Congenital Anomaly 2011 2012

8/3/2019 Congenital Anomaly 2011 2012

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General Objective

By the end of the class the student will beable to identify different congenital

anomalies that are present at birth


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Specific Objectives:

By the end of this lecture, the student should be ableto:

Identify the surgery related differences between

adult and young children.

Discuss etiology, assessment criteria and therapeutic

management of gastrointestinal, respiratory,

skeletal and orthopedic anomalies.

Use the nursing diagnosis to develop nursing careplans for children with gastrointestinal, respiratory,

skeletal and orthopedic anomalies


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Causes of Fetal Malformation

DrugRadiation

VirusesGenetic Traits


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Surgery Related Differences between Young

Children and Adults

The metabolic rate of the infant and

young children is much greater

proportionately than that of adult.

The body tissues of the child heal

quickly because of his rapid rate of

metabolism and growth.


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The child usually needs proportionately less

analgesic than adult patient to obtain relative

comfort after surgical procedures.

The child lacks the reserve physical resources

that are available to the adult. His general

condition may change very rapidly , almost

without warning.

Abnormal fluid loss is more serious in the infant

and young child than in the adult. Fluid intake

and output must be calculated very carefully.


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Some newborns with birthdefects are critically ill.They may need to betransported to medical

centers or specializedpediatric hospitals.

General Aspects of

Pre & Post-Operative Pediatric Care


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Thus if acutely ill infant is to be transferred,

provision should be made to ensurethat the trip will be safe and thatthere will be no appreciable

deterioration of the infants condition


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Safe means of transportation with aheated portable incubator andavailable oxygen supply is needed so asto maintain the infants bodytemperature and O2 therapies in theblood.

Equipment for suctioning to removesecretions is needed (as in esophagealatresia).


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A nurse should participate in the

transfer of the baby to observe himduring that time and give appropriatecare in emergency (e.g. preventtorsion of an omphalocele, proper

position of infant).

All pertinent infant informationshould accompany the infant as hegoes from one health agency toanother


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Pre-operativecare


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Psychological preparation of the child(according to his age). This aimed toprevention of fears common to children likefear of separations, fear of death.

The nurse should explain to the child andhis families what is going to occur.

Except in emergency situations, childrenshould preferably be free of respiratorycomplications and signs of malnutrition.


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Most children must have nothing by mouthbefore surgery but the length of the time

the child remains NPO will depend on age(for newborns and infants 3-4 hours isusually enough).

The incision over or the part involved in

surgery must be washed and inspected.Shaving may be needed.The mouth should be checked for loose

teeth or for dentures (particularly in

children of 6-8 years of age). Any loose ormissing teeth should be charted in thechilds record.


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Remove barrettes and pins from the childs

hair.Clothing should be warm and loose. The

child should be dressed in a hospital gownand under pants only.

Check the childs identification band to seethat is legible and secure. If not it needs tobe replaced prior to surgery.

Pre-medication: sedatives and analgesicsare usually given two hours before surgeryexcept in emergency situation.
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The nurse should chart whether the child

has passed urine and had a bowel movement.Enemas are never done routinely but may beordered in some conditions.

Prior to taking the child to surgery,

especially in newborns and infants, nostrilsshould be carefully cleansed (hard crustsmay be softened with a solution of sodiumbicarbonate, normal saline, or even warm

water). This is necessary to because crustsmay be obstructing the airway.


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The child may need to take his favoritetoy with him to surgery. Ideally, heshould be allowed to keep the toy withhim until he is under the anesthetic.

Parents should be allowed to

accompany their children to theoperation site if they so desire.

Parents should be told where to wait

during the surgery, whether the child willgo to recovery room after surgery ordirectory to his unit.


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After return from the operating room, thechilds general condition must be closely

observed

Vital signs, especially temperature.

Airway must be kept patent newbornbabies must be kept in warm cot orincubator.


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Unit the child is responsive andalert; he should be kept on his side

(for secretion and vomitus to getout from mouth).

Observe conditions and placementof dressing. Check and mark anyapparent drainage from wound.

Intravenous fluids should bechecked for correct rate of flowand for possible infiltration.


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The child should be carefully handled andshould be protected from harming himself by

use of appropriate restraints.Any urinary catheter should be connected to

drainage bag and stabilized properly to bed.

Observe patients skin color and temperatureas well as any sings of shock :

(a) low blood pressure

(b) rapid pulse

(c) cold moist pale or cyanotic skin

(d) dilated pupils

(e) restlessness.


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Oral fluids may be started often after thefollowing criteria are observed (a) color ofaspirate is clear (b) peristaltic movements

are heard (c) flatus or gases are passed. Oral fluids should be started while infusion

still on. If well tolerated then infusion isgradually discontinued.

Routine postoperative diet is modifiedaccording to childs age, but in general itchanges from clear of liquid, full liquid, softand then regular diet.

Sedatives are used according to prescribedorders and childs needs.


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For children who can walk, early progressiveambulation is the rule (expect in few cases),

this will help to restore GIT function andprevent complications of pneumonia, thethrombosis, and pressure areas.

If too young to go out of bed, the nurseshould turn the child frequently and give himgood skin care and help him to breath deeplyat intervals.


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It is defined as a structuraldefect present at birth.

Some anomalies arecompatible with life andtreatment could be delayed,

while others are incompatiblewith life and must berepaired immediately


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Gastrointestinal Anomalies


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Anomalies of the Mouth

It is a congenital fissure in the upper lip. It is

generally located at one side or the other of

the midline in the center of the nares. It maybe unilateral or bilateral
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It is incomplete fusion of the palatal shelves.The opening may involve only the soft palate

or the hard palate or both.

It may be a single midline cleft or twobilateral clefts.Cleft palate may occur in connection with

cleft lipCause:Both cleft lip and palate result from failure of

or incomplete union of embryonic structuresof the face.


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Clinical Manifestations

Observable defects.


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Complications associated with cleft lipand cleft palate

Feeding difficulties

Ear infections and hearing loss

Speech and language delayDental problems


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Feeding baby with cleft palate

Breast-feeding is allowed. It will take extra timeand patience.

Hold an infant in an upright position to help

keep the food from coming out of the nose. Small, frequent feedings are recommended.

There are many types of bottles and nipples that

can assist with feeding an infant with cleftpalate.


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NUK nipple

This nipple can be placed on regular bottles oron bottles with disposable bags. The hole canbe made larger by making a criss-cross cut inthe middle.


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Mead Johnson Nurser

This is a soft, plastic bottle that is easy tosqueeze and has a large crosscut nipple.


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Haberman Feeder

This is a specially designed bottle system with avalve to help control the air the baby drinks andto prevent milk from going back into the bottle.


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Neonatal RespiratoryDistress

Congenital

Diaphragmatic Hernia

Esophageal Atresia with

Tracheoesophageal

Fistula


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Esophageal Atresia and

Tracheoesophageal Fistula: (T.E.F.)

It is congenitalmalformation representa failure of the

esophagus to developa continuous passage.

These defects may

occur separately or incombination.


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Definition Oesophageal atresia is defined: as an

interruption in the continuity of the

esophagus with or without fistula to thetrachea.

Tracheoesophageal Fistulais definedas abnormal fistulous communicationbetween the trachea and the esophagus

with Atresia of the esophagus singly or incombination.

Etiology:


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Etiology:

Unknown.


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Types: Oesophageal

Atresia with a distaltracheo-oesophageal fistula

OesophagealAtresia with aproximal fistula


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Oesophageal Atresia: (cont)

Oesophageal atresiacombined with fistulaefrom both

oesophagealsegments

Isolated Oesophageal

Atresia without afistula


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V = Vertebral anomalies

A = Anal anomalies

TE = Tracheoesophageal Fistula

R = Renal deformaties


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Three Cs of TEF on feeding:

Coughing.

Choking.

Cyanosis.

and, regurgitation


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Excessive salivation and droolingApnea.

Increased respiratory distress following

feeding.Abdominal distentionmight occur if

large fistula as a result of gasaccumulation.


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Regurgitation of saliva and milk fromthe obstructed upper pouch into the

trachea.Reflux of acidic content through the

fistula into the lung leading to

pneumoniatis.Gastric distention by air from trachea

leading to diaphragmatic elevation.


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Insertion of catheter gently into theesophagus meets with resistance if the

lumen is blocked but passes unobstructed ifthe lumen is patent.

Radiographic visualization of opaque

catheter inserted into the hypopharynxhelps to determine patency and / oralternate channels.

Endoscopy for isolated fistula.


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Pre-operative Care:

No oral feeding.

Give Intravenous (I .V) fluids. Keep infant/ child warm, by placing

him in incubator or use warmer.


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Give humidified oxygen for respiratorydistress.

Put infant/ child in sitting or semi-sitting

position.

Make suction for mouth, pharynx and endof pouch.

P t ti C


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Post-operative Care:

Place infant/ child in humidified air andwarm him.

Maintain I. V. fluids.

Suctioning whenever needed, if he can notswallow saliva.

Observe respiration, report immediately ifthere is respiratory difficulties are presentor atelectasis.

If there is gastrostomy tube feeding it


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If there is gastrostomy tube feeding, itmust not be elevated more than 4 inches(10 cm) above stomach level to preventreflux to healed esophagus.

Use pacifier to satisfy infants need for

sucking.

Start oral feeding gradually whenesophagus anastomosis has healed, butcontinue gastrostomy feeding until theinfant takes adequate amount per mouth.

Teach parent before infants discharge

how to care for him at home.

3 A li


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3.Anomalies

of the

Stomach

andDuodenum


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Hypertrophic PyloricStenosis


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Is hypertrophy of the muscle surrounding the

pylorus that leads to narrowing of the pyloric

canal and makes it difficult for the stomach to

empty. It occurs mainly in male infants.


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It is a protrusion of the stomach through theesophageal hiatus in the diaphragm (thehiatus is abnormally wide) forceful vomitingbegins between one week and one month of

age and may contain blood. The vomitingcauses anemia, weight loss, dehydration andmalnutrition if unchecked.


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It occurs when there ishypertrophy of thecircular muscle of thepylorus leads toconstriction of thepyloric canal andobstruction of the gastricoutlet


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The circular muscle of the pylorus isgrossly enlarged as a result of bothhypertrophy (increased size) andhyperplasia (increased mass).


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This produces severe narrowing ofthe pyloric canal between the

stomach and the duodenum.Consequently, the lumen at thispoint is partially obstructed over aperiod of time, inflammation andedema further reduce the size ofthe opening until the partialobstruction may progress to

complete obstruction.


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Vomiting: usually start in 2nd to 3rd weekof life. And becomes forceful andprojectile.

Hungry and irritable later becomelethargic.

Dehydration.

Malnutrition.Constipation.

Loss of skin turgor.


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Palpable olive like mass in right upper quadrant.

Visible, peristaltic waves usually form the left to

right.

Radiographic or ultrasound revealed

hypertrophied pyloric muscle.

Narrow pyloric sphincter revealed in barium

swallow. (Positive string sign).


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Surgical relief of the

pyloric obstruction by

Pyloromyotomy


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Observe and record:

Vital signs especially that indicateselectrolyte imbalance.

Vomitus for color and amount. Stool.

Sucking fingers which indicates

hunger. Collect specimens.

A i t i di ti t t


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Assist in diagnostic tests, e.g.,blood for sodium, potassium, and

bicarbonate. With hold oral feedings and give

and monitor I.V fluids, as

prescribed. Lavaging the stomach with

isotonic solution as ordered.

Educate parents about the diseaseand its care. Provide emotionalsupport to parents.


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If doctor order to feed the infant,

feed him in semi uprightposition, feed slowly and bubblesfrequently to prevent vomiting.

Handle the infant as little aspossible after feeding.

Warm the infant/ child.

Prevent infection. Change position frequently.


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Provide mouth and skincare.

Weight infant daily toassess dehydration and

malnutrition. Intake and output chart.

Stimulate the infant. Use pacifier to meet infants

need for suckling.


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Adequate fluid and nutrition: Give I.Vuntil breast or formula feeding can betolerated. Also glucose in water or

electrolyte solution are givenfrequently in small amount andincrease it gradually.

Observe for signs of complications:Sings of inflammation, shock andabdominal distention.


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Parent education about thecare of infant, especiallyposition at feeding andhow tokeep incision clean.

Teach parent about the

importance of follow- up care.


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Anomalies

of theIntestine


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It is incomplete

development orabsence of the anus in

its normal position inthe perineum.


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It is an interruption in the continuity of theintestine which may take the form of a

septum in the lumen of the intestine,stenosis, complete atresia of varying lengths,separated blind ends of bowel, or multipleatresias. It may occur at any point in either

small or large intestine.


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Vary according to:

Age when symptoms are recognized.

The length of the affected bowel. Occurrence of complication, i.e.,

entrocolitis.


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In the newborn:

The primary signs and symptoms are:

Failure to pass meconium within 24 48hrs after birth.

Food refusal.

Vomiting.

Abdominal distention.

During infancy:


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g y

Inadequate weight gain. Constipation.


Explosive watery diarrhea. Episodes of diarrhea and vomiting.

Bloody diarrhea, fever and severe lethargyare ominous signs because they signify the

presence of enterocolitis

During childhood:


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Constipation. Passage of ribbon like, foul smelling stool.


Fecal impaction recurs frequently. Poor appetite, poor growth


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In neonate: diagnosis is usually based onclinical signs of intestinal obstruction and

failure to pass meconium. On examination, the rectum is empty of

feces, the internal sphincter is tight andleakage of liquid stool.

Barium enema and rectal biopsy. Non-invasive procedure may be used asanorectal manometry.


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Therapeutic Management:

Treatment is primarily surgical removal ofAganglionic portions of the bowel inorder to permit normal bowel motilityand establish continence by improvedfunctioning of the internal analsphincter.


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It is incomplete development orabsence of the anus in its normalposition in the perineum


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Narrowing of the anorectal canal thatmay occur at any point or extendedits entire length.


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The rectum ends in a blind pouch below(Low) or above (High) the levator animuscles.


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A thin membrane persistsacross the anal opening.

The anus is normal but the rectal canal isnot continuous. This type is very rare

A t C it i


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Assessment Criteria:

Failure to pass meconium stool in the first24 hours after birth.

Absence or stenosis of the anorectalcanal.

Anal membrane.

External fistula to the perineum.

Diagnostic Procedures:


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Diagnostic Procedures:

During the initial assessment of thenewborn perineum, which should includedetermination of the rectum patency with

small finger or rectal tube if a lesion issuspected.

X- ray examination.

Ultrasound. Computed tomography scan.


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Acquired Intestinal Defect


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qIntussusceptions

It is an acquired type of intestinalobstruction. It is the invaginationor telescoping of a segment ofintestine into the segmentimmediately distal to it.


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Intussusception is one of the mostfrequent causes of intestinal obstructions

in children between the ages of 3months and 5 years.

Half of the cases occur in children

younger than 1 year, and most of theothers occur in children during the secondyear.


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Exact cause of Intussusception is uncertain but there areseveral theories as:

Greater disparity between the size of the ileum andileocecal value in infants is believed to encouragetelescoping at this point.

Distorted peristalsis due to change of diet (weaning).

Enlarged lymph nodes along the G.I.T. that occur withrespiratory infections, cystic fibrosis, foreign bodies, G.Ipolyps.

Hyperperistalsis.Diarrhea, constipation.

Mobile ileocecal junction.


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Ileocecal value. (Ileocolic)

Ileoileal.

Colocolic.


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Sudden acute abdominal pain, whichbecomes progressively more severe.Child screams and draws the knees

into the chest.Child is healthy and normal

between episodes of pain.

Vomiting. Lethargy.

Passage of red current jelly stool. (Stoolmixed with blood and mucus)


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mixed with blood and mucus).

Palpable sausage- shaped mass in upperright quadrant.

Empty right lower quadrant (dance sign). Anorexia, discomfort.

Tender, distended abdomen.

Eventually fever, perforation, peritonitisand shock are serious complications ofIntussusception.



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The initial treatment of choice is non-surgical hydrostatic reduction.

Traditionally by barium enema:

In this procedure, correction of theinvagination is carried out at the sametime as the diagnostic testing.

The force exerted by the flowing barium isusually sufficient to push the invaginatedportion of the bowel into its originalposition.


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Abdominal Wall Defects


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It is herniation of intestines into theumbilical cord, i.e., a protrusion, at birth,

of part of intestine through a defect in theabdominal wall at the umbilicus.

Omphalocele
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p

There is a sac that covers the defect,which composed of transparent

avascular membrane from which theumbilical cord extends.


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It is a congenital defect that differs fromomphalocele in that it is fissure of theabdominal wall to the right of theumbilicus and does not involve the site ofthe insertion of the umbilical cord.

Protrusion of the small intestine and part of
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Protrusion of the small intestine and part oflarge intestine usually accompany it.There is no sac covering the intestine,therefore, the bowel is irritated byamniotic fluid during gestation andbecomes edematous and inflamed.

Umbilical Hernia


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It is protrusion of the omentum and small intestinethrough the imperfect closure or weak umbilicalring, especially when infant cries.

Inguinal Hernia


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It is protrusion of abdominal contents through theinguinal canal into scrotum.

Nursing Management:
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Pre-operative Care:

For cases of gastrochisis, omphalocele, umbilical andinguinal hernias.

Do not apply pressure on herniated area.

Nasogastric suctioning for omphalocele / gastrochisis.

Apply sterile dressing over herniated area foromphalocele/ gastrochisis.

Keep the child quiet, no crying in particular in cases ofharnias, especially umbilical hernia. Give warm bath,suck pacifier to calm the infant.

Educate parent about strape the defect has no valueto decrease hernia.

Post- operative Care:


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Post- operative Care:

Routine post-operative care, in addition to: For omphalocele and gastrochisis, the infant is fed by

peripheral Hyperalimentation Omphalocele infant then fed after that through

gastrostomy (increase amount of glucose water, then

milk).For all cases : Observe are of defect for signs of infection. Maintain clear airway. Change position to prevent atelectasis. Emotional support for parents, especially in case of

omphalocele where the operation is done in steps.


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Defect of

RespiratorySystem


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It is defined as a congenital obstruction of the posteriornares at the entrance to the naso-pharynx.

It may be unilateral or bilateral

Etiology:
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The obstruction is usually caused by a

membrane, but in rare cases by a boneygrowth.

Assessment

In bilateral obstruction there is mouthbreathing and difficulty in taking feedings.There may be dyspnea because the infantcant obtain enough oxygen.


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Treatment


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Treatment

It should be treated as early as possible

since it is one cause of asphyxia of thenewborn.

If the obstruction is unilateral, the infant

may do well unless infection occurs andpersist on the side opposite theobstruction.

2 Diaphragmatic Hernia


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2. Diaphragmatic Hernia

It is a protrusion of the abdominal viscera, mainly theintestine, through a defect in the diaphragm into thechest cavity. It usually occurs in the left side of the chest.The infant has large chest in comparison with his small

abdomen.

Assessment
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Assessment

The infant usually develops respiratorydistress and possibly cyanosis.

Treatment

Surgery. If operation is not doneimmediately, the prognosis is very poorand the patient will probably die within thefirst month of life.


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Spina Bifida:


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It is a defective closure of the vertebral

column. Whether the defect will havedevastating consequences for theneonates depends entirely on the siteand the extent of the anomaly.

Spina Bifida Occulta


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Usually the 5th lumber and 1st sacralvertebrae are affected with no protrusionof interspinal contents the spinal cordand its cover the skin over the defectmay reveal a dimple, small fatty mass, ora tuft of hair. The defect is only in thevertebra, the spinal cord and meneiges is

normal and not visible.

Meningocle


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It is a protrusion through the spinal bifida,which forms a soft, saclike appearance alongthe spinal axis and contains spinal fluid andmeninges within the sac and covered with skin.

Meningomyelocele
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It is a more serious defect in which thespinal cord and /or nerve roots as well asmeningoel covering protrude through the

spina bifida. The degree and extent ofneurogenice defect depend on the levelof the defect. The higher the level of the

defect, the greater the defect.

If in the lumbosacral
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If in the lumbosacralarea, the defect is

usually associatedwith a flaccidparalysis of the lowerextremities, absent

sensation to the levelof the lesion, and lossof bowel and bladdercontrol.

Hydrocephalus
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The abnormal increase in cerebrospinal fluid volumewithin the intracranial cavity due to a defect in thecerebrospinal fluid drainage system, intracranialpressure increases, the scalp veins dilate, and the

cranial structure begin to separate

Nursing Management of Meningocele andM i l l
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Meningomyelocele

Nursing Diagnosis: High risk for infection.

High risk for injury.

High risk for skin impaired integrity.

Altered nutrition: Less than bodyrequirements
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Epispadias

Mieatual opening located on dorsal or


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Mieatual opening located on dorsal orsuperior surface of the penis.

Hypospedias

Urethral opening located behind glandspenis or anywhere along ventral (lower)

surface of penile shaft.N .B.: infants with epispadias and

hypospadias should not be circumcised

before repair of the defect because thesurgeon may wish to use apportion ofthe foreskin for plastic repair.

3-Phimosis

Narrowing or stenosis of
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Narrowing or stenosis ofpreputial opening of

foreskin. In Severecases, circumcision orvertical division andtransverse, suturing of

foreskin.4-Hydrocele

Fluid in scrotum.Therapeutic management

is surgical repairindicated if spontaneousresolution notaccomplished in 1 year.

5-Polycystic Kidney:
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The infant has enlarged kidneys filled withcysts at birth. If the condition is bilateralthe infant will not pass urine but if it isunilateral the condition may be missed untillater in life.

6-Wilms Tumor (Ebryoma)

l f h k d h
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It is a malignant tumor of the kidney that arisesfrom an embryonic structure present in the childbefore birth; the tumor is felt as an abdominalmass. It is important that the necessary fordiagnosis because handling appears to increasethe danger of metastasis.
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Cl bf
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Clubfoot

Flexion at the ankle with inversion ofthe heel and fore foot.

Torticollis

It is a condition in which there is a lateralinclination and a rotation of the headaway from the midline of the body with

limitation of the range of motion of theneck.

Congenital Dislocation of the Hip
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In this condition the femur head is

completely dislocated from theacetabulum. The infant shows limitedability to abduct the hip, asymmetry of

the gluteal skin folds and inguinalcreases, and shortening of the affectedleg
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Before cast application Child should be allowed to play with a doll that has a

cast so that he understands what will be done.

The nurse must set up the cast materials and hold theextremity in alignment.

Before the cast is applied, the extremities are checkedfor any abrasions, cuts, or other alterations in skinsurface and other items that might cause constrictionfrom swelling; such objects are removed.

Identification bands are placed on a non-injuredextremity if hospitalization is anticipated.

After cast application

The cast must remain uncovered to allow is


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The cast must remain uncovered to allow isdryness from inside out.

After the cast is applied, the extremities arechecked for any sings of compromise.

Observations such as pain, swelling,

discoloration (pallor or cyanosis) of theexposes portions, lack of pulsation andwarmth, or the inability to move the exposedparts are reported immediately.

Turning the child in a plaster cast at least
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Turning the child in a plaster cast at leastevery 2 hours to prevent complications fromimmobility.

The body part can be elevated, therebyincreasing venous return.

Periodically the circumscribed blood stainedarea should be outlined with a ball point pento assess the amount of blood.

Parents need instructions on drying andcaring for the cast and checking for signsthat indicate the cast is too tight.


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Congenital Anomaly 2011 2012

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