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:
By the end of this lecture, the student should be able to: 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 care plans for children with gastrointestinal, respiratory, skeletal and orthopedic anomalies

Causes of Fetal Malformation

Genetic Traits Viruses

Drug Radiation

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.

 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.

General Aspects of Pre & Post-Operative Pediatric Care

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 infants condition

 Safe means of transportation with a

heated portable incubator and available oxygen supply is needed so as to maintain the infants body temperature and O2 therapies in the blood. Equipment for suctioning to remove secretions is needed (as in esophageal atresia).

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 omphalocele, proper position of infant). should accompany the infant as he goes from one health agency to another

All pertinent infant information

Pre-operative care

 Psychological preparation of the child

(according to his age). This aimed to prevention of 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.
Except in emergency situations, children should preferably be free of respiratory complications and signs of malnutrition.

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). 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 or missing teeth should be charted in the childs record.

Remove barrettes and pins from the childs

hair. Clothing should be warm and loose. The child should be dressed in a hospital gown and under pants only. Check the childs identification band to see that is legible and secure. If not it needs to be replaced prior to surgery. Pre-medication: sedatives and analgesics are usually given two hours before surgery except in emergency situation.

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 conditions. 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.

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.
Parents should be allowed to

accompany their children to the operation site if they so desire.

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.

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

Vital signs, especially temperature. Airway must be kept patent newborn babies must be kept in warm cot or incubator.

 Unit the child is responsive and alert; he should be kept on his side (for secretion and vomitus to get out from mouth). Observe conditions and placement of dressing. Check and mark any apparent drainage from wound. Intravenous fluids should be checked for correct rate of flow and for possible infiltration.

The child should be carefully handled and

should 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 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.

 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 childs age, but in general it changes from clear of liquid, full liquid, soft and then regular diet. Sedatives are used according to prescribed orders and childs needs.

For children who can walk, early progressive ambulation is the rule (expect in few cases), this will help to restore GIT 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.

It is defined as a structural defect present at birth. Some anomalies are compatible with life and treatment could be delayed, while others are incompatible with life and must be repaired immediately

Gastrointestin al Anomalies

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 may be unilateral or bilateral

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 two bilateral clefts. Cleft palate may occur in connection with cleft lip Cause: Both cleft lip and palate result from failure of or incomplete union of embryonic structures of the face.

Clinical Manifestations

Observable defects.

Complications associated with cleft lip and cleft palate

Feeding

difficulties Ear infections and hearing loss Speech and language delay Dental problems

Feeding baby with cleft palate

Breast-feeding is allowed. It will take extra time and 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 cleft palate.

NUK nipple

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

Mead Johnson Nurser

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

Haberman Feeder
This is a specially designed bottle system with a valve to help control the air the baby drinks and to prevent milk from going back into the bottle.

Neonatal Respiratory Distress

Congenital Diaphragmatic Hernia

Esophageal Atresia with Tracheoesophageal Fistula

Esophageal Atresia and 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.

Definition

Oesophageal atresia is defined: as an interruption in the continuity of the esophagus with or without fistula to the trachea. Tracheoesophageal Fistula is defined as abnormal fistulous communication between the trachea and the esophagus with Atresia of the esophagus singly or in combination.

Etiology: Unknown.

Types:

Oesophageal Atresia with a distal tracheooesophageal fistula

Oesophageal Atresia with a proximal fistula

Oesophageal Atresia: (cont)

Oesophageal atresia combined with fistulae from both oesophageal segments Isolated Oesophageal Atresia without a fistula

 V = Vertebral anomalies
A = Anal anomalies TE = Tracheoesophageal Fistula R = Renal deformaties

Three Cs of TEF on feeding:

Coughing. Choking. Cyanosis. and, regurgitation

 Excessive salivation and drooling Apnea. Increased respiratory distress following

feeding. Abdominal distentionmight occur if large fistula as a result of gas accumulation.

 Regurgitation of saliva and milk from

the 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.

 Insertion of catheter gently into the

esophagus meets with resistance if the lumen is blocked but passes unobstructed if the lumen is patent. Radiographic visualization of opaque catheter inserted into the hypopharynx helps to determine patency and / or alternate channels. Endoscopy for isolated fistula.

Pre-operative Care: No oral feeding. Give Intravenous (I .V) fluids. Keep infant/ child warm, by placing him in incubator or use warmer.

 Give humidified oxygen for respiratory distress. Put infant/ child in sitting or semi-sitting position. Make suction for mouth, pharynx and end of pouch.

Post-operative Care:
Place infant/ child in humidified air and warm him. Maintain I. V. fluids. Suctioning whenever needed, if he can not swallow saliva. Observe respiration, report immediately if there is respiratory difficulties are present or atelectasis.

 If there is gastrostomy tube feeding, it must not be elevated more than 4 inches (10 cm) above stomach level to prevent reflux to healed esophagus. Use pacifier to satisfy infants need for sucking. Start oral feeding gradually when esophagus anastomosis has healed, but continue gastrostomy feeding until the infant takes adequate amount per mouth. Teach parent before infants discharge how to care for him at home.

3.Anomalies of the Stomach and Duodenum

Hypertrophic 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.

 It 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.

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.

 Vomiting:

usually start in 2nd to 3rd week of life. And becomes forceful and projectile. Hungry and irritable later become lethargic. Dehydration. Malnutrition. Constipation. Loss of skin turgor.

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).

Surgical relief of the pyloric obstruction by Pyloromyotomy

 Observe and record:

Vital signs especially that indicates electrolyte imbalance. Vomitus for color and amount. Stool. Sucking fingers which indicates hunger.

Collect specimens.

 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 disease and its care. Provide emotional support to parents.

 If doctor order to feed the infant, feed him in semi upright position, feed slowly and bubbles frequently to prevent vomiting. Handle the infant as little as possible after feeding. Warm the infant/ child. Prevent infection. Change position frequently.

 Provide mouth and skin care. Weight infant daily to assess dehydration and malnutrition. Intake and output chart. Stimulate the infant. Use pacifier to meet infants need for suckling.

 Adequate fluid and nutrition: Give I.V until breast or formula feeding can be tolerated. Also glucose in water or electrolyte solution are given frequently in small amount and increase it gradually. Observe for signs of complications: Sings of inflammation, shock and abdominal distention.

 Parent education about the care of infant, especially position at feeding andhow to keep incision clean. Teach parent about the importance of follow- up care.

Anomalies of the Intestine

It is incomplete development or absence of the anus in its normal position in the perineum.

 It 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.

There is congenital absence of parasympathetic ganglion nerve cells of a part of intestine usually in the distal end of the descending colon.

Vary according to: Age when symptoms are recognized. The length of the affected bowel. 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:
Constipation. Passage of ribbon like, foul smelling stool. Abdominal distention. Fecal impaction recurs frequently. Poor appetite, poor growth

 In neonate: diagnosis is usually based on clinical signs of intestinal obstruction and failure to pass meconium. On examination, the rectum is empty of feces, the internal sphincter is tight and leakage of liquid stool. Barium enema and rectal biopsy. Non-invasive procedure may be used as anorectal manometry.

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.

 It is incomplete development or absence of the anus in its normal position in the perineum

Narrowing of the anorectal canal that may occur at any point or extended its entire length.

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

A thin membrane persists across the anal opening.

The anus is normal but the rectal canal is not continuous. This type is very rare

Assessment Criteria:
Failure to pass meconium stool in the first 24 hours after birth. Absence or stenosis of the anorectal canal. Anal membrane. External fistula to the perineum.

Diagnostic Procedures:
During the initial assessment of the newborn perineum, which should include determination of the rectum patency with small finger or rectal tube if a lesion is suspected. X- ray examination. Ultrasound. Computed tomography scan.

Acquired Intestinal Defect Intussusceptions

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: Greater disparity between the size of the ileum and ileocecal value in infants is believed to encourage telescoping at this point. Distorted peristalsis due to change of diet (weaning). Enlarged lymph nodes along the G.I.T. that occur with respiratory infections, cystic fibrosis, foreign bodies, G.I polyps. Hyperperistalsis. Diarrhea, constipation. Mobile ileocecal junction.

Ileocecal value. (Ileocolic) Ileoileal. Colocolic.

 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. Vomiting. Lethargy.

 Passage of red current jelly stool. (Stool mixed with blood and mucus). Palpable sausage- shaped mass in upper right quadrant.

Empty right lower quadrant (dance sign). Anorexia, discomfort. Tender, distended abdomen. Eventually fever, perforation, peritonitis and shock are serious complications of Intussusception.

Therapeutic Management:
The initial treatment of choice is nonsurgical 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.

Abdominal Wall Defects

It is herniation of intestines into the umbilical cord, i.e., a protrusion, at birth, of part of intestine through a defect in the abdominal wall at the umbilicus.

Omphalocele

There is a sac that covers the defect, which composed of transparent avascular membrane from which the umbilical cord extends.

It is a congenital defect that differs from omphalocele in that it is fissure of the abdominal wall to the right of the umbilicus and does not involve the site of the insertion of the umbilical cord.

Protrusion of the small intestine and part of large intestine usually accompany it. There is no sac covering the intestine, therefore, the bowel is irritated by amniotic fluid during gestation and becomes edematous and inflamed.

Umbilical Hernia
It is protrusion of the omentum and small intestine through the imperfect closure or weak umbilical ring, especially when infant cries.

Inguinal Hernia
It is protrusion of abdominal contents through the inguinal canal into scrotum.

Nursing Management: Pre-operative Care:

For cases of gastrochisis, omphalocele, umbilical and inguinal hernias. Do not apply pressure on herniated area. Nasogastric suctioning for omphalocele / gastrochisis. Apply sterile dressing over herniated area for omphalocele/ gastrochisis. Keep the child quiet, no crying in particular in cases of harnias, especially umbilical hernia. Give warm bath, suck pacifier to calm the infant. Educate parent about strape the defect has no value to decrease hernia.

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.

Defect of Respiratory System

It is defined as a congenital obstruction of the posterior nares at the entrance to the naso-pharynx. It may be unilateral or bilateral

Etiology: The obstruction is usually caused by a membrane, but in rare cases by a boney growth. Assessment In bilateral obstruction there is mouth breathing and difficulty in taking feedings. There may be dyspnea because the infant cant obtain enough oxygen.

Treatment 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.

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

 Assessment The infant usually develops respiratory distress and possibly cyanosis. Treatment Surgery. If operation is not done immediately, the prognosis is very poor and the patient will probably die within the first month of life.

Spina Bifida:
It 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.

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.

Meningocle
It 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.

Meningomyelocele
It 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 of the defect, the greater the defect.

 If in the lumbosacral area, the defect is usually associated with a flaccid paralysis of the lower extremities, absent sensation to the level of the lesion, and loss of bowel and bladder control.

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 structure begin to separate

Nursing Management of Meningocele and Meningomyelocele

Nursing Diagnosis: High risk for infection. High risk for injury. High risk for skin impaired integrity. Altered nutrition: Less than body requirements

Epispadias Mieatual opening located on dorsal or superior surface of the penis. 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. In Severe cases, circumcision or vertical division and transverse, suturing of foreskin. 4-Hydrocele Fluid in scrotum. Therapeutic management is surgical repair indicated if spontaneous resolution not accomplished in 1 year.

5-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.

6-Wilms 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 metastasis.

Clubfoot
Flexion at the ankle with inversion of the heel and fore foot.

Torticollis
It 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.

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

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 the extremity in alignment. Before the cast is applied, the extremities are checked for any abrasions, cuts, or other alterations in skin surface and other items that might cause constriction from swelling; such objects are removed. Identification bands are placed on a non-injured extremity if hospitalization is anticipated.

After cast application

The cast must remain uncovered to allow is dryness from inside out. After the cast is applied, the extremities are checked for any sings of compromise. Observations such as pain, swelling, discoloration (pallor or cyanosis) of the exposes portions, lack of pulsation and warmth, or the inability to move the exposed parts are reported immediately.

 Turning the child in a plaster cast at least every 2 hours to prevent complications from immobility. The body part can be elevated, thereby increasing venous return. Periodically the circumscribed blood stained area should be outlined with a ball point pen to assess the amount of blood. Parents need instructions on drying and caring for the cast and checking for signs that indicate the cast is too tight.