Choanal Atresia: Types and Manifestations

Choanal Atresia

• Atresia is the closure of a normal opening
• Choanal Atresia is a condition where the posterior nares are obstructed by either membranous septum or bone, occurring either bilaterally or unilaterally
• Incidence includes being the most common congenital malformation of the nose, occurring in 1:7,000 live births, and is often associated with other congenital anomalies in 50% of cases, particularly in females
• The cause is unknown

Types of Choanal Atresia

• Unilateral type (usually the right-60% of cases) may go undiagnosed until the child has persistent unilateral nasal drainage and seems to have more nasal obstruction than the degree of infection suggests
• Bilateral type causes neonates to become apneic or cyanotic at birth, requiring resuscitation to prevent asphyxia/severe hypoxia

Clinical Manifestations of Choanal Atresia

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

Diagnosing Choanal Atresia

• Generally recognized shortly after birth while the infant is in the hospital
• Physical exam provides proof of nose obstruction
• Diagnosis confirmed by passing a firm catheter (Fr. 6) through each nostril 3-4 cm into the nasopharynx
• Radiography using contrast material helps determine the exact location of obstruction
• Fiberoptic endoscope looks into the nose to ensure the airways are open and assess healing after surgery
• A CT scan evaluates the extent of the choanal atresia once a feeding tube fails to pass through the nasal passage; this detects the composition (membranous or bony), the thickness of the atresia, the depth of the nasopharynx, and any associated skull base anomalies

Management of Choanal Atresia

• Airway support-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 because the anatomy is more developed, facilitating easy access to the posterior choanae, and intraoperative blood transfusion is not needed for repair at this age
• Transnasal approach requires less operative time and causes slightly less morbidity related to the incision, but the risk of the nasal passages closing later may be higher
• Transpalatal approach allows for better exposure and more accurate bone removal
• In both approaches, the doctor places nasal stents (small plastic tubes) into the passages to keep them open, and after 6 or more weeks, the doctor removes the stents under general anesthesia
• A possible complication is restenosis, which requires dilatation and reoperation

Nursing Care for Choanal Atresia

• Ensure Airway support through close observation, using a cardiorespiratory monitor
• Suction mucous carefully to maintain breathing, especially before each feeding
• Hydration and nutrition, including a feeding tube for bilateral choanal atresia until defect is corrected
• Unilateral cases require feeding slowly with a small nipple in an upright position with frequent pauses to permit breathing

Croup

• Croup is a broad grouping of UPPER AIRWAY illnesses resulting from inflammation and narrowing of the larynx (voice box), trachea (windpipe), and bronchi (larger branching air tubes)

Incidence of Croup

• Common in children aged 5 and younger, particularly those born prematurely, peaking at the 2nd year
• Signs and symptoms are typically most severe in children 3 and younger because their airways are narrower and more susceptible to blockage during infection
• Croup usually occurs in the winter or early spring, and a child can get croup more than once

Causes of Croup

• Mostly caused by VIRUSES, like the PARAINFLUENZA VIRUS (accounting for most cases), adenovirus, respiratory syncytial virus, influenza, and measles
• Occasionally caused by BACTERIA or an allergic reaction

Changes in the Upper Airway During Croup

• The epiglottis and larynx swell, occluding the airway
• The trachea swells against the cricoid cartilage, causing restriction

General Clinical Manifestations of Croup

• Croupy cough- loud, harsh, “brassy” or “barking” cough (similar to a the noise of a seal barking) due to swelling around the vocal cords (larynx) and windpipe (trachea) and when the cough reflex forces air through this narrowed passage, the vocal cords vibrate with a barking noise
• Dysponia (hoarseness), as laryngeal involvement often dominates the clinical picture
• Inspiratory Stridor- high-pitched or squeaking noise when breathing in due to narrowing of the airway
• Respiratory Distress from swelling or obstruction of the larynx

Types of Croup

• Acute Laryngitis affects the glottic area and is least serious; causative agent is a virus with an allergic/psychosomatic component onset is typically sudden occuring at night, and tending to recur
• Spasmodic Bronchitis affects the subglottic area and is the most common, serious viral croup that progresses as it goes untreated; causative agent can be parainfluenza, influenza A & B, RSV, Mycoplasma pneumonia
• Bacterial Tracheitis affects the upper trachea and is guarded requiring close observation if required; causative agent is usually staphylococcus aureus
• Acute Epiglotitis affects the supraglottic area and is considered most life threatening; causative agent is usually HIB

Onset of Croup

• Acute Laryngitis typically presents with afebrile, mild distress
• Spasmodic Bronchitis starts gradual, from URI to respiratory distress
• Bacterial Tracheitis progresses rapidly as a URI;
• Acute Epiglotitis progresses rapidly within hours

Clinical Manifestations of Croup

• Bacterial Tracheitis causes high fever and thick,purulent tracheal secretions from a URI with no response LTB therapy
• Acute Epiglotitis causes high fever, toxic appearance, dysphonia, dysphagia, drooling, and distressed respiratory effort

Treatment of Croup

• Bacterial Tracheitis is treated with Antibiotics
• Acute Epiglotitis is treated with Antibiotics and Airway Protection

Diagnosing Croup

• Relies on history (fever, cold symptoms, recent viral infection) and clinical manifestations
• An X-ray of the neck (not always required) can rule out foreign body obstruction/epiglottitis, revealing a steeple sign where the top of the airway narrows to a point
• WBC and differential counts can distinguish between bacterial and viral illnesses

Management of Croup

• The goal is to maintain a patent airway and provide adequate respiratory exchange
• Humidity (High humidity with cool mist provides relief for most children and decreases swelling of laryngeal tissue)
  • Mild cases are manageable at home with a cool air vaporizer
  • Spasm is sometimes relieved by sudden exposure to cold air like breathing in cool night air or air from an open freezer or taking the child to a cool basement/garage.
  • Warm mist from steam from hot running water in a closed bathroom
• If the condition does not improve or becomes severe, the child needs hospitalization with a Mist Tent/Croupette to provid cool, moist air with oxygen if extra oxygen and moisture is needed
• Medications. including Racemic Epinephrine (nebulized) which causes mucosal vasoconstriction and subsequent decrease in edema, working fast but symptoms sometimes "relapse" within 2 hours
• Corticosteroids aid in anti-inflammatory effects to decrease subglottic edema
• Antibiotics address bacterial croup
• Antipyretics adress fever
• IVF for hydration if fever causes fluid loss and sore throat results in inadequate intake
• An artificial airway=ET intubation/tracheostomy may be needed
• Extubation occurs when spasm and edema subside allowing the child to manage secretions successfully (usually within 24-36 hours)

Nursing Care for Croup

• Ineffective airway clearance due to laryngeal edema, mucosal inflammation, and decreased energy/fatigue
• Altered tissue perfusion (cardiopulmonary) from partially obstructed airway
• High risk for fluid volume deficit because of inability to meet body requirements and increased metabolic demands
• Fear/anxiety from acute illness, hospitalization, and uncertain course of illness and treatment
• Knowledge deficit from diagnosis, treatment, prognosis, and home care needs

Nursing Care for Croup

• Maintain patent airway and adequate respiratory exchange
• Assess respiratory status every 2-4 hours or more frequently as needed; record and report changes promptly to the physician
• Early warning signs of impending airway obstruction include tachycardia, tachypnea, substernal/suprasternal and intercostal retractions, flaring of nares, and increasing restlessness
• Infants and preverbal toddlers require constant monitoring
• Emergency suction and tracheostomy intubation tray must be at the bedside
• Care in a croupette -device that provides cool humidification with administration of O2 or compressed air
  • Keep the child dry: linens and gown need to be changed and hair towel dried to prevent dampness that may lead to chilling which increases energy use
  • Keep loose edges tucked under the mattress to prevent oxygen and moisture loss within the tent
  • Situated near the nurses station and resuscitation equipment at bedside
  • No battery/friction/fur toys, which are a fire hazard
• Administer medications as prescribed
• No visual inspection of mouth and throat except during "E" intubation or Laryngospasm may occur due to severe irritation and hypersensitivity of the airway muscles
• Axillary temperature is preferred over oral
• Head of bed should be raised not supine to facilitates ventilation and prevent feelings of choking
• Suction as needed for bacterial tracheitis
• Balanced fluid and nutrition are promoted
• NPO in severe respiratory distress (RR>60/min) to prevent aspiration and to decrease the work of breathing
• Assess for dehydration from fever and sore throat
• IVF as prescribed
• Encourage fluid intake
• Clear and high caloric liquids as tolerated
• Antipyretics are prescribed
• Promote rest and comfort through a quiet environment with less stress, encouraging calmness
• Support and educate the child and parents by allaying anxiety and providing opportunities to express their feelings to reduce guilt and self-blame
• Involve in plan of care and provide education regarding the illness

Bronchiolitis

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

Causes of Bronchiolitis

• Viral, generally caused by a respiratory syncytial virus (RSV) that is most cases, and easily spread direct contact/droplet
• Bacterial cause is Mycoplasma pneumonia

Incidence of Bronchiolitis

• Usually affects infants with the most common age occurring at 6 months old, rarely affects anyone over 2 years old
• Occurs more in boys
• Time of year: epidemics at late fall and early spring (Oct-Mar)

Risk Factors for Bronchiolitis

• Children in day care are more likely to get bronchiolitis
• Exposure to cigarette smoke increases risks; babies at home with a parent who smokes are less likely
• Those at high risk for asthma also are more prone
• Most children exposed to an adult or another child with a cold in the previous week develop bronchiolitis

Pathophysiology of Bronchiolitis

• RSV invades the mucosal cells lining bronchioles
• Invaded cells die as the virus bursts from inside the cell and spreads to adjacent cells
• Cell debris clogs and obstructs bronchioles
• The airway becomes irritated, causing swelling
• Leads to increased mucus production and bronchospasm
• Partial obstruction results in air trapping
• Complete results in emphysema (hyperinflation) and atelectasis
• Respiratory Acidosis and Hypoxia

Clinical Manifestations of Bronchiolitis

• Preceded by URI, resulting in nasal stuffiness, mild fever progressing to increased respiratory distress (after 1-2 days)
• Tachypnea
• Paroxysmal cough
• Irritability
• Wheezing
• Retraction
• Dyspnea
• Diminished breath sound
• Apnea

Diagnosis and Management of Bronchiolitis

• A history (exposure to cold the previous week) and physical exam
• Nasal or nasopharyngeal culture showing presence of RSV through ELISA/IFA where a (+) indicates needs to isolate/roomed together or placed in the same ward to minimize spread
• Chest X-ray can show hyperaeration and consolidation (similar to that of pneumonia), as well as collapse of alveoli/atelectasis which presents a major risk factor for COPD later in life
• Respiratory acidosis may be revealed through testing of the arterial blood gases [ABG=Hypercapnea (↑pCO2); hypoxemia (↓pO2)]
• CBC shows leukocytosis, 个ESR

Management of Bronchiolitis

• Can be managed at home; hospitalize when the case gets worse
• Cardiorespiratory monitor and pulse oximetry
• Oxygen therapy via mask, nasal canula, or high humidity mist tent
• Hydration is possible through oral/IV routes, but the patient may be NPO, if tachypnea, weakness, and fatigue
• Medications: Nebulizer therapy, bronchodilator, corticosteroids, beta agonists, antipyretics, mild sedative, and antibiotics only if there are (+) secondary bacterial infections, and antiviral therapy -Ribavirin (Virazole for high risk infant and child

Ribavirin

• Controversial-cost, benefit, safety and efficacy depending on the case
• Aerosolized, delivered via particulate generator thru mask, ventilator tubing or tent
• Pregnant health care providers should not care for a child receiving ribavirin
• If with contact lenses, wear goggles-may dissolve it
• Wait for a few moments before opening the tent to prevent contamination

Prevention of Bronchiolitis in High Risk Children

• RSV-IGIV (RESPIGAM) which is less preferred and Intereferes with MMR and varicella vaccines needs to be deferred for 9 months after Respigam infusion because volume of drug may not be well tolerated by infants
• PALIVIZUMAB (SYNAGIS) is preferred for most high risk and does not interfere with MMR, and varicella vaccine with just mild pain and transient erythema at the site

Nursing Diagnosis for Bronchiolitis

• Ineffective airway clearance
• Ineffective breathing pattern
• High risk for fluid volume deficit
• Fear/anxiety due to treatments, hospitalizations, and acute illness
• Knowledge deficit about treatments and diagnosis

Nursing Care for Bronchiolitis

• Promote balanced fluid and nutrition include NPO when severe and give hand washing practice
• Maintain Respiratory Function
• Promote Rest and Comfort
• Isolation methods if there are other with infected with RSV

Cystic Fibrosis

• A chronic multisystem disorder caused by exocrine gland dysfunction, often inherited as an autosomal recessive trait
• Exocrine gland dysfunction causes mucus that is abnormally thick, obstructing small passageways of affected organ
• Autosomal recessive inheritance characteristics require both parents to be unaffected but carry the trait with the illness occurring in 1 of 4 children in the family
• Males and females are affected equally; therefore, an affected individual married to unaffected individual will have = all children will be carriers

Incidence of Cystic Fibrosis

• About US=30,000 affected; worldwide=70,000
• Occurs in 1:3000 live births in US
• Most common among Caucasians with ancestors from northern Europe
• About 1,000 new cases of cystic fibrosis are diagnosed each year and more than 70% of patients are diagnosed by age two
• Also more than 40% of the CF patient population is age 18 or older
• In 2005, the predicted median age of survival was almost 37 years

Common Manifestations of Cystic Fibrosis

• Progressive chronic lung disease
• Pancreatic enzyme deficiency-acinar cells primarily affected
• Sweat gland dysfunction-electrolyte composition is greatly affected

Respiratory System Manifestation of Cystic Fibrosis

• Symptoms of cystic fibrosis are produced by the stagnation of mucus in the airway resulting in bacterial colonization by S aureus, P. aeroginosa, and H influenza (repeated episodes of bronchitis and pneumonia)
• Emphysema and atelectasis occur as the airways become increasingly obstructed; and chronic hypoxemia causes contraction of the muscle fibers in pulmonary arteries leading to pulmonary hypertension/cor pulmonale
• S/S include dyspnea, cyanosis, and/or clubbing of fingers and toes, which leads to death-lung failure

Gut/Pancreas Manifestation of Cystic Fibrosis

• Obstruction of pancreatic ducts, fibrosis, and atrophy of pancreas is possible
• The earliest manifestation in newborn presents as: meconium ileus (small intestine blocked with thick putty like, tenacious mucilaginous meconium) where abdominal distention occurs without passage of stool
• In addition to the absence of enzymes, malabsorption of CHO, fats, and CHON, causing the patients to struggle with malnutrition
• Because there is a loss of nutrients and an inability to absorb fat soluble vitamins, the patient may experience failure to thrive
• Unabsorbed food fractions excreted in the stool produce steatorrhea in the stool produce steatorrhea as well

F's of Cystic Fibrosis

• Frothy, foul-smelling, fat-containing, stool that floats

Manifestations in Relation to the Skin for Cystic Fibrosis Sufferers

• Abnormally increased concentrations of Na and Cl in sweat
• Parents report that infants taste salty when kissed/ with salt frosting
• Dehydration and electrolyte imbalances, especially during hot seasons , resulting in heat exhaustion

Manifestations in Relation to the Reproductive System for Cystic Fibrosis Sufferers

• Delayed puberty in females
• Males are usually sterile due to persistent plugging and blocking of the vas deferens from tenacious seminal fluid
• Females can conceive but may struggle from increased mucus in the vaginal tract
• Pregnancy can cause increased stress on the respiratory system of the mother

Complications Associated with Cystic Fibrosis

• Chronic respiratory infection – pneumonia
• Pneumothorax
• Chronic respiratory failure
• Cor Pulmonale

Diagnostic Tests for Cystic Fibrosis

• History and clinical manifestations
• Sweat chloride test and fecal fat test
• Trypsin and chymotrypsin
• Secretin stimulation test for Pancreatic function
• Chest X-ray to show Hyperinflation
• Pulmonary function test to determine the lung’s function
• Upper Gl and small bowel tests in a series
• Serum Immunoreactive trypsin (IRT) and prenatal genetic testing
• Amniocentesis
• Chorionic Villus Sampling
• Increased sweat electrolyte concentration through the sweat chloride test -Alternative Names include: Sweat Test; Sweat Chloride; Iontophoretic Sweat Test; Pilocarpine Iontophoresis -Standard diagnostic test for CF where the patient is given a medical product to stimulate the production of sweat is stimulated and collected , then the sweat electrolytes are measured -A low-level electric current is applied to the test area, and a current is applied for 5 to 12 minutes, and a paper disk is then placed over the test site and covered with paraffin for an airtight seal
• Chloride concentration is greater than 60 mEq/L is a (+) test result
• Quantitative stool/fecal determination will show the the absence of pancreatic enzymes.
• This tests also evaluates fat absorption because the liver, gallbladder, pancreas, and intestines work together for it’s absorption; Requires bile from the gallbladder and enzymes from the pancreas, and the intestines (24/72 hrs to collect stool)
• There is then a trypsin and chymotrypsin test; If normal there is normal pancreas function and there enzymes are detected in a a drop of emulsified stool on a thin layer of gelatin will be digested and clear

In Relation to The Secretin Stimulation Test

• This test is responsible for the measurement of the pancreatic response to a hormone called, secretin
• A NGT must also be a part of the procedure must have the doctor insert
• Should have the contents measured over an entire 2 hours It may be determined that the patient has a chronic issues by the use of chest -rays
• PFT(Pulmonary Function Test) are also a measurement that must be used
• Serum Immun reactive may be be tested in Newborn; Upper GI and small Bown Series
• For early testing in Utero there need to be both A blood testing AND Amniocentesis

Medical Management for Cystic Fibrosis Patients

Patients will under respiratory theirapy via treatments using treatments that will come through the 3 diffrent medications such as such as medications that:Mucolytics,Bronchodilators and Dornase Alfa is another drug to ensure decreasing of mucus. Chest Physio is one of the most important factors used in all cases This is what has been used to remove secretion such as with bilateral percussion, Vibration and Forceful coughing.Oxygen can also improve and provide medical treatments for Cystric Fibrosis. Infections: Antibiotics should be used and used with the sputum. Nutrients: Vitamins (multivitamins) Pancreate Supplements will be taken with every snack. High cal, prot, and moderate high fat diet.

Nursing Implications for CYSTIC Fibrosis Patients

There can be an affect on the air way This can further cause breathing problems .You can make it to where there will be a huge infections. There may be a shortage of nutrients and fear anxiety because there will be a shortage or lack of development. This can lead to many knowledge deficits. A huge part of taking care of a CF patient is making sure there is going to be pulmonary help such as through vibration, suction and postural drainage. The RN will administer meds. It may also be needed to do some special salt care. The patient will be on a High cal, and moderate fat diet or even may need a genetic counseli