Professional Documents
Culture Documents
Assessment .
Cleft lip: slight notch to a complete separation from the
floor of the nose
Cleft palate: nasal distortion, midline or bilateral cleft and
variable extension from the uvula and soft and hard palate
Difficulty in swallowing
Increase in upper respiratory infections
The blue lines indicate incisions.
Surgical Treatment
Cheiloplasty
o Lip reduction - the process of surgically reducing
the size of the lip or lips in order to reduce the
appearance of abnormally large or protruding lips
and the process of forming an artificial tip or part
of the lips by using a piece of healthy tissue from
some neighboring part
Postoperative Interventions
Interventions Cleft Lip
Assess the ability to suck, swallow, handle normal o A lip protector device or Logan bar may be taped
secretions, and breathe without distress securely to the cheeks to prevent trauma to the suture
Assess fluid and calorie intake daily line
o Position the child on the side lateral to the repair or
Monitor daily weight
on the back
Modify feeding techniques: plan to use specialized feeding
o Avoid prone position to prevent rubbing of the
techniques, obturators and special nipples and feeders
Hold the child in an upright position and direct the formula surgical site on the matter
to the side and back of the mouth to prevent aspiration o After feeding, cleanse the suture line of formular or
Feed small amounts gradually and burp frequently serosanguineous drainage with a cotton-tipped swab
dipped in normal saline
Position on the side after feeding
o Apply antibiotic ointment to the site as prescribed
Keep suction equipment and a bulb syringe at the bedside
o Elbow restraints should be used to prevent the infant
Teach the parents special feeding or suctioning techniques
from injuring or traumatizing the surgical site
o ESSR Method
-Soft elbow or jacket restraints to keep the child
from touching the repair site Treatment
-Remove restraints at least every 2 hours to assess Relieve constipation with stool softeners and rectal
skin integrity and to allow for exercising the arms irrigations
Monitor for signs and symptoms of infections at the Surgery- surgical removal (resection) of the abnormal
surgical site section of the colon, followed by reanastomosis
Keep the surgical site clean and dry a. temporary colostomy – neonatal period
relieve obstruction and allow the normally
Cleft Palate Repair innervated, dilated bowel to return to its normal
The child is allowed to lie on the abdomen size
Feedings are resumed by bottle, breast or cup b. Complete surgical repair
Oral packing may be secured to brush his or her teeth o Done when child weighs 9 kg (20lpbs)
Instruct the parents to avoid offering hard food items to the o Pull through procedure to excise portions of the
child such as toast or cookies bowel
Repairs the colon by connecting the
Other Interventions functioning portion of the bowel to the anus.
Avoid contact with sharp objects near the surgical site The typical method for treating
Avoid the use of oral suction or placing objects in the Hirschsprung’s in younger patients.
mouth such as tongue depressor, thermometer, straws, o Colostomy is closed.
spoons, forks, or pacifiers
Provide analgesic for pain
Instruct the parents to monitor for signs of infection at the The Swenson, Soave, Duhamel, and Boley procedures all
surgical site, such as redness, swelling or drainage vary slightly from each other:
Encourage the parents to hold the child The Swenson procedure leaves a small portion of the
Initiate appropriate referrals for speech impairment or diseased bowel.
language-based learning difficulties The Soave procedure leaves the outer wall of the
colon unaltered. The Boley procedure is just a small
modification of the Soave procedure. The term
HIRSCHPRUNG’S DISEASE "Soave-Boley" procedure is sometimes used.
Congenital aganglionosis or aganglionic megacolon; The Duhamel procedure uses a surgical stapler to
congenital aganglionic megacolon, connect the good and bad bowel.
Congenital disorder of the colon in which certain nerve
cells, known as ganglion cells, are absent, causing chronic Interventions: Medical Management
constipation. Maintain low-fiber ; high-calorie, high-protein diet;
Results in mechanical obstruction because of inadequate parenteral nutrition
motility in an intestinal segment Stool softeners
The lack of ganglion cells is in the myenteric plexus, Daily rectal irrigations with normal saline to promote
which is responsible for moving food in the intestine adequate elimination and prevent obstruction
Assessment
characteristic triad of symptoms:
o impairments in social interaction;
o impairments in communication;
o Repetitive behavior.
Other aspects,
o Atypical eating, are also common but are not essential for
diagnosis.]
Communication impairment
first year of life
Spina Bifida Cystica
May include delayed onset of babbling, unusual
Protrusion of the spinal cord and/or its meninges occurs
gestures, diminished responsiveness, and vocal patterns
that are not synchronized with the caregiver. Defect results in incomplete closure of the vertebral and
neural tubes, resulting in a saclike protrusion in the lumbar
Second and third years,
or sacral area with varying degrees of nervous tissue
Autistic children have less frequent and less diverse
involvement
babbling, consonants, words, and word combinations; their
gestures are less often integrated with words.
Spina Bifida Occulta
Repetitive behavior Occulta is Latin for "hidden.“ ; mildest forms of spina
bifida.
Stereotypy is repetitive movement, such as hand flapping,
making sounds, head rolling, or body rocking. Spinal cord remains intact and usually is not visible
Compulsive behavior is intended and appears to follow Meninges are not exposed on the skin surface
rules, such as arranging objects in stacks or lines. Neurological deficits are not usually present
Sameness is resistance to change; for example, insisting The skin at the site of the lesion may be normal, or it may
that the furniture not be moved or refusing to be have some hair growing from it; there may be a dimple in
interrupted. the skin, a lipoma, a dermal sinus or a birthmark
Ritualistic behavior involves an unvarying pattern of
daily activities, such as an unchanging menu or a dressing
ritual.
Restricted behavior is limited in focus, interest, or
activity, such as preoccupation with a single television
program, toy, or game. Self-injury includes movements
that injure or can injure the person, such as eye poking,
skin picking, hand biting, and head banging
Interventions
Determine the child’s routines, habits, and preferences and
Meningocele
maintain consistency as much as possible
Protrusion involves meninges and a sac-like cyst that
Determine the specific ways in which the child
contains CSF in the midline of the back, usually in the
communicates
lumbosacral area
Facilitate communication through the use of picture boards
Spinal cord is not involved
Evaluate the child for safety
Neurological deficits are usually not present
Implement safety precautions as necessary for self-
injurious behaviors such as head banging
Monitor for stress and anxiety
Avoid placing demands on the child
Initiate referrals to special programs as required
Provide support to parents
o The child is at high risk for latex allergy
Myelomeningocele HYDROCEPHALUS
Protrusion of the meninges, CSF, nerve roots, and a portion An imbalance of CSF absorption, or production caused by
of the spinal cord occurs malformations, tumors, hemorrhage, infection or trauma
The sac (defect) is covered by a thin membrane prone to Characterized by
leakage or rupture An abnormal increase in CSF volume within the
Neurological deficits are evident intracranial cavity and
enlargement of the head in infancy
Hydrocephalus- Types
Noncommunicating
Communicating
Noncommunicating Hydrocephalus
Obstruction in the system between the source of CSF
production (ventricles) and the area of its reabsorption
(subarachnoid space)
Caused by
Congenital defects- Arnold-Chiari malformation,
Assessment Dandy-Walker cyst, aqueduct stenosis
Depends on the spinal cord involvement Acquired conditions – infections, trauma,
Visible spinal defect spontaneous intracranial bleeding and neoplasms
Flaccid paralysis of the legs
Altered bladder and bowel function Communicating Hydrocephalus
Hip and joint deformities Failure in the absorption system; cause unknown
Hydrocephalus Excessive production of CSF-tumor or unknown
causes
Interventions
Evaluate the sac and measure the lesion Normal Flow of CSF
Perform neurological assessment
Monitor for increased ICP which might indicate
developing hydrocephalus
Measure head circumference; assess the anterior fontanel
for fullness
Protect the sac; cover with a sterile, moist (normal saline),
nonadherent dressing to maintain the moisture of the sac
and contents and change dressing every 2 to 4 hours as
prescribed
Place in a prone position to minimize tension on the sac
CSF Choroid plexus lateral ventricles
and the risk of trauma; the head is turned to one side for
foramen of Monro ), third ventricle aqueduct of
feeding
Sylvius (Cerebral aqueduct) fourth ventricle,
Change the dressing covering the sac whenever it becomes
foramina of Magendie (Median aperture) and foramina of
soiled because of the risk of infection; diapering may be
Luschka (Lateral apertures) subarachnoid space
contraindicated until the defect has been repaired
over brain and spinal cord reabsorption into
Use aseptic technique to prevent infection venous sinus blood via arachnoid granulations
Assess the sac for redness, clear or purulent drainage,
abrasions, irritation and signs of infections The CSF is produced at a rate of 500 ml/day. Since the brain
Early signs of infection include elevated temperature can contain only 135 to 150 ml, large amounts are drained
(axillary), irritability, lethargy and nuchal rigidity primarily into the blood through arachnoid granulations in
Assess for physical impairment such as hip and joint the superior sagittal sinus. Thus the CSF turns over about 3.7
deformities times a day. This continuous flow into the venous system
Prepare the child and family for surgery dilutes the concentration of larger, lipoinsoluble molecules
Administer antibiotics preoperatively, as prescribed for penetrating the brain and CSF.
infection
Teach the parents and eventually the child about long-term
home care
o Positioning, feeding, skin care and ROM Pathophysiology
o Performing clean intermittent catheterization Tumorobstruction of the flow of CSFincreased ventricular
o Administer propantheline (Pro-Banthine) as pressurethinning of the cerebral cortex and cranial bones (frontal,
prescribed to improve continence parietal and temporal areas)
o Implement a bowel program including a high-fiber Floor of the 3rd ventricle bulges downward
diet, increased fluid, suppositories as needed
Compression of the optic Basal ganglia, brainstem
Surgical Management
nerve Extracranial Shunt Procedure
& hypophysis cerebri and cerebellum 1. Ventriculoperitoneal (VP) shunt
Dilation of sella turcica choroid plexus atrophied 2. Ventriculoatrial (VA) shunt)
3. Ventriculopleural shunt
Hydrocephalus- Clinical Manifestations 4. Ventriculo-gall bladder shunt
Infants
o Excessive head growth (maybe seen up to age 3) Ventriculoperitoneal shunts
o Delayed closure of the anterior fontanelle Diverts CSF from lateral ventricle or the spinal
o Fontanelle tense and elevated above the surface of subarachnoid space to the peritoneal cavity
the skull A tube is passed from the lateral ventricle through an
o Signs of increased intracranial pressure occipital burr hole subcutaneously through the posterior
o Alteration of muscle tone of the extremities including aspect of neck and paraspinal region to the peritoneal
clonus or spasticity cavity through a small incision in the RLQ
Later Physical Signs
o Forehead becomes prominent (bossing) Ventriculoatrial shunt
o Scalp appears shiny with prominent scalp veins A tube is passed from the dilated lateral ventricle through a
o Eyebrows and eyelids may be drawn upward, burr hole in the parietal region of the skull
exposing the sclera above the iris It then is passed under the skin behind the ear and into a
o Infant cannot gaze upward, causing “sunset eyes” vein down to a point where it discharges into the right
atrium or superior vena cava
o Strabismus, nystagmus, and optic atrophy
A one-way pressure-sensitive valve will close to prevent
o Difficulty holding head up
reflux of blood into the ventricle and open as ventricular
o Child may experience physical or mental
pressure rises, allowing fluid to pass from the ventricle
developmental lag into the bloodstream
Older Children
With closed sutures and present with signs of increased Ventriculoperitoneal shunt
ICP Diverts CSF to the pleural cavity
o Vomiting
Indicated when the VP or VA route cannot be used
o Restlessness and irritability
o High-pitched, shrill cry Ventricle-gall bladder shunt
o Rapid increase in head circumference (infants) Diverts CSF to the common bile duct
o Tens, bulging fontanelle (infants) Used when all other routes are unavailable