6.

CLEFT AND LIP PALATE

The maxillary and median nasal processes normally fuse between weeks 5 and 8 of intrauterine
life.
- weeks 5 and 8 median and nasal process

Cleft lip
- ranges from small notch in the upper lip, total separation of the lip and facial structure up into
the floor of the nose, with even the upper teeth and gingiva absent.
- unilateral or bilateral.
- nose is generally flattened
- boys > girls.

Cleft Palate
- an opening of the palate is usually on the midline and may involve the anterior hard palate, the
posterior soft palate, or both.
- It may occur with a cleft lip.
- girls > boys
- result of polygenic inheritance or environmental influences

palate: roof of oral cavity

- hard palate and soft palate

Etiology
- familial tendency or from transmission of multiple genes.
- aided by teratogenic factors present during weeks 5 to 8 of intrauterine life (viral infection
or possibly a deficiency of folic acid)
- multifactorial inheritance ↑ incidence in relatives and monozygotic twins.(identical twins/1
ovum
-
pathophysiology
Clinical Manifestations
• Defects are readily apparent at birth.
Cleft lip
- a notched upper lip border, nasal distortion, and may include unilateral or bilateral
involvement.
Cleft palate
- visible or palpable gap in uvula, soft palate, hard palate, and/or incisive foramen with
exposed nasal cavities and associated nasal distortion.

Diagnostic Test
- Sonogram
● in utero Inspection
- depressing the newborn's tongue with a tongue blade → total palate and the extent of
the cleft palate

Medical Management
- Surgical correction
● cleft lip: at 1 to 3 months of age,
● cleft palate: between 6 and 18 months of age to allow the anatomic change in the palate
contour that occurs during the first year of life.
● Repair of the cleft palate may require several stages of surgery as the child grows.

Medical Management
- Early correction of cleft lip enables more. normal sucking patterns and facilitates
bonding.
• enables development of more normal speech patterns.
• defects may require the use of orthodontics

Surgery
- Cheiloplasty (Plastic surgery of the lips)
● 1-3 mos - for normal sucking patterns and facilitates bonding and so as not to remove
sucking reflex
- Palatoplasty (reconstruction of the palate) done as early as 4-6 or
● 12-18 mos to allow normal palatal growth & not to impair speech (6-18 months)
● Re-sched OR if with URTI
Nursing Management
1)Assess for problems with feeding, breathing, parental bonding, and speech.
2)Ensure adequate nutrition and prevent aspiration.
a)Provide special nipples of feeding devices
b)Hold the child in a semi-upright position; direct the formula away from the cleft and toward the
side and back of the mouth to prevent aspiration.
c)Feed the infant slowly and burp frequently to prevent excessive swallowing of air and
regurgitation.
d)Stimulate sucking by gently rubbing the nipple against the lower lip.

Nursing Management
1)Support the adjustment infant's and parents' emotional and social
2)Provide preoperative care.
- Reinforcing the health care provider's explanation and surgical procedures.
- Providing mouth care to prevent infection.
3) Provide post operative care.
- Post-op positioning
- Cheiloplasty: side lying
- Palatoplasty: prone ( to facilitate drainage
4)Provide child and family teaching

Nursing Management

1)Post-op care
- Assess airway patency and V/S check for edema andrespiratory distress
- Keep tension on the suture line.
 ● Position the child with cleft lip on her back, in an infant seat, or propped to avoid injury to
the operative site; position the child with cleft palate on the abdomen to facilitate
drainage. on a s
- Clean the suture line: antibacterial ointment
1)Post-op care
- Use elbow restraints: maintain suture line integrity. Remove 2 hours for skin care and
range-of-motion exercises.
- Feed the infant: rubber-tipped medicine dropper, bulb syringe,
- Manage pain.: analgesics
- feeder, or soft bottle-nipple
- Attempt to keep the child from putting tongue up to palate sutures
7. IMPERFORATED ANUS (ANAL ATRESIA)

Imperforate Anus

4 types
1. Atresia (anal or rectal) - body orifice or passage in the body is abnormally closed or absent
2. Agenesis (anorectal) - failure to develop during embryonic stage, most dangerous type
3. Stenosis (too small opening)
4. Membranous-has anal opening but with a thin tissue layer

Etiology
•The cause is unknown.
- abnormal development of the fetus
• Boys > girls.
- May occur as an additional complication of spinal cord disorders (part of germ layer is
iisa lang)
Medical Management
REPAIR:
• simple anastomosis of the separated
bowel segments.
• Complications:
• a fistula to the bladder or vagina is present.
• If the repair will be extensive → temporary
colostomy → final repair when the infant is
older (6 to 12 months).

Medical Management
● Position post-op: sidelying
● Perform anal dilation as prescribed (use water soluble lubricant, insert dilator no more
than 1-2cm deep)
● Complication
- INCONTINENCE for a lifetime due to sacral anomalies and improper surgery

Nursing Management
● Preoperative Care
● NPO
● NGT-for decompression to relieve vomiting and prevent pressure on other abdominal
organs or the diaphragm from the distended intestine.
● IVT or TPN- fluid and electrolyte balance.

Postoperative Care
- Begin small oral feedings of glucose water, formula, or breast milk when bowel sounds
are present and NGT removed.(auscultate bowel sounds and check for flatulence or if
the baby has already passed gas)
- Do not to give high-residue foods
- No rectal temperature taking
- No enemas, suppositories, or any other intrusive rectal procedures.

Post Operative care

- Give stool softener daily
- Clean the suture line well by irrigating it with NSS.
- Place a diaper under, not on the infant
 - A side-lying position is best. .
- An infant may need rectal dilatation (gently inserting al lubricated cot-covered finger into
the rectum)

8. HIRSCHSPRUNG'S DISEASE/ AGANGLIONIC MEGACOLON

- absence of ganglionic innervation to the

- muscle of a section of the bowel-lower portion of the sigmoid colon just above the anus.
- →no peristaltic waves to move fecal material → chronic constipation or ribbon-like
stools
- proximal portion of the bowel dilates ---distending the abdomen.

Etiology
- familial, congenital defect
- Failure of ganglion nerve cell precursors along the GI tract between the 5th and 12th
weeks of gestation.
- males > females
- abnormal gene on chromosome 10
Clinical Manifestations
- Neonates: failure to pass meconium, reluctance to ingest fluids, abdominal distention,
and bile stained vomitus.
- Infants: failure to thrive, constipation, abdominal distention, vomiting and episodic
diarrhea.
- Older infants: anorexia, chronic constipation, foul smelling and ribbon-like stools,
abdominal distention, visible peristalsis, palpable fecal mass, malnourishment or poor
growth

Clinical Manifestations
- Rectal examination --- rectum empty of stool, a tight anal sphincter and stool leakage.
- * Warning signs signifying enterocolitis (inflammation of the Intestines);explosive, bloody
diarrhea, fever, and severe prostration (exhaustion)

Diagnostic Test
- Barium enema: megacolon
- Rectal biopsy: Confirmatory of ganglionic cells---absence
- Anorectal manometry --- records the reflex pressure response of the internal anal
sphincter- external resphincter contracts normally but fails to relax.

Medical Management
- Repair: dissection and removal of the affected section, with anastomosis of the intestine.
- two-stage surgery:
● 1st: temporary colostomy
● 2nd bowel repair at 12 to 18 months of age
 - In a few instances in which the anus is deprived of nerve endings, a permanent
colostomy may be established.

Nursing Management
• Assess
- signs of enterocolitis
- bowel functioning
- passage of meconium in neonates.
• Promote adequate hydration.
• Note and record: frequency and characteristics of stools.
- measure abdominal girth to assess for increasing distention.

Nursing Management
• Promote adequate nutrition
• Small and frequent feedings
- Administer enemas- to relieve constipation. Avoid taking temperature rectally
•Administer prescribed medications
• systemic antibiotic given with an enema to reduce intestinal flora.
- stool softener
- Decrease discomfort
• Elevate HOB
• Change the child's position frequently
- Assess for any respiratory difficulty
• Support the child and parents.
• Prepare the child and the parents for procedures and treatments, which may include:
- Manual dilation of the anus dietary management
• cleansing enemas
● Surgery to remove the aganglionic, nonfunctioning segment of the colon, followed by
anastomosis in three stages:
- Temporary colostomy before definitive surgery to allow the bowel to rest and the child to
gain. weight
- Reanastomisis by means of an abdominoperineal pull-through about 9 to 12 months
later
- Closure of the colostomy about 3 months after the pull-through procedure.

POST OPERATIVE CARE

- Assisting with symptomatic treatment
● enemas, low-fiber, high-calorie, high-protein diet, and occasionally the use of TPN.
- Preparing the bowel for surgery
● saline enemas, systemic antibiotics, and colonic antibiotic irrigations to decrease bowel
flora; bowel prep is not necessary for the neonate whose bowel is sterile.
- Keeping the child on NPO during the initial period after surgery.
- Monitoring 1&O including NGT drainage.
- Prevent contamination.
 - Initiating oral fluids after bowel sounds are identified.
- Providing ostomy care if indicated:
preparation of the skin, application of the collecting appliance, control of odor, and
monitoring of problems such as ribbon-like stools, excessive diarrhea, bleeding and
failure to pass stools or flatus.
- Instruct the family regarding home care

9. NEURAL TUBE DEFECTS

- defects of the CNS involving the cranium or spinal cord, may be cystic or noncystic
- multifactorial inheritance.
- Many children (80% to 85%) with myelomeningocele form of spina bifida have
hydrocephalus.

Types of Disorders

1. Anencephaly
- absence of the cerebral hemisphere.
- the upper end of the neural tube fails to close in early intrauterine life.
- increase AFP
- no brain formation
2. Microcephaly
- brain growth is so slow
- associated with an intrauterine infection (TORCH) as rubella, cytomegalovirus, or
toxoplasmosis(toxoplasma gondi)
- from severe malnutrition or anorexia in early infancy.