1. GI DYSFUNCTION

2. Anatomic division of Abdomen

3. GI Anatomy

4. Structural Defects

5. Cleft Lip and/or Cleft Palate.
Cleft Lip and/or Cleft Palate
ETIOLOGY: believed to be a combination of chromosome and environmental factors at a critical point in embryonic development. Some association with maternal smoking in first trimester.
PATHOLOGY: A facial malformation that occurs during embryonic development.

6. Types of Cleft Lip & Palate

7. Cleft Types
Cleft Lip: can vary from a small notch to a complete cleft extending to the base of nares. May involve dental structures.
Cleft Palate: can occur alone or with CL and may extend into hard and soft palate in midline or bilaterally and extend through nasal cavity.

8. NURSING CARE
Facial deformities are particularly disturbing to parents and may generate a strong negative reaction=high risk for initial maternal detachment. Emphasize infant’s other physical qualities and emotional needs. How nurse handles infant is very important.
Feeding is a problem. CL & CP  ability to suck because infant cannot create negative pressure. Liquid escapes through cleft in nose. Hold infant upright. Use special nipple with large opening (less need to suck). If still a problem, use asepto syringe with soft rubber tubing (p.914), place in back of mouth and control flow with plunger. Rinse mouth with water after feeding.

9. Feeding the infant with CL/CP

10. Protect operative site.
Post-Operative care
Protect operative site.
For CL, infant kept supine or in infant seat. A thin, arched metal device – Logan bow – taped to cheeks securely, protects suture line.
Analgesia – opioids, acetaminophen, possibly sedation
Elbow restraints to keep hands from site. Jacket restraint for older infant to prevent rolling over. Release one at a time periodically and ROM limbs, cuddle infant.
Keep from crying, stresses suture line, ask parent to stay.
Feed liquids and advance to regular diet for age

11. Post-Operative care
Clean suture line with cotton swab dipped in saline, apply antibiotic ointment if ordered. Keep suture lines free from infection, which will improve cosmetic effect of healing.
Suctions any secretions VERY gently.
May have oral packing for 2-3 days forcing him to nose breathe.
Analgesia
Blenderized or soft diet with no hard foods initially

12. Nursing Interventions : Post operative Repair of Cleft Lip & Cleft Palate
Refer to early intervention for speech therapy evaluation, almost all children require some form of speech therapy.
Refer to a good pediatric dentist that specializes in the care of these children. Assist parents in insurance issues. The child usually will require extensive, expensive & dental treatment.
Refer infant/child to audiologist as these babies have a high % of hearing loss from repeated middle ear infections.

13. Esophageal Atresia
ETIOLOGY: unknown, higher % of preemies born with defect.
PATHOLOGY: a rare malformation of the esophagus failing to develop as a continuous tube, defect occurs equally in males/females. Mother may have polyhydramnios during pregnancy % of casesproximal esophagus terminates in a blind pouch and distal segment is connected to the trachea or primary bronchus by a fistula.
Diagnosis is almost immediately after birth. Infant may have excessive frothy saliva in mouth, If fed, infant swallows normally but suddenly coughs, gurgles, chokes and fluid is aspirated or returns via nose and mouth. After suctioning, infant is eager to eat again, but problem repeats.

14. Types of esophageal atresia

15. Nursing Care of the child with esophageal atresia:
Assessment:
If TEF suspected (baby coughs, gags with first feeding , has frothy secretions) , immediately make child NPO
Intervention:
Place supine with head  30, minimizes reflux of gastric secretions into distal esophagus and possibly into trachea if a fistula is present. High risk of aspiration pneumonia.

16. Nursing Interventions Post operative care of the child with esophageal atresia:
Infant will return from RR with GT, NG tube and chest tube, Foley catheter, prepare parents. Check skin from damage from secretions and irritation. Apply ointment around site, may have drainage collection bag
IV fluids and strict I&O
Intermittent or continuous suctioning via NG tube of blind proximal pouch.
Gastrostomy tube may be inserted to release air and gastric fluid. If baby goes home with GT, teach parents care. Tube may also be used for feedings.

17. Nursing Interventions Post operative care of the child with esophageal atresia:
Surgical repair may be done once or in steps. End-to-end anastamsois or graft of colon if ends cannot meet. Complications include leak at site, strictures which need to be dilated, GERD, tracheomalacia (weakness in tracheal wall from dilated proximal pouch compressing trachea from fetal life and not allowing it to develop normally).
GT feedings until anastamsois is healed and oral feedings tolerated approx days post op. Sterile water, then frequent, small feedings of formula. GT is removed when baby is able to tolerate feeds well and is adequately nourished.
100% survival in healthy full-term without severe respiratory distress.

18. Obstructive Disorders

19. Pyloric Stenosis
ETIOLOGY: unknown. Increase in full-term, male, Caucasian infants.
PATHOLOGY: circular muscle of pylorus is hypertrophied (greatly enlarged) which produces severe narrowing of opening between stomach and duodenumpartial obstructioninflammation and edemacomplete closure.
Often palpable as an olive shaped mass in RU abdomen to right of umbilicus. Hyperperistaltic waves may be seen from left to right. Projectile vomiting (a diagnostic hallmark) , usually stale milk, nonbilious, begins at 1-10 weeks of age with vomiting minutes after feeding. Infant is hungry right after, eats eagerly, no pain, chronic weight loss, dehydration, distended upper abdomen.

20. Pyloric Stenosis

21. Nursing Care of the child with pyloric stenosis:
Assessment:
Restore fluid and electrolyte balanceNPO with IV fluids, monitor I&O and specific gravity. Also record and vomiting and stools
V/S and capillary refill, s&s of dehydration, urinary output (0.5-2ml/Kg/hr), weigh daily, skin turgor, mucus membranes dry, depressed fontanels, no tears.
Infant is prone to metabolic alkalosis from loss of H ions, K and Na, CL from vomiting.
Delay surgery until imbalances are corrected.

22. Nursing Care of the child with pyloric stenosis:
Interventions
NG tube to suction secretions and for stomach decompression
Skin and mouth care
Keep flat or with head slightly elevated and with elbow restraints
Tell parents infant may continue vomiting hrs post op. NG tube, IV fluids until taking adequate fluids orallyI&O.
Analgesia for pain
Feed glucose water followed by breast milk or formula, advance diet as tolerated, and record response to feeding
Check suture site for inflammation/infection and teach parents

23. Intussusception ETIOLOGY: 90% unknown cause
PATHOLOGY: a telescoping or invagination of one portion of intestine into another, site of ileocecal valve is most common (ileum invaginates into cecum and colon) intestinal obstruction, necrosis, perforation, sepsis and death follow.
Occurs in previously healthy child (any age) with sudden episode of acute abdominal pain.
Child screams in pain and draws knees up to chest, but is comfortable between episodes. Progresses to tender abdomen, distended with palpable, sausage-like mass in RUQ, vomiting, fever, prostration and s&s of peritonitis. Currant jelly stools (stool mixed with blood and mucus) is a diagnostic, but late sign.

24. Intussusception

25. NURSING CARE: Interventions:
explaining to parents need for immediate intervention, occurs too rapidly, parents are often stunned
IV with I&O, NG tube restoration of fluid and electrolyte imbalance, possibly antibiotics
Report passage of any brown, normal stool immediately (may have corrected itself)
Continually assess V/S for shock

26. Medical Treatment
Initial management involves performing a barium enema in an attempt to reduce intussusception90% successful, have surgery if not.

27. Imperforate Anus
ETIOLOGY: unknown, classified as high, intermediate or low defect.
Any child with imperforate anus requires a comprehensive multisystem physical exam this is rarely an isolated defect. There are usually other anomalies.
PATHOLOGY: Inspection of anal area and patent anal opening is part of newborn assessment. No opening is found or very stenosed. Ultrasound confirms if defect continues internally is anus is just covered by a membrane. Infant does not pass meconium; however, a fistula may exist from vagina in girls or meconium may be mixed with urine from fistula to bladder or urethra.

28. Imperforate Anus Nursing Care: Assessment:
Assess for patency of rectum upon admission to newborn nursery by inserting a rectal thermometer
check for meconium in 24 hrs following birth, report if no passage or stool appears in vagina or urine
Interventions:
IV, NG tube, I&O
Keep site scrupulously clean, use zinc oxide ointment for skin if stool continuously dribbles

29. Medical Management – Surgical repair
Repair of Stenosis involves manual dilations, may also need excision of anal membrane if imperforate, followed by dilations.
Higher defects: colostomy for one year followed by “pull-through” repair. However, a functioning internal sphincter is necessary to achieve continence or child needs a bowel program to achieve it Toilet training is delayed even with successful procedure and child may not be fully continent or have completely normal bowel function until late childhood or adolescence

30. Post-operative nursing care:
Side lying to keep pressure off perineal sutures. If infant has colostomy, teach parents care of stoma, drainage bag, etc.
Encourage breastfeeding, breast milk is a natural stool softener. If formula fed, increase fiber, give stool softener

31. .
Disorders of Motility

32. Hirschprung’s Disease (congenital aganglionic megacolon)
ETIOLOGY: absence of ganglion cells in one or more segments of colon, usually rectum and proximal portion of large intestine.
PATHOLOGY: results in absence of peristalsis with accumulation of contents and distention proximal to defect (megacolon), may lead to ischemia and enterocolitis of small intestine and colon causing death.

33. Aganglionic Megacolon

34. Hirschprung’s Disease
Nursing Assessment:
In newborns, suspect when failure to pass meconium. Infants: failure to thrive, chronic constipation, episodes of vomiting and diarrhea. In children, ribbon-like, foul smelling stools, visible mass with palpable fecal mass. Infant appears poorly nourished and anemic.
Medical Management:
Surgery to remove aganglionic portions and restores normal function. Repaired in 2 steps: colostomy for one year to relieve distention and allow bowel to return to its normal size. Followed by fully corrective procedure with closure of colostomy, resection of aganglionic portion and “pull through” anastomosis of normal bowel.

35. Nursing care of the child with Hirscprungs disease:
Pre-op:
depends on age of child and overall condition
Improve nutritional status for surgery. Diet may be low fiber, high calorie and high protein or TPN
Monitor for s&s of enterocolitis and bowel perforation fever, vomiting, increased irritability, increased tenderness or distention of abdomen, V/S + BP for shock
Fluid and electrolyte replacement, IV, I&O
Measure abdomen with paper tape and mark spot to be consistent, report any increase immediately
Bowel prep with saline enemas until clear, antibiotic enemas, systemic antibiotics prophylactically
Explain to child and/or parents and teach colostomy care and skin protection and involve them in care.

36. Malabsorption Syndromes.
Malabsorption Syndromes

37. Celiac Disease
ETIOLOGY: exact cause unknown, but there appears to is inherited predisposition influenced by environmental factors
PATHOLOGY: characterized by intolerance to the protein gluten, found in wheat, barley, rye and oats. Child is unable to digest gluten, resulting in damage to mucosa of small intestine. More common in northern Europe, rarely in Asian or AA children.
S&S noted several months after child starts diet containing gluten, usually between 1-5 years: impaired fat absorption causes steatorrhea (stools excessively large, pale, oily, frothy, very foul-smelling and float in the bowl). Muscle wasting especially in legs and abdomen, anorexia, abdominal distention. Celiac crisis with acute, severe episode of diarrhea and vomiting may be precipitated by infection, immunizations, dehydration, and emotional upsets. Treatment with gluten-free diet, corn and rice are allowed grains.

38. Nursing Interventions for the child with celiac disease
Help child adhere to strict dietary management. Improvement occurs within a day or two of removing gluten from diet with weight gain and improved appetite. Diarrhea and steatorrhea improve in several weeks.
Child and parents need to understand that “cheating” will result in return of symptoms and increase chance of complicationshigh risk of GI lymphoma.
Must learn to read labels for hidden sources of gluten, ex. Wheat is often added to bake goods or used as a thickener.
Adolescents may not be able to have typical foods – hot dogs, pizza, spaghetti etc.
Diet is generally high in calories and protein with simple carbohydrates, fruits and vegetables, low in fat. Must be continued for life
May need supplement of fat soluble vitamins
Prognosis is generally excellent

39. Phenylketonuria – PKU
ETIOLOGY: genetic disease caused by recessive trait, primarily affecting white children of northern European ancestry.
PATHOLOGY: absence of an essential amino acid necessary to metabolize phenylalanine, resulting in lack of tyrosine, needed to produce melanin, epinephrine and thyroxin.
Child is typically blonde; blue eyed and fair skinned. Accumulation of levels in CNS result in mental retardation with bizarre or schizoid behavior, screaming, biting, head banging, and catatonic reactions. Elimination in urine results in child having a musty odor.
Diagnosed by Guthrie Test, mandatory on all newborns (heel stick) – done during Newborn Screen

40. Phenylketonuria – PKU Nursing Interventions
Help parents understand that PKU is treated with dietary restrictions of phenylalanine until adolescence. Diet is begun immediately after diagnosis
Refer parents to a nutritionist for counseling
Infant formula low in phenylalanine is used: Lofenalac. Because breast milk is low in phenylalanine, mother may choose to feed with close monitoring of levels in infant
Mothers with PKU must resume diet before pregnancy to prevent damage to fetus if positive. Mother has 50% chance of transmission
Since phenylalanine is a necessary amino acid, daily allowable amount are calculated for each child and changed for growth and development
Most high protein foods, meats and dairy products are avoided.

41. .
Colic

42. Colic
Intermittent abdominal pain or cramping manifested by loud crying and drawing legs up to abdomen. Usually crying continues 3 or more hours/day and resolves spontaneously around 3 months of age.
Slight chance it may be due to milk allergy, but most commonly no cause can be found and infant eats well, gains weight, and thrives.
However, because of perceived “difficult” temperament, child-parent relationship may be affected.