Common Acquired and Congenital Anomalies

Congenital Anomalies
Occur in about 2% - 3% of all live births (Steele, 1997)
ées 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
Variations in clefts of lip and palate

Etiology
Defective development of embryonic primary palate occurring on 7 th 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)
Sonogram of fetus w/ cleft lip
Signs and symptoms
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
Cheiloplasty
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
Specialty feeding devices

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:
Maintain patent airway
Position properly for drainage:
Cleft lip: side-lying, never prone
Cleft palate: prone, never supine
Suction gently and carefully: insert suction catheter along non-operative side
Prevent injury to suture line:
Maintain Logan bar over suture line post cheiloplasty
Proper position and positional changes
Apply bilateral elbow restraints
Remove Q2 hrs, one @ a time, under supervision
Provide arm exercises
Prevent sucking – use rubber-tipped syringe/dropper, paper cup
Minimize crying
Anticipate needs
Soothing tactile stimulation
Diversion
Play activities – exc. Pacifiers, teethers
Logan bar – apparatus used to protect surgical incision
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
Imperforate Anus
Definition:
A congenital anorectal malformation in w/c the rectum ends in a blind pouch
connecting it to the vagina (recto-vaginal fistula), or to the urethra (rectourethral
fistula)
Incidence:
1 in 5000 live births
More commonly in boys
Variations

Etiology
Failure of upper bowel to meet w/ pouch from perineum èabnormal positioning of
the caudal hindgut by the anorectal septum in the 7 th - 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 defecaion 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
Sonogram

Treatment: Surgery
Anoplasty for simple type
Full-through operation w/ or w/o temporary colostomy

Nursing Care
Early detection
Observe newborn for passage of meconium 1st 24 hrs – confirms anal patency

Provide preoperative care

Observe NPO; pacifier to suck
Maintain NGT to decompress stomach
Provide warmth
Monitor VS
Prepare parent for surgery and for temporary colostomy, if necessary

Provide postoperative care:

Prevent infection
Meticulous skin care; perirectal care, observing strict aseptic techniques
Administer and maintain IVF
Monitor rate of flow; strict I&O measurement; daily weight
Provide oral feedings
w/ colostomy, begin oral feedings slowly once stools have been passed;
I&O; w/ pull-throughs – oral feedings slowly once peristalsis begins
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
S/Sx:
Newborns:
No meconium stools
Bile-stained vomitus
Abdominal distention
Feeding difficulties
Abdominal pain: irritability, crying
Infants:
Chronic constipation – hallmark of megacolon
Explosive diarrhea
Bile-stained vomitus
Abdominal distention
Failure to thrive/malnutrition
Older children
Chronic constipation
Ribbon-like stools (pellet-like)
Abdominal distention
Palpable fecal mass
Visible peristalsis
Fecal odor of breath
Anemia
malnutrition
Diagnosis
History and physical exams
Barium enema
Abdominal x-ray
Rectal biopsy: confirms megacolon
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
Incidence:
Hyperactivity of infant’s digestive tract è intussusception
Age of onset: 3 to 12 months
More common in males (3:1)
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