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Lecture 1

NURS 3012 Lecture 1: NURS 3012 Topic 1 - Hernias, DDH, Congenital Club Foot, Hypospadias, GER & Pyloric Stenosis

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NURS 3012
Lori Robbins

Topic 1 Hernias, DDH, Congenital Clubfoot, Hypospadias, GER, & Pyloric Stenosis o Congenital anomalies are abnormalities that are present at birth, even if not diagnosed until months or years later. o Anomalies can occur at conception but often happen in the embryonic period, but can also develop in early fetal period • Down Syndrome occurs at conception • Neural tube defects occur in the embryonic period (3-5 week gestation) • Orofacial clefts develop in the fetal period (cleft lip 4-7 weeks, cleft palate 6- 9) o The anomaly can also develop as a result of an environmental insult, which may occur later in pregnancy o When a child is born with an anomaly, it becomes the role of the nurse caring for the family to help them move forward • This may include helping families face immediate surgery, preparing them for multiple medical interventions, or for a lifetime of needing specialized care • Parental disappointment and disillusion add to the complexity of the nursing care needed for these infants Hernias o Hernias occur when an organ or tissue protrudes through the muscle wall in an abnormal opening • These are usually reducible • Hernias that cannot be reduced are called incarcerated hernias • The concern of hernias is when they become strangulated. • Strangulation occurs when the organ protruding through the opening is constricted to the extent that circulation is impaired or when the protruding organs encroach on and impair the function of other structures • Thus, a strangulated hernia is one in which the blood supply to the herniated organ is impaired o Umbilical hernias are common in infants • These occur when there is a weakness in the abdominal wall around the umbilicus, or when an incomplete closure of the abdominal wall occurs, allowing intestinal contents to protrude through the umbilical ring • These are small, reducible hernias that usually close by themselves in 1-2 years • Usually no medical interventions are necessary. Surgery is required if the hernia is greater than 1.5-2cm by 2 years age, doesn’t close by age 4-6, or if it becomes strangulated • Complications of this hernia is rare o Inguinal hernias are common in children • These are protrusions of the peritoneum through the abdominal wall in the inguinal canal • These hernias occur mostly in males, is frequently bilateral, and may be visible as a mass in the scrotum • Hernia incarceration is the most common cause of bowel obstruction in infants and children. Signs of this include a tender, firm mass, poor feeding, and an inconsolable child • There is risk for poor bowel circulation/bowel death with these hernias • These hernias are always surgically repaired because of the risk o Diaphragmatic hernias typically occur in infants and are life threatening • These hernias occur when there is a defect in the diaphragm, allowing abdominal structures to enter the thoracic cavity • Can usually be detected through U/S in pregnancy • This causes an impairment to developing lung tissue • Signs include severe respiratory distress, scaphoid abdomen, and shock • Treatment should include respiratory support (mechanical ventilation), gastric decompression, and hydration (IV) • After children have stabilized, surgery may take place o Another type of herniation include abdominal wall defects, including gastroschisis and omphalocele • These conditions increase risk for infection, bowel perforation, and fluid/loss and dehydration (especially with gastroschisis) • If these conditions are detected through ultrasound, the mom will have C- section o Gastroschisis occurs when intestinal loops protrude through the abdomen lateral to the umbilicus (R>L) • There is no sac covering the extruded viscera with gastroschisis • This is usually an isolated defect • Risk for gastroschisis increases in young mothers o Omphalocele occurs when there is a herniation of abdominal viscera through abdominal ring • Covered by the amnion and peritoneum • There is usually associated anomalies with this defect o These abdominal wall defects require urgent surgical closure, which may be complete or staged depending on the size of the defect Developmental Dysplasia of the Hip (DDH) o The term DDH describes a broad spectrum of disorders related to abnormal development of the hip, in which preluxation, subluxation or dislocation occurs • Preluxation (or dysplasia) is mild, and occurs when the ligaments are loose around the hip joint, allowing the femur to move abnormally in the acetabulum • Subluxation is the most common and occurs when the loose joint allows the femoral head to move partway out of the acetabulum • Dislocation is severe and occurs when a shallow acetabulum allows the femoral head to partly or fully slip out of the socket o Risk factors for DDH include: • Gender – more common in females than males • Birth order – the first born child is more prone • Family history • Intrauterine position – more common in breech positioning • Delivery type – vaginal delivery increases risk • Circulating maternal hormones • Post natal positioning – swaddling babies may increase risk • Other anomalies – spinal cord, nerve, muscle disorders, and prematurity o Diagnosing DDH may be difficult as it is often not detected at the initial examination after birth, and thus all infants should be carefully monitored at follow-up visits for 1 year of life o A physical exam in the Newborn Screening should include the Barlow & Ortolani tests, as well as to look for the Galleazzi sign • Barlow test – is conducted by guiding the hips into mild adduction and applying slight forward pressure with the thumb. If the hip is unstable, the femoral head will slip over the acetabulum, producing a palpable sensation of subluxation/dislocation (+ sign) • Ortolani test –With slow abduction, a dislocated hip will reduce with a described palpable “clunk” (+ sign) • Galleazzi sign – performed by flexing an infant’s knees when they are supine so that the feet touch the surface and the ankles touch the buttocks. If the knees are not level then the test is +, indicating potential DDH ** Note that the Ortolani test and Barlow test will disappear in the first 2 weeks of life. o Serial clinical exams – limited hip abduction o X-ray is not reliable in diagnosing DDH in early infancy, as ossification of the femoral head does not normally take place until 3-6 months of life • The cartilaginous head can be visualized directly by ultrasonography, however. • In children from 4-6 months of age, radiography can be utilized to aid diagnostics o DDH is also shown with limp, toe walking or waddling gait and Positive Trendelenburg • However, this is only a sign in walking children. It is ideal to diagnose DDH before walking age • The Trendelenburg test is carried out by asking the patient to stand unassisted on each leg (flex one leg) • The affected slide slips, so the unaffected side drops o Treatment of DDH is begun as soon as the condition is diagnosed, as early intervention is most favourable to normal restoration • The longer treatment is delayed, the more severe the deformity, the more difficult the treatment, and the less favourable the prognosis is • Treatment varies with the child’s age and extent of the deformity • The goal of treatment is to obtain and maintain a safe, congruent position of the hip joint and to promote normal hip joint development o Treatment of Newborns – 6 months of age usually includes the use of a Pavlik harness: • The Pavlik harness centers the femur in the acetabulum with the hip abducted and flexed • Babies using the Pavlik harness should wear an undershirt and socks under the straps, as well as their diaper. • BID-TID skin inspection & daily massage under the straps to prevent skin breakdown • Avoid the use of lotions and powders • The Pavlik Harness usually loses its effectiveness after 6 months, as the child’s mobility and strength increases • Usually transfers to an abduction brace up to 1 year • Although less common, sometimes skin traction will be used, followed by the use of hip spica casting and then abduction bracing o Treatment of babies 6-18 months of age • In this age group, the dislocation is not recognized until the child begins to walk • Gradual reduction by traction is usually used for about 3 weeks • Following traction, the child undergoes an attempted close reduction of the hip; if the hip is not reducible, an open reduction is performed • After reduction, the child is placed in a hip spica cast for 2-4 months until the hip is stable, at which time a abduction brace is applied o Because of the restriction associated with the hip spica cast, there is education that needs to be done with the family • Address elimination challenges •
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