UMBILICAL HERNIA - Protrusion of the Omentum or Small Intestine thru congenital weakness OR opening of the umbilical ring Incidence: Appears 6 months and generally disappears spontaneously by 1 year Symptom: Soft tissue swelling covered by skin that looks like a FINGER protruding from umbilicus when infant cries, strains, or coughs. Management: Manipulation but sometimes can strangulate. Surgery is indicated if strangulation occurs. 4. INGUINAL HERNIA protrusion of hernial sac thru abdominal wall, inguinal opening, or into scrotum.

Incidence: Present @ birth or appear later stage and adulthood; Common in Males and often on the RIGHT although maybe BILATERAL. S/S: 1. 2. 3. Mass in the groin SILK GLOVE SENSATION (Diagnostic Inguinal Hernia) Risk of Incarcenation (intestines become trapped in the sac), causing strangulation of bowel, gangrene, rupture, and even death. (d/t sepsis)

Treatment: Surgery: HERNIORRAPHY less risk of complications when elective, rather than emergency. PreopManagement: Non Constipating Diet remove or lessen fiber; increase water intake Postoperative Care: 1. 2. DAT diet Non Constipating Foods 3. Infant can be as active as he desires when healing has taken place.

DIAPHRAGMATIC HERNIA

HIATAL HERNIA