Cleft palate (palatoplasty; maximize speech production and growth of midface)
DIAGNOSIS OF CLEFT LIP AND PALATE
Newborn Screening
Transabdominal ultrasound can be performed as early as 13 to 14 weeks’ gestation
The upper lip, alveolar arches, nostrils, and primary and secondary palates is inspected and palpated.
NURSING MANAGEMENT OF CLEFT LIP AND PALATE
a.Feeding
Best accomplished with the infants head in an upright position
Special bottles for feeding (HabermanBottle)
b. POST-OP
Cleft lip: avoid prone position
Cleft palate: prone position
Elbow restraints (remove at intervals)
Avoid use of suction, tongue depressor, thermometers, spoons, and straws
Hirschsprung Disease is also known as congenital aganglionic megacolon
HirschsprungDisease - congenital anomaly in which inadequate motility causes mechanical obstruction of the intestine (failure of the bowel to relax)
Ganglion cell - responsible for relaxing the colon
THERAPEUTIC MANAGEMENT OF H.S DISEASE
a.Surgery ( Two Stages)
1st: Creationoftemporary ostomy site to relieve obstruction
2nd: Complete corrective surgery or Soave endorectalpull-through (remove the segment of the colon that has no ganglion cell)
b. Anorectalmyomectomy (very short-segment disease)
Clinical manifestations of Hirschsprung disease
Abdominal distention
Feeding intolerance
Bilious vomiting
Failure to pass meconium within the first 24 to 48 hours after birth.
Constipation
Ribbon-like and foul smelling stool
Diagnosis of Hirschsprung Disease
History and Physical examination
Rectalbiopsy for presence or absence of ganglion cells.
Barium Enema ( injection of Barium Sulfate via enema)
Anorectal manometry (measures the capacity of the rectum to pass the stool)
NURSING CONSIDERATION OF H.S DISEASE
Observe the passage of meconium (if the baby was able to excrete meconium within 24 hrs)
Helping parents to adjust to the congenital disorder
Preparing the parents for the medical-surgical procedure
Detect abdominal distention
Intussuception - Telescoping or invagination of the proximal segment to a more distal segment that results in lymphatic and venous obstruction
CLINICAL MANIFESTATION OF INTUSSUCEPTION
• Sudden onset of abdominal pain
• Inconsolable crying
• Drawing up of knees on the chest
• Bilous vomiting
• Palpable sausage-shaped mass in the abdomen
• CurrantJelly-like stool
DX OF INTUSSUCEPTION
• Subjective findings
• Barium enema
• Abdominal radiography
• Rectal exam (mucus and blood)
NURSING MANAGEMENT OF INTUSSUCEPTION
Assist in establishing DX
Explain the basic defect to the parents and how it is corrected
TREATMENT OF INTUSSUCEPTION
radiologist-guided pneumo-enema (air enema); not recommended if there are signs of perforation
Intravenous fluids
NG decompression
antibiotic therapy
surgical intervention
Appendicitis - inflammation of the vermiform appendix, the small sac near the end of the cecum, and is the most common cause of emergency surgery in children.
ETIOLOGY OF APPENDICITIS
a fecalith (hard fecal mass)
parasitic infestations
stenosis
hyperplasia of lymphoid tissue
a tumor
CX MANIFESTATIONS OF APPENDICITIS
Colicky
Pain in the periumbical area
Blumbergsign (pain upon removal of palpation (pressure) rather than application of pressure)
Rovsingsign (palpating other quadrants but the pain was felt on RLQ)
Psoassign (px in supine position; apply your hand (pressure) on the right knee and ask the px to lift it. px will feel pain on RLQ)
Obturatorsign (px in supine position; bend and lift the right knee, then rotate it. px will feel pain on RLQ)
Diagnosis of appendicitis
Elevated whitebloodcell count (above 10,000/mm3 )
Abdominal ultrasound
CT scans
NURSING MANAGEMENT OF APPENDICITIS
a.Assist in establishing diagnosis
b. Instruct the child to pint a finger on the painful region
c. Light palpation induced pain
d. PRE-OP CARE
Don’t administer painrelievers (paracetamol); it can mask the ruptureappendicitis that causes relief of pain
PX is NPO
Administer IV fluids and antibiotics
e. POST-OP CARE
Dressing covering the incision
Antibiotics
If px has perforated appendix, remain NPO until bowel function return
Give Morphine for pain reliever
Hypertrophic pyloric stenosis (HPS) - circumferential muscle of the pyloric sphincter becomes thickened, resulting in elongation and narrowing of the pyloric channel.
CLINICAL MANIFESTATION OF HPS
projectile vomiting
fails to gain weight
dehydration
olive-sized mass in the right upper quadrant may be evident
DIAGNOSIS OF HPS
History and Physical examination
Ultrasonography (determine the diameter and length of the pyloric muscle)
Blood test (determine if the child is dehydrated or has electrolyte or acid–base imbalance)
Upper GI radiography
NURSING MANAGEMENT OF HPS
• Careful regulation of fluid therapy
• Minimizing weight loss
• Promote rest and comfort
• Preventing infection
• Reestablishment of normal feeding patterns
• Encourage the parents to become involved in caring for the child
Laparoscopic pyloromyotomy is the preferred surgical method to correct pyloric stenosis
Celiacdisease is a sensitivity or abnormal immunologic response to protein, particularly the gluten factor of protein found in grains—wheat, rye, oats, and barley.
CLINICAL MANIFESTATION OF CELIAC DISEASE
impaired growth
chronic diarrhea
abdominal distention
muscle wasting with hypotonia
poor appetite
lack of energy
DIAGNOSIS OF CELIAC DISEASE
Presence of antigliadin and antiendomysial immunoglobulin and their disappearance when gluten is removed from the diet
Villous atrophy and hyperplasia in children who eat a diet with gluten
a.Due to inflamed bowel the child must avoid highfiberfoods
nuts, raisins, raw vegetable, raw fruits with skin, until inflammation subsides
NURSING CONSIDERATION OF CELIAC DISEASE
helping the child adhere to the dietary regimen.
explaining the disease process to the child and parents,
the specific role of gluten in aggravating the disorder, and the foods that must be restricted.
Hypopituitarism (dwarfism) - diminished or deficient secretion of pituitary hormones
2 CATEGORIES OF DWARFISM
a.DisproportionateDwarfism
The body size is disproportionate, and some parts of the body are small while others are of average size or above average size
b. ProportionateDwarfism
The body is proportionately small; all parts of the body are small to the same degree
CLINICAL MANIFESTATION OF DWARFISM
An average-size trunk
Short arms and legs
Short fingers
Limited mobility at the elbow
A disproportionate large head
Progressive development of bowed legs
Progressive development of lower back
An adult height around 4 feet (122 cm)
NURSING CONSIDERATION OF DWARFISM
identifying children with growth problems
special emotional adjustments for these children
key person in helping to establish a diagnosis
observed carefully for signs of hypoglycemia; generated by tests with insulin
PITUITARY HYPERFUNCTION (GIGANTISM) - Excess GH before closure of the epiphyseal shafts results in proportional overgrowth of the long bones until the individual reaches a height of 2.4 m (8ft) or more.