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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. Introduction: 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 understood. 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: Definition: 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 lip. 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 occurs. 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 defect. 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 metastasis. 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 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 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 atresia). 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 record. 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 conditions. 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 infiltration. 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 bed. 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 diet. 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. Goal: Nurse: Provide adequate nutritional intake. Patient: Will receive optimum nutrition. Interventions: 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. Goal: Nurse: facilitate family’s acceptance of infant. Patient (family): will demonstrate acceptance. Interventions: 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. Intervention: 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. Intervention: Position to allow for drainage of mucus (partial side- lying position, semi-Fowler position). Expected Outcome: Child manages secretions with out aspiration. Nursing Diagnosis: Altered nutrition; less than body requirements related to physical defect, surgical procedure. Goal 1: Nurse: Provide adequate nutrition intake. Patient: Will receive optimum nutrition. Intervention: 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. Goal: Nurse: Relive discomfort. Patient: Will experience optimum comfort level. Intervention: 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. Goal: Nurse: Support the family. Patient: Will receive adequate support. Intervention: 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. Etiology: Unknown. Types: Clinical Manifestation of TEF: Excessive salivation and drooling. Three Cs of TEF: -Coughing. -Choking. -Cyanosis. 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 infant. Goal: Nurse: Support the family. Patient: Will receive adequate support. Intervention: 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. Pathophysiology: 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: Preoperative: 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. Pathophysiology: 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 defect. Failure of the internal anal sphincter to relax contributes to clinical manifestations of obstruction because it prevents evacuation of solids and gas. Intestinal distention and ischemia may occur as a result of distension of the bowel wall. 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 objective: 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. Intussusception: 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. Incidence: 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. Etiology: 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. Pathophysiology: 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 Intussusception. 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: Preoperatively: 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. Postoperatively: 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, inflammation. 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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