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What are physical defects that develop during fetal life? congenital abnormalities abnormalities can be two types 1. genetic ( inherited) or 2. environmental exposure an environmental agent (chemical or drug) that injures the embryo or fetus is called teratogen Mutagenic substances cause genetic mutation ( a chemical change in the genetic structure) The maternal diet is important Pediatric patients are vulnerable to hypothermia & hyperthermia Physiological mechanisms that normally regulate temperature in an adult are what from an infant? absent or undeveloped infants and children tend to lose more heat than they generate relatively large skin surface area & low body weight contribute to rapid lowering of body temperature in infants children and infants have extensive peripheral circulation, which contributes to relatively rapid cooling Loss of body heat occurs through 1. radiation 2. conduction 3. evaporation 4. anesthetics infants lack what warming mechanism? shivering What is less of a risk than hypothermia in infants hyperthermia the misuse of warming devices can cause hyperthermia very quickly What is used to maintain a safe core temperature 1. warm air blankets 2. water filled mattresses 3. overhead heat lamps Maintaining the correct amount and type of fluids in infant body spaces is called fluid balance It is important to maintain proper fluid balance infants and children have little blood reserve who is responsible for monitoring blood loss all surgical team members Hemostasis is critical in pediatric surgery an important anatomical difference between the adult and child respiratory system is the airway One of the leading causes of death in a child is failure to monitor the airway in medical and trauma emergency Preparing a child for surgery is much the same way as adults but you need to prepare for a child's more extreme fears because they do not understand pediatric patients may be given what before surgery to calm them anxioloytic (varies from institution) anxiolytic is anxiety reducing What is now an accepted practice for children in the surgery room the presence of parents for induction ( reduces the need for premedication) the goal of psychosocial care is to help patients and family develop strategies to cope with the perioperative experience Pediatric patients tend to their environment is predictable ways according to their developmental age What are the two greatest fears of children undergoing surgery 1. fear of the unknown 2. fear of separation from family because children are small in size, they are at high risk for adverse effects from overdosing pediatric patients are vulnerable to risks related to their 1. age 2. size 3. stage of development Neonates are transported to the operating room is a heated Isolette an isolette is environmentally controlled and equipped with monitoring devices toddlers may walk to the surgical department or transported by crib the only in difference in ESu between an adult and child are the size of the active and return elctrodes In positioning a child be sure to use appropriate size padding. -Cleft lip and cleft palate occur with in the first 8 weeks of embryonic development What fails to close normally in cleft lip or palate the philtrum & palate cleft palate is sometimes seen with cleft lip cleft palate and cleft lip are no related to each other, they are separate malformations the term branchial refers to "gills" and is derived from the science of embryology When branchial fail to develop, they may persist as cysts or fistulas a thyroglossal duct cyst or fistula is the result of incomplete closure of the this duct what fails to form during development of the thyroid gland during embryonic life thyroglossal gland choanal artesia is a congenital anomaly of the anterior skull base, characterized by stricture or blockage of the passage between the nasal sinuses and the pharyngeal nasal airways where is the graft taken to reconstruct the ear intercostal cartilage what is divided & released in pyloromyotomy, to allow normal passage of food the pyloric outlet muscles esophageal atresia is the absence of or a gap in the esophagus the goal to fix esophageal atresia is to join the two sections and provide continuity of the structure What is tracheoesophageal fistula often associated with EA tracheoesophageal fistula is a defect that develops between the esophagus, and trachea causing saliva, stomach contents and food to go into the lungs omphalocele is a congenital anomaly in which abdominal viscera develop outside the body contained with in a peritoneal sac gastroschisis is similar to omphalocele except there is no peritoneal covering over the abdominal viscera hirschsprung disease is congenital absence of ganglionic cells in the colon. the result of hischsprung disease is the bowels failure to function in peristalsis The pull through method for hisschsrung disease involves removal of the diseased portion of the bowel and anastomosis to the rectal muscles intussusception is an emergency bowel that telescops back in on its self is called intussusception intussusception causes obstruction & ischemia volvulus is rotation of the bowel around the mesenteric attachment, or twisting on itself emergency medical response is required of a volvulus to prevent gangrene & peritonitis bladder exstrophy is part of a complex congenital defect in which the bladder develops outside the body in open configuration baldder exstrophy also involves other structures of the genitourinary tract, skeletal system and other systems orchiopexy is surgical correction for an undescended testicle patent ductus arteriosus occurs when the duct fails to close normally after birth patent ductus arteriosus causes arterial blood to continue its fetal path back to the lungs coarctation of the aorta is a congenital narrowing that obstructs the normal flow of blood through the thoracic aorta to the lower body an artial septal defect is a congenital anomaly in which a hole in the interatrial septum allows blood from the left atrium to flow to the right atrium a ventricular septal defect causes increased pulmonary pressure by allowing blood fromthe left ventricle to flow into the right ventricle and to the lungs ventricular septal defect leads to congestive heart failure tetralogy fallot is a combination of congenital defects the combination of congential defects of tetralogy fallot is pulmonary stenosis ventricular septal defect right ventricular hypertrophy dextroposition (displacement) of the aorta pectus excavatum is a defect of the chest wall marked by overgrowth of the costal cartilages and a sunken appearance in the sternum to repair a pectus excavatum what technique would you use Nuss minimally invasive surgical technique spina bifida is a birth defect associated with incomplete closure of a section of the vertebral column. myelomeningocele is the herniation of the spinal cord, meninges and nerve roots through the skin