Ateneo de Zamboanga University

College of Nursing
NCM 109j – PEDIA

MARIDEL B. DAGALEA RN MN

Common Acquired and Congenital Anomalies (INFANCY)

Congenital Anomalies:

Occur in about 2% - 3% of all live births (Steele, 1997)

-es to 6% by 5 years
-When more anomalies are diagnosed
-Interdisciplinary team approach – vital for providing holistic care:
-Surgical treatment
-Rehabilitation and education of the child
-Psychosocial & financial assistance for the parents
-Parental disappointment and disillusion add to the complexity of the nursing care needed for these
infants.

Gastrointestinal System Anomalies:

Cleft Lip and Palate
Definition:
Cong. facial malformation characterized by non-fusion of facial processes (cleft lip), and
non-fusion of tissue and bone of the hard and soft palate (cleft palate) happening during
embryonic life
 *(A) incomplete unilateral cleft of the lip, (B) unilateral cleft of the lip, alveolus, and palate, (C)
bilateral cleft of the lip, alveolus, and palate, (D) isolated (median) cleft palate

Etiology:
Defective development of embryonic primary palate occurring on 7th to 8th week of fetal life, and
defective development of embryonic secondary palate (cleft palate) occurring about the 7th to 12th
weeks of fetal life

Incidence:
Cleft lip more frequent, males
Cleft palate higher in females

Risk/predisposing factors:
Multiple genetic factors
Cleft lip – familial tendency
Environmental factors:
Maternal infection (viral infections)
Radiation exposure
Alcohol ingestion
Tx w/ meds like: corticosteroids, some tranquilizers, antiepileptics
Signs and symptoms:
Visible facial defect (cleft lip)
Visible defect on physical examination (cleft palate)
Sucking difficulties
Due to inability to create suction (cleft lip)
Swallowing difficulties (more in cleft palate); feeding difficulty (most common prob. presented
by parents)
Abdominal distention
Susceptibility to infection
Respiratory (aspiration pneumonia),
Mouth (oral thrush),
Ear (otitis media)
Diagnostic Tests:
Sonogram while in-utero
Physical assessment
Treatment:
Correction/repair through plastic surgery in later life
Cheiloplasty – repair of cleft lip
Done in infancy: 2 – 3 mos (2 – 10 weeks, Pilliteri)
Often follows “RULE OF 10”
10 weeks old
10 lbs in weight
10 gms in hemoglobin
Treatment:
Uranoplasty
Repair of cleft palate
Best done in toddlerhood before speech training period:
18 – 36 months
Complications;
Speech problems/faulty speech/speech disturbance
Hearing problems/loss
Body image problems
Dental problem: malposition of teeth
Infection
Nursing Care;
Provide preoperative care:
Careful feeding: small, frequent, slow w/ infant in upright position; w/ frequent burping,
using appropriate feeding tools:
Cleft lip: rubber-tipped medicine dropper or syringe
Cleft palate: large-holed, soft nipple or paper cup

Preoperative Care:
Prepare parents for surgical procedures:
Arrange for a multidisciplinary meeting to discuss the short- and long-term plans of care
Provide psychological support:
Be present during initial mother-newborn contact
Don’t show discomfort in caring for infant
Verbalize positive traits of infant
Allow expression of feelings and concerns
Show pictures of “before and after surgery”
Provide postoperative care:
Provide mouth care/wash q after feeding, w/ water to prevent infection
Avoid rubbing motion; pat gently to dry
No fork, spoon, and straw, no ice drop
Liquid diet, except milk
Could form curds – difficult to clean
Prevent infection
Antibiotics as ordered
Meticulous mouth care, suture line cleansing and proper drying

Etiology:
Failure of upper bowel to meet w/ pouch from perineum ➔abnormal positioning of the caudal
hindgut by the anorectal septum in the 7th - 8th week of gestation
Signs and symptoms:
Absence of anal opening on inspection
Non-insertion of rectal thermometer (not practiced anymore)
Progressive abdominal distention
Difficult defecation or inability to defecate
Absence of meconium passage in 1st 24 hours
Passage of meconium from inappropriate opening (vagina or urethra)
(-) “wink” reflex
Diagnostic tests/procedures:
Wangesteen-rice method/ x-ray
Sonogram
Infant held upside – down
To allow swallowed air to rise to the end of the blind pouch of the bowel
Helpful also to estimate distance the intestine is separated from perineum or extent of correction
necessary

Postoperative care:

Provide parental teaching on colostomy care:

Empty pouch as needed; skin care:
Clean peristomal area w/ mild soap and water, dry thoroughly; apply clean
pouch
Use skin barrier as ordered
Psychological support:
Visual and tactile stimulation
Support parents

Hirschsprung’s Disease
(Congenital Aganglionic Megacolon)
A mechanical obstruction of the bowels due to the absence of autonomic parasympathetic
nerve ganglion cells in the distal bowel resulting in inadequate bowel motility
Etiology: unknown
Incidence:
Higher in children w/ Down’s syndrome
Higher in males – 4:1 male-female ratio
 Predisposing/precipitating factors:
Familial factor, Down syndrome

Treatment: Surgery
Preop care
NPO; pacifier
Prepare/assist NGT insertion
Monitor I&O
Provide warmth
Meet emotional needs thru soothing touch, pacifier, consistent care
Low-residue, high-CHON and high-calorie diet, if appropriate (childhood)
Parenteral nutrition as ordered
Bowel cleansing:
Liquid diet
Stool softeners as ordered
Digital removal of stools
Daily isotonic saline enemas/colonic irrigation
Post-op Care:
Monitor respiratory status: VS, I&O, electrolytes, stools
Maintain hydration and nutrition: monitor for abdominal distention
Keep incision site clean and dry
Assess colostomy fxning; colostomy care: meticulous skin care
Pain relief: analgesics PRN
Prevent complications:
Respiratory infection: coughing, deep breathing, turning Q2H
Skin infection: meticulous skin care
 Patency of NGT
Psychological support

Intussusception:
Characterized by the invagination or telescoping of the intestine along any point of the intestinal
tract (usually in the ileocecal valve), resulting in intestinal obstruction and interference w/ the
passage of the intestinal contents
Most common cause of bowel obstruction in children
Etiology:
Generally unknown
Associated w/ celiac disease, cystic fibrosis, and intestinal polyps

S/Sx:
Recurrent colic
Abrupt intestinal pain elicited by pulling knees up to the chest, as evidenced by crying
and screaming
“Currant jelly” stools
w/ blood, mucus, and pus
Abdominal distention w palpable “sausage-shaped” mass @ the right upper quadrant
Empty lower right quadrant = Dance sign
Vomiting, prostration, schock
Fever and progressive acute illness
Complications:
Peritonitis, shock, or death if untreated
Dxnosis:
HX taking, clinical signs
Barium enema
May also reduce the intussusceptions
Treatment:
Barium or saline enema to effect hydrostatic reduction of intussusceptions
Reduction by air pressure insufflations
Surgical correction
If medical mgt is unsuccessful and child has peritonitis and shock

Nursing Care:
Monitor VS, electrolyte levels, urine, stools
Psychological support
Observe for passage of stools or barium after hydrostatic reduction
Postoperative care:
Fluids and electrolytes status:
Urine output evidence of good hydration:
1mL/kg/hr
Monitor return of bowel sounds and stools
Observe for sign of recurrent obstruction

Otitis Media
Inflammation of the middle ear
Most prevalent dse of childhood after RTI (Kelley, 2008)
Extremely serious dse of childhood
Permanent damage can occur to middle ear structures ➔hearing impairment
ETIOLOGY:
S. pneumoniae, H. influenzae (< 5 yrs old)
Incidence:
6 – 36 mos.; 4 – 6 yrs
Males; w/ cleft palate
Formula-fed; constant pacifier use
Held in a more slanted position – allows milk to enter the eustachian tube
Highest in winter and spring
Homes in which a parent smokes cigarettes
Assessment:
Infants: extremely irritable;
pull/tug @ affected ear
To gain relief from pain
Therapeutic Management:
Most resolve spontaneously w/o therapy
Ampicillin, Amoxicillin (for H. influenzae)
Today – antibiotic therapy may add to bacterial resistance = no longer routinely prescribed
Analgesic, antipyretic
Nasal decongestant only for 3 days (rebound effect)

Therapeutic Management:

Parents’ education:
Caution parents about conductive hearing loss up to 6 mos. after acute infection
Possible hearing loss
Conductive hearing loss after 6 mos (or has other sx) = shd be examined again
for new infection or serrous otitis media
Chronic or persistent otitis media = staphylococcus
Tx: Cephalosporin
Otitis Media w/ Effusion (OME):
occurs when fluid, called an effusion, becomes trapped behind the eardrum in one or both ears,
even when there is no infection.
In chronic and severe cases, the fluid is very sticky and is commonly called "glue ear.”

Signs and symptoms:

usually not painful
child complains of "plugged up" hearing
Feeling like "being under water."
may impair children's hearing
loud talking, not responding to verbal commands, and turning up the television or radio.
Most episodes resolve w/in 3 months
30 - 40% of children may have recurrent episodes.
Only 5 - 10% of episodes last longer than 1 year.
Other S/Sx;
Sound of popping or ringing in the ears
Drop in hearing of 20 – 40 dB
Due to fluid content
Occurs most frequently in children 3 -10 yrs of age
Therapeutic Management:
Aimed @ supplying air to the middle ear
Mild involvement:
Daily antihistamine or nasal decongestant
shrink mucous membrane of eustachian tube
Enlarged adenoids: adenoidectomy
Fluid from middle ear:
Tympanocentesis
Therapeutic Management:
When myringotomy tubes are in place:
Water shd not be allowed to enter child’s ears
Bathe rather than shower (allowed if w/ ear plugs)
Swimming and hair washing – w/ ear plugs
Teach parent to continue meds
support to accept insertion of myringotomy tubes, and other procedures (cutting of eardrum)