2nd Lecture: Nervous-Sytem-Physical-and-Developmental-Disorders

Children with Pectus excavatum are likely to develop these conditions...

- COPD - Rickets

A form of assessment to check for Talipes Disorder...

Straightening all newborn's feet to the midline

A condition in which some toes or fingers are fused together usually caused by simple webbing...

Syndactyly

HYDROCEPHALUS: Therapeutic Mgmt.

A. Acatazolamide (Diamox) B. Laser surgery to reopen the route of flow or bypassing the point of obstruction by shunting the fluid to another point of absorption. C. Shunting procedure involves threading a thin polyethylene catheter under the skin from the ventricles to the peritoneum. Fluid drains by this route into the peritoneum and is absorbed across the peritoneal membrane into the body circulation. D. Prognosis is improving everyday as shunting and surgical procedures become more common and more effective. The ultimate prognosis for a child depends on whether brain damage occurred before shunting and if a shunt is in place, whether the parents can recognize when it needs to be replaced to prevent increased intracranial pressure

SOME TYPES OF NEURAL TUBE DISORDERS

A. Anencephaly B. Microcephaly C. Spina Bifida Occulta D. Meningocele E. Myelomeningocele F. Craniosynostosis G. Arnold-Chiari Disorder

HYDROCEPHALUS: Postoperative care

A. Assess for signs of increased intracranial pressure after surgery B. Have NGT until bowel sounds return and the tube can be removed. C. Introduced fluid gradually in small quantities after removal of the tube D. Support the head well when moving them to avoid strain E. Hold head with the whole palm F. No contraindications for breast feeding exist G. Note how the child sucks H. Observe constipation I. Urge parents to increase fluid and roughage in the diet as a preventive measure J. Observe the pump site daily for signs of swelling or redness K. Have child sleep with his head slightly elevated at night to help ensure fluid flow through the tube L. Wash the head daily and position it every 2 hours to relieve pressure. • M. As the child grows taller, the shunt will eventually need to be replaced for proper functioning. N. Use helmet to protect the head O. Preschool and school age parents need to confer to make the nurse/ teacher aware that the child has a shunt in place and that the child may need special head protection

What is Spina Bifida Occulta?

occurs when the posterior laminae of the vertebrae fail to fuse. This occurs most commonly at the fifth lumbar or first sacral level but may occur at any point along the spinal cord. Noticeable as a dimpling at the point of poor fusion; abnormal tufts of hair or discolored skin may be present Benign disorder (1 in every 4 child

HYDROCEPHALUS: signs of increased intracranial pressure after surgery

tense fontanelles, increasing head circumference, irritability or lethargy, decreased level of consciousness, poor sucking, vomiting, an increase in blood pressure, increasing temperature, and decrease pulse and respiratory rates.

What is a Subluxated hip?

the femur, "rides up," due to the flat acetabulum.

What is Myelomeningicele?

the spinal cord and the meninges protrude through the vertebrae the same with a meningocele. The difference is that the spinal cord ends at the point, so motor and sensory function is absent beyond this point.

What is a Obstructive Hydrocephalus?

there is a block to such passage of fluid

THERAPEUTIC MGMT:" Talipes Disorder

- Cast extending to above the knee to ensure firm correction. - Teach parents on how to assess for blueness and coldness, as well as the blanch test for good circulation. - crying episodes must be evaluated carefully. - changing cast every 1-2 weeks - cast is removed at 6 weeks - performing passive foot exercises for several months on a daily basis. - infant wearing DENIS BROWNE SPLINTS.

Cause of Developmental hip dysplasia?

- Generally unknown, but probably due to polygenic inheritance pattern.

What are the 4 separate types of Talipes Disorder?

- Plantarflexion - Dorsiflexion - Varus Valgus deviation.

DEVELOPMENTAL HIP DYSPLASIA: General Guidance

- Surgery for final correction. - Visiting Orthopedist frequently - teaching parents to bond with children despite casts - bringing experiences to the infant - assuring that the pts will be able to catch up with normal developmental steps.

For patients with Pectus Excavatum, what happens to the thoracic organs?

- The lung volume decreases due to the minimized space; - The Heart is displaced more to the left.

ABSENT OR MALFORMED EXTREMITIES: Cause/s

- maternal drug ingestion - virus invasion - amniotic band formation (AB Syndrome)

Developmental hip dysplasia: Assessment cues

- one leg is shorter - unequal skin folds revealed via sonogram, also through X-rays and MRI which reveal the lateral placement of the femur.

DEVELOPMENTAL HIP DYSPLASIA: Therapeutic Management

- use of splints or casts - Use if traction to bring the femur in a good position within the acetabulum - Splint correction: @ initial hospital stay using 2-3 diapers between legs #FREJKA SPLINT = like a huge confining diaper - keeping splints at all time except when bathing or changing diapers - need for good diaper area care - swadling is not good, or bringing the legs together in general. #PAVLIK HARNESS - adjustable chest halter that abducts the legs. reduces therapy to 3-4 weeks. - assess for redness on areas of harness - ineffective if removed frequently.

Expound BARLOW'S SIGN

1. Hold the hips and knees at 90 degrees flexion, apply a backward pressure and adduct the hips. Note any feeling of the femoral head slipping * A feeling of the femur head slipping out of the socket posterolaterally is a positive Barlow's sign indicative of hip instability associated with developmental hip dysplasia

EXPLAIN THE procedure for ORTHOLANI'S SIGN...

1. Lay the infant supine and flex the knees at 90 degrees at the hips 2. Place your middle fingers over the greater trochanter of the femur and your thumb on the internal side of the thigh over the lesser trochanter 3. Abduct the hips while applying upward pressure over the greater trochanter, & listen for a clicking sound * normally, no sound is heard. A clicking or clunking is a positive Ortholani's sign & occurs when the femoral head re-enters the acetabulum

MYELOMENINGOCELE: Postoperative care

A. Child will be placed on the abdomen until the skin incision has healed B. Careful precaution of the feces and urine not to touch the incision area C. Hold the baby in as normal feeding position D. Remind parents when to burp the baby (do not tap the back over the disorder E. Parents must make routine of inspecting lower extremities and buttocks for irritation or infection

HYDROCEPHALUS: Types

A. Communicating hydrocephalus/ Extra ventricular hydrocephalus B. Obstructive Hydrocephalus

HYDROCEPHALUS: Nature

A. Congenital B. Acquired

MYELOMENINGOCELE: Characteristics accompanied

A. Flaccidity B. Lack of sensation of the lower extremities C. Loss of bowel and bladder control D. They may have accompanying talipes (clubfoot) disorders and hip dysplasia E. 80% with myelomeningocele infants have hydrocephalus due to lack of a subarachnoid membrane for CSF absorption; higher myelomeningocele occurs on the cord the more likely it is that hydrocephalus will accompany it. F. CT scan, MRI and sonogram will reveal the size of the skull disorder

What happens to infants with Anencephaly?

A. Infants with anencephaly may have difficulty in labor because the underdeveloped head does not engage the cervix well. B. Children cannot survive with this disorder because they do not have cerebral function

HYDROCEPHALUS: Assessment

A. It can be detected on a sonogram B. Infants fontanelles widen and appear tense C. Suture lines on the skull separate D. Head diameters enlarges E. Scalps becomes shiny F. Scalp veins become prominent G. Brow bulges H. Eyes become sunset I. Increase intracranial pressure (decrease pulse, decrease respirations, increased temperature and blood pressure) J. Shrill, high-pitched cry K. Impaired motor function because of heavy head L. It can be demonstrated by sonography , CT scan, or MRI M. Skull xray film may reveal the separating sutures and thinning of the skull. N. Transillumination against the skull with the child in a darklight will reveal the skull is filled with fluid rather than solid brain.

MYELOMENINGOCELE: Assessment

A. May be discovered during intrauterine life by sonogram, fetoscopy, amniocentesis or analysis of AFP in the maternal serum. B. Infants may born cesarean section to avoid pressure and injury to the spinal cord C. Assess the newborn if they have lower motor function D. Assess the nature and pattern of voiding and defecation

HYDROCEPHALUS: Causes

A. Overproduction of fluid by a choroid plexus in the firstor second ventricle as could occur from a growing tumor B. Obstruction of the passage of fluid in the narrow aqueduct of Sylvius (foramina of Magendie & Luschka)- infections such as meningitis or encephalitis, hemorrhage from trauma, or tumor C. Occur form interference with the absorption of CSF from the subarachnoid space if a portion of the subarachnoid membrane is removed as occurs with surgery for meningocele, or after extensive subarachnoid hemorrhage

NEURAL TUBE DISORDERS: Some risk factors and Detection

A. Polygenic inheritance pattern B. Poor nutrition (deficient in folic acid-major contributing factor C. The risk of bearing a 2nd child with neural tube disorder once one child is born with such disorder increases as 1 in 20 D. Serum assessment is done at week 15 of pregnancy when AFP reaches its peak concentration E. Sonogram help to determine the presence of the disorder

Development of a baby with Microcephaly...

A. Prognosis for normal life is guarded and depends on the extent of restriction of brain growth and the cause. B. Infant is cognitively challenged because of lack of functioning brain tissue

MICROCEPHALY: Causes (infection)

A. Rubella B. Cytomegalovirus C. Toxoplamosis

MYELOMENINGOCELE: Perioperative care

A. Use sterile gloves and sterile linens when caring for the infant B. Position the infant carefully (prone or supported on their side) C. Placing a piece of plastic or sturdy plastic wrap below the meningocele to prevent feces from touching the open lesion D. Sterile wet compress of saline, antiseptic or antibiotic gauze over the lesion to keep the sac moist. E. Any seepage of clear fluid from the disorder should be reported promptly (check for glucose in urine & mucus

myelomeningocele: therapeutic mgmt.

A. surgery to replace the contents that are replaceable and to close the skin disorder to prevent infection B. Before surgery is done only after the infant has survived the newborn period. Currently it is done as soon after birth as possible (within 24-48 hrs C. Parents needs to be cautioned that the prognosis for the child depends on the extent of the disorder. D. Parents need a great deal of support of care for a child with myelomeningocele because the child has multiple challenges; they continue to have paralysis of the lower extremities and loss of bowel and bladder control

What is a disorder commonly accompanied by Lumbosacral myelomeningocele?

ARNOLD-CHIARI DISORDER:

What is Acatazolamide?

Brand name: Diamox For promoting excretion of fluid.

ARNOLD-CHIARI DISORDER: Cause

Caused by overgrowth of the neural tube in weeks 16-20 fetal life

HYDROCEPHALUS: CSF

Cerebrospinal fluid is formed in the first and second ventricles of the brain and then passes through the aqueduct of Sylvius and the fourth ventricle to empty into the subarachnoid space of the spinal cord, where it is absorbed

What do you call the improper formation and function of the hip socket?

Developmental hip dysplasia

What is the appropriate action should the passive stretching exercises prove to be ineffective in alleviating Torticollis?

If not effective within 1 year, surgery may be done.

What is a Communicating Hydrocephalus?

If the fluid can reach the spinal cord

What is Hydrocephalus?

Is an excess of CSF in the ventricles or the subarachnoid space. In the infant whose cranial sutures are not firmly knitted, this excess fluid causes enlargement of the skull.

A type of exercise activity that is taught to parents with children suffering from Torticollis as an early intervention...

Passive stretching Exercises

What condition manifests as an anterior protrusion of the Sternum?

Pectus Carinatum

What do you call the indentation found at the lower portion of a child's sternum?

Pectus Excavatum

What condition refers to the presence of additional/excess number of fingers and/or toes?

Polydactyly

What is ARNOLD-CHIARI DISORDER?

The anomaly is a projection of the cerebellum, medulla oblongata and fourth ventricle into the cervical canal

What anomaly results from the injury of the sternocleidomastoid muscle upon birth?

Torticollis (Wry neck)

MICROCEPHALY VS. CRANIOSYNOSTOSIS

True microcephaly must be differentiated from craniosynostosis (normal brain growth but premature fusion of the cranial sutures) which also causes decreased head circumference. Infants with craniosynostosis have abnormally closed fontanelles and often show bulging (bossing of the forehead and signs of increased intracranial pressure). Craniosynostosis can be relieved by surgery and brain growth will be normal

Generally, what characteristic of the baby is found on most cases of Torticollis?

Wide shoulders

What is Micricephaly?

a disorder in which brain growth is so slow that it falls more than three standard deviations below normal on growth charts. It is associated with intrauterine infection such as rubella, cytomegalovirus and toxoplasmosis. It can be a result of severe malnutrition or anoxia in early pregnancy.

What is Anencephaly?

absence of the cerebral hemispheres. It occurs when the upper end of the neural tube fails to close in early uterine life. It is revealed by an elevated level of AFP in the maternal serum or amniocentesis and confirmed by a sonogram

What is a Spica Cast?

if a hip is fully dislocated or subluxation is severe, an infant may be placed immediately in frog-leg cast or spica cast. The child may first be placed in a Bryant's traction for a week to better position of the hip. The hip is then placed in an abducted position and a large hip spica cast or an A-line cast. (change every 6-9 months).

What is Meningocele?

if the meninges covering the spinal cord herniate through unformed vertebrae, a meningocele occurs. The anomaly appears a protruding mass usually approximately the size of an orange at the center of the back. It generally occurs at the lumbar region although it might be present anywhere along the spinal canal. The protrusion may be covered by a layer of skin or only clear dura mater.

What is a #FREJKA SPLINT?

like a huge confining diaper