Choanal Atresia: Types and Symptoms

Choanal Atresia

• Atresia is defined as the closure of a normal opening
• Condition where posterior nares are obstructed by membranous septum or bone
• Obstruction can be either bilaterally or unilaterally
• Most common congenital malformation of nose
• Incidence is 1:7,000 live births
• Condition is associated with other congenital anomalies in 50% of cases like craniofacial syndromes and skull based defects (encephalocele)
• More common in females
• Cause is unknown

Types of Choanal Atresia

• Unilateral which occurs usually on the right in 60% of cases
  • It can go undiagnosed until child presents with persistent unilateral nasal drainage
  • Neonates may have respiratory infection and seem to have more nasal obstruction not proportional to degree of infection
  • Careful questioning reveals an unusual susceptibility to upper respiratory symptoms
• Bilateral
  • Neonates become apneic or cyanotic at birth
  • Requires resuscitation to prevent asphyxia/severe hypoxia and may need an artificial airway

Clinical Manifestations of Choanal Atresia

• Difficulty breathing after birth which may result in cyanosis, unless infant is crying
• Inability to nurse and breathe at same time
• Unable to pass a catheter through each side of nose into throat
• Persistent one-sided nasal blockage/discharge

Diagnosis of Choanal Atresia

• Generally recognized shortly after birth in the hospital
• Diagnosed by physical exam for obstruction of the nose
• Confirmed by passing a firm catheter (Fr. 6) through each nostril 3-4 cm into nasopharynx
• Radiography using contrast material determines exact location of obstruction
• Fiberoptic endoscope looks into nose to ensure airways are open and to assess healing after surgery
• CT scan evaluates atresia after feeding tube fails by detecting composition (membranous or bony), thickness, depth of nasopharynx, and skull base anomalies

Management of Choanal Atresia

• Airway support via oral intubation or tracheostomy
• Surgery to cure the obstruction
  • Surgery may be delayed until the child is 2 or 3 years old if only one nasal passage is blocked
  • Anatomy is sufficiently developed to facilitate easy access to posterior choanae
  • Intraoperative blood transfusion not normally required for repair at this age
  • Transnasal approach- requires less operative time, causes slightly less morbidity related to incision, poses higher risk of nasal passages closing later
  • Transpalatal approach- provides better exposure and more accurate bone removal
• Doctor stitches tubes (small plastic) called nasal stents to passages to keep them open
• Stents removed after 6+ weeks under general anesthesia
• Restenosis can occur, requiring dilatation and reoperation

Nursing Care for Choanal Atresia

• Airway support with close observation and connect to cardio respiratory monitor
• Careful suctioning of mucous to maintain breathing, especially before feedings
• Provide hydration and nutrition; feeding tube for bilateral atresia until corrected
• For unilateral, feed infant slowly with small nipple in upright position and frequent pauses to permit breathing.

Croup

• Term applied to broad classification of UPPER AIRWAY illnesses
• Results from inflammation and narrowing of larynx or voice box, trachea or windpipe, and bronchi or larger branching air tubes

Incidence of Croup

• Most common in children age 5 and younger, especially those prematurely born
• Peak is second year
• Signs and symptoms are most severe in children age 3 and younger due to narrower airways being more likely to become blocked during infection
• Occurs in winter or early spring
• A child can get croup more than once

Causes of Croup

• Primarily caused by VIRUSES; parainfluenza virus accounts for most cases
• Other viruses include adenovirus, respiratory syncytial virus, influenza, and measles
• Occasionally caused by BACTERIA or allergic reaction

Changes in Upper Airway in Croup

• Epiglottis and larynx swell, occluding the airway
• Trachea swells against cricoid cartilage, causing restriction

General Clinical Manifestations of Croup

• Croupy cough-loud, harsh, "brassy" or "barking" cough similar to the noise of a seal barking
  • Result of swelling around vocal cords (larynx) and windpipe (trachea)
  • When cough reflex forces air through narrowed passage, vocal cords vibrate with barking noise
• Dysponia is the medical term for Hoarseness
  • Often dominates clinical picture due to laryngeal involvement
• Inspiratory stridor- high-pitched or squeaking noise when breathing in; created by narrowing of airway
• Respiratory Distress
  • Caused by swelling or obstruction of the larynx

Types of Croup

• Acute Laryngitis
  • Part Affected: Glottic
  • Severity: Least Serious
  • Causative Agent: Viral with allergic/psychosomatic component
  • Onset: Sudden, typically occurs at night and tends to recur
  • Manifestation: Afebrile and Mild respiratory distress
  • Treatment: Humidity; severe Racemic epinephrine, mild Home, but must seek medical help if (+)s/s of respiratory distress
• Spasmodic Laryngitis
  • Part Affected: Subglottic
  • Severity: Most Common, Most Serious if Viral Croup progresses if untreated
  • Causative Agent: Viral-Parainfluenza, Influenza A & B, RSV, Mycoplasma pneumonia
  • Onset: Gradual, Starts as a URI and progresses to symptoms of Respiratory Distress
  • Manifestation: Low Grade Fever and Non Toxic Appearance
  • Treatment: Humidity and Racemic Epinephrine
• Bacterial Tracheitis
  • Part Affected: Upper Trachea
  • Severity: Guarded, Close observation if required
  • Causative Agent: Bacterial, Usually Staphylococcus Aureus
  • Onset: Progressive from URI
  • Manifestation: High Fever and Thick Purulent Tracheal Secretions; URI Appears as Viral Groupy cough, but there is no response to LTB therapy
  • Treatment: Antibiotics
• Acute Epiglottitis Part Affected: Supraglottic
  • Severity: Most life-threatening
  • Causative Agent: Bacterial, Usually HIB
  • Onset: Rapidly Progressive (Hours)
  • Manifestation: High Fever, Toxic Appearance, 4 D's- Dysphonia,Dysphagia, Drooling and Distressed effort
  • Treatment: Antibiotics and Airway Protection

Diagnosis for Types of Croup

• Diagnosis made by history like fever or cold symptoms, recent viral infection
• Diagnosis depends on clinical manifestations
• X-ray of neck not usually required but is used to rule out foreign body obstruction or epiglottitis -Steeple sign is where top of airway narrows to a point
• WBC and used a differential count needed to distinguish between bacterial and viral illnesses

Management of Croup

• Maintain patent airway and provide for adequate respiratory exchange
• Humidity with cool mist provides relief for most children and decrease swelling of laryngeal tissue
• Mild cases are managed at home with a cool air vaporizer
• Spasms are relieved by sudden exposure to cold air by breathing air from freezer or cool garage
• Warm mist can be used by steaming from hot water in closed bathroom
• Avoid hot steam in a pan to prevent burns
• Hospitalize if conditions do not improve or become severe
  • Mist Tent/Croupette provides cool, moist air with oxygen for patients who need extra oxygen and moisture
• Medications- Racemic Epinephrine nebulized -Causes mucosal vasoconstriction and subsequent decrease in edema -Rapid action but sometimes symptoms "relapse" within 2 hours which makes assessments important
• Corticosteroids provide anti-inflammatory effects to decrease subglottic edema
• Antibiotics for bacterial croup
• Antipyretics for fever
• IVF for hydration for patients with fever, fluid loss, and sore throat with inadequate intake
• Artificial Airway accomplished through ET intubation/tracheostomy
  • Generally extubated until spasm and edema are no longer a problem
  • The child is able to manage secretions successfully, usually within 24-36 hours

Nursing Diagnosis of Croup

• Ineffective airway clearance related to laryngeal edema, mucosal inflammation, and decreased energy or fatigue
• Altered tissue perfusion relating to partially obstructed airway
• High risk for fluid volume deficit relating to inability to meet body requirements and increased metabolic demands
• Anxiety and fear related to acute illness, hospitalization, and uncertain course of illness and treatment
• Knowledge deficit related to diagnosis, treatment, prognosis, and home care needs

Nursing Care of Croup

• Maintain a patent airway and provide adequate respiratory exchange with close monitoring
• Assess respiratory status every 2-4 hours and document and report changes promptly to physician
• Early signs of impeding airway obstruction include tachycardia, tachypnea, sternal retractions, flaring nares, and restlessness
• Infants and preverbal toddlers need continuous supervision to monitor respiratory status
• For older children, a means of communication must be available for nursing staff
• Keep emergency suction and tracheostomy intubation tray at the bedside
• CROUPETTE is a device that provides cool humidification with O2 or compresses air
  • Keep the child dry by changing linens and gowns and drying hair frequently
  • Tuck loose edges under mattress to prevent oxygen and moisture loss in tent
  • Room near nurses station and resuscitation equipment at bedside
  • No to battery, friction, or fur toys because of fire hazard
• Administer medications as prescribed
• Avoid visual inspection of mouth and throat unless "E" intubation can be done because laryngospasm can occur due to irritation and hypersensitivity
• Use axillary site, not oral for temperature
• Position head of bed up, not supine, and ensures improved aeration -Supine position compromises the function of diaphragm
• Suction PRN for bacterial tracheitis

Promote Balanced Fluid and Nutrition

• NPO if respiratory distress is severe with RR>60/min to decrease work of breathing
• Assess for signs and symptoms of dehydration like fever and sore throat
• Administer IVF as prescribed
• Encourage fluid intake
• Provide clear and caloric liquids if tolerated
• Antipyretics as prescribed

Promote Rest and Comfort

• Provide quiet environment with less stress, which prevents anxiety and crying
• Crying increases oxygen consumption and can precipitate laryngospasm

Support and Education

• Allow an opportunity to express feelings to reduce guilt and self-blame
• Involve in the plan of care and provide information about the illness

Bronchiolitis

• Lower respiratory illness when an infecting agent causes inflammation and obstruction of bronchioles

Causes of Bronchiolitis

• Generally viral; respiratory syncytial virus (RSV) causes most cases and easily spreads by direct contact/droplet
  • Handwashing
• Other viruses: adenovirus or parainfluenza
• Bacterial –Mycoplasma pneumonia

Incidence of Bronchiolitis

• Age affected: usually infants at 6 months of age; rare after 2 years
• Affects boys more often
• Time of year-epidemics at late fall to early spring from October to March

Risk Factors for Bronchiolitis

• Children in daycare are usually more likely to get Bronchiolitis
• Kids exposed to cigarette smoke are at even higher risk
• Babies who attend daycare are less likely to get bronchiolitis than those who stay home with parent who smokes
• Formula-fed infants are more prone to bronchiolitis
• High risk for asthma are prone to getting bronchiolitis
• Have been exposed to an adult or another child with a cold in the previous week

Pathophysiology of Bronchiolitis

• RSV invades mucosal cells leading bronchioles
• Invaded cells die as virus bursts from inside cell to adjacent cells
• Cell debris clogs and obstructs bronchioles
• Airway becomes irritated
• Swelling occurs as well as increased mucus secretion
• Bronchospasm occurs, which leads to partial obstruction and air trapping, as well as complete obstruction, emphysema, and atelectasis
• This results in respiratory acidosis and hypoxia

Clinical Manifestations of Bronchiolitis

• Preceded by URI, manifested as nasal stuffiness, mild fever progressing to increased respiratory distress after 1 to 2 days
• These symptoms can be tachypena or paroxysmal cough irritability
• Can also include wheezing, retraction,dyspnea, diminished breath, and apnea

Diagnostic Tests for Bronchiolitis

• Includes history like exposure to cold the previous week and physical exam
• Nasal or nasopharyngeal culture- presence of RSV through enzyme linked immunosorbent assay (ELISA)/immunoflourescent antibody ( IFA)
  • (+)=isolate/roomed together or placed in the same ward to minimize spread
• Chest X-ray shows hyperaeration and consolidation similar to pneumonia
  • Shows collapse of alveoli/atelectasis is a major risk factor for COPD in later life
• Pulse oximetry reading
• ABG= Respiratory acidosis includes Hypercapnea and hypoxemia
• CBC=leukocytosis and ↑ESR

Management of Bronchiolitis

• Most can be managed at home; can be hospitalized when conditions become worse
• Cardiorespiratory monitor and pulse oximetry
• Oxygen therapy via mask, nasal canula, or high humidity mist tent
• Hydration through oral/IV may be on NPO due to tachypnea and fatigue until acute crisis has passed
• Medications
  • Nebulizer therapy provides bronchodilators, corticosteroids, and beta agonists to directly act inflammatory and obstructed airways
  • Antipyretics for fever
  • Mild Sedatives
  • Antibiotics for (+) secondary bacterial infection with otitis media
• Antiviral Therapy for high risk infant/child: Ribavirin or Virazole
  • Aerosolized thru a small particulate generator thru mask, ventilator, tubing or tent
  • Must have case to case assessment
  • Health providers who are prengant should not be caring for child getting Ribavirin
  • Wear goggles because it dissolves contact lenses
  • Wait few moments after before opening the tent

Prevention of Bronchiolitis

• RSV-IGIV (RESPIGAM)
• Palivizumab preferred
• Vaccine administration: interferes with MMR and varicella
• Disadvatange/Advantage of medication: volume of drug may not be well tolerated by infants given to children w heart defects

Nursing Diagnosis of Bronchiolitis

• Ineffective airway clearance due to increased airway secretions, decreased energy, and air trapping
• Ineffective breathing pattern due to inflamm Tacheobronchial tree and progression of Bronchiolitis
• High riks for fluid volume deficit due to inability to meet body requirements and increased metabolic demands
• Fear or anxiety due to treatment plan
• Knowledge Deficit

Nursing Care for Bronchiolitis

• Maintain respiratory function
• Promote balance of nutrition
• Provide breathing support
• Group infected children

Cystic Fibrosis

• Chronic multisystem disorder characterized by exocrine gland dysfunction and is inherited as an autosomal recessive trait
• Exocrine gland dysfunction: mucus produced by exocrine glands is abnormally thick
• Autosomal recessive inheritance characteristics
  • Both parents must be carries
  • Occurs in 1 of 4 children

Incidence of Cystic Fibrosis

• US=30k affected; worldwide=70k
• Occurs in 1:3000 births in U.S.
• Most common among Caucasians
• Abour 1000 new cases are detected
• More than half of patients are diagnosed by age two
• About 40 percent are diagnosed after age 18
• In 2005, predicted age of survival was 37 years

Causes of Cystic Fibrosis

• defect in a gene known as"Cystic Fibrosis Transmembrane Conductance Regulator"(CFTR)

Manifestations of Cystic Fibrosis

• Progressive chronic lung disease
• Pancreatic enzyme deficiency is primarily affected
• Sweat gland dysfunction electrolyte

Cystic Fibrosis

• Respitory tract affected by the stagnation of mucus in the airway
• Bacteria include P aerogiosa, H influenza colonization and pneumonia
• Airway is constricted
• Cor primonale occurs = Dyspnea cycanssis
• Lung Failure
• Intestinal issues include bile restriction
• Liver damage
• Diabetes
• Gut problems

Gut Pancreas Involvement (4Fs)

• Frothy (bulky and large quantity)
• Foul-smelling
• Fat containing (greasy)
• Float
• Rectal prolapsed bulky stools
• Skin and reprodution is affected as well

Reproductive and Skin

• Abnormally increased
• Parent report that infants taste salty when kissed
• Heat excersions in hot season and elevated
• Puberty is delayed in females

Male Cystic Fibrosis

• Are sterile
• Mucus plug can make difficult
• Pregnany can lead to failure

Complications of Cystic Fibrosis

• Pulminale and failure
• Infection risk
• Phenomo Thorex

Tests of Cystic Fibrosis

• History, and electrolyte concentration
• Normal chloride less than 140
• Electrolyte highly suggestitive
• Check sweat
• Fecal, trypsin, chest x ray,
• Genetic testing

CF Sweat Testing

• Chol ride electroytes measure
• Low current to test site
• Fast
• Choline billius
• Pulinary restriction

Medical Management of Crstic Fibrosis

• Theraphy before CPT
• Meds such as Brochodilator
• Pulmozyme

Nursing Diagnosis of CB

• Check that all lungs clear secretions
• Nutrition with digestive and vitamins
• Transplant may be needed