d.
Cyanosis
I. ESOPHAGEAL ATRESIA
DEFINITION
- Congenital malformation of the esophagus in which
it fails to develop as a continuous passage due to
faulty embryonic development.
- It could be of the simple type, in which the
esophagus ends blindly and does not connect to the
stomach.
- It could also be of the tracheoesophageal fistula
types, in which the esophagus (upper or lower ends)
connects with the trachea through a fistula.
PREDISPOSING/PRECIPITATING FACTORS
1. small-for-gestational age (SGA), prematurity
2. Maternal polyhydramnios
3. Presence of associated anomalies: anorectal
malformations, genitourinary defects, congenital
heart defects
ETIOLOGY
- Defective development during the fourth and fifth
weeks of gestation
INCIDENCE
- Most common form has the proximal end of the
esophagus ending blindly in a closed pouch and the
lower distal end communicating with the trachea and
forming a fistula
SIGNS AND SYMPTOMS
1. Early signs in the neonate
a. Excessive drooling
b. 3Cs: choking, coughing, cyanosis
c. Inability to pass the catheter through the nose to
the stomach
2. Later in infants
a. Excessive salivation/excessive mucus
b. Return of fluid/feeding through the nose and
mouth
c. Coughing
e. Abdominal distention
DIAGNOSTIC TESTS/PROCEDURES
1. X-ray
2. Failure to pass a radiopaque catheter into
esophagus
COMPLICATIONS
1. Respiratory distress/failure
2. Infection: aspiration pneumonia
SURGERY IN 24-48 HOURS
1. Temporary gastrostomy tube, primarily for feeding
2. Esophageal anastomosis for simple esophageal
atresia
3. Esophageal anastomosis with the division of the
fistula for tracheoesophageal fistula/TEA
NURSING CARE
1. Provide preoperative care.
a. Suction oropharynx
b. Oxygen PRN; humidify air
c. Keep upright; change position to prevent pneumonia
d. Maintain NPO, oral hygiene
e. Monitor I&O
f. Observe for signs of respiratory distress
g. Keep warm
2. Provide postoperative care.
a. Position properly: head of bed elevated 30 degrees.
a. Turn every two hours; stimulate crying to
expand the lungs.
b. Suction as ordered.
c. Provide high humidity to liquefy discretions:
55% to 65% humidity in incubator.
d. Monitor:
 Signs of respiratory distress
 I&O
 Daily weight
 Signs and symptoms of infection
b. Maintain nutrition and hydration.
 Monitor IV fluids.
 Observe NPO; resume oral feedings 10-14
days after surgery. Provide pacifier to meet
emotional need.
c. Administer oxygen as ordered; provide chest tube
care.
d. Maintain strict aseptic technique to surgical site.
e. Give ordered antibiotics to prevent infection.
f. Provide physical comfort/touch. Encourage parents
to cuddle, rock infant.
g. Provide discharge instructions:
  Suctioning
 Feeding techniques
 Signs and symptoms of respiratory distress III. PYLORIC STENOSIS
 Signs of esophageal stricture: dysphagia, DEFINITION
choking, coughing - Congenital hypertrophy/hyperplasia of the muscles
of the pylorus, causing destruction of the pyloric
sphincter
II. CHALASIA / GASTROESOPHAGEAL REFLUX
DEFINITION ETIOLOGY
- Abnormal relaxation of the sphincter of the stomach - Unknown
resulting in self-limiting vomiting
INCIDENCE
ETIOLOGY - Higher in Whites and male
- Unknown; common in babies of tense mothers
COMPLICATION
COMPLICATIONS - Metabolic alkalosis from vomiting and loss of
- Metabolic alkalosis and dehydration hydrochloric acid

SIGNS AND SYMPTOMS

1. self-limiting, non-projectile, non-bile vomiting
2. Regurgitation after feeding
3. Dehydration:
a. Sunken fontanel
b. Rapid, thread pulse
c. Absence of tears
d. Scanty, concentrated urine
e. Poor skin turgor
f. Dehydration fever
4. Increased hunger
5. Weight loss
NURSING CARE
1. Observe correct feeding techniques.
a. Feed infant in upright position; keep him/her
upright for 30 minutes more after feeding.
b. Feed slowly; burp during and after feeding.
c. Refeed with a thicker formula, which is more
difficult to vomit.
d. Allow play before feeding time to relax the
mother, and therefore child is prevented from
vomiting as he gets relaxed, too.
e. Do not overfeed. Overfeeding is the most
common cause of infant vomiting. To prevent
overfeeding, consider the infant’s gastric
capacity.
2. Provide psychological support.
3. Observe for signs of dehydration; the earliest sign of
dehydration in infants is sunken fontanels.
SIGNS AND SYMPTOMS
1. Non-bile, non-projectile vomiting
2. Increased hunger, dehydration
a. Sunken fontanels (first sign of dehydration in
infants)
 Sunken eyeballs
 Dry mucus, thirst
 Poor skin turgor/non-elastic skin
 Lethargy, weakness
 Oliguria
 Fever
b. Visible gastric (left-right) peristalsis
c. Olive-shaped mass at the right upper quadrant
d. Abdominal distention
e. Constipation or decreased number of stools
f. Failure to thrive; decreased weight
DIAGNOSTIC SIGNS/PROCEDURES
1. Physical assessment and history-taking
2. Serum electrolytes
3. Upper GI series: delayed gastric emptying time and
narrowing of pyloric sphincter
TREATMENT
1. Medical: measures to prevent vomiting, IV fluids
2. Surgical: pyloroplasty, pyloromyotomy, Fredet-
Ramstedt-creation of longitudinal incision into the
muscles of the pylorus to create a gaping wound
NURSING CARE
1. Preoperative care
a. Maintain NPO with IVF and NGT.
b. Observe, monitor I&O, vomiting, NGT drainage,
stools, and weight.
c. Keep warm.
2. Postoperative care
a. Maintain a patent airway: suction PRN, side-
lying position initially, then head up position on
infant seat.
b. Monitor for signs of respiratory shock.
c. Provide adequate nutrition and hydration.
 Monitor I&O, IV, and weight.
 Feed (only by an RN) about 2-8 hours or 4-6
hours after surgery.
 Frequent burping: before, during, and after
feeding.
 Position on infant seat after feeding, with
head turned to one side. Provide minimal
handling to prevent vomiting.
IV. CLEFT LIP & CLEFT PALATE
DEFINITION
- Congenital facial malformation characterized by non-
fusion of facial processes (cleft-lip), and non-fusion
of tissue and bone of the hard and soft palate
happening during the embryonic life (cleft-palate)
ETIOLOGY
- Defective development of embryonic primary palate
occurring on the 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 is more frequent than cleft palate and more
common in males; cleft palate is higher in incidence
in females
SIGNS AND SYMPTOMS
1. Visible facial defect (cleft lip); visible defect on
physical examination (cleft palate)
2. Sucking difficulties due to inability to create suction
(primarily in cleft lip)
3. Swallowing difficulties (more in cleft palate); feeding
difficulty is the most commonly present problem by
parents of these infants.
4. Abdominal distention
5. Susceptibility to infection: respiratory infection
(aspiration pneumonia), mouth infection (oral thrush),
and ear infection (otitis media)
DIAGNOSTIC TESTS/PROCEDURES
1. Physical assessment and findings
TREATMENT
1. Correct/repair through plastic surgery in later life
a. Cheiloplasty: repair of cleft lip
 Done in infancy: 2 to 3 months
 Often follows the ‘Rule of 10:’ ten weeks old,
10 pounds in weight, and ten grams of
hemoglobin
b. Uranoplasty: repair of cleft palate
 best done in toddlerhood before the speech
training period; 18 to 36 months
POSSIBLE COMPLICATION OF CLEFT LIP/CLEFT
PALATE
1. Speech problems / faulty speech / speech
disturbance
2. Hearing problems / loss
3. Body image problem
4. Dental problem or malposition of teeth
5. Infection
NURSING CARE
1. PROVIDE PREOPERATIVE CARE
a. Careful feeding with small, frequent and slow
feeding with the infant in an upright position and with
frequent burping, using appropriate feeding tools
 Cleft lip: rubber-tipped medicine dropper or syringe
 Cleft palate: large-holed, soft nipple or paper cup
b. Prepare parents for surgical procedure of the child
 Arrange for multidisciplinary meeting to discuss the
short and long term plans of care
 Provide psychological support:
 Be present during the initial mother-newborn
contact
 Do not show discomfort in caring for the infant
 Verbalize the positive traits of the infant
 Allow expression of feelings and concerns
 Show pictures of “before surgery” and “after
surgery”
2. PROVIDE POSTOPERATIVE CARE
a. Maintain a 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 the non-operative side
 Provide humidified air; cool mist tent care (palate)
b. Prevent injury to suture line:
 Maintain the LOGAN bar over suture line post
cheiloplasty
 Proper position and positional changes
 CHEILOPLASTY – back or side, never PRONE
 Apply bilateral elbow restraints:
 Remove every 2 hours, one at a time, under
supervision
 Provide arm exercises
 Prevent sucking. Use of rubber-tipped syringe
or dropper (CLEFT LIP) and paper cup (CLEFT
PALATE)
 Minimize crying:
 Anticipate child’s needs
 Provide soothing tactile stimulation
 Provide diversion
 Provide play activities
 Crib mobile
 Nothing that will be put into the mouth ---
- never teethers nor pacifiers
 Provide mouth care/wash every after feeding using
diluted H2O2 to prevent infection.
 Avoid rubbing motion during suture line
cleansing
 Pat gently to dry
 Do not use spoon, fork and straw; No ice drop
 Provide liquid diet, except milk, which could form
curds that would be difficult to clean
c. Prevent infection
 Administer antibiotics as ordered
 Provide meticulous mouth care, suture line cleansing
and proper drying
V. PHENYLKETONURIA (PKU)
DEFINITION
- An inborn error of metabolism transferred as an
autosomnal recessive gene in which there is
decreased liver enzyme phenylalanine hydroxylase,
resulting in the absence of decreased metabolism of
the amino acid phenylalanine to tyrosine needed to
form melanin:
1. Phenylalanine rises in the blood, causing brain
damage and mental retardation.
2. The absence of melanin causes blond hair, blue
eyes, and fair skin.
3. The dietary management of ‘no phenylalanine’ for
six to eight years and a low-protein diet results in
phenylalanine deficiency.
ETIOLOGY/PREDISPOSING FACTORS
- Condition inherited through an autosomal recessive
gene. It takes two parent-carriers before one out of
four children (25%) can have the disease.
SIGNS AND SYMPTOMS
1. Signs of phenylalanine deficiency: diarrhea, anorexia,
lethargy, anemia, and skin rashes (D-A-L-A-S)
2. Melanin deficiency: blond hair, blue eyes, and fair
skin.
3. Abnormal neurologic development: mental
retardation.
4. Musty odor of urine
DIAGNOSTIC TESTS/PROCEDURES
1. Guthrie capillary blood test. A screening test done
for two to four days after birth:
a. The screening test may result in a false negative
because the newborn had no adequate protein
intake during testing.
b. To prevent a false negative result, there should be
adequate preparation: high-protein diet (milk-feeding)
for 24-48 hours prior to screening test.
2. Ferric chloride urine test. An out-patient follow-up
screening test done in the community by the
community health nurse:
a. effective only when the infant is over two weeks old,
when brain damage may have occurred;
b. Ferric chloride (10%) is dropped onto the infant’s
diaper wet with urine. If PKU is present, the spot on
the diaper will turn green.
TREATMENT
1. Dietary treatment: phenylalanine-free formula;
lofenalac- a milk-free formula- for six to eight years
to prevent mental retardation.
2. Low-protein, low-phenylalanine diet after full brain
development
NURSING CARE
1. Implement dietary management:
a. Lofenalac to prevent high levels of phenylalanine, 6. Passage of meconium from inappropriate opening
which can cause mental retardation. (vagina or urethra)
b. Restrict foods high in phenylalanine and protein:
meat, eggs, green vegetables, and fruits. Apple may
be given, as it is low in phenylalanine.
c. Dietary management in PKU is to maintain
phenylalanine at a safe level: between 3-7
mg/100mL; high levels can cause mental retardation.
d. Nutrition referrals as needed.

2. Provide health teaching

a. The need for genetic counseling and screening of

future children. Early screening is a MUST to
prevent mental retardation.
b. Instruct on indicated and contraindicated foods and
the need for compliance with dietary regimen.

3. Provide psychological support: explain that mental

retardation is prevented with early implementation of
dietary regimen.

NURSING CARE
1. Early detection (the most important single
responsibility of the nurse with respect to imperforate
anus): observe the newborn for the passage of
meconium in the first 24 hours

What used to be the practice of taking the newborn’s

initial temperature per anus to check for anal patency is
no longer considered as appropriate today. The passage
of meconium confirms anal patency and not the ability to
check temperature per anus

2. Provide preoperative care.

a. Observe NPO; provide pacifier to suck
b. Maintain NGT to decompress the stomach
c. Provide warmth
d. Monitor VS
e. Prepare parents for surgical procedures and for
temporary colostomy, if necessary
VI. IMPERFORATE ANUS
DEFINITION 3. Provide postoperative care:
- A congenital anorectal malformation in which the a. Prevent infection
rectum ends in a blind pouch or in a fistula  Provide meticulous skin care; provide perirectal
connecting it to the vagina (recto-vaginal fistula), or care, observe strict aseptic techniques
to the urethra (rectourethral fistula)
b. Administer and maintain IV fluids
ETIOLOGY  Monitor rate of flow- the single most important
- Abnormal positioning of the caudal hindgut by the action in caring for a child with IF infusion
anorectal septum in the 8th week of gestation.  Maintain strict I & O measurement.
 Check daily weight.
INCIDENCE
- Male to female ratio is 1:1; most common congenital c. Provide oral feedings
anomaly that is not compatible with life.
 With colostomy, begin oral feedings slow once stools
SIGNS AND SYMPTOMS have been passed
1. Absence of anal opening on inspection  Monitor I & O
2. Non-insertion of the rectal thermometer  With pull-through, begin oral feedings slowly when
3. Progressive abdominal distention intestinal peristalsis returns
4. Difficult defecation or inability to defecate
5. Absence of meconium passage in the 1st 24 hours d. Provide parenteral teaching on colostomy care
 2. Barium enema
 Empty pouch as needed 3. Abdominal x-ray
 Change pouch as necessary 4. Rectal biopsy: confirms megacolon
 Provide skin care:
 Clean the perilostomal area with a mild soap and TREATMENT: SURGERY
water; dry thoroughly; and apply a clean pouch 1. Bowel resection with temporary colostomy
 Use skin barrier as ordered to protect skin from 2. Abdomino-perineal pull-through by about one year
irritation
NURSING CARE
e. Provide psychological support
1. Provide preoperative care
 Provide age-related visual and tactile stimulation
 Support parents: explain diagnostic examination, a. Observe NPO; give a pacifier.
treatment regimen, and colostomy care and that it is b. Prepare for/assist in NGT insertation
temporary. c. Monitor I&O.
d. Provide warmth.
e. Meet emotional needs through soothing touch,
VII. HIRSCHSPRUNG’S DISEASE (CONGENITAL pacifier, and consistent care.
AGANGLIONIC MEGACOLON) f. Administer low-residue, high-protein, and high-
DEFINITION calorie diet if appropriate (childhood).
- A mechanical obstruction of the bowels due to the g. Provide parental nutrition as ordered.
absence of autonomic parasympathetic nerve h. Provide bowel cleansing:
ganglion cells in the distal bowel resulting in  Liquid diet
inadequate bowel motility  Stool softeners as ordered
 Digital removal of stools
ETIOLOGY  Daily isotonic saline enemas/colonic irrigation.
- Unknown real cause Volume of liquid:
 Infant: 150-250 mL
INCIDENCE  Toddler: 250-350 mL
- Higher in children with Down’s Syndrome; higher in  Preschool: 300-500 mL
males (4:1 male-female ratio)  School-age: 500-700 mL

PREDISPOSING/PRECIPITATING FACTORS 2. Provide postoperative care

- Familiar factor, Down’s Syndrome a. Monitor respiratory status: VS, I&O, electrolytes; and
stools
SIGNS AND SYMPTOMS b. Maintain hydration and nutrition: oral fluids as
1. NEWBORNS ordered as soon as bowel sounds return; advanced
a. No meconium stools diet as tolerated. Monitor for abdominal distention.
b. Bile-stained vomitus c. Keep incision site clean and dry.
c. Abdominal distention d. Assess for correct colostomy functioning; provide
d. Feeding difficulties colostomy care: emphasize meticulous skin care.
e. Abdominal pain: irritability, crying e. Provide pain relief: analgesics PRN.
f. Prevent complications:
2. INFANTS  Respiratory infection: Coughing, deep breathing,
a. Chronic constipation-the hallmark of megacolon turning every two hours
b. Explosive diarrhea  Skin infection: meticulous skin care
c. Bile-stained vomiting
d. Abdominal distention 3. Provide psychological support.
e. Failure to thrive/ malnutrition a. Stroke, hold, and cuddle infant.
b. Explain to parents diagnostic and treatment
3. Older Children procedures.
a. Chronic constipation-hallmark
b. Ribbon-like stools (stools like pellets)
c. Abdominal distention VIII. INTUSSUSCEPTION
d. Palpable fecal masses DEFINITION
e. Visible peristalsis - A condition characterized by the telescoping of the
f. Fecal odor of the breath intestine along any point of the intestinal tract
g. Anemia (usually in the ileocecal valve), resulting in intestinal
h. Malnutrition obstruction and interference with the passage of
intestinal contents.
DIAGNOSTIC TESTS/PROCEDURES
1. History and physical examination ETIOLOGY
- Generally unknown; associated with celiac disease,
cystic fibrosis, and intestinal polyps
INCIDENCES
1. Hyperactivity of infant’s digestive tract
intussusception
2. Age of onset: three to twelve months
3. More common in males (male-to-female ratio-3:1)
SIGNS AND SYMPTOMS
1. Recurrent colic: abrupt intestinal pain elicited by
pulling the knees up to the chest, as evidenced by
crying and screaming
2. “Currant jelly” stools: stools with blood, mucus, and
pus
3. Abdominal distention with palpable “sausage-
shaped” mass at the right upper quadrant
4. Vomiting, prostration, shock
5. Fever and progressive acute illness
COMPLICATIONS: peritonitis, shock, or death
untreated.
DIAGNOSTIC TESTS/PROCEDURES
1. History taking, clinical signs
2. Barium enema; may also reduce
intussusceptions
TREATMENT
1. Barium or saline enema to effect hydrostatic
reduction of intussusceptions
2. Reduction by air pressure insufflations
3. Surgical correction if medical management is
unsuccessful and child has peritonitis and shock
NURSING CARE
1. Monitor VS, electrolyte levels, urine, and stools.
2. Provide psychological support to the infant (cuddling,
pacifier, consistent care, and parents:
a. Explain the nature of the infant’s condition.
b. Allow parents to express feelings, anxiety, and
concern.
3. Observe for passage of stools or barium after
hydrostatic reduction.
4. Provide postoperative care:
a. Monitor fluids and electrolyte status
b. Monitor return of bowel sounds and stools.
c. Observe for signs of recurrent obstruction.
IX. HIATAL HERNIA
DEFINITION
- The herniation or sliding upward of a portion of the
stomach into the thorax through the esophageal
hiatus.
Types:
1. Sliding hiatal hernia: the sliding of a portion of the
stomach into the thorax at the stomach lie within the
chest.
2. Paraesophageal hiatal hernia: herniation of a portion
of the stomach into the chest through a patent
esophageal hiatus with a normally in the reflux of
gastric juices and inflammation of the lower portion
of the esophagus.
ETIOLOGY
- Exact cause unknown
PREDISPOSING/PRECIPITATING FACTORS
1. Congenital weakening of the muscles of the
diaphragm around the esophagogastric opening
2. Associated with gatroesophageal reflux
3. Psychologic factors: abnormal mother-infant
relationship
INCIDENCE: the sliding hiatal hernia is more common
SIGNS AND SYMPTOMS
1. Fullness after eating and upper abdominal pain:
if usual symptoms
2. Heartburn with positional changes, especially after
feeding/meals
3. Regurgitation several hours after feeding/meals
4. Rumination: uncommon but serious form of
the regurgitation occurs in the latter half of infancy
5. Difficulty swallowing (dysphagia)
6. Respiratory distress signs in infants for severe form
DIAGNOSIS
1. Esophagoscopy reveals an incomplete cardiac
sphincter
2. Barium swallow displays a protrusion of the gastric
mucosa through a hiatus
TREATMENT
1. Primary treatment is directed at gastroesophageal
reflux and not at the hernia; 6 weeks’ trial with
intensive medical therapy (shorter period if with
recurrent aspiration and apnea)
a. Drug therapy: antacids (Cimetidine) and before
meals or at bedtime cholinergics
b. Thickened feedings with cereals
c. Careful burping
d. Prone position with head elevated 30 degrees
after feeding
2. Surgery: reduction of the hiatal hernia through either
an abdominal or thoracic approach if medical
treatment fails
COMPLICATIONS
1. Respiratory distress
2. Recurrent aspiration
3. Apnea
NURSING CARE
1. Provide a bland diet with six small feedings per day

2. Administer prescribed medications to reduce acidity
and discomfort.
3. Improve maternal-infant relationship (absence of
which is recognized as an important factor to
repetitive self-stimulating behavior causing
symptoms.)
a. Visual stimulation; eye-to-eye contact
b. Auditory stimulation; talk, sing, read stories to
child
c. Tactile stimulation: soothing touch, cuddling
4. Provide routine pre-op care. In addition, prepare
parents for chest tubes (if thoracic approach) and
NG intubation.
5. Provide routine post-op care. In addition:
a. Promote lung expansion: position on semi-
fowler’s; maintain close chest drainage.
b. Prevent or decrease gastric distension: maintain
NG drainage.
6. Provide discharge parental teaching.
a. Sit up position for feedings/meals and up to two
hours later
b. Give small, frequent meals slowly with rest
periods to prevent distension.
c. Avoidance of tight diapers or garters around the
abdomen.
d. Importance of treating persistent coughing.
X. BILIARY ATRESIA
DEFINITION
- A congenital absence (or fibrosis) of bile ducts,
causing obstruction of bile flow. Bile maybe within
the liver (intrahepatic) or in the main biliary system
or bile passages (extrahepatic), the most common
site
ETIOLOGY
- Exact cause unknown
PREDISPOSING/PRECIPITATING FACTORS
1. Prenatal factors/insult: maternal drug intake,
alcoholism, viral infection
2. Prenatal and intranatal vascular factors causing fetal
hypoxia
SIGNS AND SYMPTOMS
1. Pathologic jaundice: persists after 2 to 3 weeks post-
birth
2. Elevated direct and indirect bilirubin
3. Liver enlargement (hepatomegaly)
4. Abdominal distention
5. Clay-colored/putty-like stools
6. Dark-colored urine
7. Itching (pruritus) and scratching
8. Malnutrition (avitaminosis A-D-E-K) from impaired fat
metabolism
9. Bleeding episodes because of clotting disturbances
due to decreased vitamin K
10. Nonspecific lethargy, poor feeding
DIAGNOSTIC TESTS/PROCEDURES
1. Physical feedings
2. Serum bilirubin levels, direct and indirect
3. Confirmatory liver biopsy
TREATMENT
1. Under 2 months: jejunal segment to the liver to
double-barrel stoma
2. Liver transplant
NURSING CARE
1. Accurate history-taking; focus on the history of
neonatal jaundice, stool, and urine.
2. Monitor VS, urine, stools, bilirubin levels, and
abdominal girth.
3. Meet child’s nutritional needs.
a. Administer a special formula (portagen,
pregestimil) that does not require bile to digest;
encourage parental participation.
b. Administer ordered fat-soluble vitamins:
Vitamins A, D, E, and K
c. Feed in a head-up (30 degrees) position or use
infant seat.
d. Weigh daily.
4. Promote comfort: tepid or cool compresses for
intching.
5. Promote bonding; provide age-related stimulation
6. Provide psychological support to parents.
a. Encourage verbalization of feelings, fears, anxiety.
b. Explain all diagnostic and surgical procedures.
 Provide teaching on ostomy and its care.
 Arrange a discussion with the multi-disciplinary
team about liver transplant.
c. Refer to community resources for support, as needed.
RENAL
Renal diseases get worse slowly over at least 3
months. It can lead to permanent kidney failure. Kidney
disease has many possible causes, such as blood loss,
medicines, urinary blockage, genetic disease, or
infections. Treatment may include changes in diet,
medicines, dialysis, or surgery.

FLUIDS AND ELECTROLYTES

Water, a primary component of body fluids, is
the most abundant substance in the body. Water plays a
vital role in several physiologic
processes such as digestion, absorption and use of
nutrients, distribution, waste excretion, and perfusion
and maintenance of hemodynamics (Jain 2015; Schmidt
2010). Homeostasis of fluids and electrolytes occurs by
complex systems that ensure proper functioning.
Pediatric patients undergo rapid growth and
development associated with changing
pharmacokinetics from birth to adulthood. An
understanding of these ongoing dynamic changes is
essential for maintaining a patient’s fluid balance,
avoiding fluid and electrolyte derangement and
optimizing pharmacotherapy.

COMMON FLUID AND ELECTROLYTE DISORDERS

1. UTI
UTI occurs more often in females than in males:
about 8% in girls and 2% in boys (Lum, 2012).
Urinary pathogens seem to enter the urinary tract most
often as an ascending infection from the perineum and
are gram-negative rods such as Escherichia coli.
UTIs occur more often in girls than boys because the
urethra is shorter in girls, and because it is located close
to the vagina and anus, vulvovaginitis or rectal bacteria
can easily spread to the urethra.
Changing diapers frequently can help reduce the risk for
infection in infants. Girls should be taught early (when
they are toilet-trained) to wipe themselves from front to
back after voiding and defecating to avoid contaminating
the urethra.
It’s important that UTIs are treated so they do not
damage the bladder lining and/or spread to involve the
kidneys (pyelonephritis) and cause permanent damage.
Girls who have one UTI should be thoughtfully assessed
and, if a second UTI occurs, should be referred to a
fellowship-trained pediatric urologist to determine
whether any congenital urologic anomaly such as an
urethral stenosis or vesicoureteral reflux exists.
Any male older than the age of 3 months who presents
with UTI should also be evaluated by a pediatric
urologist.
ASSESSMENT
Although it may be possible to locate a UTI
precisely as urethritis, cystitis, urethritis, or
pyelonephritis, the
SIGNS AND SYMPTOMS
In young children often are not clear-cut, so all
types are lumped together and referred to as UTIs.
– The typical symptoms that occur in older
children or in adults—pain on urination,
frequency, burning, and hematuria—may not
be present in young children, so UTI is
suspected when a child has a fever with no
demonstrable cause on physical examination.
– If the infection is confined to the bladder
(cystitis), the child may have a low-grade fever,
mild abdominal pain, and day- or nighttime
enuresis.
– If the infection progresses to pyelonephritis, the
symptoms are generally more acute, with high
fever, abdominal or flank pain, vomiting, and
malaise.
– Urine for culture can be collected using a clean
catch technique, suprapubic aspiration, or
catheterization, so bacteria from the vulva or
foreskin do not contaminate the sample and give
a false result. Suprapubic aspiration is generally
limited to infants.
– Catheterization, also frightening and a potential
source of infection, is limited in children of all
ages.
– Urine obtained from suprapubic aspiration is
generally sterile, so any growth from this source
is significant.
– A clean-catch urine specimen is said to be
positive for bacteriuria if the bacterial colony
 count is more than 100,000 per milliliter. A
count of less than 10,000 per milliliter is
considered a negative culture.
– If the count is between 10,000 and 100,000 per
milliliter or if the urine is positive for proteinuria
(which could happen because of the presence of
bacteria), the count is usually repeated. In
addition to identifying the responsible organism,
microscopic examination of the urine specimen
may indicate the presence of red blood cells
(hematuria) caused by bacterial irritation of the
bladder mucosa. The presence of either red or
white blood cells or bacteria tends to make urine
more alkaline, so the pH of the sample will be
elevated (>7). Surveillance urine cultures, once
a common practice, are no longer
recommended.
THERAPUETIC MANAGEMENT
The medical treatment for UTI is the:
 oral administration of a broad-spectrum
antibiotic such as sulfamethoxazole-
trimethoprim (Bactrim) or amoxicillin, (Paintsil,
2013).
 Nitrofurantoin is also a good choice for UTIs
because it is a broad-spectrum antibiotic that
concentrates in the urine and can be used for both
treatment and prophylaxis. In addition to the
antibiotic, a child needs to drink a large quantity of
fluid to “flush” the infection out of the urinary tract .
 Cranberry juice is often recommended as being
highly effective in acidifying urine and making it more
resistant to bacterial growth.
 Water is still the best choice; If the child
experiences moderate to severe pain on
urination that interferes with the ability to void,
suggest the child sit in a bathtub of warm water and
void into the water.
 A mild analgesic, such as acetaminophen
(Tylenol), may help reduce pain enough to allow
voiding. Remind parents that with a UTI, treatment
with antibiotics must be continued for the full
prescription or the infection will return. A repeat
clean-catch urine sample obtained after
approximately 7 days of antibiotic dosing is
indicated for some children who have had multiple
recurrent UTIs. This is referred to as a “test of cure,”
and the goal is to confirm that the bacterial infection
has been obliterated and is not simply being partially
suppressed while the child is on antibiotics and will
surge back to culture-positive levels when the
antibiotics
are discontinued. After recurrent UTIs, children may
be prescribed a prophylactic antibiotic for 6 months
while measures are taken to optimize voiding habits
and hydration.
2. GLOMERULONEPHRITIS
 inflammation of the glomeruli of the kidney, may
occur as a separate entity but usually occurs in
children as an immune complex disease after
infection with nephritogenic streptococci (most
commonly subtypes of group A betahemolytic
streptococci) where complement, a cascade of
proteins activated by antigen–antibody reactions,
plugs or obstructs glomeruli. Immunoglobulin G (IgG)
antibodies against streptococci can be detected in
the bloodstream of children with acute
glomerulonephritis, proof the illness follows a
streptococcal infection (Kambham, 2012).
Intravascular coagulation occurs in the minute renal
vessels; ischemic damage from this leads to scarring
and decreased glomerular function. The glomerular
filtration rate decreases, leading to
an accumulation of sodium and water in the
bloodstream. The inflammation of the
glomeruli allows protein molecules to escape into
the urine.
ASSESSEMENT
 Acute glomerulonephritis is most common in children
between the ages of 5 and 10 years, the age group
most susceptible to streptococcal infections.
 Boys appear to develop the disease more often than
girls; it occurs more often during the winter and
spring, as do pharyngeal streptococcal infections.
The child typically has a history of a recent
respiratory infection (within 7 to 14 days) or
impetigo (within 3 weeks). All children who have
had a “strep” throat, tonsillitis, otitis media, or
impetigo caused by a streptococcal infection,
ideally, should have a urinalysis 2 weeks after
the infection to evaluate that glomerulonephritis
is not occurring.
 The disorder is announced by a sudden onset of
hematuria and proteinuria. The hematuria is
usually so extreme that the child’s urine
appears tea-colored, reddish-brown, or smoky.
After these initial urine changes, the child
develops oliguria. Specific gravity of urine
becomes elevated. Hypertension from
hypervolemia occurs. The child may have
abdominal pain, a low-grade fever, edema,
anorexia, vomiting, orheadache.
 There may be cardiac involvement such
as orthopnea, cardiac enlargement, enlarged
liver, pulmonary edema, a galloping heart rhythm,
or heart failure because of the difficulty in
managing the excessive plasma fluid. Blood
analysis will indicate a lowered blood protein level
(hypoalbuminemia) caused by the massive
proteinuria. As the child’s blood volume expands, a
mild anemia will also develop. As in all inflammatory
diseases, the erythrocyte sedimentation rate will
increase. Because the glomeruli cannot filter
properly, concentrations of urea, nonprotein
nitrogen (BUN), and creatinine in blood will
increase. If blood pressure reaches 160/100 mmHg
as part of the expanding circulatory volume,
encephalopathy may occur, with symptoms of
headache, irritability, seizures, vomiting, coma or
lethargy, and perhaps transitory paralysis, symptoms
all caused by cerebral ischemia (vasoconstriction of
 cerebral vessels that occurs to reduce cranial
pressure).

THERAPEUTIC MANAGEMET
 A course of antibiotics may be prescribed to be
certain all streptococci are removed from the
child’s system.
 Diuretics are of little value because obstructed
glomeruli cannot be made to function, although a
course of ethacrynic acid or furosemide (Lasix)
may be tried. If heart failure occurs, keeping the
child in a semiFowler’s position,
digitalization, and oxygen administration are
helpful. If diastolic blood pressure rises to more
than 90 mmHg, antihypertensive therapy with
an antihypertensive such as labetalol will be
prescribed.
 Phosphate binders, such as aluminum
hydroxide to reduce phosphate absorption in the
gastrointestinal tract, or a potassium-removing
resin agent, such as sodium polystyrene
sulfonate (Kayexalate), may be necessary in
children who have rising phosphate and
potassium levels because the kidneys are
unable to clear these from the circulation.
 Diet is controversial. Although restricting
salt may limit edema and limiting
protein intake may reduce the amount of protein
lost in urine, most children who are losing large
quantities of protein actually need more protein
to supplement this loss. Weighing the child
every day and calculating intake and
output are important assessments to follow the
course and extent of the disease.
 After 1 or 2 weeks, they can attend school and
engage in their usual activities, although
competitive activity is limited until kidney function
has returned to normal (about 2 months).
Caution parents that the results of a urine
protein test may remain abnormal for up to a
year, so if their child has this test done as a
routine screening procedure at a health checkup,
they don’t worry that the finding means
reinfection or the beginning of further disease.
 Glomerulonephritis
– Immunoglobulin G (IgG) antibodies against
streptococci can be detected in the bloodstream
GLOMERULUS – Inflammation of the glomeruli
– Intravascular coagulation occurs in the minute
renal vessels with ischemic damage that leads
to scarring and decreased glomerular function
 glomerular filtration rate decreases
 accumulation of sodium and water in the
bloodstream.
allows protein molecules to escape into the urine

PREDISPOSING FACTOR
1. Low socioeconomic status: overcrowding
2. Cold climate: high incidence of URTI
3. Streptococcal infections: strep throat, dental
abscess, tonsillitis, pharyngitis, otitis media, and
impetigo contagiosa

Glomerulonephritis
 Inflammation of the glomeruli of the kidney
 may occur as a separate entity but usually
occurs in children as an immune complex
disease after infection with nephritogenic
streptococci (most commonly subtypes of group
A betahemolytic streptococci)  Gross hematuria - tea-colored, reddish-
 a cascade of proteins activated by antigen– brown
antibody reactions, plugs or obstructs glomeruli.  Moderate proteinuria – Smoky urine
Hypertension from hypervolemia
(BUN), and creatinine increase.

OTHER SIGNS & SYMPTOMS

3. Oliguria with increased urine specific gravity
4. Moderate edema: facial and periorbital (around
the eyes) in the morning, and abdominal and
extremity edema later in the day
5. Moderate hypertension
6. Anorexia
7. Lethargy, irritability
8. Diarrhea due to bowel edema

DIAGNOSTIC TESTS/PROCEDURES
1. Urinalysis
2. Serum protein, creatinine, blood urea nitrogen (BUN)
Elevated antistreptolysin O (ASO) titre and rethrocyte
sedimentation rate (ESR)

TREATMENT
1. Bedrest during the acute stage or until edema,
hematuria and hypertension subside; may have
bathroom privilege.
2. Medications
A- ntibiotic therapy ( penicillin)
A- ntihypertensive
A- nticonsulvant (for hypertensive
 encephalopathy)
A- nti-inflammatory
D- igitalis
D- iuretics (rarely used; limited value)
3. diet: normal protein, moderate sodium, and
low potassium until urine output is normal; may
restrict fluid intake if edematous

NURSING CARE
1. Implement bedrest. Assist with bathroom
privileges.
2. Monitor:
a. VS (especially BP), temperature
b. Daily weight, I & O
c. Nature of urine: should improve from
smoky to clear, tea to amber yellow, and
scanty to normal volume
3. Increase/force fluids.
4. Provide proper diet
5. Provide health teachings, with focus on the
preventive measures:
a. Seek prompt medical treatment at the
first sign of the disease.
b. Prevent/prompt treatment of
streptococcal infection
c. Completion of ordered antibiotic
treatment for streptococcal infections
d. Increase fluid intake: at least 2.5 to 3
liters per day.
e. Urinary hygienic measures:
 Empty bladder promptly, frequently,
and completely whenever there is
an urge to void.
 For females:
– Use cotton underwear.
– Remove sanitary
napkins/pantyliners from
front to back
– Hold sanitary napkins on the
posterior side to prevent UTI
– Limit/avoid tub bathing.
6. Provide parental/child teaching for home care:
a. Daily weighing; report sudden excessive
b. increase in weight.
Bedrest with bathroom privileges
Monitor urine: volume, appearance.

1-5
RESPI
GASTRO
Hema

M6-7

FLUID RENAL ENDO NEURO AT MUSCULO


I. NEPHROTICS SYNDROME (NEPHROSIS)
DEFINITION
– A chronic renal disorder characterized by
alteration in the glomerular basement membrane,
allowing increased glomerular membrane
permeability to plasma protein; with remission
and exacerbation; acute phase lasts for four
weeks
ETIOLOGY
– Unknown
INCIDENCE
 Higher in the preschool age; peak age at two to
three years
 Slightly higher in males (60%)
 a collection of symptoms due to kidney damage
 includes protein in the urine, low blood albumin
levels, high blood lipids, and significant swelling
SIGNS AND SYMPTOMS
1. Excessive proteinuria causing urine to be dark,
“foamy” and cloudy
2. Hypoproteinemia
3. Decreased urine output (oliguria)
4. Severe, generalized edema (anasarca)
5. Insiduous weight
6. Microscopic hematuria
7. Normal blood pressure
8. Anorexia, pallor, fatigue
9. Susceptibility to infection
DIAGNOSTIC TESTS/PROCEDURES
1. Urinalysis for proteinuria, increased specific
gravity
2. Blood works: low serum protein
(hypoproteinemia)
TREATMENT
1. Bedrest during the acute edematous phase.
2. Steroid: corticosteroids (oral prednisone)
3. Immunosupressants to those who do not
respond to steroids or for whom steroids are
contradicted.
NURSING CARE
1. Maintain complete bedrest initially; ambulate
gradually after the acute edematous phase
2. Monitor VS including BP every two hours; urine
volume and protein, including specific gravity
every voiding; serum protein and electrolytes;
daily abdominal girth and weight.
3. Administer corticosteroids (oral prednisone) as
ordered.
4. Provide proper diet:
a. Offer small, frequent meals.
b. Give a no-added-salt diet during the
acute edematous phase
c. Give a high-protein, high-carbohydrate
diet, unless there is renal failure or
azotemia. In such a case, maintain a
low-protein diet.
5. Provide meticulous care of edematous skin.
a. Change position every two hours
b. Provide support to edematous scrotum:
use scrotal support.
c. No band aid on edematous skin.
6. Protect child from infection.
a. Avoid exposure to people with infection.
b. Administer antibiotics as ordered.
7. Meet psychological need.
a. Allow child and parents to express
feelings and concerns.
b. Provide quiet, age-appropriate
diversional and educational activities
c. Encourage peer visits/interactions.
8. Provide discharge instructions/teaching.
a. Administration of medication.
b. Testing of urine for protein.
c. Weighing daily.
d. Preventing infection.
e. Adhering to dietary regimen: high-
protein, increased potassium, and low-
sodium.
II. RENAL NEOPLASM
 A renal neoplasm in
childhood characterized
by a solitary growth that
is sharply demarcated
and encapsulated and
may occur in any part
of the kidney,
compressing it.
III. WILM’S TUMOR
ETIOLOGY
1. Exact cause unknown
2. Familial factor
PREDISPOSING/ PRECIPITATING FACTORS
1. Thirty percent risk of developing Wilm’s Tumor if
a parent had bilateral or familial Wilm’s tumor
2. Parents or siblings with hemihypertrophy
3. Associated with congenital genitourinary
anomalies
5 STAGES OF WILM’S TUMOR
 STAGE I- unilateral tumor limited to the kidney
and can be completely excised; with 90 percent
cured with multimodial therapy
 STAGE II- tumor extends beyond the kidney but
can be completely excised; with 90 percent
cured with multimodial therapy
 STAGE III- with a residual nonhematogenous
extension of the tumor, confined to the abdomen
 STAGE IV- with hematogenous metastasis
affecting most frequently the lungs
 STAGE V- bilateral renal involvement
concordant in time
SIGNS AND SYMPTOMS
1. Often asymptomatic
2. Unilateral smooth, firm abdominal mass: most
frequent sign; accidentally discovered in routine
physical examination
3. Abdominal distention, abdominal pains, and
vomiting.
TREATMENT
1. Surgical removal of the kidney containing the tumor,
even if pulmonary metastases are present: usual
immediate management.
2. Chemotherapy
3. Radiation
a. Unilateral disease: preoperative radiation
not recommended
b. Bilateral disease: preoperative radiation is
treatment of choice (to cause shrinkage of
the tumor and allow partial nephrectomy,
with salvage of the greatest amount of
normal kidney)
4. Prognosis: Better in children if diagnosed early,
before the age of 2 years
NURSING CARE
1. AVOID PALPATING THE ABDOMEN! This can
cause rupture of the tumor capsule and
precipitate spread.
2. Prepare child for surgical removal of the kidney
and adrenal gland and provide routine post-
abdominal surgery.
3. Provide appropriate care to a child with
combined chemotherapy and radiation.
a. Chemotherapy: indicated for all stags;
usually runs for 6-15 months; includes
vincristin, dactinomycin, and doxorubicin
b. Radiation: indicated for a large tumor
that is not completely resectable or if
already with metastasis
4. Support normal growth and development of the
child.
5. Provide psychological support and appropriate
referrals.

4. Hypertension (60% of cases)

Hypertension results from renal ischemia secondary to
renal artery compression. If prolonged and severe, it
may cause congestive cardiac failure.

DIAGNOSTIC TESTS
1. Computed tomography (CT): most helpful;
identifies intrarenal origin of tumor, which rules
out neuroblastoma, detects extent of tumor
(including involvement of great vessels), and
evaluates the opposite kidney
2. Urinalysis: reveals microscopic hematuria
3. Intravenous pyelography: reveals intrarenal
mass
4. Roentgenograms (x-ray) and CT scan: reveal
pulmonary metastasis

 Reproductive disorders of the female and male
reproductive tract are problems in a baby's
reproductive organs that occur while baby is
growing in her mother's body.
 A baby starts to develop its reproductive organs
between weeks 4 and 5 of pregnancy. This
development continues until the 20th week of
pregnancy.
– Common reproductive disorders in males
include structural alterations in the penis or
testes such as phimosis and cryptorchidism,
inflammation such as balanoposthitis, and, in
adolescents, testicular cancer.
1. PHIMOSIS AND PARAPHIMOSIS
– Phimosis is the inability to retract the foreskin
from the glans of the penis. The foreskin is tight
at birth and may even be held fast by adhesions
and so, in newborns, cannot (and should not) be
retracted.
– After a few months, the adhesions dissolve and
the foreskin become retractable; if it does not,
the infant has phimosis (Shahid, 2012). If a
foreskin is extremely tight, it can interfere with
voiding.
– Balanoposthitis may develop because the
foreskin cannot be retracted for cleaning.
Circumcision of newborns (discussed in is no
longer routinely advised but is used to relieve
phimosis.
– Paraphimosis is the inability to replace the
prepuce over the glans once it has been
retracted. This is an
emergency situation to address before
circulation to the glans is impaired.
2. CRYPTORCHIDISM
– Cryptorchidism is failure of one or both testes to
descend from the abdominal cavity into the
scrotum (Braga & Lorenzo, 2017).
– Normally, testes descend into the scrotal sac
during months 7 to 9 of intrauterine life. They
may descend any time up to 6 months after birth,
but they rarely descend after that time and a
referral to specialist is warranted (Fantasia,
Aidlen, Lathrop, et al., 2015).
– The cause of undescended testes is unclear.
Testes apparently descend because of
stimulation by testosterone; hence, a lower than
usual level of testosterone production
– may prevent descent. Fibrous bands at the
inguinal ring or inadequate length of spermatic
vessels may prevent descent. The condition is
found in about 3 out of every 1,000 male
newborns; it occurs most often in premature or
low–birth-weight babies (Gaylord & Petersen-
Smith, 2013)
ASSESSMENT
– Early detection of undescended testes is
important because the warmth of the abdominal
cavity may inhibit development of the testes,
ultimately affecting spermatogenesis.
– After the age of 1 year, sperm production
deteriorates rapidly in undescended testes, and
the testes may even undergo a malignant
change (Braga & Lorenzo, 2017).
– Anchoringthe testes in the scrotal sac does not
guarantee malignancy can be prevented, but it
will allow the boy to perform preventive
measures such as testicular self-examination.
– Some boys may be diagnosed with
undescended testes when, if an examining room
is chilly, the testes have retracted to make
palpation assessment difficult. Excessive
palpation or stroking of the inner thigh may also
stimulate the cremasteric reflex and cause
retraction. In these instances, testes descend
when the child is standing or after a warm bath.
– Laparoscopy is effective at identifying whether
an undescended testis is at the inguinal ring
(true undescended testis) or ectopic (still in the
abdomen). Because testes arise from the same
germ tissue as the kidneys, the kidney function
of a child with ectopic testes is usually evaluated
as well. If undescended testes and other factors
such as ambiguous genitals pose questions
 about the child’s gender, a karyotype may be minimized by applying ice for the first few hours
done to determine the child’s true gender postoperatively.

THERAPEUTIC MANAGEMENT
 Because the testes sometimes descend
spontaneously during the first year of life,
treatment is usually delayed for 1 year, possibly
2 years. Boys may be given a short course of
chorionic gonadotropin hormone for about 5
days to see if testicular descent can be
stimulated. If this is not successful, surgery
(orchiopexy) by laparoscopy will then correct the
condition

3. HYDROCELE
– Testis descends into the scrotum in utero, it is
preceded by a fold of tissue, the processus vaginalis.
Occasionally, fluid (termed a hydrocele) collects in
this fold. In utero, the fluid can be revealed by
ultrasound. At birth, the fluid causes the scrotum of
the newborn to appear enlarged (Panabokke,
Clifford, Craig, et al., 2016).
– Its presence can be revealed by ultrasound
or transillumination (the shining of a light through
the scrotal sac causes the area to glow). If the
hydrocele is uncomplicated, the fluid will gradually
be reabsorbed, so no treatment is necessary. The
child’s parents can be assured that the hydrocele is
only excess fluid and the scrotal enlargement is not
caused by an abnormal testis, tumor, or hernia.
Hydroceles may form later in life due to inguinal
hernias (abdominal contents extruding into the
scrotum through the inguinal ring, with
accompanying fluid). If this happens, when the
hernia is repaired, the hydrocele will be reabsorbed.
Injection of a drug to decrease fluid production
(sclerotherapy) may also be effective for older
youths.

4. VARICOCELE
– A varicocele is abnormal dilation of the veins of the
spermatic cord. It is important to identify varicoceles
in adolescents because, although it may not cause a
difference, the increased heat and congestion in the
testicles is a possible cause of subfertility (Fine &
Poppas, 2012). If fertility becomes a concern, the
varicocele can be surgically removed. The
adolescent will experience some local tenderness
and edema for a few days after surgery. This can be

I. EPISPADIAS / HYPOSPADIAS
 EPISPADIAS - a condition in which the urethral
opening is located behind the glans penis or on
the dorsal segment
 HYPOSPADIAS – the urethral opening is on the
ventral or undersurface of the penis with ventral
curvature of the penis (chordee) causing
constriction
ETIOLOGY
– Unknown
– Tends to be FAMILIAL or may occur from a
MULTIFACTORIAL GENETIC focus.
ASSESSMENT
1. Obvious malposition of the urethral orifice
2. May have short chordee or a fibrous band that
causes the penis to curve downward (cobra-
head appearance)
3. Also inspect for cryptorchidism which are often
found in conjunction with hypospadias
TREATMENT
 For minor conditions in which the urethral
opening is still on the glans, no treatment is
needed
 MEATOTOMY (in newborn) a surgical procedure
in which the urethra is extended to a normal
position to establish better urinary function
 RELEASE OF adherent chordee when the
child is age 12-18 months.
 URETHROPLASTY is done for severe
cases when child is about 2-3 years old
(toilet training period) or before the child
enters kindergarten school
EFFECTS IF not CORRECTED
 Hypospadias be corrected before school age, or
the child will look and feel not normal
 In later years a meatal opening at the inferior
penile site may interfere with fertility ( sperms
will not be deposited close to the female cervix
during coitus)
NURSING CARE
1. Participate in prompt diagnosis
a. Careful and thorough assessment of the
genitourinary system of the newborn.
b. Identify signs like MISPLACED urinary
meatus and inability to make a straight
stream of urine
2. Diaper normally. Provide the usual perianal care
3. Provide psychological care to parents who are
disappointed over their baby’s defect. Allow
verbalization of concerns or feelings
4. At the time of surgery, provide support to the
child, who may have a fear of mutilation
5. Provide post operative care:
6. Assess for bladder spasm. Administer
anticholinergics as ordered
7. Maintain urinary drainage catheter to allow
urinary output to prevent tension against the
urethral sutures
8. Check orders for dressing changes and change
dressings as ordered
9. Encourage high fluid intake
10. An analgesic may be prescribed
II. BLADDER EXSTROPHY
 A midline closure defect that occurs during the
embryonic period of gestation (first 8 weeks)
 The bladder lies open and exposed on the
abdomen.
 More common in males than females (ratio of
2:1)
ASS
ESS
ME
NT

wall of the bladder and no anterior skin covering
on the lower anterior abdomen
 Bladder appears bright red and continually
drains urine from the open surface
 Defects of external genitalia where the urethra is
abnormally formed
 Widened symphysis pubis
 Defects of the external genitalia
 Displaced anal opening
 Physical examination
 Renal functions tests
 Urinalysis
TREATMENT
1. Initial surgery: closure of abdominal wall within the
first few days of life (preferably during the first 48
hours of life) to prevent changes in the bladder wall
from becoming established
2. Subsequent operations: reconstruction of the
bladder and genitalia
NURSING CARE
1. Assess anal patency, urinary continence, history
of UTI (in the older child); monitor adequacy of
urine output.
2. Maintain integrity of the exposed bladder
mucosa until surgical correction is performed.
At birth:
a. Allow drainage of urine.
b. Prevent the bladder mucosa from drying
with the use of a non-adhering plastic
wrap. Avoid the use of petroleum jelly
gauze, which can dry out, adhere to the
mucosa, and damage tissues when the
dressing is removed.
3. Administer ordered antibiotics.
4. Provide psychological support to the parents.
a. Encourage verbalization of concerns and
fears.
b. Promote bonding between the newborn and
the parents

1. POLYCYSTIC OVARY SYNDROME
– Polycystic ovary syndrome (PCOS) is the
most frequent cause of ovulation failure
seen today.
– It is found in about 10% of women of
childbearing age (Connor, 2012).
– Adolescents with the syndrome begin to
develop an increased androgen (male
hormone) level, which then prevents
follicular ovarian cysts from maturing, a
situation that leads to typical symptoms
of irregular or missed menstrual cycles,
acne, excessive hair growth (hirsutism),
being overweight, male pattern baldness,
type 2 diabetes, and, most important, an
absence of ovulation.
ASSESSMENT
Assessment for the disorder includes a thorough history
and physical exam, a pelvic exam:
– to determine the consistency and size of ovaries,
and perhaps an ovarian ultrasound for the same
purpose.
– Serum androgen and glucose levels will also be
assessed. Because the exact cause of
polycystic ovaries is not known, treatment is
aimed at relieving the symptoms.
– Many adolescents with the syndrome are obese,
therefore, weight loss by increasing lean meat,
fruits, and vegetables and decreasing the
amount of concentrated carbohydrates in their
diet is encouraged.
– This eating pattern also lowers blood glucose
levels, improves the body’s use of insulin, and
helps to normalize testosterone secretion. If a
woman is morbidly obese, bariatric surgery may
be recommended because this will achieve the
same result.
MANAGMENT
– COC (combined oral contraceptives) may be
prescribed because this changes the ratio of
estrogen and testosterone produced, leading to
better regulated menstrual cycles.
– To prevent type 2 diabetes from
developing, metformin (Glucophage) may be
prescribed, which is yet another method to reduce
blood glucose levels.
– If the adolescent or woman wants to become
pregnant, fertility medications such as a course
of clomiphene (Clomid) to stimulate ovulation may
be suggested.
– Two final therapies to help achieve pregnancy are
in vitro fertilization (IVF) and ovarian drilling, a
surgery technique done by laparoscopy that
reduces the size of the ovaries and limits the amount
of testosterone the ovaries are able to produce.
– To decrease hair growth and reduce acne
symptoms, antiandrogens such as
spironolactone (Aldactone) or finasteride
(Propecia) can be tried.
– Caution women that finasteride is teratogenic and so
should not be used ifthey intend to become pregnant,
and it should be discontinued during pregnancy
(Mysore & Shashikumar, 2016). PCOS is a
perplexing disorder because it produces such a wide
range of symptoms, and responses to therapy may
not be immediate.
2. VULVOVAGINITIS
– Inflammation of the vulva or vagina is
accompanied by pain, odor, pruritus, and a
vaginal discharge (Rome, 2012).
– Vaginal bleeding may also be present.
– The condition may occur in a girl of any age, but
it tends to be more frequent as girls reach
puberty probably because the change to adult
pH and the presence of vaginal secretions make
the vagina more receptive to infections.
HERE ARE SOME TIPS THAT MIGHT HELP:
 Wash the area twice a day with mild,
nonperfumed soap and water, and pat dry to
remove secretions and decrease irritation.
 Always wash and dry from front to back to
prevent spreading rectal contamination forward.
 Take a tub bath or apply warm, moist
compresses three times a day to soothe
the area and to keep it free of irritating drainage.
 After drying the cleansed area, apply cornstarch
for comfort and to absorb residual moisture.
 Avoid bubble baths and feminine hygiene sprays
because the ingredients in these may cause
additional local irritation as well as may
contribute to urinary tract infections.
 Take acetaminophen (Tylenol) or a nonsteroidal
anti-inflammatory drug (NSAID) such as
ibuprofen every 4 hours. These are both
 analgesics and so relieve pain and reduce Daily washing of the perineum and frequent
itching, a mild pain sensation. changing of tampons or sanitary pads during
 Avoid scratching, which may increase abrasions menstruation helps prevent chafing or stasis of
and introduce a secondary infection. Instead, menstrual blood and so helps prevent irritation
apply a cold compress to relieve the itching and odor.
sensation.
 Wear cotton underwear, which allows air to
circulate and moisture to evaporate rather than
nylon or silk, which prevents air circulation and
retains moisture.
 Sleep without underwear.
 Use an anesthetic spray or hydrocortisone
cream if prescribed.
If an antibiotic has been prescribed for a vaginal
infection, take it conscientiously because only
after the infection subsides will the vaginal
discharge and itching clear.

3. PRESCHOOL, SCHOOL-AGE CHILDREN AND

ADOLESCENTS
– Vaginal discharge may occur before menarche, but
bleeding is rarely seen at this age. If vaginal
bleeding does occur, it is usually caused by irritation
caused by an inserted foreign object into the vagina,
infestation of pinworms, or daily bubble baths (which
can lead to urinary tract infections [UTIs] as well as
vulvar irritation). It could also be due to urethral
bleeding from cystitis (bladder infection) or rectal
pruritus, which has led to scratching and rectal
bleeding.
– Precocious puberty is yet another disorder that
must be ruled out. Finally, whether sexual
maltreatment has occurred must be investigated
because it could be a cause of any bleeding,
tenderness, or infection. If there is a foreign body in
the vagina, it obviously should be removed by a
vaginal examination. This may be difficult for young
girls to accept because, unless a small speculum is
used, vaginal manipulation and stretching can be
painful.
– Application of a local antibiotic ointment or warm
baths afterward may be prescribed to prevent
infection and inflammation after removal of the
object. A few preschool- or school-age children
develop a vaginitis due to Streptococcus or to
– Escherichia coli introduced from the anus by
improper perineal care after voiding or bowel
movements. A tight hymen then traps the
microorganisms in the vagina and leads to infection.
– The girl may need an antibiotic prescribed for this
and needs to be reminded to wipe from front to back
after voiding or bowel movements to help prevent
this from occurring again.

 As a girl enters puberty, she may notice a slight

vaginal discharge caused by increased vaginal
secretions. As long as she has no other
symptoms, you can reassure her this is normal.
To keep from developing vulvar irritation, caution
girls to wear cotton underpants rather than nylon
(so that moisture is absorbed better) and to dry
the vulva thoroughly after bathing or swimming.

The endocrine system keeps our bodies in
balance, maintaining homeostasis and guiding proper
growth and development. A single hormone may affect
more than one of these functions and each function may
be controlled by several hormones.
Although the endocrine glands are the body's
main hormone producers, some non-endocrine organs—
such as the brain, heart, lungs, kidneys, liver, thymus,
pancreas, skin, and placenta — also produce and
release hormones. Hormones communicate this effect
by their unique chemical structures recognized by
specific receptors on their target cells, by their patterns
of secretion and their concentrations in the general or
localized circulation. Hormones functions as
reproduction and sexual differentiation, for development
and growth, for maintenance of the internal environment
and regulation of metabolism and nutrient supply. Any
abnormality in our endocrine system disrupts the normal
growth and development of the children.
TYPE 1 DIABETES MELLITUS
– Type 1 diabetes mellitus is a disorder that involves
an absolute or relative deficiency of insulin, which is
in contrast to type 2, where insulin production is only
reduced Type 1 diabetes is equal in incidence in
boys and girls and affects approximately 1 of every
500 children and adolescents in the United States
(Sherr & Weinzimer, 2012)
ETIOLOGY
– The disease apparently results from immunologic
damage to islet cells in susceptible individuals. Why
autoimmune destruction of islet cells occurs is
unknown, but children with the disorder have a high
frequency of certain human leukocyte antigens
(HLAs), particularly HLA-DR3 and HLA-DR4, located
on chromosome 6, that may lead to susceptibility. If
one child in a family has diabetes, the chance that a
sibling will also develop the illness is higher than in
other families because siblings also tend to have
one of the specific HLA that are associated with the
disease.
DISEASE PROCESS
– Insulin can be thought of as a compound that opens
the doors to body cells, allowing them to admit
glucose, which is needed for functioning. It does not
play a major role in glucose transport into the brain,
erythrocytes, leukocytes, intestinal mucosa, or
kidney epithelium. These cells, therefore, can
survive insulin deficiency but not glucose deficiency.
– If glucose is unable to enter body cells because of a
lack of insulin, it builds up in the bloodstream
(hyperglycemia). As soon as the kidneys detect
hyperglycemia (greater than the renal threshold of
about 160 mg/dl), the kidneys attempt to lower it to
normal levels by excreting excess glucose into the
urine, causing glycosuria, accompanied by a large
loss of body fluid (polyuria).
– Excess fluid loss, in turn, triggers the thirst response
(polydipsia), producing the three cardinal symptoms
of diabetes: polyuria, polydipsia, and hyperglycemia.
Because body cells are unable to use glucose but
still need a source of energy, the body begins to
break down protein and fat.
– If large amounts of fat are metabolized this way,
weight loss occurs and ketone bodies, the acid end
product of fat breakdown, begin to accumulate in the
bloodstream (creating high serum cholesterol levels
and ketoacidosis) and spill into the urine as ketones.
– Potassium and phosphate, attempting to serve as
buffers, pass from body cells into the bloodstream.
From there, they are evacuated, causing a loss of
these important electrolytes.
– Untreated diabetic children, therefore, lose weight,
are acidotic due to the buildup of ketone bodies in
their blood, are dehydrated because of the loss of
water, and experience an electrolyte imbalance
because of the loss of potassium and phosphate in
urine. Because large amounts of protein and fat are
being used for energy instead of glucose, children
lack the necessary components for growth; they
therefore remain short in stature and underweight.
ASSESSMENT
– Although children may be prediabetic for some time,
the onset of symptoms in childhood is usually abrupt.
Parents notice increased thirst and increased
urination (which may be recognized first as bed-
wetting [enuresis] in a previously toilet-trained child).
The dehydration may cause constipation.
LABORATORY STUDIES
– In some children, diabetes is detected at a routine
health screening. For others, although the disease
has been progressing internally for some time,
outward symptoms have such an abrupt onset that
the child is in a coma from acidosis and
hyperglycemia by the time it is detected.
– Laboratory studies usually show a random plasma
glucose level greater than 200 mg/dl (normal
range, 70 to 110 mg/dl fasting; 90 to 180 mg/dl
not fasting) and significant glycosuria Two
diagnostic tests, the fasting blood glucose test
and the random blood glucose test, are used to
– confirm diabetes.
A diagnosis of diabetes is established if one
of the following three criteria is present on two
separate occasions:
– Symptoms of diabetes plus a random blood
glucose level greater than 200 mg/dl
– A fasting blood glucose level greater than 126
mg/dl
– A 2-hour plasma glucose level greater than 200
mg/dl during a 75-g oral glucose tolerance test
(GTT)
 Typically, a GTT involves the oral ingestion of a
concentrated glucose solution followed by blood
glucose levels drawn at fasting (baseline), after 1
hour, and after 2 hours.
 The test is difficult for children to undergo because it
requires them to fast for 8 hours, drink an overly
sweet solution, and submit to painful, intrusive
procedures (routine application of
lidocaine/prilocaine [EMLA] cream to finger stick or
venipuncture sites and use of intermittent infusion
devices greatly reduces this problem).
 Do not take blood for glucose analysis from
functioning IV tubing to try to help with pain
because the glucose in the IV solution will cause
the serum reading to be abnormally high.
OTHER DIAGNOSTIC TESTS
– If diabetes is detected, the diagnostic workup also
usually includes an analysis of blood samples for pH,
partial pressure of carbon dioxide (PCO2), sodium,
and potassium levels; a white blood cell count; and a
glycosylated hemoglobin (HbA1c) evaluation.
– Normally, the hemoglobin in red blood cells carry
only a trace of glucose. If serum glucose is
excessive, however, excess glucose attaches itself
to hemoglobin molecules, creating HbA1c. In
nondiabetic children, the usual HbA1c value is 1.8 to
4.0. A value greater than 6.0 reflects an excessive
level of serum glucose.
– Measuring HbA1c has advantages because it not
only provides information on what is the child’s
present serum glucose level but what the serum
glucose levels have been during the preceding 3 to 4
months (red blood cells have a life span of 120 days).
– If the potassium level of the blood is low, a child may
need an electrocardiogram to observe for T-wave
abnormalities, the mark of potassium deficiency. The
white blood cell count of a child with diabetes may
be elevated even though no infection is present,
apparently as a response to the ketoacidosis.
– The presence of infection must always be suspected,
however, because it is often a precipitant to a
diabetic crisis. For this reason, nose and throat
cultures may be obtained as well.
THERAPEUTIC MANAGEMENT
Therapy for children with type 1 diabetes involves
five measures:
– insulin administration, regulation of nutrition and
exercise, stress management, and blood
glucose and urine ketone monitoring. T
THE INITIAL REGULATION OF INSULIN
– When children are first diagnosed with diabetes,
they are usually hyperglycemic and perhaps
ketoacidotic. To correct the metabolic imbalance,
they are given insulin administered IV at a dose
of 0.1 to 0.2 units per kilogram of body weight
per hour. This initial IV infusion of insulin is then
gradually reduced once the blood glucose level
is lower than 200 mg/dl. Ideally, within 12 hours,
the acidosis is considerably less than when a
child was admitted to the hospital, and the
serum glucose level is near the normal range.
– The insulin given for emergency replacement
this way is regular (short-acting) insulin such as
Humulin-R because this is the form that takes
effect most quickly.
– It may seem that in a child with diabetes in a
state of acidosis, the administration of glucose
would not be warranted. Because the child is
being given insulin, however, body cells soon
become ready to use glucose, so they require,
incorporate, and use available glucose quickly. If
more glucose is not provided, cells are forced to
continue to break down fats and protein, and the
acidosis can increase, not decrease. Glucose,
therefore, may be added to the infusion.
– After 24 hours, as the child’s serum glucose
returns to normal, oral feedings may replace the
IV route.
– Further management in the days after this first
crucial 24-hour period is based on serum
glucose determinations.
– A child may remain on regular insulin given SC
alone (given three or four times a day) for the
first 1 or 2 days. Typically, intermediate-acting
insulin is then added as soon as oral fluids are
taken, usually on the second day of therapy.

1. GROWTH HORMONE DEFICIENCY
– If production of human growth hormone (GH, or
somatotropin) is deficient, children are not able
to grow to full size (Dörr, Boguszewski, Dahlgren,
et al., 2015).
– As a result, children may appear well
proportioned but measure well below the
average on astandard growth chart.
– Deficient production of GH may result from a
nonmalignant cystic tumor of embryonic origin
that places pressure on the pituitary gland or
from increased intracranial pressure as a result
of trauma. In most children with hypopituitarism,
however, the cause of the defect is unknown; it
may have a genetic origin.
– If hypopituitarism is not treated, predicting
exactly what height a child will reach is difficult
because height varies with each individual.
Without treatment, however, most children will
not reach more than 3 or 4 ft in height.
ASSESSMENT
– The child with deficient production of GH is
usually normal in size and weight at birth. Within
the first few years of life, however, the child
begins to fall below the third percentile of height
and weight on growth charts.
– The face appears infantile because the mandible
is recessed and immature, and the nose is
usually small.
– The child’s teeth may be crowded in a small jaw
(and may erupt late). The child’s voice may be
high pitched, and the onset of pubic, facial, and
axillary hair and genital growth will be delayed.
– The history, physical findings, and a decreased
level of circulating GH contribute to the
diagnosis.
– A pituitary tumor must be ruled out as the cause
of decreased GH production. Sudden halted
growth suggests a tumor; gradual failure
suggests an idiopathic involvement.
– A history of vision loss, headache, an increase in
head circumference, nausea, and vomiting
(signs of increased intracranial pressure) also
suggest a pituitary tumor.
– Growth failure may be so marked in some
children that the parents may be suspected of
child neglect.
– As part of the history taking, evaluate the family
history for traits of short stature or constitutional
delay (familial late development). If at all
possible, obtain estimates of the parents’ height
and siblings’ height and weight during their
periods of growth.
– Assess the child’s prenatal and birth history for
any suggestion of intrauterine growth restriction.
– Assess also for any severe head trauma that
could have injured the pituitary gland or chronic
illness, such as a heart, kidney, or intestinal
disorder that could have contributed to the
decreased level of growth.
– Take a 24-hour nutrition history to see if “picky
eating habits” are extensive enough to halt
growth.
– Be certain to assess not only the child’s actual
height but also his or her feelings about being
short.
– A physical assessment, including a funduscopic
examination, neurologic testing, and blood
analysis for hypothyroidism, hypoadrenalism,
hypoaldosteronism, and growth factor–binding
proteins are also helpful in ruling out a lesion or
tumor.
– Bone age is established by a wrist X-ray
(epiphyseal closure of long bones is delayed
with GH deficiency but is proportional to the
height delay).
– A skull series, computed tomography (CT)
scanning, magnetic resonance imaging (MRI), or
ultrasound will be prescribed to detect possible
enlargement of the sella turcica, which would
suggest a pituitary tumor.
THERAPEUTIC MANAGEMENT
– GH deficiency is treated by the administration of
intramuscular recombinant human growth
hormone (rhGH) usually given daily at
bedtime, the time of day at which GH normally
peaks.
– In addition, some children may need
suppression of luteinizing hormone–releasing
hormone (LHRH, or gonadotrop in releasing
hormone [GnRH]) to delay epiphyseal
closure.
– Other children may need supplements of
gonadotropin or other pituitary hormones if these
are determined to be deficient as well.
– GH has been used irresponsibly by athletes in
the hope that it will improve muscle growth and
overall stamina.
– Caution children that the use of the drug when
there is no medical reason for it is potentially
dangerous and so they should not share the
drug with friends or take excessive doses
themselves (Baumann, 2012). Because they
have delayed epiphyseal closure, if treatment is
begun early, children can expect to reach a
height individually targeted for them. Once
epiphyseal lines of long bones close (with
adolescence), GH will be tapered and stopped.
2. GROWTH HORMONE EXCESS
– An overproduction of GH usually is caused by a
benign tumor of the anterior pituitary (an
adenoma).
– If the overproduction occurs before the
epiphyseal lines of the long bones have closed,
excessive or overgrowth will result. Weight will
become excessive also, but it is proportional to
height.
 – The skull circumference typically exceeds usual,
and the fontanels may close late or not at all.
– After epiphyseal lines close, acromegaly
(enlargement of the bones of the head and soft
parts of the hands and feet) begins to be evident.
– The tongue can become so enlarged and
thickened that it protrudes from the mouth,
giving the child a dull, apathetic appearance and
making it difficult to articulate words. If the
condition remains untreated, a child may reach a
height of more than 8 ft.
THERAPEUTIC MANAGEMENT
– If X-rays or ultrasounds of the skull reveal that
the sella turcica is enlarged or that a tumor is
present, laser surgery to remove the tumor or
cryosurgery (freezing of tissue) is the primary
treatment.
– If no tumor is present, a GH antagonist such as
bromocriptine (Parlodel) taken orally or
octreotide (Sandostatin) taken by injection
can slow the production of GH.
– When GH secretion is halted in this way, other
hormones may also be affected; therefore, the
child may need to receive supplemental thyroid
extract, cortisol, and gonadotropin hormones in
later life.
– A more permanent therapy is irradiation or
radioactive implants of the pituitary gland, again
to halt GH production. It is difficult for a child
always to be bigger and taller than playmates,
and problems such as buying clothes or fitting
into airline seats continue to be very real and
distressing in adulthood.
– Counseling them about maintaining self-esteem
and making the adjustments necessary to
accommodate their larger-than-usual size is a
nursing responsibility.
3. DIABETES INSIPIDUS
– Diabetes insipidus is a disease in which there is
decreased release of ADH by the pituitary gland.
– This causes less reabsorption of fluid in the
kidney tubules.
– Urine becomes extremely dilute, and a great
deal of fluid is lost from the body. Diabetes
insipidus may reflect an X-linked dominant trait,
or it may be transmitted by an autosomal
recessive gene.
– It may also result from a lesion, tumor, or injury
to the posterior pituitary, or it may have an
unknown cause.
– In a rare type of diabetes insipidus, pituitary
function is adequate, but the kidneys’ nephrons
are not sensitive to ADH (a kidney-related
etiology).
ASSESSMENT
– The child with diabetes insipidus experiences
excessive thirst (polydipsia) that is relieved only
by drinking large amounts of water; there is
accompanying polyuria. The specific gravity of
the urine will be as low as 1.001 to 1.005
(normal values are more often 1.010 to 1.030).
– Urine output may reach 4 to 10 L in a 24-hour
period (normal range, 1 to 2 L), depending on
age. Because so much fluid is lost, sodium
becomes concentrated or hypernatremia occurs
with symptoms of irritability, weakness, lethargy,
fever, headache, and seizures.
– The signs and symptoms usually appear
gradually.
– Parents may notice the polyuria first as bed-
wetting in a toilet-trained child or weight loss
because of the large loss of fluid.
– If the condition remains untreated, the child is in
danger of losing such a large quantity of water
that dehydration and death can result. MRI, CT
scanning, or an ultrasound study of the skull
reveals whether a lesion or tumor is present.
– A further test is the administration of vasopressin
(Pitressin) to rule out kidney disease.
– For this, after the child’s urine output has been
measured to establish a baseline, vasopressin is
administered. The drug decreases the blood
pressure, alerting the kidney to retain more fluid
in order to maintain vascular pressure.
– If the fault that is causing the dilute urine is with
the pituitary gland, not the kidneys, the child’s
urine output will decrease; if the fault is with the
kidneys, urine will remain dilute and excessive in
amount because the diseased kidneys cannot
concentrate fluid.
THERAPEUTIC MANAGEMENT
– Surgery is the treatment of choice if a tumor is
present. If the cause is idiopathic, the condition
can be controlled by the administration of
desmopressin (DDAVP), an arginine
vasopressin.
– In an emergency, this drug can be given
intravenously (IV). For long-term use, it is given
intranasally or orally
– If desmopressin is given as an intranasal spray,
this may cause nasal irritation; the route will not
be effective if the child develops an upper
respiratory tract infection with swollen mucous
membranes.
– Caution children that they will notice an
increasing urine output just before the next dose
is due so they can arrange their day according to
where bathrooms are located.
4. CONGENITAL HYPOTHYROIDISM
– Thyroid hypofunction causes reduced production
of both T4 and T3. Congenital hypofunction
(reduced or absent function) occurs as a result
of an absent or nonfunctioning thyroid gland in a
newborn.
– Congenital hypothyroidism or an indication that
the infant’s thyroid is not functioning well may
not be noticeable at birth because the mother’s
thyroid hormones (unless she ingested less than
 usual amounts of iodine) maintain adequate – The treatment for true hypothyroidism is the oral
levels in the fetus during pregnancy. administration of synthetic thyroid hormone
– The symptoms of the disorder become (sodium levothyroxine).
apparent during the first 3 months of life in a – A small dose is given at first, and then the dose
formula-fed infant and at about 6 months in a is gradually increased to therapeutic levels.
breastfed infant. Because congenital – The child needs to continue taking the synthetic
hypothyroidism leads to both severe, thyroid hormone indefinitely to supplement that
progressive physical and cognitive which the thyroid does not make.
challenges, early diagnosis is crucial. – Supplemental vitamin D may also be given to
prevent the development of rickets when, with
ASSESSMENT the administration of thyroid hormone, rapid
– A screening test for hypothyroidism is mandatory bone growth begins (Kim, 2016).
at birth in the United States in all 50 states – Further cognitive challenges can be prevented
(using the same few drops of blood obtained for as soon as therapy is started, but any degree of
a phenylketonuria [PKU] blood spot test) (Kollati impairment that was already present cannot be
et al., 2017). reversed, making the disorder one of the most
– If an infant should miss this screening procedure, preventable causes of mental development
an early sign that parents report is that their child delay known (LaFranchi, 2014).
sleeps excessively, but because the tongue is – Be certain the parents know the rules for long-
enlarged, they notice respiratory difficulty, noisy term medication administration with children,
respirations, or obstruction. particularly the rule about not putting medicine in
– The child may also suck poorly because of a large amount of food (T4 tablets must be
sluggishness or choking from the enlarged crushed and added to food or a small amount of
tongue. formula or breast milk) and being certain they
– The skin of the extremities usually feels cold, dry, have medicine during holidays or vacations.
and perhaps scaly, and the child does not – Periodic monitoring of T4 and T3 helps to ensure
perspire. an appropriate medication dosage.
– Pulse, respiratory rate, and body temperature all – If the dose of thyroid hormone is not adequate,
become subnormal. the T4 level will remain low and there will be few
– Prolonged jaundice may be present due to the signs of clinical improvement. If the dose is too
immature liver’s inability to conjugate bilirubin. high, the T4 level will rise and the child will show
Anemia may increase the child’s lethargy and signs of hyperthyroidism: irritability; fever; rapid
fatigue. pulse; and perhaps vomiting, diarrhea, and
– On a physical exam, the hair is brittle and dry, weight loss.
and the child’s neck appears short and thick.
– The facial expression is dull and open mouthed
because of the infant’s attempts to breathe
around the enlarged tongue.
– The extremities appear short and fat; as muscles
become hypotonic, deep tendon reflexes
decrease and the infant develops a floppy, rag-
doll appearance.
– Generalized obesity usually occurs. Dentition will
be delayed, or teeth may be defective when they
do erupt.
– The hypotonia affects the intestinal tract as well,
so the infant develops chronic constipation; the
abdomen enlarges because of intestinal
distention and poor muscle tone.
– Many infants have an umbilical hernia. Infants
have low radioactive iodine uptake levels, low
serum T4 and T3 levels, and elevated thyroid-
stimulating factor. Blood lipids are increased.
– An X-ray may reveal delayed bone growth. An
ultrasound reveals a small or absent thyroid
gland. Untreated, the condition will result in
severe irreversible cognitive deterioration or
delay (Kollati et al., 2017).

THERAPEUTIC MANAGEMENT
– Transient hypothyroidism usually fades by 3
months’ time.

I. SPINA BIFIDA
DEFINITION
– A congenital defect of the spinal/neural tube in
which there is an incomplete closure of the
spinal column due to one or two missing
vertebral arches, and accompanied by varying
degrees of protrusion of CNS contents through
the bony defect. 80% of cases involve the
lumbosacral region.
ETIOLOGY
– Usually occurs during the 4th week of embryonic
life, but the exact cause is unknown.
INCIDENCE
– Higher incidence if a sibling has had the
condition
PREDISPOSING FACTORS
– Hereditary and environmental factors.
CLASSIFICATIONS OF SPINA BIFIDA
1. Spina bifida oculta. With missing L5 and S1
and seen as small sac or dimple at the lower
back; usually asymptomatic and seldom creates
health problems; often, no treatment is needed.
2. Meningocele. With a sac-like cyst that contains
meninges and spinal fluid that protrudes through
the body defect.
3. Meningomyelocele. With a herniated sac of
meninges, spinal fluid, and a portion of the
spinal cord and its nerves, which protrude
through the defect in the spine. It is the most
severe form.
SIGNS AND SYMPTOMS
1. Visible sac-like structure or dimpling of the skin
at any point on the spinal column.
2. Neurological function rarely interrupted in
Meningocele, but not so with meningomyelocele.
3. Associated defects/problems found in the third
type:
a. Hydrocephalus
b. Bowel and bladder dysfunction may
have difficulty attaining autonomy
c. Paralysis of the lower extremities
d. Orthopedic defects: club foot, hip
dislocation
e. CNS impairment: cool and dry skin with
no sweating
4. Associated meningitis: the sac is covered with a
thin membrane which easily can be penetrated
by microorganisms, causing infection.
DIAGNOSTIC TESTS/PROCEDURES
1. Prenatal detection if a sibling has had the
disease:
a. Ultrasound
b. Amniocentesis: elevated alpha-
fetoprotein (AFP) at about 14 to 16
weeks’ gestation
2. Postnatal detection
a. Meningeal sac inspection
b. Clinical signs and symptoms
c. Transillumination
d. X-ray of skull and spine
COMPLICATIONS
1. Hydrocephalus: leading complication of
meningomyelocele
2. Partial or complete motor impairment
3. Bowel and bladder dysfunction
4. Kyphosis, scoliosis
5. Meningitis
TREATMENT
– Sac closure and treatment of associated defects
and problems
NURSING CARE
1. Promote early and prompt diagnosis and
treatment by inspecting the back of every
newborn for any sac-like structure or dimpling.
Report right away if present.
2. Protect the sac from pressure, irritation, and
infection.
a. Meticulous skin care: top priority
 Clean the site with hydrogen
peroxide (H2O2) and sterile
saline solution
 Cover the sac with saline soak
(sterile gauze wet with sterile
 NSS); or apply gauze moist with
sterile NSS every two to four
hours, as ordered.
b. Prone position with hips abducted (best
position);positional changes every two
hours: prone-left-prone-right, NEVER
supine!
c. No diapers: these compress the sac
d. Careful handling of the child
3. Maintain nutrition and hydration.
a. Use soft nipples for feeding.
b. Elevate the head for feeding.
c. Feed slowly with proper burping. Be
careful not to touch the sac at the lower
back when burping.
d. Maintain strict I&O.
4. Promote adequate elimination.
a. Crede maneuver: milk the bladder every
4 hours to stimulate emptying.
b. Provide intermittent aseptic
catheterization, as ordered.
5. Monitor for signs of complications:
a. Hydrocephalus. The first sign is
excessive increase in head
circumference. (Greater than one inch
per month)
b. Increased intracranial pressure (ICP).
Early signs include tense and bulging
fontanels, high-pitched, shrill cry, and
projectile vomiting.
c. Urinary tract infection (UTI).
Prevention is the best treatment: avoid
stool contamination of the urinary
meatus. Administer antibiotics as
ordered.
d. Meningitis, inflammation of the
meninges can manifest as fever,
seizures, altered level of consciousness
(LOC), and signs of increased ICP.
e. Hip dislocation. Signs include difficulty
with hip abduction, an asymmetrical hip,
and popliteal and ankle lines. Prevent
orthopedic problems with good body
alignment, hip abduction (pillows
between the legs), and passive range of
motion (ROM) exercises every four
hours.
6. Provide health teaching to parents:
a. General home care
b. Signs and symptoms of complications
c. Maintenance of developmental progress
d. Treatment and surgical procedures:
 Sac closure
 Skin grafting over the sac
 Shunting for hydrocephalus
7. Provide psychological support.
II. HYDROCEPHALUS
DEFINITION
– Congenital or acquired increased enlargement
of the head due to excessive accumulation of
cerebrospinal fluid (CSF) within the ventricles of
the brain
ETIOLOGY
– Variable
1. Choroid plexus’ increased production of CSF
2. Obstruction of CSF circulation
3. Inadequate reabsorption of CSF
PREDISPOSING/PRECIPITATING FACTORS
1. Congenital malformation
2. Infection: meningitis, encephalitis
3. Tumor
4. Trauma: accidental head injury (intranatal or
postnatal)
SIGNS AND SYMPTOMS
1. Enlarged head circumference (HC): increase
in HC in greater than one inch per month
(normal HC rate of increase: 0.5 to 1 inch per
month); the first sign
2. Sunset sign: the hallmark sign
3. Head lag after three to four months: the
developmental sign
4. Dilated cephalic veins, widening of sutures,
shiny scalp
5. Signs of ICP
a. Early signs: tense and bulging
fontanels (for infants), high-pitched,
shrill cry, projectile vomiting
b. Late signs: increasing lethargy,
constant headache that increases in
intensity, changes in vital signs, and
altered LOC- the most important
measure of the child’s condition
6. Opisthotonus (arching of the back)
7. Feeding difficulties

TREATMENT
SURGICAL SHUNTING
1. Ventriculoperitoneal (V-P) shunt connects the
lateral ventricle of the brain to the peritoneal
cavity; most commonly used shunt in children;
does not require frequent revisions and is easier
to revise.
2. Atrioventricular (A-V) shunt connects the
lateral ventricle to right atrium of the heart.
3. Ventriculoureteral (V-U) shunt connects the
lateral ventricle of the brain to the ureter;
requires unilateral nephrectomy which, may
cause recurrent fluid and electrolyte imbalance.
POSTSURGERY COMPLICATIONS
1. Shunt blockage
2. Infections
3. Seizures
NURSING CARE
1. Assess daily head circumference.
2. Protect the child’s head:
a. Meticulous skin care of the head/scalp
b. Frequent turning (every two hours)
c. Use of sheepskin protector under the head
d. Use of ‘doughnut’ ring (clean towel/cloth
rolled into a ring) for the ears to relieve
pressure
3. Maintain nutrition and hydration.
a. Small, frequent feedings
b. Holding of infant, eye-to-eye contact
during feeding
c. Slow feeding, frequent burping
d. Rest after feeding
e. Strict monitoring of I&O
4. Provide psychological support
a. Infants
 Provide physical contact and tactile
stimulation: touching and stroking
 Provide psychological stimulation with
the use of a crib mobile.
 Provide consistent ‘caring’ and
parenting
 Anticipate infant’s needs for trust
development.
b. Parents
 Prepare for procedures and tests:
explain all procedures and tests using
understandable terms. For example, in
preparation for shunting, the nurse
explains that ‘shunting is to bypass the
obstruction to the flow of fluid, or to
remove excess fluid.’
5. Provide postoperative care.
a. Proper position:
 Flat for the first 24 to 48 hours; not to be
cuddled or lifted, even if the infant cries
 Head elevation (30 to 45 degrees up)
after 48 hours to promote CSF drainage
and decrease ICP
 Turning every two hours, but NOT to the
operative side
b. Frequent monitoring: VS, HC, LOC, I&O,
fontanels, reflexes, and signs of increased
ICP
c. Pumping of shunt, as ordered (if shunt has a
valve) to maintain patency
d. Assessment for early signs of complications
 Shunt blockage: signs of increased ICP
such as irritability, tense fontanels,
lethargy, projectile vomiting, increased
BP and temperature, decreased pulse
and respiratory rates (opposite of the VS
in shock)
 Infection: pulmonary (fever, signs of
respiratory distress) and shunt (fever,
signs of increased ICP)
6. Provide parental teaching:
 Shunt care: the need for pumping of the
shunt and shunt revision, as indicated.
 Prevention of infection
 On growth and development: if condition
is treated early and adequately, the head
may remain larger than normal, but
intelligence may be normal. The child will
need mental stimulation or mental feeding.
III. MICROCEPHALY
DEFINITION
– A congenital condition in which the head is
smaller than the chest, usually because of a
small brain (micrencephaly)

ETIOLOGY
– Unknown

TREATMENT
– No need for surgical treatment (craniotomy)

MEDICAL AND NURSING MANAGEMENT

– Meet the child’s physical, nutritional, and
psychologic needs (if purely asymptomatic).

IV. ARNOLD-CHIARI DEFORMITY

DEFINITION
– Projection of the cerebellum, medulla oblongata
and 4th ventricle into the cervical canal, causing
spinal or cranial Meningocele or
meningomyelocele

ETIOLOGY
– Overgrowth of the neural tube in the 16th to 20th
weeks of fetal life.

SIGNS AND SYMPTOMS

– Similar to those present in a child with
hydrocephalus and meningomyelocele.

1. Associated defects: meningomyelocele in 50%

of cases
2. Hydrocephalus from aqueduct stenosis

PROGNOSIS
– Depends on the extent of the defect and the
surgical procedures

MEDICAL/NURSING MANAGEMENT
– See Meningomyelocele and Hydrocephalus

I. CLUBFOOT
DEFINITION
– Congenital twisting of one or both foot in the
uterus, resulting to deformity of the muscles and
bones of the foot/feet that prevent it/them from
manipulated into the normal position
ETIOLOGY
– Real cause unknown
INCIDENCE
– Twice as common in males as in females with
the unilateral slightly more common than the
bilateral type
PREDISPOSING/PRECIPITATING FACTORS
1. Anomalous or arrested embryonic development
2. Abnormal position in the uterus
3. Restricted movement in the uterus
4. Environment causation
SIGNS AND SYMPTOMS
Talipes equinovarus is the most common type of
clubfoot and has the following characteristics:
1. Inner area of the foot is turned upward
2. Anterior half of the foot is adducted.
3. The foot is in plantar flexion.
DIAGNOSTIC TESTS/PROCEDURES:
– Inspection
TREATMENT
1. Foot exercises and gentle manipulation
instituted early.
a. True clubfoot, or the rigid type, is not
readily corrected by manipulation and
exercises.
b. The flexible type is easily corrected by
exercises and manipulation
2. Serial casting for immobilization with weekly
manipulation because infant growth is rapid.
3. Denis-Browne splint: metal crossbar with affixed
shoes.
4. Surgery followed by casting for the rigid form of
clubfoot:
a. To release tight ligaments
b. For tendon transfer
COMPLICATIONS: crippling of the child if condition is
not corrected.
NURSING CARE
1. Detect early. Inspect all newborns at birth for any
skeletal defects.
2. Perform ordered exercises and manipulations.
a. Do ROM exercises every shift with the
unaffected extremities
b. Give parental instructions on foot exercises
and manipulations
3. Maintain affected limb/limbs in cast and provide cast
care: serial casting is started early after birth with
weekly manipulations and cast changes to
accommodate the rapid growth of the child.
4. Monitor neurovascular (NV) status:
a. Color: pinkish
b. Capillary refill: briskly or less than 3 seconds
c. temperature: warm
d. Pedal pulses: (+)
e. Edema: (-)
f. sensation: (+) to touch
g. Movement: positive wiggling of toes
5. Examine the cast for skin breakdown, pressure area,
pain, infection, and bleeding. Foul odor is the first
sign of infected cast.
6. Keep cast dry and clean.
a. Protect from water and urine.
 Use plastic wrappers
 Use a Bradford frame as ordered.
 Position child properly: slight elevation
of head part of the bed.
b. No baby powder near the cast
 c. No small toys that can be placed inside the
cast.
7. Keep the cast elevated and exposed to air to
enhance drying up. Petal the cast to protect the skin.
8. Use the palm of the hands when handling a wet cast.
a. Instruct parents on cast care.
 Turning child every two hours.
 Provide skin care.
b. Provide age-related diversion and play materials.

II. CONGENITAL HIP DYSPLASIA/CONGENITAL HIP

DISLOCATION
DEFINITION
– Congenital malrotation or malformation of the
hip present at birth

ETIOLOGY
– Unknown

PREDISPOSING/PRECIPITATING FACTORS
1. Familial tendency
2. Endocrine factor: the maternal hormone
estrogen secreted toward late pregnancy cause
laxity of hip capsule and ligaments
3. Breech presentation
4. Postnatal position an infant carry: knees
together or adduction of legs

INCIDENCE
1. More common in females; female- to male-ratio-
7:1
2. Bilateral hip involvement in 25% of cases
3. Lowest incidence in the Far East doe to the way
infants are carried: legs widely abducted
4. higher in breech presentation

SIGNS AND SYMPTOMS

1. Infant and Younger child
a. Asymmetrical gluteal folds, popliteal
lines and ankle lines
b. Shortening of the limb on the affected
side
c. Widened/broadening of perineum from
bilateral dislocation
d. Limited/restricted hips abduction
e. Positive Ortolani’s sign: ‘clicking’ when
affected limb is abducted; caused by
femoral head slipping over acetabulum
2. Older child
a. Flattening of buttocks on affected side
b. Positive Trendelenburg sign: while the
child is standing on the affected side,
there is downward pelvic tilting or
dropping/dipping on normal side,
instead of upward