Stage 4: Individuating-reflexive—Adolescence beco- me more sceptical and begin to compare the religious
1. Non-communicating hydrocephaly (due to developmental malformation), or obstructive
hydrocephaly, is caused by a CSF-flow obstruction ultimately preventing CSF from flowing into the subarachnoid space (either due to external compression or intraventricular mass lesions).
l Foramen of monro obstruction may lead to dilation of one or, if large enough (e.g., in colloid cyst), both lateral ventricles.
l The aqueduct of sylvius, normally narrow to begin with, may be obstructed by a number of genetically or acquired lesions (e.g., atresia, ependymitis, haemorrhage, and tumour) and lead to dilation of both lateral ventricles as well as the third ventricle.
l Fourth ventricle obstruction will lead to dilatation of the aqueduct as well as the lateral and third ventricles.
l The foramina of luschka and foramen of magendie may be obstructed due to congenital failure of opening (e.g., Dandy-Walker malformation).
2. Communicating hydrocephaly, also known as non-obstructive hydrocephaly
l It is caused by impaired cerebrospinal fluid resorption in the absence of any CSF-flow obstruction between the ventricles and subarachnoid space. It has been theorized that this is due to functional impairment of the arachnoid granulations, which are located along the superior sagittal sinus and is the site of cerebrospinal fluid resorption back into the venous system. Various neurologic conditions may result
in communicating hydrocephalus, including subarachnoid/intraventricular haemorrhage, meningitis, Chiari malformation, and conge- nital absence of arachnoidal granulations (Pacchioni’s granulations). Scarring and fibrosis of the subarachnoid space following infectious, inflammatory, or haemorrhagic events can also prevent resorption of CSF, causing diffuse ventricular dilatation.
l Surgery to repair a myelomeningocele.
l Adhesions between meninges at the base of the brain of meningeal haemorrhage.
l Rarely, a tumour in the choroids plexus that causes an overproduction of CSF.
Pathophysiology
CSF is formed by the choroid plexus mainly in the lateral ventricles. It is absorbed into the venous system through the arachnoid villi, CSF circulates within the ventricles and the subarachnoid space. (It is a colorless fluid, consisting of water with traces of protein, glucose and lymphocytes.) Any defect in this causes the problem.
Clinical Feature
Infancy—Head enlarges, suture line separate
l AF becomes tense and bulging.
l Setting sun eyes.
l Macewen sign (cracked pot sound).
l Fontal enlargement or bossing with depressed eyes.
l Infant reflexes persist.
l Failure in the development.
l Convulsion, nystagmus.
l Increased Cranial Pressure.
l Child has difficulty to turn the head or raising.
l Irritability to support the head when upright or lethargy.
l Vomiting not related to food intake.
l Distended scalp vein, thin, shiny and fragile looking scalp skin and weak muscles.
l High–pitched cry.
Investigation
l Physical examination and assessment findings of clinical features.
l Head circumference.
l Computerized axial tomography.
l Angiography.
l MRI.
l Light reflexion—Light reflects off the opposite side of the skull with skull transillumination.
Complications
l Physical injury
l Delayed growth and development
l Decreased intracranial adaptive capacity
Fig. 4.2: Child with ventricular peritoneal shunt
Treatment
Medical therapy—towards reduction of production of CSF.
Surgical therapy
1. Remove the obstruction.
2. Ventricular bypass into a normal intracranial channel.
3. Drainage of fluid from a lateral ventricle into an extra cranial compartment (Right atrium or peritoneum) by the way of artificial passage or shunt.
m Ventriculoatrial shunt
m Ventriculo peritoneal shunt.
Mechanical Complication of Shunting
l Shunt malfunction, shunt failure
l Kinking of the tube
l Shunt infection
l Shunt block (clogged)
l Plugging or separation of the tubing and
l Bacterial infection
l Recurrent hydrocephaly due to failure of shunt
l Increased intracranial pressure.
Nursing Care Preoperative Care
Preoperatively the infant should be observed carefully for the signs of increased intracranial pressure.
l Daily head circumference to be measured
l Fontanels and suture lines are gently palpated for size, signs of bulging, tenseness and separation
l Psychological aspect of care
l Adequate nutrition—small and frequent feeding
l Prevent pressure sores.
Postoperative Care
l Routine post operative care and observation.
l Assess the shunt function, if not functioning the cerebrospinal fluid will begin to accumulate again and a number of physical symptoms will develop (headaches, nausea, vomiting, photophobia/light sensitivity), some extremely serious, like seizures.
l Position the baby on unoperated side to prevent pressure on the shunt value.
l Observe the sign of increased intracranial pressure.
l Prevent infection—observe the sign of elevated vital signs, poor feeding, and vomiting, decreased response and seizure activity.
l Administration of antibiotics.
l Emotional support to the parents.
l Prevent post operative complications.
l Administer comfort measures.
Craniosynostosis
Premature closure of single or multiple sutures of cranial vault, face, and base of skull during the first 18 months of life. This condition occurs in upto 2000 births.
Causes
Unknown 15% have autosomal dominant inherited syndromes such as Crouzon syndrome.
Diagnosis
l Clinical appearance
l Palpitation of the skull reveals a bony ridge along a suture
l Skull X-ray
l CT scan
l MRI Treatment
l Reconstructive surgery is performed to protect mental development and vision.
Nursing Care
Observe the child for symptoms of increased intracranial pressure like early signs and report immediately.
l Headache
l Visual disturbances, diplopia
l Nausea and vomiting
l Dizziness
l Slight change in vital signs
l Seizures
l Infant has the signs of bulging fontanels
l Wide sutures, increased HC
l Dilated scalp veins
l High piched, catlike cry.
THE EYE
Congenital Cataracts
Definition: Opacity of crystalline lens Pathophysiology
Prevents light rays from entering eye and refracting them on retina.
Clinical Feature
l Gradually less able to see objects clearly
l May lose peripheral vision
l Gray opacities of lens
l Strabismus
l Absence of red reflex Treatment
l Surgical treatment to remove cloudy lens and replace lens.
l Must be treated early to prevent blindness from amblyopia.
Strabismus
“Squint” or cross eye—malalignment of eyes.
Estropia—inward deviation of eye.
Exotropia—outward deviation of eye.
Pathophysiology
Strabismus results from muscle imbalance or paralysis, poor vision or congenital defect.
Treatment
Treatment depends on causes of strabismus
l May involve occlusion therapy (Patching stronger eye) or surgery to increase visual stimulation to weaker eye.
l Early diagnosis is essential to prevent vision loss.
Nursing Management
l Assessment of children for visual impairment is a critical nursing responsibility.
l Identifying those children whose history is at risk.
l Observing for behaviours that indicate a vision loss.
l Screening all children for visual acuity and signs of other ocular disorders such as strabismus.
If it is Infant
l At birth the nurse should observe the neonates response to visual stimuli like following a light or object and cessation of body movement.
l Lack of eye contact from the infant.
l Infancy of the child should be tested for strabismus.
l Lack of binocularity after 4 months of age is considered abnormal and must be treated to prevent amblyopia.
Note: Suspect blindness if the infant does not react to light and in any-age child if parents express concern.
MOUTH
Cleft Lip and Cleft Palate
Cleft lip with or without cleft palate is the most common craniofacial malformation. It occurs in 1 in 800 live births. Cleft palate occurs 1 in 2000 live births. Cleft lip results from failure of the bone and tissue of the upper jaw and palate to fuse completely at the midline.
Causes
l Congenital defects; in some cases inheritance plays a role.
l Part of another chromosomal abnormality.
l Prenatal exposure to teratogens or environmental factors.
l Maternal smoking.
Pathophysiology
l Development of the primary and secondary palates takes place at different times and involves different developmental processes.
l Primary palate of the maxillary process to fuse with the nasal elevation on the frontal prominence, which occurs normally during the sixth week of gestation.
l Merging of the upper lip at the midline is completed between the seventh and eighth weeks of gestations.
l Fusion of the secondary palate (hard and soft) takes place later in development, between the seventh and twelfth weeks of gestation.
l At the time the primary palate is completed, the two lateral palatine processes are situated in a vertical position at the side of the tongue.
l In the process of migrating to a horizontal position, they are, for a short time, separated by the tongue movement downwards, allowing the palatine process to fuse with each other and with the primary palate to form the roof of the mouth.
l If there is delay in this movement, or if the tongue fails to descent soon enough, the remainder of development proceeds but the palate never fuses.
Fig. 4.3: Types of cleft lip and cleft palate The above illustration shows the four variations of cleft lip and cleft palate.
A. Notch with vermilion border B. Unilateral cleft lip and cleft palate C. Bilateral cleft lip and palate D. Cleft palate
Assessment Findings
l Abdominal distention from swallowed air
l Difficulty in swallowing
l If infant has cleft lip
m Simple notch on the upper lip
m Complete cleft from the lip edge to the floor of the nostril
m On either side of the midline but rarely along the midline itself.
l If infant has cleft palate
m Opening in the palate.
m May be partial or complete.
Complications
l Speech defects.
l Dental and orthodontic problems.
l Nasal defects.
l Alterations in hearing.
l Shock, guilt and grief for the parents that may interfere with parent child bonding.
l Increased risk of aspiration, upper respiratory infection, and otitis media.
Management
Management of both defects is directed towards surgical closure of the cleft, prevention of complication
and facilitation of normal growth and development of the child.
l Cheiloplasty (cleft lip repair surgery):
m Performed between birth and age 3 years.
m Unites the lip and gum edges:
n Provides a route for adequate nutrition and sucking.
n Performed in anticipation of tooth eruption.
l Staphylorrhaphy (cleft palate repair surgery):
m Scheduled at about age 18 months:
n Allow for palate growth.
n Allow for surgery to be done before the infant develops speech patterns.
m Infant must be free from ear and respiratory infection.
l Long-term, team-oriented care to address speech defects, dental and orthodontic problems, nasal defects, and possible alterations in hearing.
l If cleft lip is detected on sonogram while the infant is in utero, foetal repair may be possible.
Nursing Management
l Maintain the nutritional status of the infant:
m To help determine an effective feeding method.
m Assess the quality of the child’s sucking by determining if the infant can form an airtight seal around a finger or nipple that’s placed in his mouth.
m Nurses should feed the infant with a specialized nipple used for cleft lip or palate infant.
m Assess the infant’s ability to swallow.
m Secure the intravenous line, administer intravenous fluid to maintain the nutritional status.
l Assess infant for the complication:
m Assess for abdominal distension from swallowed air.
m Assess respiratory status to detect signs of aspiration (choking, respiratory distress, cyanosis, cough).
l Monitor vital signs (temperature, heart rate, respiration, BP).
l Maintain intake and output chart to determine fluid volume status.
Preoperative Nursing Care for Cheiloplasty
l Administer diet appropriate for age, provide adequate nutrition.
l Assist mother with breastfeeding if this is mother’s preference, since the newborn with either defect can breast–feed.
l Position and stabilize nipple well back in oral cavity so that tongue action facilitates milk expression.
l Feed the infant slowly and in an upright position to decrease the risk of aspiration.
l Feed the infant using a commercially available cleft lip and cleft palate nipple.
l Position nipple between infant’s tongue and existing palate to facilitate compression of the nipple.
l When using the device without nipple (e.g., Breck feeder-syringe with rubber tube, Asepto syringe), deposit the formula on back of tongue to facilitate swallowing and adjust flow according to infant’s swallowing to prevent aspiration.
l Burp often during feeding to eliminate swallowing air and decrease the risk of emesis.
l Use gavage feeding if oral feedings are unsuccessful.
l Administer a small amount of water after feeding to rinse the mouth to prevent accumulating the milk to form bacterial growth.
l Give small and frequent feeding to promote adequate nutrition and prevent tiring of the infant.
l Encourage the parents to begin feeding as soon as possible so that they become adopt in feeding technique before discharge.
Postoperative Nursing Care for Cheiloplasty
l Maintain a patent airway; edema or narrowing of a previously large airway may make the infant appear to be in distress.
l Observe for cyanosis as the infant begins to breathe through his nose to detect signs of respiratory compromise.
l Maintain an intact suture line; keep the infant’s hands away from his mouth by using restraints or pinning his sleeves to his shirt.
l Anticipate the infant’s needs to prevent crying;
don’t place him in the prone position.
l Give extra care and support because the infant’s emotional needs can’t be met by sucking.
l When feeding resumes, use a syringe with tubing to administer foods at the side of the mouth to prevent trauma to the suture line.
l Place the infant on his right side after feedings to prevent aspiration.
l Monitor for pain and administer pain medication as prescribed.
Preoperative Interventions for Staphylorrhaphy
l Position the child on his abdomen or side to maintain a patent airway.
l Assess for signs of altered oxygenation to promote good respiration.
l Keep hard or pointed objects (utensils, straws, frozen dessert sticks) away from the child’s mouth to prevent trauma to the suture line.
l Use a cup to feed; do not use a nipple or pacifier, to prevent injury to the suture line.
l Use elbow restraints to keep the child’s hands out of his mouth.
l Provide soft toys to prevent injury.
l Start the child on clear liquids and progress to a soft diet.
l Rinse the suture line by giving the child a sip of water after each feeding to prevent infection.
l Distract or hold the child to try to deep his tongue away from the roof of his mouth.
ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL FISTULA
Congenital atresia of the oesophagus and trache- oesophageal fistula are rare malformations that represent a failure of the oesophagus to develop as a continuous passage.
Etiology
TA and TEF are not known
Incidence: 1 in 800 to 5000 live births
Predisposing facts—maternal polyhydramnios Pathophysiology
During normal embryologic development, the gut and respiratory tract originate as a single tube. During 4 and 5 weeks of gestation, this tube lengthens and separates longitudinally. Two parallel tubes are formed both of which are joined at the pharynx. Atresia occurs because of the defective growth of ectodermal cells. When there is incomplete fusion of the lateral walls of the foregut, there is incomplete closure of the
laryngotracheal tube and fistula is formed, generally it occurs at the level of tracheal bifurcation. If atresia there is a thin, non-canalized cord with an upper blind pouch connected to the pharynx and a lower pouch leading to the stomach.
l In fistulas commonly the cover pouch is connected through a fistulous tract with the trachea or a main stem bronchus.
l Less frequently a fistulous tract is present between the blind upper oesophageal pouch and the respiratory tree.
Types
Fig. 4.4: Types of tracheoesophageal atresia and fistula (a) Consists of a blind pouch at each end, widely
separated and with no communication to the trachea.
(b) Involves a fistula from the trachea to the upper oesophageal segment.
(c) One in which the proximal oesophageal segment terminates in a blind pouch and the distal segment is connected to trachea or primary bronchus by a short fistula at or near.
(d) Extremely rare anomalies involve a fistula from the trachea to the upper oesophageal segment or to both the upper and lower segments.
(e) Less frequently normal trachea and oesophagus are connected by a common fistula.
Clinical Manifestations
Oesophageal atresia—Infant with excessive salivation and drooling accompanied by 3 Cs
l Choking, coughing, and cyanosis.
l Cyanotic, sneezing and may stop breathing in aspirated into the trachea or bronchus.
l Cyanosis—due to laryngospasm.
l Acute gastric distention.
Investigation
l To rule out oesophageal atresia a catheter is generally passed into the oesophagus and gets resistance if the lumen is blocked.
l Aspiration of stomach content or introduce the air to see patent oesophagus.
l Radio opaque fluid is carefully instilled in the oesophagus under fluoroscopy.
l Fistulas are not patent.
Treatment
l If oesophageal atresia and fistula need surgical repair of the anomaly.
l If fistula is suspected—the infant should be stopped oral intake, start only intravenous fluids.
l Positioning of baby to avoid aspiration.
l Suctioning of baby from the mouth and pharynx.
l Catheter is placed into the upper oesophageal pouch and the infant head is kept in an upright position, so that fluid collection in the pouch is easily removed.
l Gastrostomy is performed to decompress the stomach and prevent future aspiration of gastric contents by way of the fistula.
l Antibiotics therapy is administered.
l Surgical correction consists of a thoracotomy with division and ligation of the tracheophageal fistula and end to end anastomosis.
l For preterm babies with multiple anomalies and if condition is poor then it is preferred to involve palliative surgery like gastrostomy ligation of the oesophageal pouch.
l Oesophageal replacement is usually done when child is closed and the child is fed directly by gastrostomy, whereas the upper oesophageal segment is drained by means of a cervical esophageostomy.
Nursing Management
l Detection of malformation immediately after birth.
l Suction to be done to prevent aspiration.
l If there is cyanosis and excessive mucus secretion immediately inform the physician.
l Place the infant under a radiant warmer.
l O2 is administered to help relieve respiratory distress.
l Observe the cyanosis—due to laryngospasm caused by overflow of saliva into the larynx.
l Positive pressure is contraindicated.
Preoperative Nursing Care
1. Maintain patent airway and lung expansion
m Remove accumulated secretion from oropharynx.
m Position for patent airway and prevent aspiration.
m Observe for signs of respiratory distress.
2. Recognize the defect early
m Be alert to danger signs.
m Excess salivation.
m Three Cs—coughing, choking and cyanosis.
m Acute gastric distension.
3. Prevent aspiration pneumonia
m Nothing Per Oral (NPO).
m Positioning the infant in upright position.
m Aspirate secretion through NG tube.
Postoperative Nursing Care 1. Maintain vital signs
m Pulse rate and respiratory rate to be checked every 15 minutes.
m Any alteration in vital signs, inform to physician.
2. Prevent infection at operative sites
m Observe the signs of inflammation, bleeding and other drainage.
m Clean dressing to be done.
3. Maintain fluid and electrolyte balance
m Record intake output chart.
m Maintain the IV fluid carefully.
m Measure and record gastrostomy drainage.
m Record weight daily.
4. Facilitate ventilation
m Maintain patent airway.
m Suction the secretions as needed.
m Maintain care of chest tubes and drainage apparatus.
m Position for optimum ventilation.
m Administer O2 as needed.
m Prevent aspiration of feedings.
5. Control pneumonia
m Administer antibiotics.
m Administer O2.
m Suction out the secretion.
m Position for optimum ventilation.
6. Administer comfort measures
m Provide tactile stimulation.
m Position comfortably.
m Avoid restraints.
m Administer mouth care.
m Offer pacifier frequently.
7. Support parents (Psychological)
m Encourage frequent visiting to ICU.
m Encourage participation in infant’s care.
m Encourage expression of feelings.
8. Educate parents in home care
m Teach parents about positioning.
m Signs of respiratory distress.
m Care of gastrostomy and esophagostomy.
m Care of operative site anatomies, dressing change etc.
9. Administer oral feedings
m Introduce foods at a time.
m Begin with slightly liquid feedings and progress to more solid food.
HYPERTROPIC PYLORIC STENOSIS
Pyloric stenosis is the narrowing of part of the stomach (the pylorus) that leads into the small intestine. This narrowing occurs because the muscle around the pylorus has grown too large. Most babies with pyloric stenosis begin to vomit during the second to third week of life. Babies begin with “spitting up” that turns into forceful projectile vomiting.
Incidence: It is common in males than females
m Incidence: 5 in every 1000 males and 1 in every 1000 females.
Etiology
l Unknown
l Predisposing factor—Hereditary
l Monozygotic to dizygotic twins.
Pathophysiology
The circular muscle of the pylorus is grossly enlarged as a result of both hypertrophy and hyperplasia. These produces severe narrowing of the pyloric canal between the stomach and duodenum consequently the lumen at this point is partially obstructed, over a period of time inflammation and edema further reduce the size of the opening until the partial obstruction may progress to complete obstruction. The distal portions end abruptly and are externally distinct and easily palpated. The stomach is usually dilated.
(a) Diagram of normal stomach and pylorus. Note the cross-section showing normal pyloric opening
(b) Diagram of stomach with pyloric stenosis. Note the cross-section showing how the pyloric opening is narrowed
(c) Diagram of stomach after repair of pyloric stenosis.
Note (in the cross-section) how an incision has been made in the muscle, enlarging the pylorus and reliev- ing the obstruction
Fig. 4.5: Pyloric stenosis Clinical Manifestations
Infants—1st week of life
l Initially there is only regurgitation or occasional non-projectile vomiting.
l 4 weeks after birth—Vomiting becomes projectile in nature then it may lead to complete obstruction by 4 to 6 weeks.
l Infant vomits may be ejected 3 to 4 feet from the child in a side lying position.
l Infant vomits 1 foot or more when the infant is lying on the back.
l Vomiting occurs most often shortly after a feed or several hours later.