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Congenital Anomalies


  • pg 1
									                       Congenital Anomalies

General Objective:
    By the end of the class the student will be able to identify different congenital
anomalies that are present at birth.

Specific Objectives:
     At the end of the class student should be able to:
   1. List the possible causes of fetal malformations.
   2. Define some common congenital anomalies in the newborn infant.
   3. Describe some of the surgery related differences between infants and adults.
   4. Outline important aspects in the pre and post operative care of pediatric patients.
   5. Discuss specific pre and post operative care of a child with the following: cleft lip,
      cleft palate, esophageal atresia and pyloric stenosis.
      The desired and expected outcome of every wanted pregnancy is a normal,
functioning infant with a good intellectual potential. Fulfillment of this hope depends on
numerous hereditary and environmental factors. Congenital anomalies are evident in 2%
to 3 % of all live births, but this number increases to 6% by five years of age when more
anomalies are diagnosed.

Note:    A disease or disorder that is transmitted from generation is termed genetic or
hereditary. A congenital disorder is one that present at birth and can be caused by genetic
or environmental factors or both. Some congenital disorder such as inborn errors of
metabolism and mental retardation are not malformations.

Causes of Fetal Malformation:
      Several factors are known to produces malformations of the developing fetus. These
are environmental factors like use of drugs, radiation, viruses, genetic traits in one or both
parents, and as interaction of the environmental and hereditary influences. Nurses are
mainly concerned with the environmental influences since they are controllable in many
instances so that birth defects can be prevented.
      1. Drugs:
     Drugs in general carry such a potential for the production of birth defects that their
use during pregnancy is strongly discouraged. Use of street drugs like heroin or hashish
by pregnant women could be responsible for soon congenital anomalies. Alcohol also
appears to influence fetal development and its excessive use during pregnancy can
produce fetal malformations.
      2. Radiation:
      Exposure of the mother to radiation has been shown to cause birth defects, the
earlier in pregnancy the exposure, the more extensive is the fetal damage. Exposure at
later gestational stages may result in less apparent deviations. Atmospheric or
environmental radio contamination also contributes to the risk of fetal deviations (as in
nuclear accidents or testing of nuclear weapons).
        3. Viruses:
     Like rubella virus (that causes German measles). Whether infection of the mother
during pregnancy procedures deformities in the fetus depends upon the nature of the
infectious organism, the development stage of the fetus at the time it is exposed to the
organism; other factors such as the mother’s antibody titer and other variables not yet
      4. Genetic Traits:
       Probably all human characteristics have a genetic component, including those that
produce symptoms or physical abnormalities that impair the fitness of the person. Some
disorder or disease occurs through the influence of a single gene or combined action of
many genes inherited from the parents; others result from the action of the intrauterine
environment that affects the embryonic development.
Note: contained within the nucleus of every somatic cell in the human body are more
than 200.000 genes, the genetic material responsible for programming the body’s
physiologic process and characteristics. These genes are composed of segments of DNA
(deoxyribonucleic acid) and are organized into structure called chromosomes, which are
visible only during certain stage of cell division. Alteration of a whole chromosome, a part
of a chromosome or even a single gene can manifest a genetic disorder.
Common Congenital Anomalies in the Newborn:
       An anomaly is a structural defect present at birth. Some defects may be compatible
with life but need treatment that could be delayed. Others are incompatible with life and
must be repaired immediately.
Common anomalies, of the different systems are as follows:

    Respiratory System:

         1. Laryngeal Stridor:           Noisy respiration often with a crowing sound on
         inspiration, most noticeable when the infant cries. It is due to some abnormal
         condition in or around the larynx. The infant may become cyanotic and dyspnic.
         The infant’s sternum may be retracted on inspiration because of his increased
         efforts to pull air through the trachea. Most infants with this condition stop
         sucking frequently during a feeding to maintain adequate ventilation. Symptoms
         gradually disappear by one year of age.

         2. Choanal Atresia: Is a congenital obstruction of the posterior nares at
         the entrance to the naso-pharynx. The obstruction is usually caused by a
         membrane, but in rare cases by a boney growth. It may be unilateral or bilateral.
         In bilateral obstruction there is mouth breathing and difficulty in taking feedings.
         There may be dyspnea because the infant can’t obtain enough oxygen. It should
         be treated as early as possible since it is one cause of asphyxia of the newborn.
         If the obstruction is unilateral, the infant may do well unless infection occurs and
         persist on the side opposite the obstruction.

    Gastrointestinal System:

        1. Anomalies of the Mouth:
        1. Cleft Lip: 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 may be unilateral
        or bilateral.
         2. Cleft Palate: is incomplete fusion of the palatal shelves. The opening may
        involve only the soft palate or the hard palate or both of them. It may be a single
        midline cleft or two bilateral clefts. Cleft palate may occur in connection with cleft
        2. Anomalies of the Esophagus:
        1. Esophageal Atresia: There are numerous              anatomical variations of this
        malformation. The form of highest incidence is that in which the proximal part of
        the esophagus ends in a closed pouch and the distal part communicates, with the
        trachea at its bifurcation.
         2. Chalasia of the Esophagus: Is a neuromuscular disorder in which the
        cardiac sphincter and the lower portion of the esophagus are lax (constantly relax
        and patent). It allows easy regurgitation of gastric contents into the esophagus.
        Vomiting occurs immediately after feeding, which is nonprojectile and may result
        in aspiration pneumonia or esophageal stricture, it starts within a week after birth.
3.Anomalies of the Stomach and Duodenum:
    1. Pyloric Stenosis: 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.
     2. Duodenal Obstruction: There may be complete obstruction
    (atresia) or incomplete obstruction (stenosis) of the duodenum.
     3. Hiatus Hernia: Is a protrusion of the stomach through the
    esophageal hiatus in the diaphragm (the hiatus is abnormally wide) forceful
    vomiting begins between one week and one month of age and may contain
    blood. The vomiting causes anemia, weight loss, dehydration and malnutrition
    if unchecked.
 4.Anomalies of the intestine:
    1. Imperforate Anus: There                are four types of imperforate anus:
       stenosis, membranous (imperforate anal membrane), agenesis (rectum
       has a blind end it may be high or low) the anal dimple may be present at
       the site where the anus should be, atrelic. A fistula between the rectum
       and the genitourinary tract may be present. The infant can’t pass stool and
       signs of intestinal obstruction result, mainly abdominal distension.
    2. Omphalocele: It is the protrusion of the abdominal cavity into the
       base of the umbilical cord or through a defect in the anterior abdomen.
       The protruded mass may contain only part of the intestine or almost all the
       intestine and other abdominal organs. The mass is usually covered with is
       usually covered with a transparent membrane.
    3. Intestinal Atresia: Is an interruption in the continuity of the
       intestine 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 multiple atresias. It may occur at any point in either
       small or large intestine.
    4. Diaphragmatic Hernia: Is a protrusion of the abdominal viscera,
       mainly the intestine, through a defect in the diaphragm in to the chest
       cavity. It usually occurs in the left side of the chest. The infant has large
       chest in comparison with his small abdomen. The infant usually develops
       respiratory distress and possibly cyanosis. If operation is not done
       immediately. The prognosis is very poor and the patient will probably die
       within the first month of life.
    5. Hirshsprung’s Disease: (Cong. Aganglionic Megacolon)
       There is congenital absence of parasympathetic ganglion nerve cells of a
       part of intestine usually in the distal end of the descending colon the
       affected portion has no peristalsis. It is common in males and females. It
       then hypertrophies with accumulation of feaces. Resection anastemosis is
       done as a treatment.
     6. Intussusception: It is an acquired from of intestinal obstruction. It
       is the invagination of a portion of the bowel into the portion immediately
       distal to it; thus the blood supply is out off and if the condition continuous,
       the bowel will become will become gangrenous. Death may result if the
       condition is not recognized and corrected. Sometimes a simple reduction
 Urinary System:
     1. Epispadias: Mieatual opening located on dorsal or superior surface
          of the penis.
     2. Hypospedias:              Urethral opening located behind glands penis 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 the surgeon may wish to use
          apportion of the foreskin for plastic repair.
     3.   Phimosis: Narrowing or stenosis of preputial opening of foreskin.
          Severe cases: circumcision or vertical division and trans verse, suturing
          of foreskin.
     4.   Hydrocele: Fluid in scrotum. Therapeutic management is surgical
          repair indicated if spontaneous resolution not accomplished in 1 year.
     5.   Inguinal Hernia: Protrusion of abdominal contents through inguinal
          canal into the scrotum. Therapeutic management includes: Detected as
          painless inguinal swelling of variable size surgical closure of inguinal
     6.   Polycystic Kidney: The infant has enlarged kidneys filled with
          cysts at birth. If the condition is bilateral the infant will not pass urine but if
          it is unilateral the condition may be missed until later in life.
     7.   Wilm’s Tumor (Ebryoma): It is a malignant tumor of the kidney
          that arises from an embryonic structure present in the child before birth;
          the tumor is felt as an abdominal mass. It is important that the necessary
          for diagnosis because handling appears to increase the danger of

 Skeletal Defects Affecting the Nervous System:
     1. Spina Bifida: Is a defective closure of the vertebral column. Whether
          the defect will have devastating consequences for the neonates depends
          entirely on the site and the extent of the anomaly. It is more common in
          the lumbo sacral region. It has varying degree of tissue protrusion through
          the bony cleft.
     2.     Spina Bifida Occulta: Usually the 5th lumber and 1st sacral
          vertebrae are affected with no protrusion of interspinal contents the spinal
          cord and its cover the skin over the defect may reveal a dimple, small fatty
          mass, or a tuft of hair. The defect is only in the vertebra, the spinal cord
          and meneiges is normal and not visible.
     3.   Meningocle: Is a protrusion through the spinal bifida, which forms a
          soft, saclike appearance along the spinal axis and contains spinal fluid
          and meninges within the sac and covered with skin.
     4.     Meningomyelocele: Is a more serious defect in which the spinal
          cord and /or nerve roots as well as meningoel covering protrude through
          the spina bifida. The degree and extent of neurogenice defect depend on
          the level of the defect. The higher the level the greater the defect. If in the
          lumbosacral area, the usual of the defect is associated with a flaccid
          paralysis of the lower extremities, absent sensation to the level of the
          lesion, and loss of bowel and bladder control.
     5. Hydrocephalus:              The abnormal increase in cerebrospinal fluid
          volume within the intracranial cavity due to a defect in the cerebrospinal
          fluid drainage system, intracranial pressure increases , the scalp veins
          dilate, and the cranial suture begin to separate.
 Orthopedic Anomalies:
    1. Clubfoot: Flexion at the ankle with inversion of the heel and fore foot.
    2. Torticollis: Is a condition in which there is a lateral inclination and       a
         rotation of the head away from the midline of the body with limitation of the
         range of motion of the neck.
    3.   Congenital Dislocation of the Hip: In this condition the femur
         head is completely dislocated from the acetabulum. The infant shows
         limited ability to abduct the hip, asymmetry of the gluteal skin folds and
         inguinal creases, and shortening of the affected leg.
Surgery Related Differences between Young Children
and Adults:
 1. The metabolic rate of the infant and young children is much greater proportionately
    than that of adult. Children are growing and need to be fed more frequently.
 2. The body tissues of the child heal quickly because of his rapid rate of metabolism
    and growth.
 3. The child usually needs proportionately less analgesic than adult patient to obtain
    relative comfort after surgical procedures.
 4. The child lacks the reserve physical resources that are available to the adult. His
    general condition may change very rapidly , almost without warning.
 5. 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.
General Aspects of Pre and Post-Operative Pediatric
      Some newborns with birth defects are critically ill. They may need to be transported
to medical centers or specialized pediatric hospitals. Thus if acutely ill infant is to be
transferred, provision should be made to ensure that the trip will be safe and that there will
be no appreciable deterioration of the infant’s condition.
    Transportation of the Newborn:
   1. Safe means of transportation with a heated portable incubator and available oxygen
      supply is needed so as to maintain the infant’s body temperature and 0 2 therapy in
      the blood.
   2. Equipment for suctioning to remove secretions is needed (as in esophageal
   3. A nurse should participate in the transfer of the baby to observe him during that
      time and give appropriate care in emergency (e.g. prevent torsion of an
      amphalocele, proper position of infant).
   4. All pertinent infant information should accompany the infant as he goes from one
      health agency to another.
    Pre-Operative Care:
   1. Psychological preparation of the child (according to his age) this is aimed at
       prevention fears common to children like fear of separations, fear of death. The
       nurse should explain to the child and his families what is going to occur.
   2. Except in emergency situations, children should preferably be free of respiratory
       complications and signs of malnutrition.
   3. Most children must have nothing by mouth before surgery but the length of the time
       the child remains NPO will depend on age (for newborns and infants 3-4 hours is
       usually enough).
   4. The incision over or the part involved in surgery must be washed and inspected.
       Shaving may be needed.
   5. The mouth should be checked for loose teeth or for dentures (particularly in children
       of 6-8 years of age). Any loose or missing teeth should be charted in the child’s
   6. Remove barrettes and pins from the child’s hair.
   7. Clothing should be warm and loose. The child should be dressed in a hospital gown
       and under pants only.
   8. Check the child’s identification band to see that is legible and secure. If not it needs
       to be replaced prior to surgery.
   9. Pre-medication: sedatives and analgesics are usually given two hours before
       surgery except in emergency situation.
   10. The nurse should chart whether the child has passed urine and had a bowel
       movement. Enemas are never done routinely but may be ordered in some
   11. Prior to taking the child to surgery, especially in newborns and infants, nostrils
       should be carefully cleansed (hard crusts may be softened with a solution of sodium
       bicarbonate, normal saline, or even warm water). This is necessary to because
       crusts may be obstructing the airway.
   12. The child may need to take his favorite toy with him to surgery. Ideally, he should be
       allowed to keep the toy with him until he is under the anesthetic.
   13. Parents should be allowed to accompany their children to the operation site if they
       so desire.
14. Parents should be told where to wait during the surgery, whether the child will go to
    recovery room after surgery or directory to his unit.
 Post-Operative Care:
1. After return from the operating room, the child’s general condition must be closely
    observed (a) Vital signs, especially temperature. (b) Airway must be kept patent
    newborn babies must be kept in warm cot or incubator.
2. Unit the child is responsive and alert; he should be kept on his side (for secretion
    and vomitus to get out from mouth).
3. Observe conditions and placement of dressing. Check and mark any apparent
    drainage from wound.
4. Intravenous fluids should be checked for correct rate of flow and for possible
5. The child should be carefully handled and should be protected from harming himself
    by use of appropriate restraints.
6. Any urinary catheter should be connected to drainage bag and stabilized properly to
7. Observe patient’s skin color and temperature as 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.
8. Oral fluids may be started often after the following criteria are observed (a) color of
    aspirate 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 is
    gradually discontinued. Routine postoperative diet is modified according to child’s
    age, but in general it changes from clear of liquid, full liquid, soft and then regular
9. Sedatives are used according to prescribed orders and child’s needs.
10. For children who can walk, early progressive ambulation is the rule (expect in few
    cases), this will help to restore gastrointestinal function and prevent complications
    of pneumonia, the thrombosis, and pressure areas. If too young to go out of bed,
    the nurse should turn the child frequently and give him good skin care and help him
    to breath deeply at intervals.
                            Gastrointestinal system

    Anomalies of the mouth:
Cleft lip and cleft palate:
Definition: (return to page 3)
Clinical Manifestations:
      Observable defects.
Diagnostic Evaluation:
      Readily apparent by observation and palpation (cleft palate).
Objectives of Therapeutic Management:
          -   Close defects surgically at the appropriate age.
          -   Prevent the complications.
          -   Habilitate for optimum use of residual impairments.
          -   Facilitate normal growth and development of the child.
Nursing Care plan for Infant with Cleft Lip and /or Palate Repair:

Preoperative care:
Nursing Diagnosis:
      Altered nutrition less than body requirements related to difficulty in eating.
Nurse: Provide adequate nutritional intake.
Patient: Will receive optimum nutrition.
     Administer diet appropriate for age (specify).
     Modify feeding techniques to adjust to defect.
       -Hold the child in upright position.
       -Use special feeding appliances.
       -Bubble frequently.
     Assist mother with breast-feeding if this is mother’s preference.
Expected Outcome:
     Infant consumes an adequate amount of nutrients (specify the amount).
     Infant exhibits appropriate weight gain.

Nursing Diagnosis:
      High risk for altered parenting related to infant with a highly visible physical defect.
Nurse: facilitate family’s acceptance of infant.
Patient (family): will demonstrate acceptance.
     Allow expression of feelings.
     Convey attitude of acceptance of infant and family.
     Indicate by behavior that child is a valuable human being.
     Describe results of surgical correction of defect.
       -Use photographs of satisfactory results.
      Arrange meeting with other parents who have experiences a similar situation and
       coped successfully.

Expected Outcome:
   Family discusses feelings and concerns regarding child’s defect, its repair and
     future prospects.
   Family exhibits an attitude of acceptance of infant.

Postoperative Care:
Nursing Diagnosis:
      High risk for trauma related to surgical procedure, dysfunctional swallowing.
Goal 1:
Nurse: Prevent trauma to suture line.
Patient: will experience no trauma to operative site.
     Position on back or side (CL).
     Maintain lip protective device (CL).
     Use non-traumatic feeding techniques.
     Restrain arms to prevent access to operative site.
       -Use jacket restraints on older infant.
     Avoid placing objects in the mouth following cleft palate repair (suction catheter,
       tongue depressor, straw, pacifier, small spoon).
     Prevent vigorous and sustained crying.
     Cleanse suture line gently after feeding and as necessary in manner ordered by
       surgeon (CL).
     Teach cleansing and restraining procedures, especially when infant will be
       discharged before suture removal.
Expected Outcome:
      Operation site remains undamaged.
Goal 2:
Nurse: Prevent aspiration of secretions.
Patient: will exhibit no evidence of aspiration.
     Position to allow for drainage of mucus (partial side- lying position, semi-Fowler
Expected Outcome:
      Child manages secretions with out aspiration.

Nursing Diagnosis:
      Altered nutrition; less than body requirements related to physical defect, surgical
Goal 1:
Nurse: Provide adequate nutrition intake.
Patient: Will receive optimum nutrition.
     Administer diet appropriate for age.
     Involve family in determining best feeding methods.
     Modify feeding techniques to adjust to defect
           - Feed in sitting position.
           - Use special appliances.
           - Encourage frequent bubbling.
           - Assist with breast-feeding if method of choice.
     Teach feeding and suctioning techniques to family.
     Monitor IV fluids (if prescribed).
Expected Outcome:
     Infant consumes an adequate amount of nutrients (specify amount).
     Family demonstrates ability to carry out postoperative care.
Nursing Diagnosis:
      Pain related to surgical procedure.
Nurse: Relive discomfort.
Patient: Will experience optimum comfort level.
     Administer analgesic and / or sedatives as ordered.
     Remove restraints periodically while supervised.
     Provide cuddling and tactile stimulation.
     Involves parents in infant’s care.
     Apply developmental interventions appropriate for infant’s level and tolerance.
Expected Outcome:
      Infant appears comfortable and rests quietly.

Nursing Diagnosis:
      Altered family processes related to child with a physical defect.
Nurse: Support the family.
Patient: Will receive adequate support.
     Be available to family.
     Listen to family members singly or collectively.
     Allow for expression of feelings including feeling of guilt, helplessness and their
       perception of the condition may have (or does on the family).
     Refer to community agencies or special organizations provide assistance. Financial,
       social, and support.
     Refer to genetic counseling if appropriate.
     Help family learn to expect feelings of frustration and anger toward child, reassure
       them that it is a reflection on their parenting.
     Assist family in problem solving.
     Encourage interaction with other families who have a similarly affected child.
     Introduce to family.
     Provide information regarding support groups.
     Help families learn when to accept and when to (fight).
Expected Outcome:
     Family maintains contact with health providers.
     Family demonstrates an understanding of the needs of the child and the impact of
       condition will have on them.
     Problems are dealt with early.
     Family becomes involved with local agencies and support.
        Anomalies of the Esophagus:
Esophageal Atresia with Tracheoesophageal Fistula: (T.E.F.)
      It is congenital malformation represent a failure of the esophagus to develop a
continuous passage. These defects may occur separately or in combination.

Clinical Manifestation of TEF:
    Excessive salivation and drooling.
    Three Cs of TEF:
    Apnea.
    Increased respiratory distress following feeding.
    Abdominal distention.
Diagnostic Evaluation:
           1. Insertion of catheter gently into the esophagus meets with resistance if the
              lumen is blocked but passes unobstructed if the lumen is patent.
           2. Radiographic visualization of opaque catheter inserted into the
              hypopharynx helps to determine patency and / or alternate channels.
Objective of Therapeutic Management:
          1. Prevent pulmonary complications.
          2. Provide nutrition.
          3. Repair defect surgically.
Nursing Care plan for Infant with T.O.F.:
Nursing Diagnosis:
     Potential for infection.
Nursing Goal:
     Prevent infection.
Nursing Intervention:
   Administer continues preoperative care.
   Observe for signs of inflammation, bleeding or other drainage.
Expected Outcome:
     Infant had no evidence of infection.

Nursing Diagnosis:
     Airway clearance, ineffective.
Nursing Goal:
         Recognize defect early.
         Maintain patent airway.
         Prevent aspiration pneumonia.
Nursing Intervention:
   Be alert of danger signs as:
      -Excessive salivation.
      -Three c’s: choking, coughing, and cyanosis.
   Remove accumulated secretion from oropharynx.
   Position for optimum ventilation.
   Change the position every 2 hours.
   Observe for signs of respiratory distress.
Expected Outcome:
         Defect is recognized early.
         Airway remains patent.
         Infant doesn’t aspirate secretions.

Nursing Diagnosis:
     Anxiety related to inability to swallow.
Nursing Goal:
     Administer comfort measures.
Nursing Intervention:
   Provide tactile stimulation.
   Position comfortably postoperatively.
   Avoid restraints.
   Administer mouth care.
   Offer pacifier frequently.
Expected outcome:
         Child’s rests calmly.
         Mouth remains clean and moist.
Nursing Diagnosis:
      Altered family processes related to child with a physical defect, hospitalization of
Nurse: Support the family.
Patient: Will receive adequate support.
     Educate the parents in home care.
     Administer oral feedings.
     Support the family.
     Maintain contact with family.
Expected Outcome:
     Family maintains contact with health providers.
     Family demonstrates an understanding of the needs of the child and the impact of
        condition will have on their problems are dealt with early.
     Family becomes involved with local agencies and support.

         Anomalies related to the Stomach and Duodenum:
Hypertrophic pyloric stenosis
     It occurs when there is hypertrophy of the circular muscle of the pylorus leads to
constriction of the pyloric canal and obstruction of the gastric outlet.
      The circular muscle of the pylorus is grossly enlarged as a result of both hypertrophy
(increased size) and hyperplasia (increased mass). This produces severe narrowing of the
pyloric canal between the stomach and the 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.
Clinical Manifestation:
   1.    Vomiting: usually start in 2nd to 3rd week of life. And becomes forceful and projectile.
   2.    Hungry and irritable later become lethargic.
   3.    Dehydration.
   4.    Malnutrition.
   5.    Constipation.
   6.    Loss of skin turger.
Diagnostic Evaluation:
   1.    Palpable olive like mass in right upper quadrant.
   2.    Visible, peristaltic waves usually form the left to right.
   3.    Radiographic or ultrasound revealed hypertrophied pyloric muscle.
   4.    Narrow pyloric sphincter revealed in barium swallow. (Positive string sign).
Therapeutic Management:
        Surgical relief of the pyloric obstruction by Pyloromyotomy.
Nursing Management:
   1. The infant must be rehydrated.
   2. Correction of metabolic alkalosis with parenteral fluid and electrolyte administration.
   3. N.G. insertion for gastric decompression.
   4. Preparation of the child and parents psychologically.
Hirschsprung Disease (Congenital Aganglionic Megacolon)
     It is congenital absence of ganglionic nerve fiber in the affected part of the gut leads
to mechanical obstruction caused by inadequate motility in part of the intestine.
      Congenital Aganglionic Megacolon describes the primary defect, which is he
absence of autonomic parasympathetic ganglionic cells in the submucosal and myenteric
plexuses in one or more segments of the colon.
      Lack of innervation produces the functional defect:
          Absence of peristalsis, which cause.
          Accumulation of intestinal contents and bowel distention proximal to the
          Failure of the internal anal sphincter to relax contributes to clinical
             manifestations of obstruction because it prevents evacuation of solids and
      Intestinal distention and ischemia may occur as a result of distension of the bowel
Clinical Manifestations:
     Vary according to:
   1. Age when symptoms are recognized.
    2. The length of the affected bowel.
    3. And the occurrence of complication i.e. entrocolitis.
In the newborn:
      The primary signs and symptoms are:
         Failure to pass meconium within 24 – 48 hrs after birth.
         Food refusal.
         Vomiting.
         Abdominal distention.
During infancy:
          Inadequate weight gain.
          Constipation.
          Abdominal distention.
          Explosive watery diarrhea.
          Episodes of diarrhea and vomiting.
          Bloody diarrhea, fever and severe lethargy are ominous signs because they
             signify the presence of enterocolitis.
During childhood: (chronic)
          Constipation.
          Passage of ribbon like, foul smelling stool.
          Abdominal distention.
          Fecal impaction recurs frequently.
          Poor appetite, poor growth.
Diagnostic Evaluation:
    1. In neonate: diagnosis is usually based on clinical signs of intestinal obstruction and
       failure to pass meconium.
       In infant and children: typical details of chronic constipation.
    2. On examination, the rectum is empty of feces, the internal sphincter is tight and
       leakage of liquid stool.
    3. Barium enema and rectal biopsy.
    4. Non-invasive procedure may be used as anorectal manometry: in which a catheter
       with a ballon attached is inserted into the rectum, the test records the reflex
       pressure response of internal oral sphincter to distention of the ballon.
Therapeutic Management:
      Treatment is primarily surgical removal of Aganglionic portions of the bowel in order
to permit normal bowel motility and establish continence by improved functioning of the
internal anal sphincter.
      Surgery consists of:
    1. First Stage: Temporary ostomy is created proximal to the Aganglionic segment to
       relieve obstruction and allow the normal ionervation of dilated bowel to return to its
       normal size.
    2. Second Stage: Is complete, correction. Consists of pulling the end of the bowel
       through the muscular sleeve of the rectum from which the Aganglionic mucosa has
       been removed.
Nursing Management:
     According to the child age, if disorder diagnosed during neonatal period the main
   1. Help the parents to adjust to congenital anomalies of their child.
   2. Foster infant-parent bonding.
   3. Assist them in colostomy care after discharge.
   4. Provide pre-post operative care.
    It is an acquired type of intestinal obstruction. It is the invagination or telescoping of a
segment of intestine into the segment immediately distal to it.
      Intussusception is one of the most frequent causes of intestinal obstructions in
children between the ages of 3 months and 5 years. Half of the cases occur in children
younger than 1 year, and most of the others occur in children during the second year.
     Exact cause of Intussusception is uncertain but there are several theories as:
   1. Greater disparity between the size of the ileum and ileocecal value in infants is
      believed to encourage telescoping at this point.
   2. Distorted peristalsis due to change of diet (weaning).
   3. Enlarged lymph nodes along the G.I.T. that occur with respiratory infections, cystic
      fibrosis, foreign bodies, GI polyps.
   4. Hyperperistalsis.
   5. Diarrhea, constipation.
   6. Mobile ileocecal junction.
       Intussusception causes an interference with the vascular supply as well as an
obstruction of the G.I.T. Interference with lymphatic and venous drainage leads to edema,
increased tissue pressure and capillary and venule engorgement which from a vicious
circle. With increasing edema and eventual total venous obstruction. Tissue pressure rises
until arterial flow is stopped which leads to necrosis. Intestinal cells are stimulated to
discharge mucus, which mixes with the blood to produce a current Jelly stool.
       The most common sites:
                  Ileocecal value. (Ileocolic)
                  Ileoileal.
                  Colocolic.
Clinical Manifestation:
     1. Sudden acute abdominal pain, which becomes progressively more severe.
             Child screams and draws the knees into the chest.
             Child is healthy and normal between episodes of pain.
     2. Vomiting.
     3. Lethargy.
     4. Passage of red current jelly stool. (Stool mixed with blood and mucus).
     5. Palpable sausage- shaped mass in upper right quadrant.
     6. Empty right lower quadrant (dance sign).
     7. Anorexia, discomfort.
     8. Tender, distended abdomen.
     9. Eventually fever, perforation, peritonitis and shock are serious complications of
Therapeutic Management:
    1. The initial treatment of choice is non-surgical hydrostatic reduction.
 Traditionally by barium enema:
      In this procedure, correction of the invagination is carried out at the same time as the
diagnostic testing. The force exerted by the flowing barium is usually sufficient to push the
invaginated portion of the bowel into its original position. Similar to pushing an inverted
finger (out of glove) this observed by fluoroscopy.
Now: To reduce the risk of barium peritonitis. The radiologists use water-soluble contrast
and air pressure to reduce Intussusception.
 Intravenous fluids are administered.
   Insertion of nasogastric for decompression.
   Administration of antibiotic.
    2. If there is clinical signs of shock or perforation.
       Surgical intervention is done. Involves manually reducing the invagination and
       resecting any nonviable (gangrenous) part of intestine.
Nursing Management:
    Prepare the parents for the immediate need for hospitalization. Explain the defect of
     the Intussusception and how it ‘ll be corrected briefly.
    Replacement fluids are administered.
    Nasogastric suctioning.
    Antibiotics.
    General preoperative procedures may be perfumed.
    Observe passage of barium or water-soluble contrast material with stools.
    The major nursing responsibilities are the same as the following abdominal surgery.
     Assess: bowel movement, bowel sound, distention, vomiting.
    Monitor v/s.
    Assess kidney function through urine out put, presence signs of infection,
    The parents should be educated about the risk of recurrence. If it recurs, hydrostatic
     reduction is usually attempted. A laparotomy is considered for multiple recurrences.

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